Radiologic Pathology

Radiologic
Pathology
Fifth Edition
VOLUME 1
Chest, Gastrointestinal, and Genitourinary
Radiologic Pathology Correlation
2006
2007
Editors
Angela D. Levy, COL, MC, USA
Chairman and Registrar
Chief, Gastrointestinal Radiology
Ellen M. Chung, LTC, MC, USA

Chief, Pediatric Radiology
Jeffrey R. Galvin, MD
Chief, Chest Radiology
Kelly K. Koeller, MD
Chief, Neuroradiology
Mark D. Murphey, MD
Six Week Course Director

Chief, Musculoskeletal Radiology
Paula J. Woodward, MD
Chief, Genitourinary Radiology
Associate Editor
Jean-Claude Kurdziel, MD
Illustrators
Aletta A. Frazier, MD
Dianne D. Engelby, MAMS, RDMS
Heike Blum, MFA
Department of Radiologic Pathology

Armed Forces Institute of Pathology
Washington DC, USA
American Registry of Pathology

Washington, DC
20306-6000
_____________________________________
Copyright 2006 by the American Registry of Pathology.
All rights reserved. No part of this publication may be reproduced or transmitted in any form
or by any means: electronic, mechanical, photocopy, recording, or any other information
storage and retrieval system without written permission of the publisher.
Made in the United States of America
_____________________________________
Great care has been taken to guarantee the accuracy of the information contained in this
volume. However, neither the American Registry of Pathology, Armed Forces Institute of
Pathology, nor the editors and contributors can be held responsible for errors or for any
consequences arising from the use of the information contained herein.
The opinions and assertions contained herein are the private views of the authors and are
not to be construed as official nor as representing the views of the Departments of the Army,
Air Force, Navy, or Defense.
987654321
Library of Congress Cataloging-in-publication Data [in process]
ISBN 1-933477-00-8
Preface
The Armed Forces Institute of Pathologys Radiologic Pathologic Correlation
course presented by the Department of Radiologic Pathology enters its 59th year
of educating radiology residents worldwide. For the fifth year, our staff and visiting
lecturers have contributed their lecture material and images to compile Radiologic
Pathology 2006 2007, continuing the tradition of presenting richly illustrated
material that teaches the pathologic basis of disease to improve our understanding
of the imaging appearance of disease. We hope the efforts of our authors and
editors have once again accomplished our goal of bringing the outstanding and
unique Radiologic Pathologic Correlation course to your fingertips.
Acknowledgements
The annual production of the Radiologic Pathologic Correlation course and
syllabus is made possible through the tremendous support, dedication, and
selfless service of countless individuals who work in the AFIP and the various
institutions and organizations throughout the world that believe in the importance
of teaching the principles of disease through radiologic pathologic correlation.
The Department of Radiologic Pathology of the Armed Forces Institute of
Pathology expresses our deepest appreciation and sincerest gratitude to:
- All radiologists and radiology residents who have contributed case material to
the Thompson Radiologic Pathologic Archive at the Armed Forces Institute of
Pathology,
- All pathologists in the AFIP who have donated their time and expertise to
radiologic pathologic correlation,
- All of our outstanding authors, illustrators, and department staff members who
make the course and the syllabus happen effortlessly year after year,
- And, to the extraordinary efforts of our production team, headed by JeanClaude Kurdziel, MD, who have tirelessly dedicated the spring and summer of
the last five years to the production of this syllabus.
iii
Faculty VOLUME 1
Chest Radiology
Marc S. Levine, MD
Professor of Radiology
Hospital of the University of Pennsylvania
Advisory Dean
University of Pennsylvania School of Medicine
Philadelphia, PA
and
Former Distinguished Scientist
Washington, DC
Chief, Pulmonary and Mediastinal Radiology

Washington, DC
and
Professor of Radiology and Pulmonary Medicine
University of Maryland
Baltimore, MD
Gerald F. Abbott, MD
Director of Chest Radiology

Rhode Island Hospital
and
Assistant Professor of Radiology
Brown University School of Medicine
Providence, RI
Deborah Rubens, MD
Professor and Associate Chair
Department of Imaging Sciences
University of Rochester Medical Center
Rochester, NY
and
Distinguished Scientist
Washington, DC
Staff Radiologist and Medical Illustrator

Washington, DC
and
Clinical Associate Professor of Radiology
University of Maryland School of Medicine
Baltimore, MD
Francis J. Scholz, MD
Staff Radiologist
Lahey Clinic Medical Center
Burlington, MA
and
Clinical Professor of Radiology
Tufts University School of Medicine
Boston, MA
Leonard M. Glassman, MD
Washington Radiology Associates, PC

Washington, DC
and
Clinical Professor
Department of Radiology
George Washington University Medical Center
Washington, DC
Robert K. Zeman, MD
Chairman and Professor of Radiology

George Washington University
Washington, DC
Genitourinary Radiology
Melissa L. Rosado de Christenson, MD, FACR

Clinical Professor of Radiology
The Ohio State University
Columbus, OH
and
Adjunct Professor of Radiology
Uniformed Services University of the Health Sciences
Bethesda, MD
Acting Chief, Genitourinary Radiology

Washington, DC
and
Adjunct Professor of Radiology
University of Utah School of Medicine
Salt Lake City UT
Rosita M. Shah, MD
Clinical Associate Professor of Radiology

Hospital of the University of Pennsylvania
Philadelphia, PA
Peter L. Choyke, MD
Chief Molecular Imaging Program

National Cancer Institute
Bethesda, MD
and
Professor of Radiology and Nuclear Medicine
Uniformed University of the Health Sciences
Bethesda, MD
Gastrointestinal Radiology
Chairman and Gastrointestinal Radiology Section Chief

Washington, DC
and
Associate Professor of Radiology and Nuclear Medicine
Bethesda, MD
William D. Craig, MD

Washington, DC
Bruce P. Brown, MD
Associate Professor of Radiology

University of Iowa
Iowa City, IA
iv
David S. Hartman, MD
Pennsylvania State University
M. S. Hershey Medical Center
Hershey, PA
Deborah J. Rubens, MD
Professor and Associate Chair
Department of Imaging Sciences
University of Rochester Medical Center
Rochester, NY
and
Distinguished Scientist
Washington, DC
Brent J. Wagner, MD
Chairman, Department of Radiology

The Reading Hospital and Medical Center
West Reading Radiology Associates
West Reading, PA
Jade J. Wong-You-Cheong, MD
Associate Professor of Diagnostic Radiology

Director of Ultrasound
Baltimore, MD
Table of Contents VOLUME 1

Chest Radiology
An Approach to Diffuse Lung Disease, Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3

The Idiopathic Interstitial Pneumonias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .14
Airways Disease: The Movement from Anatomic to Physiologic Assessment . . . . . . . . . . . . . . . . . . . . . . . .26
Inhalational Lung Disease (Asbestosis and Silicosis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .44
Pulmonary Lymphoid Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .54
Angiitis and Granulomatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .63
The Pulmonary Complications of Organ Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .75
The Diagnosis of Pulmonary Embolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .82
Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .93
Fungal Disease in the Thorax: Opportunistic and Primary Pathogens . . . . . . . . . . . . . . . . . . . . . . . . . . . . .100
Bronchogenic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .110
Chest Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .121
Chest Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .126
Pulmonary Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .131

Pulmonary Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .138
Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .148

Chest Seminar: Where is the lesion? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .168
Chest Seminar: Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .173
Rosita M. Shah, MD
Pneumonia: Usual and Unusual Organisms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .178
Uncommon Malignant Tumors of the Lung . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .192

Benign Tumors of the Lung and Tumor-like Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .199
Pleural Disease I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .205
Pleural Disease II and Chest Wall . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .213
Leonard M. Glassman, MD (Mammography)
Classic Breast Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .220

Basic Breast Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .229
Ductal Carcinoma in Situ (DCIS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .238
Breast Abnormalities in Young Women . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .246
The Male Breast . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .257
Benign Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .267

Malignant Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .275
Hepatic Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .284
Imaging of Chronic Liver Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .293
Benign Biliary Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .303
Biliary Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .313
Pancreatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .321
Gastric Malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .332
Abdominal Non Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .344
Small Intestinal Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .353
Colorectal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .361
Mesenteric Masses and Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .372
Idiopathic Inflammatory Bowel Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .382
Approach to Inflammatory Diseases of the Colon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .391
GI Seminar 1: Abdominal Gas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .400
GI Seminar 2: Nonneoplastic Disease of the Stomach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .405
GI Seminar 3: Pancreatic Duct . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .411
GI Seminar 4: Hepatic Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .417
GI Seminar 5: Complications of Meckel Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .422
vi
GI Seminar 6: Beyond Appendicitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .427

GI Seminar 7: Tumors and Tumor-Like Lesions of the Gallbladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .432
Robert K. Zeman, MD
Cholelithiasis and Cholecystitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .438
Marc S. Levine, MD
Inflammatory Diseases of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .444

Tumors of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .450
Radiology of Peptic Ulcer Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .455
Bruce Brown, MD
Pancreatitis: Imaging Has Made a Difference . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .460

Gastrointestinal Bleeding In The Age of the Endoscope. What Does a Radiologist Have To Contribute? . .468
Small Bowel Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .475

Acute Mesenteric Ischemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .487
Malabsorption . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .505
Familial Polyposis and Other Such . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .519
The Spleen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .531

Portal Venous Doppler . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .542
Imaging of Uterine Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .551

Approach to Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .561
Urinary Tract Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .573
Retroperitoneum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .579
Radiologic Evaluation of the Scrotum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .585
First Trimester Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .594
Fetal CNS Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .602
Fetal Body Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .607
Peter L. Choyke, MD
Cystic Diseases of the Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .614

Imaging of Prostate Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .620
Radiographic Evaluation of Urinary Stone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .624
Testicular Torsion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .630
Brent J. Wagner, MD
Imaging of Ovarian Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .637

Adrenal Imaging in Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .645
Imaging of the Urinary Bladder and Urethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .649
Jade Wong You Cheong, MD
Non-Neoplastic Disorders Of The Ovary And Adnexae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .653

Imaging of Solid Organ Transplants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .664
The Neglected Nephrogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .674

Problem Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .681
GU Seminar 1: MSAFP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .691

GU Seminar 2: Renal Calcifications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .693
vii
Chest Radiology
An Approach to Diffuse Lung Disease,

Sarcoidosis
Describing Diffuse Lung Disease
The Alveolar vs. Interstitial Problem

Alveolar or Interstitial ?
An Approach to Diffuse Lung Disease
Figure 1-1-2
Radiograph
Lung volumes
Opacity
Distribution
Ancillary findings
Computed tomography
Opacity
Distribution
Figure 1-1-1
Radiology and pathology form

a continuum of visualization
Fibrosis results in reduced lung volumes
Lung Volumes
Reduced [Figure 1-1-2]

Pathology distal to the airway
Fibrosis
IPF, asbestosis, sarcoidosis, chronic
hypersensitivity pneumonitis
Increased [Figure 1-1-3]
Pathology of the airway
Emphysema, asthma, bronchitis, constrictive
bronchiolitis, LAM
Figure 1-1-3
Airways disease results in increased

lung volumes
Chest Radiology
Distribution: Upper vs Lower
Figure 1-1-4
[Figure 1-1-4]
Plain Film and CT Opacities
Nodules
Reticulation and Lines
Ground glass
Consolidation
Cystic airspaces
Plain Film and CT Opacities
Nodules
Sarcoid
Silicosis, coal-workers
Hypersensitivity Pneumonitis
Metastasis
Reticulation and Lines
Fibrosis
IPF-lower, subpleural
Asbestosis-lower, subpleural
Sarcoidosis-peribronchovascular
Chronic hypersensitivity pneumonitis-mid and
upper lung zone
Ground glass [Shah and Miller AJR 2003]
Non-specific
Airspace, interstitial, combined
DIP, NSIP, AIP, DAD (32%)
Infection (32%)
Drug toxicity (11%)
Hemorrhage (3%)
Ground glass with reticulation
Chronic diseases tend to involve the upper lung
Fibrosis
Consolidation
Organizing Pneumonia (BOOP)
Chronic eosinophilic pneumonia
Lymphoma
Bronchoalveolar cell carcinoma
Infection
Hemorrhage
Cystic airspaces
Mimics reticulation on plain radiographs
Fibrosis and honeycombing
IPF-Lower, subpleural
LAM-Diffuse
LCH-Upper
Radiologic-Pathology Continuum
Anatomy - Secondary Lobule
As defined by Miller
Polygonal
1-2.5 cm
Smallest unit demarcated by connective tissue septa
Most useful diagnostically
Readily identified on:
HRCT
Gross examination
Histologic section
Explains HRCT appearance
Broad range of lung diseases
Especially interstitial disease
Chest Radiology
Anatomy - Secondary Lobule [Figure 1-1-5]
Core structures
Axial interstitium
Bronchiole
Pulmonary artery
Lymphatics
Septal structures
Peripheral interstitium
Pulmonary veins
Lymphatics
Parenchyma
Alveolar interstitium
Alveoli
Pulmonary capillary bed
Figure 1-1-5
The secondary lobule with lymphatics in the

interloblular septa and along
the bronchovascular bundle
Figure 1-1-6
Septal pattern on HRCT and gross specimen

Chest Radiology
Abnormal Patterns
Bronchovascular
Bronchus
Asthma, CF, bronchitis,
bronchiectasis [Figure 1-1-7]
Lymphatic
CA, lymphoma, sarcoidosis
Edema
Centrilobular [Figure 1-1-8]
Airway related
Panlobular [Figure 1-1-9]
Nonspecific
Septal
Lymphatic
CA, lymphoma, sarcoidosis
Figure 1-1-7
Figure 1-1-8
[Figure 1-1-10and 1-1-11]
Edema
Random [Figure 1-1-12]
Hematogenous spread of tumor
TB
Bronchovascular pattern
Figure 1-1-9
Centrilobular pattern
Figure 1-1-10
Sarcoidosis
Multisystem granulomatous disorder

Unknown etiology
Young and middle aged adults
Bilateral hilar lymphadenopathy, pulmonary
infiltration, eye and skin lesions
Clinical and radiologic findings supported
by evidence of noncaseating epithelioid
granulomas
Exclusion of granulomas of unknown cause
and local sarcoid reactions
ATS Statement on Sarcoidosis 1999
Sarcoidosis: Epidemiology
Worldwide
both sexes, all races, all ages
Predilection for adults
under 40 years
peak 20-29 years
U.S. prevalence
10 per 100,000 exams
Highest disease
African-American women
Panlobular pattern
Figure 1-1-11
Septal pattern
Figure 1-1-12
Sarcoidosis: Clinical Features
Asymptomatic
15-50%
Constitutional symptoms
33%
Dyspnea, cough, chest pain
33-50%
Palpable lymph nodes
33-75%
Ocular involvement
11-83%
Cutaneous involvement
20-30% Erythema nodosum, Lupus
pernio
Combined septal and

bronchovascular pattern
6
Random nodule pattern
Chest Radiology
Sarcoidosis: Laboratory Abnormalities
Figure 1-1-13
BAL
macrophages, proportions; CD4 helper
cells
Angiotensin-Converting Enzyme
Nonspecific Produced by
granuloma/macrophage
33-90%
Hypercalcemia 10%
Hypercalciuria 30%
Macrophage/granuloma extrarenal sources
of 1-25 Dihydroxyvitamin D
Anergy
Hypergammaglobulinemia
Sarcoidosis: Respiratory System

[Figure 1-1-13]
100% lung involvement

Portal of entry
Local lymph nodes
Distant organs
Disease distribution
Alveolar wall
Secondary lobule,
Axial CT
Radiograph
Non-Caseating Granuloma and Fibrosis
Sarcoidosis pathogenesis
Alveolar Distribution
Sarcoidosis and the Secondary Lobule [Figure 1-1-14]
Figure 1-1-14
Bronchovascular distribution of
granulomas in Sarcoidosis
Chest Radiology
Figure 1-1-15
Distribution of nodules in sarcoidosis
Masses in Sarcoidosis
Ground glass in Sarcoidosis
Conglomerate masses and fibrosis in sarcoidosis
Sarcoidosis: Computed Tomography [Figure 1-1-15]
Nodules
Masses
Ground Glass
Fibrosis
Conglomeration
Distortion
Emphysema
Bulla
Honeycombing
Chest Radiology
Parenchymal Disease: Radiography
Sarcoidosis: Adenopathy
Figure 1-1-16
Bilateral
Symmetrical
Nodules
Reticulonodular
Masses
Ground Glass
Hilar Retraction
Bulla
Honeycombing
Node Group
Hilar
R. Paratracheal
A-P Window
Subcarinal
Ant. Med.
Post. Med.
CXR
84
76
72
12
12
0
[Figures 1-1-16 and 1-1-17]
CT
88
100
92
64
48
16
Lymph node involvement is a hallmark of sarciodosis
Sarcoidosis:
Staging based on Adenopathy and Parenchyma
Presentation
Stage 0
Normal
Stage 1
Adenopathy
Stage 2
Adenopathy &
Parenchyma
Stage 3
Parenchyma
Figure 1-1-17
Resolution
51
65
29
49
12
20
20% develop fibrosis or Stage 4 disease
Sarcoidosis Stage I
Sarcoidosis Stage II
Sarcoidosis Stage III
Sarcoidosis Stage IV
Bilateral calcified lymph nodes are common
Sarcoidosis Progression
Sarcoidosis and the Parenchyma: Computed Tomography
Thickened Bronchovascular Bundles

Nodules
Peribronchovascular
Pleural, subpleural and septal
Consolidation and Large Nodules
Ground-Glass Opacities
Fibrosis
Chest Radiology
Thickened Bronchovascular Bundles [Figure 1-1-18]

Figure 1-1-18
Peribronchovascular opacities
in sarcoidosis
Peribronchovascular Nodules
Peribronchovascular and Pleural Nodules
Septal Lines
Ground Glass Opacities
Consolidation and Large Nodules
Fibrosis
Bronchovascular Bundle Distortion [Figure 1-1-14]
Conglomerate Mass
Fibrosis and Emphysema
Fibrosis and Honeycombing
Sarcoidosis: Diagnosis
Typical clinical and radiologic manifestations

Non-caseating granulomas
Transbronchial Bx
Endobronchial Bx
Sarcoidosis: Differential Diagnosis
Infection
Tuberculosis, Fungal (Histoplasmosis)
Pneumoconiosis
Silica, Beryllium
Malignancy
Lymphoma
10
Chest Radiology
Miliary Tuberculosis
Transbronchial Spread of Tuberculosis
Histoplasmosis
Silicosis
Berylliosis
Extrinsic Allergic Alveolitis
Sarcoidosis: Mortality
Mortality range 5-10%

Cor Pulmonale related to fibrosis
Cardiac Arrhythmia
Pulmonary Hemorrhage
Aspergilloma
Cor Pulmonale
Sarcoidosis: Cardiac Involvement
Clinical involvement 5%
Heart block, arrhythmia, mitral regurgitation, CHF (dilated cardiomyopathy)
and sudden death
Autopsy involvement 20-30%
Localized wall motion abnormalities
Anterior and apical
MRI, Echocardiograph, Thallium-201
Vignaux AJR 184 Jan 2005
Cardiac Sarcoidosis [Figure 1-1-19]

Figure 1-1-19
Sarcoid infiltration on MRI is represented as focal zones of

increased signal on T2 and early gadolinium images
Dilated Cardiomyopathy
Chest Radiology
11
Sarcoidosis: Mycetoma
Figure 1-1-20
Present in 40-50% of cystic lesions

Bullae, cavities or bronchiectasis
Hemorrhage
Steroids may convert to invasive process
Mycetoma [Figure 1-1-20]

Sarcoidosis: Therapy
Cardiac, CNS, eye involvement

Hypercalcemia
Corticosteroids
Relief of symptoms; resolution of radiologic abnormalities;
improved function
Cytotoxic agents
Methotrexate, Azathioprine
Chlorambucil, cyclophosphamide, antimalarials
Risk of recurrence
Sarcoidosis: Resolution
Sarcoidosis: Prognosis
Favorable
Acute onset, erythema nodosum,
> 80% spontaneous remission
Lfgren syndrome
Low stage
Poor
Chronic course, Lupus pernio
Older age at presentation
Hypercalcemia/nephrocalcinosis
Black race, Extrathoracic involvement
Mycetoma in a cystic space
caused by sarcoidosis
Sarcoidosis Conclusion
References
General
1. Akira M, Hara H, Sakatani M. Interstitial lung disease in association with polymyositis- dermatomyositis: longterm follow-up CT evaluation in seven patients. Radiology 1999; 210(2):333-8.
2. Bergin CJ, Muller NL. CT of interstitial lung disease: a diagnostic approach. American Jounal of Roentgenology
1987; 148:8-15.
3. Bergin C, Roggli V, Coblentz C, Chiles C. The secondary pulmonary lobule:normal and abnormal CT appearances.
American Journal of Roentgenology 1988; 15:21-25.
4. Epler GR, McLoud TC, Gaensler EA, Mikus JR Carrington CB. Normal chest roentgenograms in chronic diffuse
infiltrative lung disease. N Engl I Med 1978:298(17):934-9.
5. Epler GR. Chest films: underused tool in interstial lung disease. Journal of Respiratory Diseases 1987; 8(6):1 4-24.
6. Felson B. A new look at pattern recognition of diffuse pulmonary disease. American Journal of Roentgenology
1979; 133:183-189.
7. Calvin JR. Mon M, Stanford W. High-resolution computed tomography and diffuse lung disease. Curr Probl Diagn
Radiol 1992; 21(2):31-74.
8. Grenier P. Valeyre D, Cluze I R Brauner MW, Lenoir 5, Chastang C. Chronic diffuse interstitial lung disease:
diagnostic value of chest radiography and high- resolution CT. Radiology 1991; 179:123-132.
9. Gruden JF, Webb WR, Naidich DR, McGuinness G. Multinodular disease: anatomic localization at thin-section
CTmultireader evaluation of a simple algorithm. Radiology 1999; 210(3):711-20.
10. Gurney JW, Schroeder BA. Upper lobe lung disease: physiologic correlates. Radiology 1988; 167:359-366.
11. Heitzman ER. The lung. Second ed. St. Louis: C.V. Mosby, 1984.
12. Johkoh T, Muller NL, Cartier Y, Kavanagh PV, Hartman TE, Akira M, lchikado K, Ando M, Nakamura H.
Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 1999; 211
(2):555-60.
13. Mathieson JR. Mayo JR. Staples CA, Muller NL. Chronic diffuse infiltrative lung disease: comparison of dianostic
accuracy of CT and chest radiography. Radiology 1989; 171:111-116.
12
Chest Radiology
14. Mayo JR. Webb WR, Gould R, Stein MG, Bass I, Gamsu G, Goldberg H. High- resolution CT of the lungs: an
optimal approach. Radiology 1987; 163:507-510.
15. McLoud TC, Carrington CB, Gaensler EA. Diffuse Infiltrative lung disease: a new scheme for description.
Radiology 1983; 149(2):353-363.
16. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part 2. Am Rev Respir
Dis 1990; 142(6 Pt 1 ):1440-8.
17. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part lAm Rev Respir Dis
1990; 142(5):1206-15.
18. Muller NE, Coiby TV. Idiopathic interstitial pneumonias: high-resolution CT and histologic findings.
Radiographics 1997; 17(4): 1016-22.
19. Murata K, Itoh H, Todo G, Kanaoka M, Noma 5, Itoh T, Furuta M, Asamoto H, Torizuka K. Centrilobular lesions
of the lung: demonstration by high-resolution CT and pathologic correlation. Radiology 1986; 161 :641-645.
20. Murata K, Khan A, Rojas KA, Herman PG. Optimization of computed tomography technique to demonstrate the
fine structure of the lung. Investigative Radiology 1988; 23:170-175.
21. Murata K, Khan A, Herman R Pulmonary parenchymal disease: evaluation with high-resolution CT. Radiology
1989; 170:629-635.
22. Muller NI, Miller RR. Computed tomography of chronic diffuse lung disease. American Review of Respiratory
Disease 1990; 142:1206-1215, 1440-1448.
23. Staples CA, Muller NE, Vedal S, Abboud R, Ostrow D, Miller RR. Usual interstitial Pneumonia: correlation of CT
with clinical, functional, and radiologic findings. Radiology 1987; 162:377-381.
24. Webb WR. High resolution CT of lung parenchyma. Radiologic Clinics of North America 1989; 27(6):1085-1097.
25. Weibel ER. Looking into the lung: what can it tell us? American Journal of Roentgenology 1979; 133:1021-1031.
26. Weibel ER, Bachofen H. The Fiber Scaffold of Lung Parenchyma. In: Crystal RG, West JB, eds. The Lung. New
York: Raven Press, 1991; 787-794.
27. Weibel ER, Crystal RG. Structural Organization of the Pulmonary Interstitium. In: Crystal RG, West JB, eds. The
Lung. New York: Raven Press, 1991; 369-380.
Sarcoidosis
1. Bergin CJ, Bell DY, Coblentz CL, Chiles C, Gamsu C, Maclntyre NR, Coleman RE, Putman CE. Sarcoidosis:
correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests. Radiology 1989;
171(3):619-24.
2. Gawne-Cain ML, Hansell CM. The pattern and distribution of calcified mediastinal lymph nodes in sarcoidosis
and tuberculosis: a CT study. Clin Radiol 1996; 51(4):263-7.
3. Gleeson FV, Traill ZC, Hansell CM. Evidence of expiratory CT scans of small- airway obstruction in sarcoidosis.
AJRAm J Roentgenol 1996; 166(5):1052-4.
4. Hansell DM, Milne DC, Wilsher ME, Wells AU. Pulmonary sarcoidosis:morphologic associations of airflow
obstruction at thin-section CT. Radiology 1998; 209(3):697-704.
5. Kuhlman JE, Fishman EK, Hamper UM, Knowles M, Siegelman SS. The computed tomographic spectrum of
thoracic sarcoidosis. Radiographics 1989; 9(3):449-66.
6. Miller WT Jr, Shah RM. Isolated diffuse ground-glass opacity in thoracic CT: causes and clinical presentations.
AJR Am J Roentgenol. 2005 Feb;184(2):613-22.
7. Muller NE, Kullnig P, Miller RR. The CT findings of pulmonary sarcoidosis: analysis of 25 patients. AJR Am J
Roentgenol 1989; 152(6):1179-82.
8. Muller NE, Mawson JB, Mathieson JR. Abboud R, Ostrow DN, Champion P Sarcoidosis: correlation of extent of
disease at CT with clinical, functional, and radiographic findings. Radiology 1989; 171 (3):61 3-8.
9. Murdoch J, Muller NE. Pulmonary sarcoidosis: changes on follow-up CT examination. AJR Am J Roentgenol
1992; 159(3):473-7.
10. Newman ES, Rose CS, Maier LA. Sarcoidosis [published erratum appears in N Engl J Med 1997 Jul
10;337(2):1391 [see comments]. N Engl J Med 1997; 336(17):1224-34.
11. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. Pulmonary sarcoidosis: correlation of CT and histopathologic
findings [published erratum appears in Radiology 1994 Mar;190(3):907]. Radiology 1993; 189(1):105-9.
12. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. CT and pathological correlation of pulmonary sarcoidosis.
Semin Ultrasound CT MR 1995; 16(5):361-70.
13. Padley SP, Padhani AR, Nicholson A, Hansell DM. Pulmonary sarcoidosis mimicking cryptogenic fibrosing
alveolitis on CT. Clin Radio! 1996; 51(11):807-10.
14. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol 1985; 144(3):51328.
15. Thomas PD, Hunninghake GW. Current concepts of the pathogenesis of sarcoidosis. Am Rev Respir Dis 1987;
135(3):747-60.
16. Vignaux O. Pictorial Essay: Cardiac sarcoidosis: spectrum of MRI features. AJR Am J Roentgenol 2005
Jan;184(1):249-54.
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78(1 ):65-71.
Chest Radiology
13
The Idiopathic Interstitial Pneumonias

Chronic Diffuse Lung Disease [Figure 1-2-1 and 1-2-2]
Alveolar involvement
Surrounding airways
Fibrosis and/or cells
Alveolar wall
Alveolar space
Restrictive physiology
Decreased lung volumes
Increased attenuation
Subacute or chronic
Weeks to months
Figure 1-2-1
Figure 1-2-2

involve the alveolar walls and spaces
The lung volumes are low and

there are areas of increased density
Liebow 1975
Supporting lung structures
Inflammation
Fibrosis
Not confined to interstitium
Initiated within the airspace
Liebow, Prog Reps Dis 1975

Current List-ATS/ERS Consensus Classification
Idiopathic Pulmonary Fibrosis (IPF)

Usual Interstitial Pneumonia (UIP)
Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD)
Desquamative Interstitial Pneumonia (DIP)
Acute Interstitial Pneumonia (AIP)
Cryptogenic Organizing Pneumonia (COP)
NonSpecific Interstitial Pneumonia (NSIP)
Travis et al. Am J Respir Crit Care 2002

14
Chest Radiology
Idiopathic Pulmonary Fibrosis
Usual Interstitial Pneumonia: histologic pattern

5th-7th decade
66% > 60 years
7/100,000 women and 10/100,000 men
Insidious onset of dyspnea
6 months before diagnosis
Restrictive ventilatory defect
Rales and clubbing
Associations:
Cigarette smoke
Dusty environments: farming, wood dust, metal dust
GE reflux
Autoantibodies common (ANA, RA)
Median survival 2.5-3.5 years
Figure 1-2-3
Usual Interstitial Pneumonia: Histology
Geographic variation
Temporal variation
Fibroblast foci
Mature fibrous tissue
Extensive fibrosis
Inflammation
Minimal
No correlation outcome
Abnormal wound healing
Prognosis
Fibroblast foci
Presence and extent
Katzenstein, Am J Respir Crit Care Med 1998

Selman, Ann Int Med 2001
King, Am J Respir Crit Care Med 2001
Idiopathic Pulmonary Fibrosis Imaging [Figure 1-2-3]
Radiograph abnormal-95%
Volume loss
Reticulonodular opacities
Lower lobe
Honeycombing
Computed tomography
Peripheral and lower lobe
Reticulation and ground glass
Progress to honeycombing
Ground glass in areas of
traction bronchiectasis
The abnormalities are predominantly

peripheral and lower lung field.
There is progressive volume loss
Figure 1-2-4
Hartman, Chest 1996
IPF-Progressive Volume Loss

Idiopathic Pulmonary Fibrosis [Figure 1-2-4]
IPF and Emphysema
Typical peripheral reticulation and honeycombing

and traction bronchiectasis in a patient with IPF
Chest Radiology
15
Utilility of Biopsy for Diagnosis of IPF
Prospective, multi-center study

91 patients suspected of IPF
Clinical diagnosis
Positive predictive value with a confident diagnosis-87%
Imaging diagnosis
Positive predictive value with a confident diagnosis-96%
CT always abnormal in patients with proven IPF
Histologic diagnosis
Agreement regarding the presence or absence of IPF-85%
Agreement in patients without IPF-48%
Relevance to NSIP
Uncertain diagnosis
Discordant data
Disease other than IPF
Hypersensitivity pneumonitis
Collagen-vascular disease
Infection
Figure 1-2-5
Hunninghake, Am J Respir Crit Care Med 2001
IPF Rad-Path Discord

Smoking Related ILD
Interstitial Lung Disease [Figure 1-2-5]
Respiratory bronchiolitis
RB
Respiratory bronchiolitis-interstitial lung disease
RB-ILD
Desquamative interstitial lung disease
DIP
Smoking Related ILD

RB
Clinical
Cigarette smoke or equivalent
Asymptomatic
Pathology
Peribronchiolar macrophages
Peribronchiolar fibrosis
Imaging
Centrilobular nodules
Poorly defined 2-3 mm
Uper lobe predominance
Ground glass opacity
Bronchial wall thickening
Decreased attenuation
Emphysema
Air trapping
Reticulation
Smokers macrophages
Niewoehner, NEJM 1974 ; Remy-Jardin, Radiology 1993
16
Chest Radiology
Figure 1-2-6
Smoking Related ILD [Figure 1-2-6]

RB-ILD
Clinical
Cigarette smoke or equivalent
Dyspnea
Restrictive or mixed PFTs
Good prognosis
Pathology
Peribronchiolar macrophages
Peribronchiolar fibrosis
Imaging
Poorly defined 2-3 mm
Uper lobe predominance
Emphysema
Air trapping
Reticulation
Meyers, Am Rev Respir Dis 1987

Park, J Comput Assist Tomogr 2002
Small centrilobular nodules with an

upper lobe predominance in RB-ILD
Smoking Related ILD

DIP
Clinical
Cigarette smoke
4th and 5th decade
Uncommon
70% survival-10 years
Steroids
Pathology
Pigmented macrophages
Interstitial infiltrate
Plasma cells and eosinophils
Fibrosis
Imaging
Ground glass
Symmetrical
Basal predominance
Reticulation
Cysts
Alveolar ducts
Bronchioles
Emphysematous spaces
Figure 1-2-7
Carrington, NEJM 1978 ; Hartman, Radiology

1993
Desquamative Interstitial
Pneumonia [Figure 1-2-7
Ground glass opacities in DIP

Chest Radiology
17
Dependent Density
Figure 1-2-8
Desquamative Interstitial
Pneumonia
RB and DIP
Smoking Related ILD [Figure 1-2-8]
Acute Interstitial Pneumonia
AIP
Hammon-Rich disease
Rapidly progressive
Days-weeks
Antecedent flu-like syndrome
Mean age 50 years
50% fatal at least
Vourlekis, Medicine 2000

Histology
Exudative phase
Hyaline membranes
Edema
Inflammation
Collapse of alveoli
Organizing phase
Type II hyperplasia
Loose fibrosis
Diffuse Alveolar Damage
Smoking related interstitial lung disease with upper lobe

indistinct nodules, reticulation and well defined emphysematous
spaces combined with lowerlobe ground glass
Figure 1-2-9
Katzenstein, Am J Pathol 1986 ; Ichikado, AJR 1997

Radiography [Figure 1-2-9 and 1-2-10]
Diffuse
Airspace opacification
Costal sparing
Mechanical ventilation
Resembles ARDS
Figure 1-2-10
AIP involves all 5 lobes
Most patients are intubated with

diffuse opacities
18
Chest Radiology

Computed Tomography
Exudative phase
Figure 1-2-11
Consolidation
Bilateral
Focal sparing
Organizing phase
Distortion
Traction bronchiectasis
Ground glass
Johkoh, Radiology 1999;

Ichikado et al. Am J Respr Crit Care Med 2002
Acute Interstitial Pneumonia [Figure 1-2-11]

Cryptogenic Organizing Pneumonia
Non-specific inflammatory response

Pattern of repair
Self-perpetuating
Cryptogenic
Secondary
Connective tissues disease, hematologic
malignancy, drugs or organ transplantation
Focal
Bacteria, legionella, mycoplasma, mycobacterial,
or infarction
Lohr, Arch Int Med 1997
Focal areas of sparing are common in AIP
Terminology problem
Bronchiolitis obliterans OP (BOOP), bronchiolitis obliterans (BO),
bronchiolitis interstitial pneumonia (BIP)
Subacute presentation (3 months)
M=F
Cough, dyspnea, weight loss, fever
Restrictive PFTs
Steroid responsive
Relapse common
Figure 1-2-12
Epler, NEJM 1985

Histology
Fibroblastic plugs in alveoli

Fibrosis in the alveolar space
May be airway centered
Bronchiolitis
Cryptogenic Organizing Pneumonia [Figure 1-2-12]

Radiography
Consolidation
Unlateral or bilateral
Small Nodules
10-50%
Lung volumes
normal in 75%
Cryptogenic organizing pneumonia is

characterized by focal areas of
consolidation more common in the
lower lung fields
Chest Radiology
19

Computed Tomography
Figure 1-2-13
Consolidation 90%
Ground glass 75%
Bronchial thickening and dilatation
Small nodules along bronchvascular
bundles
Large nodules (15%)
Irregular margins
Air bronchograms
Reverse halo
Cryptogenic Organizing
Pneumonia [Figure 1-2-13 and 1-2-14]
Figure 1-2-14
Typical findings in COP with peripheral areas of consolidation.

The differential includes chronic eosinophilic pneumonia,
bronchoalveolar cell carcinoma, lymphoma and infection
Diffuse nodules may also be seen in

COP
Nonspecific Interstitial Pneumonia
Katzenstein
Described in 1994
Does not fit definition of other IIPs
UIP, RB-ILD, DIP, OP, AIP
Represents a variety of etiologies
Collagen vascular disease, drug reaction, inhaled antigen
Inadequately sampled UIP or OP
Median age 45
Onset gradual with wide range
6 months to 3 years
Better prognosis
Katzenstein, Am J Resp Crit Care 1994

Nicholson, Am J Respir Crit Care Med 2001
20
Chest Radiology
Nonspecific Interstitial Pneumonitis

Histology
3 categories
Cellular
Fibrosing
Mixed
Prognosis=fibrosis
OP common
Temporally uniform
Nonspecific Interstitial Pneumonitis

Imaging
Few reports on chest radiography

Wide variety of CT patterns
Ground glass, consolidation, reticular and honeycombing
Traction bronchiectasis=fibrosis
CT pattern indistinguishable
UIP 32%
Hypersensitivity 20%
OP 14%
Other 12%
Hartman, Radiology 2000

NSIP ATS Consensus Conference
Pathologists
Radiologists
Pulmonalogists
300 cases submitted
11 cases agreed to be NSIP by all pathologists
Imaging
Lower lobe
Peribronchiolar reticulation and distortion
Subpleural clearing
NSIP Current View

NSIP Fibrosis with IPF Imaging
Areas of NSIP commonly found in proven cases of UIP

NSIP and UIP
Different severity of injury?
Different mechanism of injury?
Prognosis in these cases is driven by the imaging
Figure 1-2-15
Katzenstein AA et al, Amer J of Surg Path 2002
NSIP in Cigarette Smokers
OP-NSIP
[Figure 1-2-15]
OP-NSIP is peribronchovascular with UIP is peripheral

Chest Radiology
21

Current List
Figure 1-2-16
Idiopathic Pulmonary Fibrosis (IPF)

Usual Interstitial Pneumonia (UIP)
Respiratory Bronchiolitis-Interstitial Lung Disease
(RB-ILD)
Desquamative Interstitial Pneumonia (DIP)
Acute Interstitial Pneumonia (AIP)
Cryptogenic Organizing Pneumonia (COP)
NonSpecific Interstitial Pneumonia (NSIP)

[Figure 1-2-16]
RB/RB-ILD [Figure 1-2-17]

RB-ILD/DIP [Figure 1-2-18]
IPF
Figure 1-2-17
Figure 1-2-18
RB-ILD
DIP
Acute Interstitial Pneumonia [Figure 1-2-19 and 1-2-20]
Figure 1-2-19
Figure 1-2-20
AIP early phase

AIP late phase with organization and fibrosis
22
Chest Radiology
Organizing Pneumonia
Figure 1-2-21
[Figure 1-2-21]
NSIP in the Literature

NSIP-IPF
NSIP-Cigarette Smokers [Figure 1-2-22]
Figure 1-2-22
Organizing pneumonia
Patients with smoking related fibrosis may have a

biopsy that demonstrates NSIP
NSIP-Hypersensitivity Pneumonitis
NSIP-Organizing Pneumonia [Figure 1-2-23]
Figure 1-2-23
Patients with organizing pneumonia may have a biopsy

that demonstrates NSIP
Chest Radiology
23
References
General
1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification
of the Idiopathic Interstitial pneumonias. This joint statement of the American Thoracic Society (ATS), and the
European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS
Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165:277-304
2. Wittram C, Mark EJ, McLoud TC. CT-histologic correlation of the ATS/ERS 2002 classification of idiopathic
interstitial pneumonias. Radiographics. 2003 Sep-Oct;23(5):1057-71.
IPF/UIP
1. Hansell DM, Wells AU. CT evaluation of fibrosing alveolitisapplications and insights. J Thorac Imaging 1996;
11(4):231-49.
2. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am U
Respir Crit Care Med 1998; 157(4 Pt 1):1301-15.
3. Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbation in idiopathic pulmonary
fibrosis. Analysis of clinical and pathologic findings in three cases. Chest 1993; 103(6):1808-12.
4. Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Resp Res 1975; 8:133.
5. Tobin RW, Pope CE, 2nd, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal
reflux in patients with idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 158(6): 1804-8.
6. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D. Interstitial
lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 1990; 176(755759).
7. Coxson HO, Hogg JC, Mayo JR, Behzad H, Whittall KP, Schwait DA, Hartley PC, Galvin JR, Wilson JS,
Hunninghake SW. Quantification of idiopathic pulmonary fibrosis using computed tomography and histology. Am
J Respir Crit Care Med 1997; 155(5):1649-56.
8. Gay SE, Kazerooni EA, Toews GB, Lynch UP, 3rd, Gross BH, Cascade PN, Spizarny DL, Flint A, Schork MA,
Whyte RI, Popovich U, Hyzy R, Martinez FJ. Idiopathic pulmonary fibrosis: predicting response to therapy and
survival. Am U Respir Crit Care Med 1998; 157(4 Pt 1):1063-72.
9. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar Ho, Schroeder DR, Offord KR. Prognostic significance of
histopathologic subsets in idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 157(1):1 99-203.
DIP
1. Gaensler EA, Goff AM, Prowse CM. Desquamative interstitial pneumonia. N Engl U Med 1966; 274(3)113-28.
2. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA.Desquamative interstitial pneumonia and
respiratory bronchiolitis-associated interstitial lung disease. Chest. 2005 Jan;127(1):178-84.
DAD/AIP
1. Bone RC. The ARDS lung. New insights from computed tomography [editorial; comment]. Jama 1993; 269(1
6):21 34-5.
2. Desai SR, Wells AU, Rubens MB, Evans TW, Hansell DM. Acute respiratory distress syndrome: CT abnormalities
at long-term follow-up. Radiology 1999; 210(1):29-35.
3. Greene R. Adult respiratory distress syndrome: acute alveolar damage. Radiology 1987; 163(1):57-66.
4. Ichikado K, Johkoh T, Ikezoe U, Takeuchi N, Kohno N, Arisawa U, Nakamura H, Nagareda T, Itoh H, Ando M.
Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AUR Am U Roentgenol 1997;
1 68(2):333-8.
5. Johkoh T, Muller NL, Taniguchi H, Kondoh Y, Akira M, Ichikado K, Ando M, Honda 0, Tomiyama N, Nakamura
H. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 1999; 211(3):859-63.
6. Katzenstein AL, Myers UL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell
kinetic study. Am U Surg Pathol 1986; 10(4):256-67.
7. Olson U, Colby TV, Elliott CG. Hamman-Rich syndrome revisited [see comments]. Mayo Clin Proc 1990;
65(12):1538-48.
8. Primack SL, Hartman TE, Ikezoe U, Akira M, Sakatani M, Muller NL. Acute interstitial pneumonia: radiographic
and CT findings in nine patients [see comments]. Radiology 1993; 188(3):817-20.
NSIP
1. Cottin V, Donsbeck AV, Revel D, Loire R, Cordier JR Nonspecific interstitial pneumonia. Individualization of a
clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med 1998; 158(4):1286-93.
2. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical
significance. Am J Surg Pathol 1994; 18(2):136-47.
24
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3.
Kim TS, Lee KS, Chung MP, Han J, Park JS, Hwang JH, Kwon OJ, Rhee OH. Nonspecific interstitial pneumonia
with fibrosis: high-resolution CT and pathologic findings. AJR Am J Roentgenol 1998; 171(6): 1645-50.
BOOP/Organizing Pneumonia
1. Akira M, Yamamoto S, Sakatani M. Bronchiolitis obliterans organizing pneumonia manifesting as multiple large
nodules or masses. AJR Am J Roentgenol 1998; 170(2):291-5.
2. Carlson BA, Swensen SJ, OConnell EJ, Edell ES. High-resolution computed tomography for obliterative
bronchiolitis. Mayo Clin Proc 1993; 68(3):307-8.
3. Chandler PW, Shin MS, Friedman SE, Myers JL, Katzenstein AL. Radiographic manifestations of bronchiolitis
obliterans with organizing pneumonia vs usual interstitial pneumonia. AJR Am J Roentgenol 1986; 147(5):899906.
4. Epler GR, Colby TV, McLoud TC, Carrington CB, Oaensler EA. Bronchiolitis obliterans organizing pneumonia. N
Engl J Med 1985; 312(3):152-8.
5. Gosink RB, Friedman Pd, Liebow AA. Bronchiolitis obliterans. Roentgenologicpathologic correlation. Am J
Roentgenol Radium Ther Nucl Med 1973; 11 7(4):81 6-32.
6. Haddock JA, Hansell DM. The radiology and terminology of cryptogenic organizing pneumonia. Br J Radiol 1992;
65(776):674-80.
7. Katzenstein AL, Myers JL, Prophet WD, Corley LS, 3rd, Shin MS. Bronchiolitis obliterans and usual interstitial
pneumonia. A comparative clinicopathologic study. Am J Surg Pathol 1986; 10(6):373-81.
8. Lau DM, Siegel MJ, Hildebolt CF, Cohen AH. Bronchiolitis obliterans syndrome: thin-section CT diagnosis of
obstructive changes in infants and young children after lung transplantation. Radiology 1998; 208(3):783-8.
9. Lee KS, Kullnig P, Hartman TE, Muller NL. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR
Am J Roentgenol 1994; 162(3):543-6.
10. Lohr RH, Boland BJ, Douglas WW, Dockrell DH, Colby TV, Swensen SJ, Wollan PC, Silverstein MD. Organizing
pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 1997;
157(12):1323-9.
11. McLoud TC, Epler GR, Colby TV, Gaensler EA, Carrington CB. Bronchiolitis obliterans. Radiology 1986;
159(1)1-8.
12. Muller NL, Cuerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC. Differential diagnosis
of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and
radiologic findings. Radiology 1987; 162(1 Pt 1):151-6.
13. Muller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR
Am J Roentgenol 1990; 154(5):983-7.
Chest Radiology
25
Airways Disease: The Movement from

Anatomic to Physiologic Assessment
Assessment of Dyspnea
A Common Clinical Problem
55 million adult smokers

15 million meet criteria for bronchitis
5 million with airway obstruction
10 million with asthma
Figure 1-3-1
Gordon Snyder
Differential Diagnosis of Airways Obstruction
Common
Emphysema, bronchitis, bronchiectasis, asthma
Uncommon
LAM, BO, panbronchiolitis, sarcoid,
alpha-1 deficiency, ABPA
Diseases with Obstructive Physiology

The Changing Role of Imaging
Diagnosis
Functional assessment
Why Pulmonary Functions are Insensitive
PFTs based on wide range of normal

80-120% predicted
Diseases with opposing physiologic processes
The silent zone of the lungs
Small airway tethered to

the pleural surface by alveolar walls
The Silent Zone of the Lungs

[Figure 1-3-1 and 1-3-2]
Small Airways
Figure 1-3-2
Peter Macklem
1970s
No cartilage
<2mm physiologists
1mm pathologists
Tethered
fiber skeleton
pleura
Weibel
Mosaic attenuation on an expiratory CT in patient with

constrictive bronchiolitis
Airways Disease
26
Chest Radiology
Diffuse Lung Disease

Airways [Figure 1-3-3]
Figure 1-3-3
Figure 1-3-4
Airways involvement
Obstructive physiology
Increased lung volumes
Airways Disease
Direct Signs [Figure 1-3-4]
Changes
Airway wall
Airway lumen
Opacities
Tubular
Nodular
Branching
Airways Disease
Indirect Signs
Mosaic density
Air trapping
Subsegmental atelectasis
Ground glass
Airways Disease
Airways involvement at the level of

the secondary lobule
Emphysema
Emphysema and Fibrosis
Alpha-1 deficiency
Langherhans Cell Histiocytosis
Bronchiectasis
Asthma
Allergic Bronchopulmonary Aspergillosis
Sarcoidosis
Diffuse Panbronchiolitis
Bronchiolitis Obliterans
Lymphangioleiomyomatosis
Tree-in-bud in a patient with a

respiratory infection
Figure 1-3-5
Emphysema
ATS Definition
Permanent enlargement of airspaces distal to the terminal

bronchiole, accompanied by destruction of the walls without
obvious fibrosis
Emphysema
Emphysema
Classification [Figure 1-3-5]
Proximal Acinar
Centrilobular
Resp bronchiole
Cigarette smoke
Upper lobes
Panacinar
Entire acinus
Alpha-1 deficiency
Lower lobes
Distal Acinar
Paraseptal
Distal acinus
Subpleura
Pneumothorax
Chest Radiology
Cigarette smoke related

emphysema is most severe in the
upper lobes
27
Airways Disease
Emphysema
Clinical Presentation
Figure 1-3-6
Cough, dyspnea and sputum

production
Hemoptysis rare
R/O cancer
Symptomatic air flow obstruction
After age 50, 20-30 years of
smoking
Cor pulmonale (late) related to
hypoxemia and loss of capillary bed
Emphysema and Cor Pulmonale

Emphysema
Pulmonary Functions
Important to identify patients at risk

Reduction in Fev1
Most reproducible
RV increases followed by TLC
Volumes and flows
Insensitive to early changes
Diffusing capacity
Sensitive but non-specific
Small airways tests
Saber trachea
Figure 1-3-7
Emphysema
Radiographic Feature
Hyperinflation
Concave diaphragm
Increased A-P diameter
Retrosternal airspace
Arterial deficiency pattern
Bulla
Cystic airspaces > 1cm
Radiography is insensitive
41% of moderate disease
66% of severe disease
Saber Trachea [Figure 1-3-6]

Emphysema and Computed
Tomography [Figure 1-3-7]
Typical low attenuation

lesions of emphysema
The Diagnosis of Mild Emphysema

Correlation of CT and Pathology Scores
HRCT detects emphysema

Before there is airflow
limitation on PFTs
HRCT excludes emphysema
Patients with moderate
to severe airflow limitation
Kuwano et al, Am Rev Respir Dis 1990
Early Emphysema
Airways Disease
28
Chest Radiology
Respiratory Bronchiolitis [Figure 1-3-8]

Smokers Bronchiolitis
Figure 1-3-8
Common change
all smokers
Pigmented macrophages
In respiratory bronchioles
Surrounding alveoli
Upper lobe predominance
Usually asymptomatic
May cause symptoms
Relationship of RB and Emphysema
Prospective study
111 subjects
Followed for 5 years
Imaged at inception TO and 5 years T1
Smokers, nonsmokers and quitters
Micronodules at TO predisposes to the development of
emphysema at T1
Micronodules and emphysema at TO predicts more rapid decline
in lung function
Remy-Jardin, Radiology 2001
Relationship of RB and Emphysema

Remy-Jardin, Radiology 2001
Respiratory bronchiolitis
14 patients
Scanning electron microscopy
Thick and thin walls
Both fibrotic
Figure 1-3-9
Nagai & Thurlbeck, Am Rev Resp Dis 1985
Normal lung volumes and

Normal flow rates
Reduced diffusing capacity
Severe
Minimal pulmonary reserve
TLC
119%
VC
126%
RV
109%
FEV1/FVC 88%
D/Va
28%
Emphysema and Fibrosis [Figure 1-3-9]

Emphysema and fibrosis

Langerhans Cell Histiocytosis
Almost exclusively cigarette smokers

Slight male preponderance
Cough and dyspnea most common
May be asymptomatic
Occasional bone lesion
Chest Radiology
29
Airways Disease

Histology [Figure 1-3-10]
Figure 1-3-10
Nodular
Interstitial lesions
Located near bronchioles
Histiocytes, eosinophils,
plasma cells and lymphocytes
Diagnosis requires
Langerhans cells
Large histiocytes
Folded nuclei
Eosinophilic cytoplasm
Path DDX
Eos pneumonia, DIP,UIP

[Figures 1-3-11 and 1-3-12]
The range of findings in Langerhans Cell Histiocytosis
Figure 1-3-12
Figure 1-3-11
Cystic lesions in LCH
Typical nodules in LCH
Figure 1-3-13

Radiographic Features [Figure 1-3-13]
Varies over time

Upper lobe
Predominance
Nodules
0.5-1.0 cm in upper lobes
Early
Cysts replace nodules
Later
Honeycomb lung
Pneumothorax 15%
Adenopathy and effusion are unusual
LCH is characterized by low attenuation areas with bizarre shapes

Airways Disease
30
Chest Radiology

EM and Immunohistochemistry
Immunoperoxidase staining
CD1a, S-100 protein
Cells in clusters in interstitium
EM
X-bodies
Langerhans cell granules
Birbeck granules
Figure 1-3-14

Clinical Course
Clinical resolution
Common
Radiographic abnormalities
Persist
Occasional progression
Fibrosis and honeycombing
May be fatal
Rapid progression

Alpha-1 Antitrypsin Deficiency
Pathophysiology
1-2% of emphysema in the US

Alpha-1 antitrypsin inactivates neutrophil elastase
Production controlled by 2 genes
Level of antitrypsin dependent on allele
ZZ homozygotes most severe
Smoking accelerates the destruction

Imaging Features
Radiograph may be normal

Lower lobe predominance
Panacinar emphysema
CT
Upper lobe involvement
Bronchiectasis
Airway thickening common
CT more sensitive
Alpha-1 antitrypsin deficiency
Alpha-1 Antitrypsin Deficiency [Figure 1-3-14]
Figure 1-3-15
Bronchiectasis
Pathophysiology
Dilatation of bronchi
Reversible form
Infection
Atelectasis
Congenital
tracheobronchomalacia
Post-inflammatory
Postobstructive
Fibrotic
IPF
Sarcoid
Williams-Campbell [Figure 1-3-15]

Williams-Campbell
Chest Radiology
31
Airways Disease
Mounier-Kuhn Syndrome
Figure 1-3-16
Bronchiectasis
Postinflammatory
Primary Ciliary Dyskinesia

Kartageners
Immunodeficiency
Postinfectious
TB, Measles, pertussis, viral
Post-toxic bronchitis
gastric acid aspiration
Immunologic
ABPA
Primary Ciliary Dyskinesia

Post Obstructive Bronchiectasis [Figure 1-3-16]
Neoplasm
Foreign body
Broncholith
Lymph node enlargement
Post obstructive bronchiectasis in a patient with

mucoepidermoid carcinoma
Figure 1-3-17
Bronchiectasis
Cough
Purulent sputum
Hemoptysis (50%)
Dyspnea
Rare
clubbing, brain abscess, amyloidosis
Bronchiectasis
Radiographic Features
Upper lobe smoking related

emphysema
Prominent markings
Crowding of Vessels
Tram Tracks
Loss of volume
Cystic spaces
Figure 1-3-18
Bronchiectasis
CT Features
Bronchi in the periphery

Signet Rings
Tram Tracks
Sensitivity
Collimation
RB-ILD
Emphysema [Figure 1-3-17]
Figure 1-3-19
RB/RB-ILD [Figure 1-3-18]

Emphysema and Fibrosis [Figure 1-3-19]
Emphysema and fibrosis

Airways Disease
32
Chest Radiology
Langherhans Cell Histiocytosis [Figures 1-3-20 to 1-3-22]

Alpha-1 Antitrypsin Deficiency [Figure 1-3-23]
Figure 1-3-21
Figure 1-3-20
Late LCH Cysts and nodules
Early LCH nodules
Figure 1-3-22
Figure 1-3-23
End-stage LCH
Lower lobe predominance in

Alpha-1 antitrypsin
Bronchiectasis
Airways
Airways involvement
Asthma
ATS Definition
Reversible airway disease

Increased airway responsiveness
Persistent airflow obstruction occurs in chronic asthmatic
Why?
6% in the American population
Rate has doubled in 20 years
Higher incidence in large cities
Asthma
Extrinsic
Family history atopy

Early onset <30 years
Seasonal symptoms
Increased IGE
Positive skin tests
Often remits
Chest Radiology
33
Airways Disease
Asthma
Intrinsic
Figure 1-3-24
No atopy
Absence of external triggers
Older age group
Increased blood eosinophils
Increased sputum eosinophils
Fixed airway obstruction
Progressive
Asthma
Pathology
Airway smooth muscle

Hypertrophy
Airway wall
Inflammation
Edema
Airway plugging
Mucus
Inflammatory exudate
Airway thickening in asthma
Figure 1-3-25
Asthma
Radiographic Features [Figure 1-3-24]
Chest roentgenogram
Often normal
Airway thickening
Chronic disease
Rapid attenuation of vessels
hypoxemia
Pneumomediastinum
pneumothorax
Hyperinflation
Adaptive
Later air trapping
Silva AJR 183 September 2004
Asthma-Hyperinflation [Figure 1-3-25]

Severe hyperinflation in which you
can see the slips of the diaphragm
as it inverts
Asthma [Figure 1-3-26]

CT Features
More sensitive than CXR

Reversible
Consolidation
Atelectasis
Mucoid impaction
Airway Narrowing
Air Trapping
Permanent
Bronchiectasis
Emphysema
Figure 1-3-26
Mucoid impaction in severe

asthma
Airways Disease
34
Chest Radiology

Primary Criteria
Figure 1-3-27
Asthma
Eosinophilia
Immediate skin test reactivity
Precipitating antibodies (IgG)
Elevated serum (IgE)
Pulmonary infiltrates
Central bronchiectasis

Presentation & Pathology [Figure 1-3-27]
Atopic individuals
Most common
Cystic fibrosis
Airways filled
Fungus
Inspissated mucous
Presentation with
Cough, fever
Hemoptysis
Worsening asthma
Seen in stable asthmatics
Good response to steroids
ABPA represents a non-invasive

colonization of the airways

Imaging [Figures 1-3-28 and 1-3-29]
Figure 1-3-28
Bifurcating opacities
Gloved-finger
Mucous filled airways
Central Bronchiectasis
Fleeting infiltrates
Pleural disease
Uncommon
Sarcoidosis and the Airways

Computed Tomography
Functional evidence of airways obstruction

Obstructive PFTs are common
Endobronchial biopsies find granulomas
Obstructive physiology correlates with
Decreased attenuation on expiratory scans
(small airways)
Reticular pattern and advanced fibrotic disease
(large airways)
Mucoid impaction in ABPA
Figure 1-3-29
Hansell et al, Radiology 1998
Endobronchial Granulomas
Small Airway Distortion
Reticular Pattern and Fibrosis
Central bronchiectasis in ABPA
Chest Radiology
35
Airways Disease
Japan most common

Rarely: Korea, China, Europe and North America
HLA BW54
M-F 2:1
Presentation
Dyspnea
Cough
Obstructive PFTs
Slowly progressive
15 yr mean survival
Erythromycin
May not be an antibacterial effect
Pathology
Discrete nodules
Early infiltration
Interstitium
Respiratory bronchioles
Alveolar ducts
Foamy histiocyte, lymphocyte and plasma cells
Late secondary ectasia
Proximal terminal bronchioles
Figure 1-3-30
Imaging Early
Radiography
Nodules 5mm
Hyperinflation
Computed Tomography
Branching opacities
Mosaic attenuation
Early Diffuse Panbronchiolitis represented by

widespread airways nodules
Diffuse Panbronchiolitis [Figure 1-3-30]
Figure 1-3-31
Imaging Late [Figure 1-3-31]
Radiography
Nodules
Cysts and bulla
Hyperinflation
Computed Tomography
Bronchiolectasis
Bronchiectasis
Constrictive Bronchiolitis
Introduction
Confusing Terminology
Obliterative Bronchiolitis
Bronchiolitis Obliterans Organizing Pneumonia
Different disease
Cryptogenic organizing pneumonia
Small Airways
Fibrosis
Inflammation
Response to
Inflammatory disorders
Infectious disorders
Airways Disease
36
Severe airway involvement in

panbronchiolitis
Chest Radiology
Figure 1-3-32
Cough, dyspnea and malaise

History
prior infection
exposure
Hypoxemia
Airway obstruction
Classification
Infection
RSV, adenovirus and mycoplasma
Toxic Inhalation
Ammonia, acid and NO
Aspiration: gastric acid
Collagen Vascular: RA
Organ Transplantation
Unknown
Obstruction
Terminal bronchiole
Respiratory bronchioles
Polyps of fibrosis
Cellular infiltration
Lymphs
Plasma cells
Histiocytes
Constrictive bronchiolitis
Imaging [Figures 1-3-33 and 1-3-34]
Hyperinflation
Localized
Diffuse
Discrete nodules
Airway associated
Mosaic pattern
Airway thickening
Bronchiectasis
Air trapping
Figure 1-3-34
Figure 1-3-33
Central bronchiectasis and mosaic

attenuation in constrictive bronchiolitis
Mosaic attenuation in constrictive bronchiolitis

Chest Radiology
37
Airways Disease
Swyer-James Syndrome [Figures 1-3-35 and 1-3-36]

Figure 1-3-35
Figure 1-3-36
Unilateral hyperlucent lung in a patient with

Swyer-James
Swyer-James Syndrome
Swyer-James Syndrome-Adenovirus
Figure 1-3-37
Exclusively women
Reproductive years
Progressive dyspnea
Chylous pleural effusions
Hemoptysis
Massive hemorrhage
Function
Obstructive defect
FEV1 is decreased
TLC and RV increased
DLCO reduced
Hypoxemia
Hypocapnia
Histology
Smooth muscle proliferation

Disorderly
Bronchioles, alveolar septa,
arteries, veins and lymphatics
Small air filled cysts
Air trapping
Figure 1-3-38
Typical thin-walled cyst in
lymphangioleiomyomatosis
Gross Features [Figures 1-3-37 and 1-3-38]
Cysts
0.2-2cm
Diffuse involvement
Enlarged thoracic duct
Enlarged lymph nodes
The upper and lower lobes

are equally involved in LAM
Airways Disease
38
Chest Radiology
Radiographic Features
Figure 1-3-39
Basilar
Lung volume
Normal
Increased
Pleural effusion
60-75%
Pneumothorax
Honeycombing late
CT Features [Figure 1-3-39]
Thin-walled cysts
More sensitive than plain film
Diffuse
Bilateral involvement
Adenopathy
Thin-walled cysts and a

pneumothorax in patient
with
lymphangioleiomyomatosis
Figure 1-3-40
Lymphangioleiomyomatosis [Figure 1-3-40]

Therapy and Prognosis
Slowly progressive course

Variable
Progression
Cor pulmonale
Respiratory insufficiency
50-80% 5 year survival
Average survival 10 years
Hormonal therapy
Oophorectomy, progesterone
Tuberous Sclerosis [Figure 1-3-41]
Minimal disease in LAM may be difficult may be

difficult to separate from emphysema
Emphysema
Figure 1-3-41

Asthma [Figure 1-3-42]
Figure 1-3-42
LAM may represent partial expression of tuberous

sclerosis
Asthma
Chest Radiology
39
Airways Disease
ABPA [Figure 1-3-43]
Figure 1-3-43
Sarcoidosis [Figure 1-3-44]

Diffuse Panbronchiolitis [Figure 1-3-45]
Constrictive Bronchiolitis [Figure 1-3-46]
LAM [Figure 1-3-47]
Physiologic Measurement
An Integral Part of Imaging
Imaging provides physiologic information

not available from pulmonary functions
Air content and blood flow can be quantified
ABPA
Airways Disease
Figure 1-3-44
Emphysema
Alpha-1 deficiency
Histiocytosis-X
Bronchiectasis
Asthma
Sarcoidosis
Sarcoidosis airways involvement
Figure 1-3-45
Figure 1-3-46
Mosaic attenuation in
constrictive
bronchiolitis
Typical findings in LAM
Figure 1-3-47
Airways Disease
40
Chest Radiology

Airways
Airways involvement
References
General
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5. Kinsella M, Muller NE, Abboud RT, Morrison NJ, DyBuncio A. Quantitation of emphysema by computed tomography
using a "density mask" program and correlation with pulmonary function tests. Chest 1990; 97:315-321.
6. Klein JS, Gamsu G, Webb WR, Golden JA, Muller NE. High-resolution CT diagnosis of emphysema in symptomatic
patients with normal chest radiographs and isolated low diffusing capacity. Radiology 1992; 182(3):817-21.
7. Kondoh Y, Taniguchi H, Yokoyama S, Taki F, Takagi K, Satake I Emphysematous change in chronic asthma in relation
to cigarette smoking: assessment by computed tomography. Chest 1990; 97:845-849.
8. Kuwano K, Matsuba K, Ikeda T, Murakami J, Araki A, Nishitani H, Ishida T, Yasumoto K, Shigematsu N. The diagnosis
of mild emphysema. Correlation of computed tomography and pathology scores. Am Rev Respir Dis 1990; 141(1):16978.
9. Miller RR, Muller NE, Vedal S, Morrison NJ, Staples CA. Limitations of computed tomography in the assessment
of emphysema. American Review of Respiratory Disease 1989; 139:980-983.
10. Muller NE, Thurlbeck WM. Thin-section CT, emphysema, air trapping, and airway obstruction [editorial;comment].
Radiology 1996; 1 99(3):621 -2.
11.Muller NE, Staples CA, Miller RR, Abboud RT. Density Mask An objective method to quantitate emphysema using
computed tomography. Chest 1988; 94:782-787.
Chest Radiology
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12. Nagao M, Murase K, Yasuhara Y, Ikezoe J. Quantitative analysis of pulmonary emphysema: three-dimensional fractal
analysis of single-photon emission computed tomography images obtained with a carbon particle radioaerosol. AJR
Am J Roentgenol 1998; 171(6):1657-63.
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211(2):541-7
14. Remy-Jardin M, Remy J, Gosselin B, Becette V, Edme J. Lung parenchymal changes secondary to cigarette smoking:
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15. Snider GE, Kleinerman J, Thurlbeck WM, Bengali Zl-i. The definition of emphysema. Report of the National Heart,
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132:182-1 85.
16. Sutinen S, Christoforidis AJ, Klugh GA, Pratt PC. Roentgenologic criteria for the recognitiion of nonsymptomatic
pulmonary emphysema. American Review of Respiratory Disease 1965; 91:69-76.
17. Uppaluri R, Mitsa T, Sonka M, Hoffman EA, McLennan G. Quantification of pulmonary emphysema from lung
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1. Brantly ME, Paul ED, Miller BH, Falk RT, Wu M, Crystal RG, Clinical features and history of the destructive lung
disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis 1988;
138(2):327-36.
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Eosinophilic Granuloma
1. Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis X: evaluation with highresolution CT. Radiology 1989; 172(1):255-8.
2. Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of lung. Clinical aspects of primary histiocytosis in
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3. Lacronique J, Roth C, Battesti JP, Basset F, Chretien J. Chest radiological features of pulmonary histiocytosis X: a
report based on 50 adult cases. Thorax 1982; 37(2):104-9.
4. Moore AD, Godwin JO, Muller NE, Naidich DP, Hammar SR Buschman DE, Takasugi JE, de Carvalho CR. Pulmonary
histiocytosis X: comparison of radiographic and CT findings. Radiology 1989; 172(1):249-54.
5. Stern EJ, Webb WR, Golden JA, Gamsu G. Cystic lung disease associated with eosinophilic granuloma and tuberous
sclerosis: air trapping at dynamic ultrafast high-resolution CT. Radiology 1992; 182(2):325-9.
Asthma
1. Backman KS, Greenberger PA, Patterson R. Airways obstruction in patients with long-term asthma consistent with
irreversible asthma. Chest 1997; 112(5): 1234-40.
2. Brown RH, Herold CJ, Hirshman CA, Zerhouni EA, Mitzner W. In vivo measurements of airway reactivity using highresolution computed tomography. Am Rev RespirDis 1991; 144(1):208-12.
3. Haraguchi M, Shimura S, Shirato K. Morphometric analysis of bronchial cartilage in chronic obstructive pulmonary
disease and bronchial asthma. Am J Respir Crit Care Med 1999; 159(3):1005-13.
4. Kinsella M, Muller NE, Staples C, Vedal S, Chan-Yeung M. Hyperinflation in asthma and emphysema. Assessment
by pulmonary function testing and computed tomography. Chest 1988; 94(2):286-9.
5. Martin J, Powell E, Shore S, Emrich J, Engel EA. The role of respiratory muscles in the hyperinflation of bronchial
asthma. Am Rev Respir Dis 1980; 121(3):441-7.
6. Paganin F, Trussard V, Seneterre E, Chanez R Giron J, Godard R Senac JP, Michel FB, Bousquet J. Chest radiography
and high resolution computed tomography of the lungs in asthma. Am Rev Respir Dis 1992; 146(4):1084-7.
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Am J Roentgenol. 2004 Sep;183(3):817-24.
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bronchopulmonary aspergillosis. Am Rev Respir Dis 1990; 142(5):1200-5.
1. Aberle DR, Hansell CM, Brown K, Tashkin DP. Lymphangiomyomatosis: CT, chest radiographic, and functional
correlations. Radiology 1990; 176(2):381-7.
Airways Disease
42
Chest Radiology
2.
3.
4.
5.
6.
Chu SC, Horiba K, Usuki J, Avila NA, Chen CC, Travis WD, Ferrans VJ, Moss J. Comprehensive evaluation of 35
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Lenoir 5, Grenier P, Brauner MW, Frija J, Remy-Jardin M, Revel D, Cordier J. Pulmonary lymphangiomyomatosis
and tuberous sclerosis: Comparison of radiographic and thin-section CT findings. Radiology 1990; 175:329-334.
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Chest Radiology
43
Airways Disease
Inhalational Lung Disease

(Asbestosis and Silicosis)
Pneumonokoniosis
It will then be necessary to embrace under a single title all essentially

identical forms of disease
the pneumonokoniosis (from Konis, dust) recommends itself
Figure 1-4-1
Zenker 1866 Hematite Mining
Inorganic Dusts
Silica
Asbestos
Coal
Iron
Beryllium
Pneumoconiosis
The accumulation of dust in the lungs and the tissue
reaction to its presence
Dust macules
Diffuse interstitial fibrosis
Diffuse alveolar damage
Alveolar proteinosis
Giant cell (GIP)
Granulomatous inflammation
Types and Sizes of Common Aerosols

Particle Deposition
Inertial impaction, sedimentation and diffusion [Figure 14-1]
10,000-20,000 liters/day
Deposition related to particle size
>10 microns deposit in nasopharynx and large airways (100%)
1-5 micron particles deposit in lung parenchyma (20%)
Particles less that 5 microns can be

deposited beyond the conducting
airways in the alveolar spaces.
Figure 1-4-2
Airway Velocity
Inertial impaction, sedimentation and
diffusion
Particle Clearance [Figure 1-4-2]
Cough, tracheobronchial and alveolar
transport
Most important
Deposition less critical
Mucociliary escalator
Outer gel, inner liquid sol
90% of particles removed within 2 hrs
Alveolar transport
Dissolution, engulfed by macrophages, removed
to lymphatics
Early Basal Deposition

Macrophages remove small particles to regional
lymph nodes.
44
Chest Radiology
Removal to Lymph Nodes

Physiologic Gradients-Airflow FRC [Figure 1-4-3]
Physiologic Gradients-Airflow TLC [Figure 1-4-4]
Figure 1-4-3
Figure 1-4-4
Alveoli in the bases are smaller than those in the

apex.
Physiologic Gradients-Blood Flow [Figure 1-4-5]
The smaller alveoli in the bases enlarge to a

greater degree than those in the apex. Therefore
most airflow is directed towards the bases
Physiologic Gradients-Lymphatic Flow [Figure 1-4-6]

Figure 1-4-5
Figure 1-4-6
There is increased blood flow and hydrostatic

pressure in the dependent vessels
The lymphatics are driven by hydrostatic pressure.

Therefore lymphatic flow is best in the dependent
lung.
Figure 1-4-7
Removal to Lymph Nodes [Figure 1-4-7]
This explains the

tendency for chronic
diseases to be upper
lobe
Chest Radiology
45
Tuberculosis
Figure 1-4-8
Silicosis
Mineralogy
Silicon
Element
Silica (SiO2)
Mineral
Silicone
Synthetic polymer
Figure 1-4-9
Adenopathy with peripheral calcification is a

hallmark of silicosis
Figure 1-4-10
Nodules with an upper lobe predominance is

typical
Figure 1-4-11
Silicoproteinosis is an acute lower lobe process
Silicosis predisposes a patient to having active

tuberculosis
46
Chest Radiology
Silicosis
Epidemiology
Figure 1-4-12
Occupational exposure predominates

3 million workers
Mining, stonecutting, engraving and foundry work
Males more commonly affected
Degree of exposure underestimated
Increased risk of neoplasia and scleroderma
Silicosis
Pathogenesis
5 million particles/cubic foot-lower threshold

100 million particles/cubic foot-100% affected
> 5 micron particle removed in nares and upper airways
80% of particles removed in hours to days
Retained particles consistently .5-.7 microns
Silicosis
Pathogenesis
Macrophages and polys concentrate

Macrophages generate oxygen-free radicals
Macrophages generate fibrogenic proteins
Immune related tissue damage
Rheumatoid factor, ANA and gamma globulin
The silicotic nodule is typical

response to inhaled silica
Silicosis [Figures 1-4-8 to 1-4-11]

Clinical manifestations
Diagnosis
Typical imaging pattern of adenopathy and nodules
Exposure to high concentration of silica
10-20 years of exposure
Simple silicosis
Asymptomatic
Symptoms with PMF
Intense exposure
Silicoproteinosis
TB and cancer
Figure 1-4-13
Simple Silicosis
Pathology [Figure 1-4-12]
Progress to mature nodules: 3 zones

Central dense fibrosis
Mid-zone concentric collagen
Peripheral dust laden cells
Simple Silicosis
Imaging manifestations [Figure 1-4-13]
Adenopathy common
Calcification 10-20%
Calcification 5-10%
Eggshell pattern
Simple Silicosis
Imaging manifestations
Well-circumscribed nodules
1-10 mm
Upper lobe and posterior
Lymphatic gradient
CT more sensitive
Pleural lesions
Candle-wax or pseudoplaques
Chest Radiology
Eggshell calcification
47
Progressive Massive Fibrosis

Pathology
Figure 1-4-14
Conglomeration of nodular lesions

Pathology definition
2 cm
Radiology definition
1 cm
Upper lung zones
Posterior
Progressive Massive Fibrosis

Imaging manifestations [Figure 1-4-14]
Progression after exposure

May fill entire upper lobe
Posterior
Usually bilateral
Carcinoma mimic
Solitary
Lower lobe
Associated emphysema
Not always smoking related
Scar emphysema
Progressive massive fibrosis
Figure 1-4-15
Silicotic Alveolar Proteinosis

High concentration of particulate silica

Acute onset
Weeks-months
Alveoli filled with PAS+ material
Similar to surfactant
Type II cell hyperplasia
Silicosis and Tuberculosis [Figure 1-4-16]

Silicosis
Computed tomographic technique
Thick sections of value in nodular diseases

Small nodules easier to differentiate from vessels
Thin sections 1-2 mm collimation at 10 mm intervals or 3-5 selected
images with prior thick section CT
High spatial frequency algorithm
Supine
No contrast
Crazy paving pattern associated

with alveolar proteinosis
Figure 1-4-16
Silica and Lung Disease
Adenopathy
Nodules
PMF
Silicoproteinosis
Tuberculosis
Cancer
Tuberculosis in a patient with silicosis

48
Chest Radiology
Asbestos
Introduction
Figure 1-4-17
Figure 1-4-18
Group of naturally occurring mineral fibers

Hydrated fibrous silicate
Flexible and strong
Corrosion, thermal and electrical resistance
500 tons - 3 million tons
60 years
9 million people exposed
Primary (mining)
Secondary (industrial)
Nonoccupational (air)
Nonoccupational Exposure
Asbestos Bodies [Figures 1-4-17 and 1-4-18]
Indicates exposure
Transparent fiber core
Iron and mucopolysaccharide coat
Predominantly amphiboles
Longer fibers are coated
< 20 microns not coated
Uncoated fibers are pathogenic
7-5000 Xs more uncoated fibers
Fibers cannot be removed
Lower posterior disease
Asbestos
Serpentine: chrysotile
Asbestos bodies are

commonly found
in urban dwellers
Figure 1-4-19
95% of commercial use

Curly and pliable
Textile manufacture
Fragments easily
Chemically unstable
Dissolves easily
Less pathogenic
Asbestos fibers are much

larger than macrophages
and therefore cannot be
removed to regional lymph
nodes
Figure 1-4-20
Asbestos
Amphiboles: amosite,
crocidolite, anthophilite,
tremolite and actinolite
5% of commercial use
Straight, broad fiber
Do not fragment easily
Long fibers (>20 microns)
Not cleared
More likely coated
Higher carcinogenic potential
Pleural effusions are the most

common early complication of
asbestos exposure
Rounded atelectasis is usually

preceded by a pleural effusion
Figure 1-4-21
Asbestos Related Chest Disease

[Figures 1-4-19 to 1-4-21]
Pleural effusions
Pleural plaques
Round atelectsis
Pleural thickening
Diffuse
Mesothelioma
Asbestosis
Lung cancer
Asbestosis is a lower
lobe subpleural process
Chest Radiology
49
Pleural Plaques
Pathology
Figure 1-4-22
Common autopsy finding

(50-10%)
Dense bands of collagen
Basket weave
Asbestos bodies absent
Uncoated fibers in dissolved lung tissue
Dose response
Between parenchymal asbestos
bodies and presence of plaques
Pathogenesis uncertain
Pleural Plaques
Postero-lateral parietal pleura

Central diaphragm
Absent
Apices and costophrenic angles
Almost always bilateral
Sharply demarcated
Millimeters to 10 cm
May calcify extensively
Highly suggestive of asbestos exposure
The visceral pleural stripe is best seen between the ribs
Figure 1-4-23
Roberts, AJCP 1971
Pleural Anatomy [Figure 1-4-22]

Pleural Plaques
Imaging
Radiography insensitive
(8-40% of autopsy cases)
Companion shadows
Fat and muscle
HRCT
Best sensitivity and specificity
Pleural Fat [Figure 1-4-23]

Pleural Plaques [Figure 1-4-24]
Fat may mimic fibrotic pleural disease
Diffuse Pleural Thickening
Smooth pleural density

CXR: > 25% of the length of the chest wall
CT: 3 mm thick, 8 cm wide, 5 cm craniocaudal
Posteromedial lower lobes
Involves costophrenic angle
Mediastinal pleural involvement
Rare
Suggests mesothelioma
Visceral and parietal pleura
Adhesions
Sequela of prior effusion?
Figure 1-4-24
Pleural Effusion
Definition
History of exposure to asbestos

Confirmation of effusion
Imaging of thoracentesis
Absence of other disease related to effusion
Absence of malignant tumor for 3 years
Epler, JAMA 1982

Visceral pleural plaques

50
Chest Radiology
Pleural Effusion
Clinical presentation
Figure 1-4-25
Most common abnormality

First 20 yrs
3% prevalence
Asbestos exposed
Small < 500 ml
Serosanguinous
Persist for weeks to 6 months
66% asymptomatic
28% recur
Pleural Effusion
Differential diagnosis
Lung cancer
Tuberculosis
Benign asbestos effusion
Mesothelioma
Round Atelectasis
Described 1928 Loeschke

Folded lung vs inflammatory reaction
Associated conditions
Asbestos exposure, CHF, infarct, TB and histoplasmosis
Preceded by effusion
Round atelectasis is associated with

pleural effusion
Round Atelectasis
Irregular fibrous thickening of the visceral pleura

Extensive pleural folding beneath the fibrosis
Layers of invaginated pleura bound by fibrous adhesions
Surrounding lung collapsed or fibrotic
Menzies, AJSP 1987
Figure 1-4-26
Round Atelectasis
Imaging criteria [Figure 1-4-26]
Well-circumscribed
Round or oval opacity
Comet tail sign
Pleural thickening
Volume loss
Asbestosis
Pathologic definition
Interstitial fibrosis
Associated with asbestos bodies
Biopsy
Not the standard of practice
Asbestosis
Dose-response relationship
Probable exposure threshold
Latency period inversely proportional to exposure level
Latency is several decades
Cigarette smoke may act synergistically
Chest Radiology
51
Round atelectasis
Asbestosis
ATS criteria 1986
Reliable history of exposure

Latency of at least 10 years
Restrictive pulmonary functions
DLco and VC <80%
Appropriate physical findings
Inspiratory crackles, clubbing
Chest radiographic abnormalities
ILO perfusion > 1/0 (s, t or u)
Asbestosis
Histology
Early
Fibrosis of respiratory bronchioles
Progression
Terminal bronchioles, alveolar ducts and alveolar septa
Minimum 2 asbestos bodies in area of fibrosis
Craighead, Arch Pathol Lab Med, 1982
Asbestosis
Chest radiography
Lower lobe
Irregular opacities
Nonspecific
Associated pleural disease
Large inter-observer variation
Low perfusion
Normal in 26% of path proven cases
Asbestosis and Cigarette Smoking

Small irregular opacities
Small opacities are related to

Dust exposure, cigarette smoke, age, radiographic technique and obesity
Cigarette smoke causes
Emphysema
Bronchiolar thickening
Asbestos causes
Asbestos workers who smoke
Have more opacities
Related to dust exposure and cigarettes
Figure 1-4-27
Asbestosis
High-resolution CT
Lower lobe and posterior

Parenchymal bands
Curvilinear subpleural line
Interstitial short lines
Honeycombing
High sensitivity
Nonspecific
Specificity increases with # of abnormalities
Prone imaging is key!
Parenchymal Bands [Figure 1-4-27]

Parenchymal bands in a patient with
asbestosis
52
Chest Radiology
Reticulonodular Opacites
Curvilinear Subpleural Line
Short Lines
Honeycombing
Asbestosis vs UIP
Asbestos exposure in the last 30 years is low

Clinical asbestosis requires substantial exposure
Asbestos exposed individuals can have other interstitial lung diseases
Band like opacities merging with the pleura are rare in UIP
Upper zone fibrosis and ground glass are rare in asbestosis
Gaensler, ARRD, 1991 Al-Jarad, Thorax, 1992
Asbestosis
High-resolution CT
Short lines and parenchymal bands are statistically most significant

Strong association with diffuse pleural disease
Multifocal
HRCT finds asbestosis in exposed individuals with normal radiographs and
PFTs
Obstructive PFTs correlate with emphysema
Aberle, AJR, 1988 Aberle, Radiology, 1988 Staples, ARRD, 1989
Asbestosis
Dependent density
Posterior blood flow

5Xs greater
Posterior alveoli
Smaller or collapsed
Less steep ventilatory gradient
Closing volumes
(10-40% of VC)
Asbestosis
Computed tomographic technique
1.5-2 mm collimation
10 mm interval
High spatial frequency algorithm
Prone
Thick section supine: CA screen?
Asbestos Related Chest Disease

Tuberculosis
Silicosis
Asbestosis
Particle Deposition and Clearance
Cough, tracheobronchial and alveolar transport
Chest Radiology
53
Pulmonary Lymphoid Disorders

The Pulmonary Lymphoid System
Lymphatics
Lymph nodes
BALT
Bronchus Associated Lymphoid Tissue
Lymphoid aggregates
Lymphocytes
Dendritic cells
Langerhans cells
Figure 1-5-1
Figure 1-5-2
The Pulmonary Lymphoid

System
[Figures 1-5-1 and 1-5-2]
Lymphatics
Originate in the pleura
Valves
Drain towards hilum
Follow interlobular septa
Accompany blood vessels
Lymph Nodes
BALT
Lymphoid aggregates
Lymphocytes
Dendritic cells
Langerhans cells

System
One set of lymphatics originate in the

Lymphatic channels continue along the
visceral pleura as demonstrated by the pulmonary veins to the hilum. A second
lines on the surface of the lung. These
set of lymphatics originates near the
lymphatics enter the lung and follow the
center of the secondary lobule and
interloblular septa in the periphery of the
follows the pulmonary arteries
lung
Lymphatics
Lymph Nodes
Encapsulated lymph nodes
Proximal bronchi
Bifurcations
Reactive lymph nodes
Peripheral and septal
Cigarettes or dust
BALT
Lymphoid aggregates
Lymphocytes
Dendritic cells
Langerhans cells
Figure 1-5-3
Figure 1-5-4
Reactive Lymph Nodes

System
Lymphatics
Lymph nodes
BALT
Bronchus Associated
Lymphoid Tissue
Lymphoid aggregates
Lymphocytes
Dendritic cells
Langerhans cells
Classic encapsulated intrapulmonary

lymph nodes are found at the
bifurcations of the first 3-4 orders of
bronchi and are demonstrated adhering
to the right main pulmonary
54
The majority of intrapulmonary lymph

nodes are probably not visible
radiographically. Almost all of these
lymph nodes are subpleural and
inferior to the carina
Chest Radiology
BALT The organizing principle [Figure 1-5-5]
Figure 1-5-5
Lymphoid collections
Bronchial epithelium
Bifurcations
Absent in the normal adult
Absent at birth
Common in young children
Reappears with antigenic stimulation
Cigarette smoke
Connective tissue disease
AIDS
Basis of pulmonary lymphoid disorders
Pulmonary Lymphoid Disorders Derivations of BALT
Hyperplasias of BALT
Follicular hyperplasia
Follicular bronchitis
Diffuse hyperplasia
Lymphoid Interstitial Pneumonia
Nodular Lymphoid Hyperplasia
Pseudolymphoma
Non-Hodgkins lymphomas
Low-Grade B Cell lymphomas
Lymphomatoid granulomatois
Immune impairment
PTLD, AIDS and other
Bronchus associated lymphoid

tissue or BALT is found along the
bronchiole, interlobular septa and
pleura. It is normally found only in
young children.
Koss, Sem Diag Pathol 1995
Follicular Hyperplasias of BALT Hyperplasia of BALT

[Figure 1-5-6]
Figure 1-5-6
Follicular bronchitis and bronchiolitis

Pathologic features
Antigenic stimulation of BALT
Lymphoid aggregates
Peribronchial
Reactive follicles
Minimal alveolar extension
Clinical
Follicular Hyperplasias of BALT Hyperplasia of

BALT
Pathologic features
Clinical
Young adults (44 yrs.)
Cough and dyspnea
Fever and weight loss
Immune deficiencies
AIDS
Congenital
Collagen vascular diseases
Sjogrens
Rheumatoid arthritis
Uncertain Etiology
Hypersensitivity reactions?
Chest Radiology
Follicular bronchitis is characterized

by hyperplastic lymphoid follicles with
reactive germinal centers distributed
along bronchioles and to a lesser
extent bronchi.
55
Follicular Hyperplasias of BALT Hyperplasia of BALT: Imaging

[Figure 1-5-7]
Radiography
Diffuse
Reticulonodular
CT
Nodules 3-12 mm
Centrilobular
Peribronchial
Ground Glass
Air Trapping
Follicular Hyperplasia Differential Diagnosis
Respiratory Bronchiolitis
Diffuse panbronchiolitis
Cystic Fibrosis
Primary ciliary dyskinesia
Diffuse Hyperplasias of BALT Hyperplasia of BALT

[Figure 1-5-8]
Figure 1-5-7
Lymphocytic Interstitial Pneumonia

Pathologic Features
Alveolar septal infiltration
Lymphocytes (T-cells)
Diffuse
Lymphoid follicles (B-cells)
Germinal centers
Peribronchial distribution
Spectrum with follicular hyperplasia of BALT (Follicular
Bronchitis)
Clinical
Almost all patients with follicular

bronchitis have centrilobular nodes
that are less than 3mm. These
nodules correlate with the
peribronchiolar distribution of
hyperplastic lymphoid follicles shown
on the histology section to your left
Figure 1-5-8
Diffuse Hyperplasias of BALT Lymphoid Interstitial

Pneumonia LIP- Hyperplasia of BALT
Pathologic features
Clinical
Women>men
4th-6th decade
Cough and dyspnea
Collagen vascular disease
Sjogrens, RA, and SLE
Bone marrow transplantation
AIDS rare in adults
Common in children
Dysproteinemia
Restrictive lung functions
LIP is characterized by diffuse

infiltration of the alveolar septa
56
Chest Radiology
Diffuse Hyperplasia of BALT Hyperplasia of BALT: Imaging
Figure 1-5-9
[Figures 1-5-9 and 1-5-10]
Radiography
Lower lung zone
Reticulonodular
CT
Ground Glass
Nodules
Centrilobular
Poorly defined
Cystic air spaces
Thickened BVBs
Adenopathy
LIP vs Lymphoma
LIP
Cysts
82%
Consolidation 18%
Large Nodules 6%
Effusions
0%
The lung windows demonstrate

diffuse hazy ground glass that
correlates with diffuse alveolar wall
thickening. The alveolar wall
thickening is primarily the result of
lymphoid infiltation
Lymphoma
2%
66%
41%
25%
Figure 1-5-10
Nodular Lymphoid Hyperplasia Hyperplasia of BALT

[Figure 1-5-11]
Pseudolymphoma
Pathologic Features
Solitary, subpleural mass
Lymphoid proliferation
Interstitial
Perivascular
B and T cells
Polyclonal pattern
Benign
Reactive germinal centers
Difficult to separate from lymphoma
Clinical
Nodular Lymphoid Hyperplasia Hyperplasia of BALT
Pseudolymphoma
Pathologic features
Clinical
Rare entity
Most cases were
lymphomas
Monoclonal B
cell proliferation
Middle age
Asymptomatic
Autoimmune
Diseases 15%
Sjorgrens
SLE
Transverse
myelitis
Surgical excision
curative
Chest Radiology
Thin walled cysts are often found

deep within the lung parenchyma.
Previous reports have suggested that
airway narrowing or obliteration
results in these cystic lesions. The
histology on the left demonstrates
complete obliteration of the
bronchiole by lymphoid infiltration.
The accompanying arteriole is
identified by its typical wall.
Figure 1-5-11
Nodular lymphoid hyperplasia or
pseudolymphoma presents as a
solitary subpleural mass of
lymphoid tissue with numerous
reactive germinal centers
57

Hyperplasia of BALT: Imaging
Figure 1-5-12
[Figures 1-5-12 and 1-5-13]
Radiography
Solitary Nodule
Focal Consolidation
CT
Air bronchograms
100%
Indistinct margins
Occasionally multiple
Adenopathy and/or effusion suggests lymphoma
The CT demonstrates the typical subpleural, solitary lesion with indistinct
margins. The bulk of the lesion
consists of a mass of lymphoid tissue
with multiple reactive germinal
centers.

Derivations of BALT
Low-Grade B Cell lymphomas
Lymphomatoid granulomatosis
Immune impairment
Figure 1-5-13
Low-Grade B-Cell Lymphoma
Pathologic features
Lymphocytic infiltration
Small lymphocytes
Alveolar wall
Peribronchiolar
Perivascular
Immunologic evidence of malignancy
Monoclonality
B-cell markers CD20
Germinal Centers
Clinical
Similar presentation to nodular lymphoid hyperplasia
5th-6th decade
Male=Female
Asymptomatic 50%
5 year survival 85-95%
Surgical resection
Rare recurrence
Air bronchograms are universally

present and the lymphoid infiltration
gradually diminishes resulting in the
classical indistinct margin
Figure 1-5-14

[Figure 1-5-14
Imaging
Radiography
Solitary nodule/mass
Multiple
Consolidation
Air bronchogram
50%
Slow Growth
CT
Consolidation
Air bronchograms
Airway narrowing or stretching
Grossly low grade B-cell lymphoma

usually presents as a single white tan
lesion that can be either well
circumscribed or indistinct. This is
well demonstrated by the gross
specimen on the left from the AFIP
archive. The disease can, however,
be multifocal as shown on the right
and has been reported as a primarily
endobronchial lesion.
58
Chest Radiology
Primary Tracheal Lymphoma
Figure 1-5-15
Extremely rare
BALT derivative
Extensive at diagnosis
Potentially curable
Lymphomatoid Granulomatosis
[Figure 1-5-15]
Pathologic features
Majority of cases are B-cell lymphomas
Reactive small T-cells
Malignant B-cells
Majority of infiltrate
Epstein-Barr Virus
Angiocentric infiltration
Necrosis
Peribronchovascular
Peripheral
Clinical
7-85 years (mean 48 yrs)
Male:Female (2:1)
Malaise and weight loss
Lung involvement 100%
Cough and dyspnea
Skin 39-53%
Nodules, ulcers and rash
CNS 37-53%
Renal 32-40%
High mortality rate 53-90%
Most proceed to lymphoma
Lymphomatoid Granulomatosis is an
angiocentric B-cell lymphoma which
often demonstrates areas of necrosis.
Figure 1-5-16
Imaging
Nodules 80%
Multiple
Bilateral (80%)
Mid and lower lobes
Cavitation 20%
Large masses
Correspond to infarcts
Diffuse reticulonodular opacities
Hilar adenopathy 25%
Chest CT on the left demonstrates a

bronchovascular distribution of
nodules that are shown to be areas of
infarction on gross examination.
LYG [Figure 1-5-16]

Pulmonary Lymphoid Disorders Derivations of BALT
Immune impairment
Posttransplantation Lymphoproliferative Disease (PTLD)
AIDS
Other forms of prolonged immune suppression
Chest Radiology
59
Lymphoma and Immune Impairment [Figure 1-5-17]
Pathologic Features
B-cell non-Hodgkins lymphoma
Driven by Epstein-Barr Virus infection
Diffuse polyclonal expansion
Reduced T-cell control
Malignant transformation
Figure 1-5-17
Lymphoma and Immune Impairment
Clinical
Spectrum of benign to malignant
Infectious mono-like
PTLD polymorphic
PTLD monomorphic
Cyclosporin shortens induction (<1 year)
May respond to reduction in
immunosuppression, anti-virals and surgery
Chemotherapy should be avoided
Heart-lung up 20%
Imaging
Nodules
May cavitate
Halo
Along bronchovascular bundles
Lymph node
Ground glass
Septa thickening
Consolidation
Effusion
Post transplant lymphomas are driven by EpsteinBarr virus, reduced T-cell surveillance and
malignant transformation. Genetic mutation may
eventually result in malignant transformation of
one of these clones, represented in purple.
PTLD
[Figure 1-15-18]
Bone Marrow Transplant

Prolonged Chemotherapy
Figure 1-5-18

Derivations of BALT
Non-Hodgkin lymphomas
Immune impairment
Posttransplantation Lymphoproliferative Disease
PTLD
AIDS
Other
58 year old male with alpha 1 antitrypsin

deficiency who underwent a double lung transplant
7 years prior to presenting with shortness of
breath, cough, fever and chills for 2 months. The
CT reveals multiple multiple indistinct nodules in a
characteristic distribution along the
bronchovascular bundles
60
Chest Radiology
Follicular Hyperplasia [Figure 1-5-19]
Figure 1-5-19
Diffuse Hyperplasia of BALT-LIP [Figure 1-5-20]

Nodular Lymphoid Hyperplasia [Figure 1-5-21]
Low-Grade B-Cell Lymphoma [Figure 1-5-22]
LYG [Figure 1-5-23]
Follicular Bronchitis.
Figure 1-5-20
Figure 1-5-21
Lymphocytic Interstitial Pneumonia (LIP).
Figure 1-5-22
Nodular Lymphoid Hyperplasia which was

formerly known as Pseudolymphoma.
Figure 1-5-23
Low grade B-cell Lymphoma
Chest Radiology
61
Immune Impairment-PTLD [Figure 1-5-24]
Figure 1-5-24
BALT - The organizing principle
Lymphoid collections
Basis of pulmonary lymphoid disorders
Post Transplant Lymphoproliferative

Disorder
References
General
1. Koss MN. Pulmonary lymphoid disorders. Semin Diagn Pathol. 1995 May;12(2):158-71.
2. Travis WD, Galvin JR.Non-neoplastic pulmonary lymphoid lesions. Thorax. 2001 Dec;56(12):964-71.
62
Chest Radiology
Angiitis and Granulomatosis

First characterized by Averill Liebow 1973

Unknown etiology
Angiitis
Cellular infiltration of blood vessel
Granulomatosis
Necrosis of lung parenchyma not related to blood vessel occlusion
Angiitis and Granulomatosis: Current List
Wegeners granulomatosis
Churg-Strauss syndrome
Allergic granulomatosis
Necrotizing sarcoid granulomatosis
Bronchocentric granulomatosis
Angiitis and Granulomatosis: General Concepts
Etiology remains unknown

Inflammatory vs. lymphoproliferative
Clinical and laboratory findings key to Dx
Adequate tissue samples are important
Must R/O infection: mycobacterial or fungal
Pathogenesis of Vasculitis
Angiitis and Granulomatosis: Differential
Multiple vessel associated nodules [Figure 1-6-1]
Metastatic disease
Squamous
Multifocal infection
Fungus, TB, bacteria
Septic emboli
Multiple pulmonary infarcts
Langerhans cell histiocytosis
Rheumatoid nodules
Figure 1-6-1
Wegeners Granulomatosis: Classic Pathology Triad
Vasculitis described 1852

Von Rokitansky
Wegener described 1936
Wegeners
Focal vasculitis of
Arteries and veins
Necrotising granulomas
Upper and lower airways
Necrotising glomerulitis
Focal
Vasculitis in the lung fits into the

differential of vessel associated
nodules
Chest Radiology
63
Wegeners Granulomatosis: Gross Pathology

Figure 1-6-2
Necrotic nodules
With and without cavitation
Parenchymal consolidation
Massive hemorrhage
Airway narrowing
Wegeners Granulomatosis: Demographics
Rare
3/100,000 in US
2nd-8th decades of life
Average age-50 years
Male=Female
Slight male predominance (4:3)
May occur in children
Wegeners Granulomatosis: Limited
Involvement of lungs alone

Clinical sparing
Kidneys
Upper respiratory tract
Biopsy positive
When clinically normal
Better prognosis
Wegeners Granulomatosis: Clinical Presentation
Classic triad
Sinusitis
Pulmonary symptoms
Renal insufficiency
Variable onset and course
Chronic URI symptoms
May persist for years before pulmonary disease
Overwhelming vasculitis
Diffuse
Solid and cavitary nodules often

coexist in patients with Wegeners
granulomatosis
Figure 1-6-3
Upper Airway
Chronic nasal obstruction

Chronic discharge
Destruction of cartilaginous nasal septum
Saddle nose deformity
Laryngeal involvement
Subglottic stricture
Eustachian tube obstruction
Otitis media
Cochlear nerve vasculitis
Pulmonary
Most commonly affected (94%)

Multiple bilateral nodules or masses
Cavitation common (30-50%)
Occasionally solitary mass or nodule
Dx difficult
All patients progress
Less common
Diffuse alveolar hemorrhage
Pleural lesion and effusions are rare
Airway narrowing is a common

complication
64
Chest Radiology
Renal
Figure 1-6-4
Tempo: insidious to explosive

Segmental necrotizing glomerulonephritis
UA: erythrocyte casts and proteinuria
Large vessel vasculitis
Wegeners Granulomatosis: Other Organ Involvement
Skin (50%)
Symmetric papulonecrotic lesion of extremities
Eye and orbit (30%)
Scleritis, conjunctivitis, optic nerve and retro-orbital mass
Nervous system (30%)
Mononeuritis multiplex
Joints
Acute arthritis follows activity of disease (+RA latex)
Wegeners Granulomatosis: Airway Involvement

Endobronchial abnormalities
59% bronchoscopy
Subglottic stenosis
Tracheobronchitis
Ulcerating
Tracheal or bronchial stenosis
Often multifocal
Variable length of involvement
CT key for evaluation
CXR often normal
Focal airway narrowing is a common

complication in Wegeners
Wegeners Granulomatosis: Radiography
Earliest lesions
Bilateral reticulo-nodular opacities
Multifocal nodules
Bilateral
5mm-10cm
Sharply marginated
Cavitation 20-50%
Evolution
Thick walls to thin walled cysts with treatment
Airspace consolidation
Figure 1-6-5
Changing Presentation
Necrosis and Hemorrhage [Figure 1-6-6]
Figure 1-6-6
Collapse due to airway narrowing in

Wegeners
Massive necrosis and

hemorrhage in Wegeners
Chest Radiology
65
Evolution with Treatment
Figure 1-6-8
Figure 1-6-7
Infections are a common complication
Figure 1-6-9
Nodules in varying stages
Wegeners Granulomatosis:
Computed Tomography [Figure 1-6-9]
Feeding vessels
88%
Cavitation
Nodules greater than 2cm
Subpleural location
Predominant
CT halo sign
Pleural based lesions
Mimic infarcts
Reveals more nodules
Diffuse Pulmonary Hemorrhage: Capillaritis

[Figure 1-6-10]
Common
Microscopic
polyangiitis
Wegeners
granulomatosis
SLE
Uncommon
Goodpastures
Anti-GBM
Collagen vascular
Idiopathic pulmonary
hemorrhage
Churg Strauss
syndrome
Behcets syndrome
IgA Nephropathy
Figure 1-6-10
Nodules with feeding vessels are

common in vasculitis
Pulmonary hemorrhage in capillaritis

66
Chest Radiology
Microscopic Polyangiitis
[Figure 1-6-11]
Microscopic polyarteritis nodosa

Most common cause of pulmonary-renal syndrome
5th decade
Male > Female
Renal, muskuloskeletal, pulmonary, GI and cutaneous
Figure 1-6-11
Microscopic
polyangiitis
Wegeners Granulomatosis: Laboratory
ANCA
Serum Antineutrophil Cytoplasmic Autoantibody
c-ANCA cytoplasmic pattern
Proteinase 3
99% specificity and 96% sensitivity in active disease
Positivity drops to 30% in remission
p-ANCA perinuclear pattern
Reacts with myeloperoxidase
positive in collagen vascular diseases
Wegeners Granulomatosis: Treatment and Prognosis
Universally fatal without treatment

Trimethoprim/Sulfa effective in localized disease
Steroids and cyclophosphamide
Remission in 93%
5 year survival 90-95%
Infectious complications
Relapse and drug toxicity require close monitoring and follow-up imaging
Relapse has different manifestations from presentation
Churg-Strauss Syndrome: Allergic Angiitis and Granulomatosis
Described by Churg and Strauss

1951
True systemic vasculitis
Associated
Asthma
Allergic rhinitis
Blood eosinophilia
Hypersensitivity response to inhaled antigen?
Chest Radiology
67
Churg-Strauss Syndrome: Pathology
Necrotizing vasculitis
Eosinophilic tissue infiltration
Allergic granulomas
Extravascular
Eosinophils
Multinucleated giant cells
Churg-Strauss Syndrome: Demographics
2nd-4th decades
28 years mean age of onset
Male=Female
Excellent response to steroids
Churg-Strauss Syndrome: Background
Late onset asthma

100%
Precedes CSS by weeks to years (30)
Severe rhinitis and sinusitis
70%
Churg-Strauss Syndrome: Prodromal Stage
Infiltration of tissues with eosinophils

Blood eosinophilia
Elevated IgE
+ rheumatoid factor
Progressive asthma, sinus pain, myocardial involvement
Lofflers like fleeting infiltrates
Abdominal pain
Diarrhea and eosinophilic peritonitis
Myalgias and neuritis
Churg-Strauss Syndrome: Vasculitic Stage
Increasingly severe and widespread symptoms

Lung
Eosinophilic consolidation, miliary to 2 cm nodules (without cavitation), and
diffuse hemorrhage
Cardiac
Coronary vasculitis and eosinophilic myocarditis (50% of mortality)
GI
Ulcerations, perforations and peritonitis
Churg-Strauss Syndrome: Computed Tomography
Parenchymal opacification
Predominantly peripheral 59%
Effusions
Nodules
12%
Bronchial thickening
Dilatation
12%
Interlobular septal thickening
6%
Worthy et. Al. AJR Feb. 1998
Churg-Strauss Syndrome: Comparison with Wegeners
CSS
High incidence of asthma
High incidence of cardiac involvement (47%)
Less severe renal and sinus disease
Associated with P-ANCA
68
Chest Radiology
Churg-Strauss Syndrome: Therapy and Prognosis
Prognosis relates to early diagnosis and therapy

High dose steroids usually effective
Cyclophosphamide in resistant cases
Therapy stopped after 6-12 months of remission
Necrotizing Sarcoid Granulomatosis

How is this related to sarcoidosis?
A distinct entity?
Katzenstein
Some reported cases are undiagnosed infections
Those with extrapulmonary involvement
Sarcoidosis
Necrotizing Sarcoid Granulomatosis: Demographics
3rd to 7th decades

Mean age 49 years
Female:male
2.2:1
Figure 1-6-12
Necrotizing Sarcoid Granulomatosis: Pathology
Non-caseating granulomas
Similar to sarcoidosis
Vasculitis
Pulmonary arteries
Pulmonary veins
Found in areas away from parenchymal granulomas
Coagulative necrosis
Widespread
Main distinction from sarcoidosis
Necrotizing Sarcoid Granulomatosis:

100% lung involvement

Cough most common symptom
Chest pain, fever and dyspnea
Weight loss and fatigue
May be asymptomatic
15-40%
Rare extrapulmonary involvement
13%
Aspergillus antigens in some patients
Koss et al, Human Pathology 1980
Necrotizing Sarcoid Granulomatosis: Imaging

[Figure 1-6-12]
Hilar adenopathy
Variable
Up to 79%
Nodules
Cavitation is common
Subpleural
Perivascular
Parenchymal opacities
Same distribution
Necrotizing Sarcoid Granulomatosis:

Prognosis and Therapy
May require no therapy

Prompt response to steroids
No reported deaths
Chest Radiology
Typical nodules in NSG
69
Lymphomatoid Granulomatosis: Etiology and Demographics

[Figure 1-6-13]
Majority of cases are B-cell lymphomas

Epstein-Barr Virus
Reactive small T-cells
Majority of infiltrate
Malignant B Cells
Age range
7-85 years
Mean age of onset
48 years
Male:Female (2:1)
Figure 1-6-13
Lymphomatoid Granulomatosis: Pathology
Angiocentric infiltration
Mixed cell population
Atypical lymphocytes, plasma cells, histiocytes
Vascular invasion
Vascular destruction
Necrosis
Peribronchovascular
Peripheral
Lymphomatoid Granulomatosis: Clinical Presentation
Lung involvement
100%
Cough and dyspnea
Skin
39-53%
Nodules, ulcers and rash
CNS
37-53%
Renal
32-40%
Malaise and weight loss
35%
LYG is a large B-cell lymphoma
Figure 1-6-14
Lymphomatoid Granulomatosis: Imaging

[Figure 1-6-14]
Nodules
80%
Multiple
Bilateral (80%)
Mid and lower lobes
Cavitation
20%
Large masses
Correspond to infarcts
Diffuse reticulonodular opacities
Hilar adenopathy
25%
Peripheral opacities in LYG
Lymphomatoid Granulomatosis: Treatment and Prognosis
Mortality rate
53-90%
Long term remissions reported
Cyclophosphamide and steroids
All who fail therapy proceed to develop lymphoma
12-47%
70
Chest Radiology
Bronchocentric Granulomatosis
Clinical and Demographics Asthmatics
Figure 1-6-15
Average age 22 years

Tissue manifestation of ABPA
Dyspnea, cough, fever, malaise and hemoptysis
Peripheral and tissue eosinophilia
No extrapulmonary findings
Clinical and Demographics Non-Asthmatics
Average age 50 years

Males=Females
Fungal infections
Histo, blastomyces, aspergillus
Mycobacterial infections
Idiopathic
Bronchocentric Granulomatosis: Pathology
Nonspecific reaction
Early invasion of mucosa
Histiocytes
Eosinophils
Asthmatics
Neutrophils
Non-asthmatics
Secondary involvement of adjacent arteries
Granulomatous destruction
Bronchial walls
Bronchopneumonia
Distal to affected airways
Mucoid impaction in patients with

BCG
Bronchocentric Granulomatosis: Imaging [Figure 1-6-15]
Figure 1-6-16
Most often unilateral

75%
Multiple or solitary nodules
Associated findings of ABPA
Bronchiectasis
Mucoid impaction
BCG and Tuberculosis

BCG and Aspergillus [Figure 1-6-16]
Treatment and Prognosis
ABPA may be clinically unsuspected

Asthmatics respond to steroids
Some cases remit without treatment
Must rule out treatable infection and Wegeners granulomatosis
Angiitis and Granulomatosis: Differential

Multiple vessel associated nodules
Metastatic disease
Squamous
Multifocal infection
Fungus, TB, bacteria
Septic emboli
Multiple pulmonary infarcts
Rheumatoid nodules
Chest Radiology
71
BCG?
Fungal Infection ?
Angiitis and Granulomatosis: Conclusion
Churg-Strauss syndrome
Allergic granulomatosis
Necrotizing sarcoid granulomatosis
Bronchocentric granulomatosis
Until specific causes are found we must devise syndromes
Etiology
Prognosis
Therapy
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62. Koss MN, Robinson RG, Hochholzer L. Bronchocentric granulomatosis. Hum Pathol 1981; 12(7):632-638.
63. Sulavik SB. Bronchocentric granulomatosis and allergic bronchopulmonary aspergillosis. Clin Chest Med 1988;
9(4):609-621.
64. Berendsen HH, Hofstee N, Kapsenberg PD, Siewertsz Van Reesema DR, Klein JJ. Bronchocentric granulomatosis
associated with seropositive polyarthritis. Thorax 1985; 40:396-397.
65. Clee MD, Lamb D, Urbaniak SJ, Clark RA. Progressive bronchocentric granulomatosis: case report. Thorax 1982;
37:947-949.
66. Felson B, Reeder MM. Gamuts in radiology, second edition. Cincinnati: Audiovisual Radiology of Cincinnati, Inc,
1967: 561-562.
67. Albelda SM, Gefter WB, Epstein DM, Miller WT. Diffuse pulmonary hemorrhage: a review and classification.
Radiology 1985; 154: 289-29
74
Chest Radiology
The Pulmonary Complications of

Organ Transplantation
Introduction
Organ transplant first performed the 1960s

Solid organ for vital organ failure
Hematopoietic stem cell (HSC)
Standard therapy
Malignant, hematologic, autoimmune and genetic diseases
Not proven in breast cancer
Pulmonary Complications in 40-60%: Multifactorial Cause
Underlying disease
Therapy for underlying disease
Graft-vs-host disease
Conditioning regimen
Chemotherapy and radiation
Solid Organ Transplant: Typical Schedule [Figure 1-7-1]
Figure 1-7-1
Solid organ transplant complications begin predominantly

after the first month post-transplant
Bone Marrow Transplant: Typical Schedule
[Figure 1-7-2]
Figure 1-7-2
Complications are usually separated into those that

occur before and after there first 100 day
Chest Radiology
75
Pulmonary Complications of Organ Transplantation
Early Pulmonary Complications
Figure 1-7-3
Pulmonary edema
Fungal infection
Diffuse alveolar hemorrhage
Bacterial infection
Viral infection
CMV and herpes
Pneumocystis carinii
Acute graft-vs-host disease
Idiopathic pulmonary syndrome
ARDS, DAD
Late Pulmonary Complications
Chronic graft-vs-host
Obstructive airways disease
Bronchiolitis obliterans
BOOP
Restrictive ventilatory defect
Late bacterial infections
Sinopulmonary
Herpes varicella zoster
Pretransplant Considerations
Traditionally, allogeneic
transplantation used bone marrow
grafts. From 1999-2002 there was a
steady increase in peripheral blood
stem cell grafts
Residual tumor
Occult infection
Operative Technique [Figure 1-7-3]
Donor aspiration
General anesthesia
150-200 aspirates
Marrow strained
Immunocompetent T-cells
Depleted with monoclonal reagents
Infusion of marrow
400-800 ml
Figure 1-7-4
Immunologic Impact
Profound neutropenia
Prolonged depression
Cellular function
Humoral function
Graft-vs-host
Direct effect
Steroids
Pulmonary Edema
[Figure 1-7-4]
Common complication
2nd-3rd week posttransplantation
Rapid onset
Dyspnea and hypoxemia
Reticulo-nodular markings
Fluid overload
Blood products, antibiotics and TPN
Cardiac
Renal dysfunction
Decreased albumin
Often accompanied by fever
Pulmonary edema in a bone marrow

transplant patient demonstrating
interlobular septal thickening and
enlarged pulmonary veins
76
Chest Radiology
Fungal Infections
Up to 45% of BMT patients

85% mortality
Aspergillus is most common
Occurs in the first 30 days posttransplantation
Symptoms:
Fever, dyspnea, cough, chest pain and hemoptysis
Predisposition
Prolonged granulocytopenia
Broad spectrum antibiotics
Nodules
Early Halo-Sign
Late Air Crescent
Fungal Infections
[Figures 1-7-5 to 1-7-7]
Invasive Aspergillosis: Diagnosis
Figure 1-7-5
Fungi normally
invade the lung
via the airway
Figure 1-7-6
BAL (69%)
Tissue Biopsy (60%)
Antigen (83%)
Computed Tomography (92%)
Caillot, J Clin Oncol. 1997
Imaging and Survival: Invasive

Aspergillosis
Caillot, J Clin Oncol. 1997
Pulmonary Hemorrhage
21% of BMT patients

12th day posttransplantation
Neutrophil recovery
Sudden onset:
Dyspnea, cough, fever and hypoxemia
Rare hemoptysis
Mortality 50-80%
Radiographic abnormalities before symptoms
Bilateral ground glass opacities
May be localized
Typical infarct with a halo of blood in an aspergillus

infection
Figure 1-7-7
Air-crescent sign in a patient with recovering cell counts
Chest Radiology
77
Pulmonary Hemorrhage [Figure 1-7-8]
Figure 1-7-8
Cytomegalovirus Pneumonia
10-40% of BMT patients

6-12 weeks posttransplantation
Mortality rate of 85%
Reactivation of latent virus in 70%
Remainder infected by CMV positive
blood products
Anti-viral therapy improves prognosis
Cytomegalovirus Pneumonia [Figure 1-7-9]
Bilateral
Ground glass
Nodules
Consolidation
Alone
66%
59%
59%
3%
Franquet et al AJR Oct 2003
Pneumocystis Jiroveci Pneumonia
<10% of BMT patients

Effective prophylaxis
Trimethoprim/sulfa
Rapid progression
Severe dyspnea
Bilateral perihilar
Ground-glass opacities
Aspergillus infection may be a cause of pulmonary

hemorrhage
Figure 1-7-9
Noninfectious Pulmonary Complication
Late-onset Noninfectious Pulmonary Complications

LONIPCs
After the first 3 months
10-23% of allogeneic grafts
Idiopathic pulmonary syndrome
Diffuse alveolar damage (DAD)
Organizing pneumonia
BOOP
Associate with GVHD
Sicca syndrome
Acive donor T-cells
Sakaida, Blood Vol 102 2003

Typical lower lobe nodules of CMV
Idiopathic Pulmonary Syndrome
Diffuse lung injury posttransplantation

Histology
Interstitial mononuclear infiltrate
DAD
12% of allogenic BMT
40-80 days posttransplantation
Risk factors
GVHD
Radiation
Fever, cough and hypoxemia
Mortality rate of 70%
Diffuse Opacities
78
Chest Radiology
Figure 1-7-10
[Figures 1-7-10 and 1-7-11]
Infectious agents
Legionella, mycoplasma, viruses
Inhalants
Ammonia, chlorine, HS
Drugs
Cytoxan, BCNU, Bleomycin
Ingestants
Kerosene, Paraquat
Shock/trauma
Sepsis
Radiation
Idiopathic
Hammon-Rich or AIP
Patients with IPS present with diffuse opacities

involving all 5 lobes
Viral Infection
Figure 1-7-11

[Figure 1-7-12]
Graft-vs-Host Disease (GVHD) :

Donor T-lymphocytes recognize the
recipients tissue as foreign
Acute GVHD
20-100 days posttransplantation
25-75% of patients
skin, gut and liver dysfunction
10% mortality
Chronic GVHD
1> 100 days posttransplantation
20-45% of patients
Features of autoimmune diseases
Sjogrens, scleroderma, biliary
cirrhosis and airway obstruction
GVHD and IPS

GVHD and Infection
In the early phase (exudative) there is diffuse consolidation

and ground glass often with peripheral clearing
Figure 1-7-12
GVHD and IPS

Radiation Pneumonitis
Related to dose of TBI

Presents within 90 days
Cough, fever, and dyspnea
Threshold lowered by chemotherapy
Mediastinal Emphysema
Correlates
Idiopathic interstitial pneumonia
Increased likelihood with more radiation
Not a serious complication by itself
May be a harbinger of pneumothorax
The late phase of IPS demonstrates traction bronchiectasis

consistent with fibrosis
Chest Radiology
79
Secondary Malignancies
Figure 1-7-13
0.02% incidence
7Xs increase
Over the general population
1 year after transplantation
Median
Hodgkins 45%
Leukemia 17%
Solid tumors 38%
Pathologic Features
B-cell non-Hodgkin s
Driven by Epstein-Barr virus infection
Bone Marrow Transplant

demonstrating mosaic
attenuation
2-13% of BMTs
Low immunoglobulin level
Chronic GVHD
Sicca syndrome
100 days posttransplantation
Gradual deterioration of PFTS
Airflow obstruction
Fixed
Reduction in diffusing capacity
Imaging
Mosaic attenuation
Expiratory accentuation
Patchy consolidation
Figure 1-7-14
Bronchiolitis Obliterans [Figures 1-7-13 to 1-7-15]

Segmental or Lobar Consolidation
Infection
Diffuse Opacities
Pulmonary edema
Hemorrhage
Viral pneumonia
Pneumocystis pneumonia
Bronchiolitis obliterans demonstrating indistinct

nodules and branching opacities
Figure 1-7-15
Rapid Progression Over 24 Hours
Bacterial pneumonia
Pulmonary edema
Hemorrhage
Progression Over Days
Aspergillus
Pneumocystis
CMV
Bronchiolitis obliterans demonstrating

bronchiectasis
80
Chest Radiology
Diffuse Opacities
Fluid Overload
BMTP: dyspnea and cough
BMTP Lymphangitic Spread
BMTP
BMTP Aspergillus
BMTP: dyspnea and cough
BMTP: Edema
Bone Marrow Transplant: Typical Schedule
References
General
1. Franquet T, Muller NL, Lee KS, Gimenez A, Flint JD.High-resolution CT and pathologic findings of noninfectious
pulmonary complications after hematopoietic stem cell transplantation. AJR Am J Roentgenol. 2005 Feb;184(2):62937
2. Kotloff RM, Ahya VN, Crawford SW.Pulmonary complications of solid organ and hematopoietic stem cell
transplantation. Am J Respir Crit Care Med. 2004 Jul 1;170(1):22-48. Epub 2004 Apr 7
Chest Radiology
81
The Diagnosis of Pulmonary Embolism

Pulmonary Embolus
Frequent
Potentially fatal
Largely undiagnosed
Baglin, J Clin Path, 1997
Pulmonary Embolus: Epidemiology
5 million episodes of DVT

300,000 embolic events
50,000 deaths
100/100,000 new cases
The Clinical Picture of PE
Predisposing factors
Pathology
Signs and symptoms
Radiography
Arterial blood gases
V/Q scanning
Computed tomography
Arteriography
Pulmonary Embolism is a Complication of Deep Venous

Thrombosis
Hull, Annals of Internal Med 1983
Sources of Pulmonary Emboli
Majority of clots
Lower extremity veins
Increasing number of clots
Upper extremities, cardiac chambers and catheters
A negative venous study
Does not rule out PE
< 50% of PE patients
Positive lower extremity study
Kelly, Ann Int Med, 1991
Embolic Events: Predisposing Causes
Stasis
Trauma
Hypercoagulable states
Predisposing Causes
1 thrombophlebitis
Bed rest
Recent surgery
Venous insufficiency
Recent fracture
Myocardial infarction
Malignancy
CHF
No Predisposition
Pulmonary Embolism
39%
32%
31%
25%
15%
12%
8%
5%
6%
82
Chest Radiology
PE and Malignancy: 10-15% of unexplained phlebitis
Gastrointestinal
Pulmonary
Genitourinary
The History and Physical are Non-Specific

Symptoms in Patients with Non-Fatal PE
Chest Pain
Dyspnea
Apprehension
Cough
Hemoptysis
Sweats
Syncope
88%
84%
59%
53%
30%
27%
13%
Signs in Patients with Non-Fatal PE
RR> 16
Rales
HR> 100
T> 37.8C
Diaphoresis
Gallop
Phlebitis
Murmur
Cyanosis
92%
58%
44%
43%
36%
34%
32%
23%
19%
Figure 1-8-1
PE and Underlying Lung Disease

The History and Physical are Insensitive
We do not know the Prevalence of PE.
Diagnostic Algorithm and Clinical
Suspicion [Figure 1-8-1]
Among the various causes of an incorrect
diagnosis, most important are: the failure to
suspect PE and, the protean nature of the
disease.
The clinical diagnosis of PE is unreliable. Many

patients are symptomatic
Morpurgo, Chest 1995
History and Physical

The Role of Clinical Suspicion
Less than 35% of fatal emboli were diagnosed antemortem
Symptoms in Patients with Fatal PE
Dyspnea
Syncope
Altered mentation
Apprehension
Chest pain
Sweatiness
Pleuritic Pain
Cough
Hemoptysis
Arrest
Chest Radiology
59%
27%
20%
17%
10%
9%
8%
3%
3%
8%
83
Pulmonary Embolism
Signs in Patients with Fatal PE
RR> 16
HR> 100
Rales
T> 37.8C
Edema
Hypotension
Cyanosis
Gallop
Diaphoresis
Phlebitis
66%
54%
42%
30%
26%
20%
12%
10%
10%
7%
Figure 1-8-2
The Chest X-Ray is Usually Abnormal

The Chest X-Ray in Pulmonary Embolism
84% had abnormal radiographs

PE(%)
Atelectasis/Infiltrate
68
Pleural Effusion
48
Pleural Opacity
35
Elevated Diaphragm
24
Decreased Vascularity 21
Prominent PA
17
Cardiomegaly
12
Westermarks Sign
7
Pulmonary Edema
4
NoPE(%)
48
31
21
19
12
28
11
2
13
PIOPED
Common Radiographic Abnormalities
Infiltrate
Pleural Effusion
Atelectasis
Diaphragm Up
2 or More
CHF
Focal Oligemia
Normal
54%
51%
27%
17%
44%
17%
2%
7%
Chest CT Findings
Atelectasis
100%
Consolidation
57%
Hamptons hump
50%
Ground glass
57%
Pleural Effusions
87%
Mosaic attenuation
Truong, ARRS, 1998
Radiographic and CT Findings
[Figure 1-8-2]
Peripheral opacities may raise

suspicion for clinically unsuspected
PE
Pulmonary Embolism
84
Chest Radiology
Figure 1-8-3
Edema
Hemorrhage
Infarction
Normal Arterial Oxygenation Does Not Exclude

Pulmonary Embolism
Arterial Blood Gases
10-15% will have a PO2 >85mm HG

A low arterial PO2 is non-specific
A respiratory alkalosis is most common
The Physiology of Pulmonary Embolism
V/Q abnormalities
Variable
Complete vascular occlusion
Rare
Complete shunt 2 to
Atelectasis
Hemorrhage
Autoregulation
Hypoxic vasoconstriction
Hypocapnic bronchoconstriction
Hemorrhage and edema are common

sequela of PE. Infarct is less common
and is more likely to occur in patients
with CHF
Levy, JAP, 1974

Dantzker, Circulation Res, 1974
Dantzker, Chest Vol. 91 no. 5
Physiologic Change with Heparinization
Ventilation
Returns more rapidly than perfusion
Perfusion
May return before ventilation
Santolicandro, Am J Res Crit Care Med, 1995
V/Q Physiology
Ventilation/Perfusion Scanning
High
Intermediate
Low
Normal
Total
Clinical Science Probability (%)

80-100 20-79
0-19 All Probabilities
96% 88%
56% 87% (103/118)
66% 28%
16% 30% (104/345)
40% 16%
4% 14% (40/296)
0%
6%
2%
4% (5/128)
68% 30%
9% 28% (252/887)
PIOPED
The Basis of Clinical Science Probability
Dyspnea
Pleuritic Pain
Cough
Leg Swelling
Hemoptysis
Palpitations
Wheezing
Angina
PE(%)
73
66
37
28
13
10
9
4
No PE(%)
72
59
36
22
8
18
11
6
PIOPED
Chest Radiology
85
Pulmonary Embolism
Traditional Approach [Figure 1-8-4]
Figure 1-8-4
The Low-Probability Lung Scan
There is an 8% mortality rate in patients with a low probability V/Q scan and
limited cardiopulmonary reserve. Hull, Archives of Internal Medicine, 1995
There is a 25%-30% disagreement between expert readers in interpreting
INTERMEDIATE and LOW probability V/Q scans. PIOPED, JAMA, 1990
A Potentially Lethal Reading
Pulmonary embolism cannot be diagnosed on clinical grounds; it can only
be suspected. Bone, Archives of Internal Medicine, 1993
The Goal of Imaging Visualization of the clot

The Role of Pulmonary CT Angiography
Initial screening
Detection of unexpected emboli
Detection of other pathology
CT Angiography
Pulmonary CT Angiography Sensitivity and Specificity
Accurately identifies emboli

Main, lobar and segmental vessels
Misses some subsegmental emboli
Indeterminate 8-10%
Constantly changing
Related to collimation, scanner speed and prevalence
Sensitivity 66-93%
Specificity 89-97%
Eng, AJR 183; 2004
Pulmonary CT Angiography Sensitivity and Specificity
3mm visualizes 40% of subsegmental arteries

3mm visualizes 75% of segmental arteries
1.25mm visualizes 75% of subsegmental arteries
1.23mm visualizes 90% of segmental arteries
Patel, Radiology, 2003
Significance of Small Emboli Standard Angiography
Good outcome
Patients with negative angio
1.5% embolize when followed 1 year
691 patients
Novelline, Radiology, 1978
Pulmonary Embolism
86
Chest Radiology
Distribution of Pulmonary Emboli
Multiple locations
> 55%
Marked preference for
Right lung and lower lobes
Subsegmental only
6-30%
PIOPED, JAMA, 1990; Oser, Radiology, 1996; Morpurgo, Chest, 1995
Significance of Small Emboli - CTA
Small emboli that need treatment

Poor cardiopulmonary reserve
Coexisting acute DVT
Recurrent PE
Small emboli that may not need treatment
Subsegmental clots without evidence of DVT
Indeterminate scan without evidence of DVT
Normal cardiovascular status
Follow-up DVT scan in 1 week
Goodman, Radiology 234; 2005
Pulmonary CT Angiography
Negative Predictive Value of a Normal CT
No prospective, consecutive studies

The safety of withholding anticoagulantsis uncertain
Negative Predictive Value of a Normal CT
n
Mayo
69
Feretti
109
Garg
78
Loomis
81
Goodmann
198
Remy-Jardin
71
Tillie-Leblond 185
Kavanagh
85
Follow-up
3m
3m
6m
6m
3m
3m
12m
9m
NPV
97%
97%
99%
100%
99%
97%
98%
99%
Pulmonary CT Angiography Intra and Interobserver Variability
Radiologys Achilles Heel

Related to clot size
Exacerbated by poor exam
Related to reader experience
Mayo, Radiology, 1997; Chartrand-Lefebre, AJR, 1999
Alternate diagnoses
11-33%
Unexpected emboli
1-4%
Storto, AJR:184 2005
Chest Radiology
87
Pulmonary Embolism
Alternative Diagnoses
[Figure 1-8-5 and 1-8-6]
Figure 1-8-6
Figure 1-8-5
Pulmonary infection is a common alternative

diagnosis is patients suspected of pulmonary
embolus
Adenocarcinoma is an important predisposition for

hypercoaguability and PE
Pitfalls in Helical CT [Figure 1-8-7 and 1-8-8]
Partial volume
Obliquely oriented arteries
Suboptimal contrast enhancement
Breathing artifacts
Lymph nodes
Figure 1-8-8
Figure 1-8-7
Adenopathy can mimic PE
Breathing artifacts should be

assessed before reading a CTA for
PE
Pulmonary Embolism
88
Chest Radiology
Technical Improvements
Multi-channel CT
Narrower collimation: 1mm
Subsecond scanning
Contrast timing
Smart prep
Test bolus: peak + 5 sec
20 seconds normal cardiac output
Caudal-cranial scanning
Workstation viewing
Cine Mode (PACS or Workstation)
Adjust window and levels for each case
Multi-planar reconstruction
Breathing artifact-coronal lung windows
Figure 1-8-9
16 Channel CT [Figure 1-8-9]
Coronal reconstrution helps separate

pulmonary arteries and veins
Paddlewheel Reformation
Simon, AJR:177 July 2001
Combined Pulmonary CTA and Venography
Increases detection of thromboembolic disease by 20%

Contiguous sections
1cm collimation
Cham, Radiology;234 2005
Combined Pulmonary CTA and Venography

Year
Loud
00
Duwe
00
Garg
00
Cham
00
Peterson 01
N
150
74
70
116
136
Sensitivity
97%
89%
100%
100%
71%
Specificity
100%
94%
97%
96%
93%
A Diagnostic Algorithm for Pulmonary Embolism
Pulmonary Embolus and Prognosis
The prognosis in PE patients is closely related to the

presence and extent of clot in the peripheral veins
Chest Pain-Dyspnea Screening

Conclusion
The clinical diagnosis of PE is unreliable

The chest radiograph is usually abnormal
V/Q readings restricted to reliable categories
Small clots are a problem for all modalities
Outcome studies are key
CT angiography is the modality of choice
Chest Radiology
89
Pulmonary Embolism
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General
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7. Falashci F, Palla A, Formichi B, Sbragia P Petruzzelli S, Guintini C, Bartolozzi C. CT evaluation of chronic
thromboembolic pulmonary hypertension. Journal of Computer Assisted Tomography 1992; 16:897-903.
8. Garg K, Welsh CH, Feyerabend AJ, Subber SW, Russ PD, Johnston RJ, Durham JD, Lynch DA. Pulmonary
embolism: diagnosis with spiral CT and ventilation-perfusion scanningcorrelation with pulmonary angiographic
results or clinical outcome. Radiology 1998; 208(1):201-8.
9. Gefter W, Hatabu H, Holland G, Gupta K, Henschke C, Pavelsky H. Pulmonary Thromboembolism: recent
developments in diagnosis with CT and MR imaging. Radiology 1995; 197:561-574.
10. Geraghty JJ, Stanford W, Landas S, Galvin J. Ultrafast computed tomography in experimental pulmonary
embolism. Investigative Radiology 1991; 27:60-63.
11. Goodman LR. Small pulmonary emboli: what do we know? Radiology. 2005 Mar;234(3):654-8.
12. Goodman LR, Curtin JJ, Mewissen MW, Foley WD, Lipchik RJ, Crain MR, Sagar KB, Collier BD. Detection of
pulmonary embolism in patients with unresolved clinical and scintigraphic diagnosis: helical ct versus
angiography. American Journal of Roentgenology 1995; 164:1369-1 374.
13. Goodman LR, Lipchik RJ. Diagnosis of acute pulmonary embolism: time for a new approach. Radiology 1996;
199:25-27.
14. Goodman LR, Lipochik RJ, Kuzo RS. Acute pulmonary embolism: the role of computed tomographic imaging.
Journal of Thoracic Imaging 1997; 12(2):83-86.
15. Goodman LR. Helical CT for initial imaging of pulmonary embolus. AJR Am 3 Roentgenol 1998; 171(4):1153-4.
16. Gurney JW. No fooling around: direct visualization of pulmonary embolism. Radiology 1993; 188:618-619.
17. Kim KI, Muller NL, Mayo JR. Clinically suspected pulmonary embolism: utility of spiral CT. Radiology 1999;
210(3):693-7.
18. Mayo JR. Remy-Jardin M, Muller NL, Remy J, Worsley DF, Hossein-Foucher C, Kwong JS, Brown MJ.
Pulmonary embolism: prospective comparison of spiral CT with ventilation-perfusion scintigraphy. Radiology
1997; 205(2):447-52.
19. Remy-Jardin M, Remy J, Artaud D, Deschildre F, Fribourg M, Beregi JP. Spiral CT of pulmonary embolism:
technical considerations and interpretive pitfalls. J Thorac Imaging 1997; 12(2):103-17.
20. Remy-Jardin M, Remy J, Deschildre F, Artaud D, Beregi JP Hossien-Foucher C, Marchdise X, Duhamel A.
Diagnosis of pulmonary embolism with spiral ct:comparison with pulmonary angiography and scintigraphy.
Radiology 1996;200:699-706.
21. Remy-Jardin M, Remy J, Wattinne L, Giraud F. Central pulmonary thromboembolism: diagnosis with spiral
volumetric ct with single-breath-hold technique-comparison with pulmonary angiography. Radiology 1992;
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Magnetic Resonance
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1996; 167:653-655.
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1996; 36:503-508.
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developments in diagnosis with CT and MR imaging. Radiology 1995; 197:561-574.
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Pulmonary Embolism
92
Chest Radiology
Tuberculosis
Tuberculosis
Leading cause of death from infectious disease

8-10 million new cases/year
2-3 million deaths/year
1/3 of world population infected
> 90% of new cases in developing countries
80% 15-59 years of age
Highest incidence
Southeast Asia: 247/100,000
Sub-Saharan Africa: 191/100,000
HIV co-infection: 60% of children, 70% of adults
Tuberculosis: History
Ancient disease
1882: Robert Koch
Isolation of M. tuberculosis
1944: streptomycin
1952: INH
Tuberculosis Pre-Antibiotic Era

Tuberculosis: United States
1953: 84,304 cases

19,707 deaths
1985: 22,201 cases
1,752 deaths
1986-1992: 20% increase in reported cases
HIV
Immigration
Congregate settings
Deteriorating TB services
MDR-TB
Decreasing TB research
Tuberculosis: United States
2002: 15,075 cases

5.2/100,000
43% decrease from 1992
4-6% of population infected
15 million people
51% Foreign-born
Mexico, the Philippines, Vietnam, India and China
U.S. -born
African Americans 25% of all cases
homeless, immunocompromised, elderly
Urban areas, coastal states, states bordering Mexico
NMWR,March 21, 2003 Vol 52
Mycobacteria
Tuberculosis complex
M. tuberculosis, M. bovis, M. africnum, M. microti
M. tuberculosis and M. bovis
> 95% of pulmonary mycobacterioses
Slow growth
Person-to-person transmission
Chest Radiology
93
Tuberculosis
M. tuberculosis: Pathologic features
Gram positive pleomorphic rod

Acid fast:
Resists decolorization with acid alcohol
Virulence related to cell wall
No endotoxin or enzymes
Caseous necrosis
Caseating granuloma
Central caseous necrosis
Rim of histiocytes, giant cells
Figure 1-9-1
Caseous Necrosis [Figure 1-9-1]

Tuberculosis: Pathogenesis
Inhaled bacteria [Figure 1-9-2]

Mid to lower lung zones
Ghon focus
Figure 1-9-2
Even though we tend to

think of TB as an upper
lobe disease, we inhale
most bacteria into the
mid and lower lung
zones
The caseating granuloma is the

hallmark of TB. The actively growing
bacilli reside in the macrophages in
the periphery
Figure 1-9-3
Physiologic Gradients-Airflow FRC

Inhaled bacteria
Mid to lower lung zones
Ghon focus
Regional lymph node spread [Figures 1-9-3]
Ranke complex
Lymphatic/hematogenous dissemination
Cell-mediated immunity
Delayed hypersensitivity
Caseous necrosis
2-10 weeks
Healing
Tuberculosis
In primary tuberculosis the ineffective

macrophages carry bacteria to
regional lymph nodes where they
proliferate and disseminate
94
Chest Radiology
Latent TB infection
+PPD
No active signs of infection
Survival of organisms [Figures 1-9-4]
Apical/posterior upper lobe
Superior segment lower lobe
Oxygen gradient
Lymphatic gradient
Bucket handle rib motion
Active TB infection [Figures 1-9-5]
5% within 2 years
5-10% lifetime risk
HIV: 50% within 2 years
Pulmonary fibrotic lesions, underweight, silicosis, DM, renal
failure, gastrectomy, jejunoileal bypass, transplantation, head
and neck cancer, prolonged immunosuppressive therapy
Tuberculosis: Clinical features
The lymphatic gradient helps explain

the upper lobe distribution of
reactivation tuberculosis
Primary TB
Postprimary TB
Disseminated TB
Figure 1-9-5
Primary Tuberculosis: Clinical features
Figure 1-9-4
Asymptomatic 65%
Nonspecific symptoms when present
Progressive primary complex
Fever, cough, hemoptysis, weight loss
Primary Tuberculosis: Radiologic features
Lymphadenopathy [Figure 1-9-6]

Children 95%, young adults 43%, elderly 10%
Right paratracheal, hilar
Peripheral enhancement, central low-attenuation
Atelectasis, overinflation [Figure 1-9-7]
Children
Anterior segements upper lobes
Medial segment middle lobe
Consolidation
Unifocal 75%
Figure 1-9-6
Segmental, lobar,
multifocal
Homogeneous, patchy,
linear, nodular
Pleural effusion
Adults 38%, children
11%
Leung, Radiology 1999, Vol 210
The lymphatic gradient helps

explain the upper lobe distribution
of reactivation tuberculosis
Lymphadenopathy is hallmark
of primary TB and is more
common in children
Chest Radiology
95
Tuberculosis
Postprimary Tuberculosis: Clinical Features
Reactivation
Fever, malaise, anorexia, weight loss, anorexia, night sweats
Dyspnea, cough, chest pain, hemoptysis
Active TB infection
5% within 2 years
5-10% lifetime risk
HIV: 50% within 2 years
Pulmonary fibrotic lesions, underweight, silicosis, DM, renal
failure, gastrectomy, jejunoileal bypass, transplantation, head
and neck cancer, prolonged immunosuppressive therapy
Figure 1-9-7
Postprimary Tuberculosis: Pathogenesis

Delayed hypersensitivity
Liquifaction
Cavitation
Airway
Vessel
Pleura
Figure 1-9-8
The lymph nodes which surround
airways may cause narrowing that
results in atelectasis
Figure 1-9-9
Postprimary TB implies
reactivation of dormant bacilli. It is
characterized by tissue destruction
Cavitation and necrosis enables spread via the airway,
blood stream or pleura
Postprimary Tuberculosis: Radiologic features
Consolidation 50-70%
Cavitation 40-45%
Nodules
Airways involvement
Tuberculosis
96
Chest Radiology
Postprimary Tuberculosis: Radiologic features
Consolidation 50-70%
Heterogeneous, nodular, linear
Apical, posterior 85%, Superior segments 14%
Cavitation 40-45% [Figure 1-9-10]
Thin or thick walls, air-fluid levels 20%
Nodules
Tuberculoma
SPN: variable borders, satellite lesions,
upper lobes
Endobronchial spread [Figures 1-9-11 to 1-9-13]
Centrilobular, tree-in-bud, 100% by CT
Hematogenous spread
Miliary 1-3mm, random
Airways involvement
Bronchiectasis, bronchitis, airway narrowing
Figure 1-9-10
Cavitation implies a large number of bacilli speeds

the progression of disease
Figure 1-9-11
Figure 1-9-12
Endobronchial spread leads to airways nodules

Endobronchial spread leads to
airways nodules
Figure 1-9-13
Postprimary Tuberculosis-Cavitation
Thoracoplasty
Oleothorax
Plumbage [Figure 1-9-14]
Postprimary Tuberculosis-Nodules
Postprimary Tuberculosis-Airways
Endobronchial spread leads to airways nodules
Chest Radiology
97
Tuberculosis
Postprimary Tuberculosis: Assessment of Activity
Cannot discern activity from a single film

Inactive disease
radiographic stability
6mos
Negative cultures
Suggestive of active disease
Cavitation
Consolidation
Ground glass
Centrilobular opacities
Figure 1-9-14
Lee et al, Chest, 1996
Tuberculosis: Complications
End-stage disease
Hemoptysis
Bronchial arteries in chronic cavities
Mycetoma
Rassmussen (pulmonary artery) aneurysm
Chest wall involvement
Pericardial involvement
Empyema
BPF, empyema necessitatis
Images demonstrate broncho-esophageal fistula

which was a complication of plumbage
Hemoptysis-Bronchial Artery
Hemoptysis-Mycetoma
End-Stage Lung
Tuberculosis-Chest Wall
Tuberculosis-Pericardial
Tuberculosis: HIV/AIDS
CD4>200
Well formed granulomas
Upper lobe cavities, consolidation and nodules
CD4<200
Poorly formed granulomas
Adenopathy, consolidation and miliary disease
CD4<60
No hypersensitivity reaction
Organisms spread from GI tract
Miliary Disease
Tuberculosis and AIDS

Tuberculosis and AIDS-Low CD4
Tuberculosis: Diagnosis
Conventional methods
Acid-fast smear: 1 day
Culture: 1-2 weeks
Identification: 2-3 weeks
Drug susceptibility testing: 3-4 weeks
Radiometric methods
Polymerase chain reaction (PCR)
HPLC
Tuberculosis
98
Chest Radiology
Summary
Primary TB
Consolidation
Ipsilateral lymphadenopathy
Pleural effusion
Postprimary TB
Consolidation
Cavitation
Apical/posterior upper lobe nodules
Tracheobronchial spread
Tuberculosis Pre-Antibiotic Era

References
General
1. Leung AN.Pulmonary tuberculosis: the essentials. Radiology. 1999 Feb;210(2):307-22
Chest Radiology
99
Tuberculosis
Fungal Disease in the Thorax:

Opportunistic and Primary Pathogens
Fungal Disease in the Thorax: Overview
Opportunistic invaders
Aspergillus species
Candida
Mucormycosis
Primary pathogens
Histoplasma capsulatum
Blastomyces dermatitidis
Coccidioides immitis
Opportunistic Invaders
Immunocompromised host
Mucosal disruption
Reduced cellular and/or humoral immunity
Ubiquitous
Lack dimorphism
Multiple organisms may occur
Figure 1-10-1
Primary Pathogens
May infect healthy individuals

Dimorphism
Saprophytes in the soil
Spores via germination
Most disease mild or subclinical
Fulminant or chronic disease may occur
Specific geographic regions
Endemic
Coccidioidomycosis
The mycelial form of Histoplasmosis is found in
soil that has been enriched with bird droppings.
The fungus then releases conidia or spores
Blastomycosis
Histoplasmosis
Histoplasmosis: Epidemiology and Ecology
Endemic fungal disease

Ohio, Mississippi and St. Lawrence river valleys
Reported worldwide but relatively rare outside of the United States
Infection rate up to 95% in endemic areas
Point sources associated with aerosolization
Earth moving, bird husbandry and spelunking
Dimorphic fungus
Clinical
Histoplasmosis: Epidemiology and Ecology
Endemic fungal disease

Dimorphic fungus
Mycelial form in high nitrogen soil
Guano from birds and bats
Yeast within the infected host
Clinica
Histoplasmosis [Figure 1-10-1]
Fungal Diseases
100
Chest Radiology
Histoplasmosis: Pathology [Figure 1-10-2]
Early sequence of infection

Mycelia produce micronidia
Micronidia reach alveolar spaces
2-5 microns
Histoplasmosis: Pathology [Figure 1-10-3 and 1-10-4]
Early sequence of infection

Lymphocytes and macrophages replace polys
Micronidia transform to conidia or spores
Spores transform into budding yeast
Macrophages phagocytose and kill yeast
Late sequence of infection
Lymphocyte-mediated cellular immunity
Necrosis
Fibrosis
The fungal
spores are able
to reach the
alveolar level,
bypassing the
upper airway
defenses
because of
their small size
which is less
than 5 microns
Figure 1-10-2
Histoplasmosis: Pathology
Distinction from tuberculosis

Histoplasmosis relatively benign
Immunity to histoplasmosis short lived
20% lose immunity each year
Continuous reinfection
Primary and postprimary not appropriate
Histoplasmosis: Clinical
Asymptomatic
95-99% of infection in endemic areas
Parenchymal opacities in 10-25%
Small inoculum or prior infection (cellular
immunity) and moderate inoculum
Symptomatic
Acute
Moderate vs large inoculum
Chronic
Disseminated
Late complications
Histoplasmoma
Broncholithiasis
Mediastinal granuloma
Mediastinal fibrosis
Signs and symptoms

Flulike: fever, chills, cough
Retrosternal pain
Mediastinal lymph node involvement
Erythema nodosum in women
Arthralgia
Shorter incubation with prior exposure
Histoplasmosis: Acute Radiology
Poorly defined areas of consolidation

Single or multiple
Hilar lymph node enlargement
Numerous discrete nodular shadows in heavy
exposure
3-4 mm
Symptoms precede radiographic change
Nodules change to punctate calcifications
Chest Radiology
From three to 5
days following
inhalation the
spores germinate
and release
yeast forms. The
yeast within the
alveoli are rapidly
phagacytosed by
macrophages
Figure 1-10-4
Histoplasmosis: Acute Clinical
Figure 1-10-3
101
Lymphocytemediated
cellular
immunity
develops at 1014 days
controlling the
infection
through a
necrotizing
granulomatous
response
Fungal Diseases
Acute Histoplasmosis [Figures 1-10-5]
Figure 1-10-5
Acute Histoplasmosis large inoculum

Acute Histoplasmosis [Figure 1-10-6]
Histoplasmosis: Chronic Pulmonary
Histoplasmosis
Emphysema and bullous disease a common

predisposition
Two possible mechanisms
Hypersensitivity reaction in preexisting
emphysematous space
Few organisms
Colonization or minimal invasion
Thick walled bulla filled with fluid may
clear spontaneously
Progressive loss of volume
Similar to TB
Fibrosis, cavitation and granulomatous
inflammation
Acute histoplasmosis is associated with areas of

consolidation and ipsilateral hilar and mediastinal
enlargement
Chronic Histoplasmosis [Figure 1-10-7]
Figure 1-10-6
Histoplasmosis
Disseminated
Clinical
Rare entity (1/100,000-1/500,000)
Most patients immunocompromised
30% infants < 2 years
20% immunocompromised
50% apparently normal (transient
compromise)
Reduced macrophage function
Parasitization of macrophages
Intracellular survival and multiplication
Radiology
Miliary nodules (1-3 mm)
50% of disease associated with AIDS
purely extrathoracic
Normal radiograph
Positive blood or bone marrow
biopsy
Fungal Diseases
Bilateral soft tissue nodules imply a large innoculum.

The nodules disappear over months leaving behind
small calcific densities
102
Chest Radiology
Disseminated Histoplasmosis [Figure 1-10-8]
Figure 1-10-7
Histoplasmosis: Late Complications
Histoplasmoma
Broncholithiasis
Mediastinal granuloma
Histoplasmosis: Histoplasmoma
Solitary nodule (.5-3 cm)

Sharply defined
Smaller satellite lesions
Central or diffuse calcification
Diagnostic of benign lesion if less that 3 cm
May increase in size
Similar reaction to fibrosing mediastinitis
Hilar calcification common on ipsilateral side
Fungal nodules account for 30% of all solitary
nodules
87% are less than 2.5 cm in diameter
Histoplasmoma [Figure 1-10-9]
Chronic histoplasmosis resembles post-primary TB but

usually represents a hypersensitivity reaction in patients
with emphysema
Broncholith
Figure 1-10-8
Histoplasmosis: Mediastinal granuloma
Pathology
Direct infection of hilar and mediastinal lymph
nodes
Clinical
Often asypmtomatic with discovery of a
mediastinal mass on chest radiograph
SVC or esophageal obstruction less common
Radiology
Middle mediastinal mass
Subcarinal or paratracheal
Enhancing capsule with low attenuation center
Mass may be low signal on T2 weighted MR
because of fibrous tissue or calcification
Disseminated Histoplasmosis presents with miliary

nodules and macrophages filled with organisms
Figure 1-10-9
Histoplasmomas are the residua of a prior area of

pneumonitis. They typically demonstrate concentric rings of
calcification but may remain uncalcified especially in older
individuals
Chest Radiology
103
Fungal Diseases
Mediastinal Granuloma [Figure 1-10-10]
Figure 1-10-10
Histoplasmosis: Fibrosing
Mediastinitis
Pathology
Proliferation of acellular collagen and
fibrous tissue within the mediastinum
Most cases in the United States are an
immunological response to H. capsulatum
Focal form: paratracheal and
subcarinal
Calcification
Idiopathic form
Diffuse, infiltrating
Noncalcified
Multiple mediastinal compartments
Clinical
Signs and symptoms of obstruction to
mediastinal structures
Superior vena cava, pulmonary veins
or arteries, central airway or
esophagus
Mediastinal granuloma is the result of direct infection of

mediastinal lymph nodes. Acutely the lymph nodes
demonstrate low attenuation with an enhancing capsule
Figure 1-10-11
Fibrosing Mediastinitis [Figure 1-10-11]

Blastomycosis
Blastomycosis: Epidemiology and Ecology
Ecological niche [Figure 1-10-12]

Difficult to establish
Saprophyte in an unidentified resevoir within
reach of man and dogs
Survives only in wet soil with a high PH and high
organic content
Soil probably contaminated rather than the
natural resevoir
Point sources in dead and decaying material
near rivers, streams and swamps
Dimorphic fungus
Mycelium in natural habitat
Releases spores (conidia) into the air
Budding yeast
(8-15 microns) in vivo
Broad based
Clinical
Less common than Histoplasmosis
High risk of symptomatic disease although most
cases are probably asymptomatic
Males more commonly affected (3:1-15:1)
Exposure in heavily wooded areas
Variable course
Symptoms of acute pneumonia
Abrupt onset, fever, chills, cough and
pleuritic pain
Occasional rapid progression
> Hematogenous dissemination: skin,
bone and genitourinary tract
> ARDS
Chronic disease similar to tuberculosis
Fibrosing mediastinitis due to h. capsulatum usually

presents as a mediastinal mass associated with
calcification
Figure 1-10-12
Blastomycosis is usually associated with activity in

damp, wooded areas
Fungal Diseases
104
Chest Radiology
Blastomycosis: Pathology
Initial inflammatory response is neutrophilic

Small collections of cells to hundreds of milliliters of pus
Rapidly followed by chronic inflammatory response
Lymphocyte, histiocytes and plasma cells
Langhans giant cells
Granulomas
Both responses may coexist
Organisms more common in supperative area
Progression
Coalescence of patchy consolidation
Airway perforation
Cavitation
Ulcerative bronchitis is common
Blastomycosis: Pathology
Initial response is neutrophilic

Chronic inflammatory response
Both responses may coexist
Progression
Coalescence of patchy consolidation
Airway perforation
Cavitation
Ulcerative bronchitis is common
Figure 1-10-13
Blastomycosis: Radiologic Manifestations
Consolidation most common

Upper lobe 2:1
Rounded, ill-defined
Masslike opacities
Central or paramediastinal
Carinoma mimic
Solitary nodules
Air bronchograms (88% CT)
Cavitation
Nodules
Intermediate size
Remote from consolidation
Satellite lesions
Miliary disease
Hematogenous dissemination
Blastomycosis often with an upper lobe areas of masslike consolidation
Asymptomatic Mass [Figure 1-10-13]

Figure 1-10-14
Consolidation
Solitary Pulmonary Nodule
Similar to Postprimary TB
Disseminated Disease [Figure 1-10-14]
Mass and Dissemination
Blastomycosis: Treatment
Pulmonary disease may be self-limited even if

extensive
Extrapulmonary disease requires treatment
Amphotericin B IV or oral Keotconazole
Miliary disease may complicate infection with
Blastomycosis
Chest Radiology
105
Fungal Diseases
Coccidioidomycosis [Figure 1-10-15]

Coccidioidomycosis: Epidemiology
and Ecology
Figure 1-10-15
Ecological niche
Dimorphic fungus
Clinical
Acute Disease
100,000 new cases each year, essentially all
in the southwest
No racial, sex or age predilection in acute
disease
Most inhabitants of the endemic area
infected in the first year of exposure
Incubation period 10-16 days
60% are asymptomatic
Symptoms when present include
Fever, pleuritic chest pain, cough
Valley Fever: allergic form with erythema
nodosum or multiforme
Severity of disease related to immune status Coccidioidomycosis is associated exclusively with the
desert southwest
and race
Filipinos, African Americans and
Hispanics more likely to suffer dissemination
Chronic Disease (5%)
Symptoms persist without dissemination
May be mildly immunocompromised
Dissemination
Rare occurrence
Immunocompromise
Non-Caucasian (Filipino, African American and Hispanic)
Early dissemination more common and carries a poor prognosis
Mortality rate or 50% even with early treatment
Coccidioidomycosis: Pathology
Lung the usual portal of entry

Neutrophilic response early
Especially in response to ruptured spherules
Spherules ingested by macrophages
Granulomatous and giant cell reaction follows
Necrosis may occur
Coccidioidomycosis: Radiologic Manifestations
Acute Disease
Consolidation most common (75%)
Usually unilateral, hilar or basal
Segmental or lobar
Multifocal nodular or patchy opacities
Peribronchiolar thickening
Hilar or mediastinal adenopathy (20%)
Mediastinal adenopathy may herald dissemination
Pleural effusion 20%
Small, unilateral
Coccidioidoma
Fungal Diseases
106
Chest Radiology
Acute Disease
Coccidioidoma [Figure 1-10-16]
Coccidioidomycosis: Radiologic Manifestations
Acute Disease
Coccidioidoma
Area of prior consolidation
Round and well circumscribed
1.5cm average (up to 6cm)
Usually single
Marked enhancement with contrast CT
Caseating chronic granulomatous inflammation
Chronic Disease
Cavitation
Occur in areas of consolidation
May be thin or thick walled
Pneumothorax or empyema may result
Chronic progressive pneumonia
Figure 1-10-16
Chronic Coccidioidomycosis [Figure 1-10-17]

A coccidioidoma is the resdua of an area of infectious
consolidation
Coccidioidomycosis: Radiologic
Manifestations
Chronic Disease
Cavitation
Chronic progressive pneumonia
Indolent course similar to TB
Biapical fibronodular lesions
Hilar and mediastinal adenopathy
Hilar retraction
Persistently positive sputum
High complement fixing antibody titer
Non-Caucasian
Disseminated Disease
Miliary or reticular nodular pattern
Less well circumscribed that TB
Lymphadenopathy is common
Pericardial effusion
Skin, bone, meninges or upper genitourinary
tract
Figure 1-10-17
Thin-walled cavities are suggestive of chronic

coccidioidomycosis
Chest Radiology
107
Fungal Diseases
Dissemination Miliary Nodules

Histoplasmosis
Acute Histoplasmosis [Figure 1-10-18]
Histoplasmosis Solitary Nodule
[Figures 1-10-19 and 1-10-20]
Histoplasmosis large inoculum [Figure 1-10-21]

Histoplasmosis [Figure 1-10-22]
Disseminated Histoplasmosis [Figure 1-10-23]
Chronic Histoplasmosis [Figure 1-10-24]
Fibrosing Mediastinitis [Figure 1-10-25]
Blastomycosis
Figure 1-10-18
Coccidioidomycosis
Fungal Disease in the Thorax: Overview
Opportunistic invaders
Aspergillus species
Candida
Mucormycosis
Primary pathogens
Histoplasma capsulatum
Blastomyces dermatitidis
Coccidioides immitis
Paracoccidioides brasiliensis
Symptomatic patients with acute histoplasmosis may present

with solitary or multifocal areas of consolidation and associated
adenopathy
Figure 1-10-19
Figure 1-10-20
As the infection heals the inflammatory area rounds up and is

surrounded by a fibrous capsule. Over a prolonged period
calcification may develop in the nodule and regional lymph
nodes
Fungal Diseases
The nodule may enlarge by adding fibrous tissue to the

periphery
108
Chest Radiology
Figure 1-10-21
Figure 1-10-22
If the patient inhales a large number of spores then numerous

patches of consolidation may round up into well circumscribed
nodules
Figure 1-10-23
As they heal the patient will be left with numerous calcifications
Figure 1-10-24
Patients with reduced immune function may present with

hematogenous spread of disease and miliary nodules
Figure 1-10-25
Patients with underlying emphysema may develop chronic
histoplasmosis which in most cases represents a
hypersensitivity reaction to a small number of organisms
Fibrosing mediastinitis represents an exuberant fibrous reaction

within the mediastinum which may result in damage to
mediastinal structures. Most focal cases of fibrosing
mediastintis in the United States are due to H. capsulatum
Chest Radiology
109
Fungal Diseases
Bronchogenic Carcinoma
A 20th Century Disaster
Histological Classification of Tumors
World Health Organization

Lung tumor editions
1967
1981
1999
2004
Improve communication
Consistent treatment
Basis for comparative studies
Prognosis
Changes in the 1999/2004 WHO
Subclasses of adenomas
Preinvasive lesions
Adenocarcinoma
Definition of BAC
Neuroendocrine tumors
Biphasic and pleomorphic tumors
Incidence of Lung Cancer

Gazdar, Semin Oncol, 1988
Histological Typing of Lung Tumors
Based on light microscopic criteria

Classified by the best differentiated region
Graded by the most poorly differentiated region
Histologic heterogeneity is the rule
Histological Typing of Lung Tumors
Prognosis: Small cell vs. non-small cell

Stage determines prognosis in non small cell
>95% of 1 lung tumors
Adeno
Squamous
Large cell
Small cell
Combination of above
Lung Cancer Demographics
Most common cancer in males world-wide

Leading cause of cancer mortality in women and men (United States)
Mortality rates in women began increasing in 1935 and surpassed breast ca in
1987
Age-Adjusted Cancer Death Rates Males vs Females

Cancer 49: 1999
Bonchogenic Carcinoma
110
Chest Radiology
Lung Cancer Etiology Cigarette smoking
85-90% of lung cancer deaths

25% of lung cancer in non-smokers attributed to passive smoke
Risk related to:
Number of cigarettes smoked
Depth of inhalation
Age at which smoking began
Central tumors
Cough
Wheezing
Hemoptysis
Pneumonia
Extrapulmonary invasion
Pain
Pancoast Syndrome
SVC Syndrome
Metastases
Paraneoplastic Syndromes
Asymptomatic 10%
Paraneoplastic Syndromes
Cachexia, malaise and fever
Ectopic hormone production
ACTH
ADH
Hypercalcemia
Clubbing and HPO
Thrombotic endocarditis
Non-bacterial
Migratory thrombophlebitis
Lung Cancer and Clotting

Squamous Cell Carcinoma
Terminology
Squamous
Flattened cells
Epidermoid
Mimics differentiation of the epidermis
Rapid local growth
Distant metastases later
Strong association
Cigarette smoking
Pancoast Syndrome
Hyperparathyroidism
Parathyroid-like substance
Most common to present as radiographically occult
Chest Radiology
111
Preinvasive Lesions: Squamous Dysplasia
Similar to cervical Ca
Squamous metaplasia
Progression
Dysplastic epithelium
Carcinoma in situ
Full thickness dysplasia
Precursor
Invasive squamous cell Ca
Figure 1-11-1
Squamous Cell Carcinoma:

Microscopic features
Individual cell keratinization

Eosinophilia
Keratin pearls
Well differentiated tumors
Intercellular bridges
Figure 1-11-2

Gross Features [Figure 1-11-1]
Central lesion
Polypoid, endobronchial,
exophytic growth
Central necrosis common
Bronchial wall invasion
Common
Positive cytology
Proximal growth
Along bronchial mucosa
The majority of squamous cell

cancers are central lesions
Squamous Cell Carcinoma: Radiologic Features [Figure 1-11-2]
Hilar or perihilar mass

Often focal
Consolidation
Must clear completely
Atelectasis
Peripheral nodule or mass
30%
Cavitation
Squamous cell cancers are

predominantly central and
endobronchial as exemplified by this
tomogram
Atelectasis [Figures 1-11-3 to 1-11-5]

Figure 1-11-3
Figure 1-11-4
Atelectasis in an adult smoker is lung cancer until

proven otherwise
Typical central squamous cell carcinoma

112
Chest Radiology
Cavitation [Figure 1-11-6]
Figure 1-11-5
Pancoast Tumor:
Superior Sulcus Tumor
Characteristic pain
8th cervical
2nd thoracic trunk
Horners Syndrome
Destruction of bone
Hand muscle atrophy
Pancoast, JAMA, 1992
Small Cell Lung Cancer
Rapid growth
Considered metastatic at presentation
Poorest survival
Strongest association with cigarette
smoking
Goldens S sign
Small cell carcinoma

Pure histology
Variant
Combined
Elimination
Oat cell
Intermediate type
WHO, 2004
Small Cell Lung Cancer: Microscopic Features
Small, uniform cells

Scant cytoplasm
Necrosis is common
Often extensive
>10 mitosis per 10 HPF
Average 60-70
Neuroendocrine morphology
Neuroendocrine markers
75%
Light microscopy diagnosis
Figure 1-11-6
WHO, 2004
Cavitation is most common in squamous cell cancer

Chest Radiology
113
Small Cell Lung Cancer: Gross Features
Figure 1-11-7
Large
Central mass (90%)
Bronchial compression
No endobronchial lesion
Proximal growth
Along submucosa
Extensive necrosis
Hemorrhage
Small Cell Lung Cancer: Radiologic

Features
[Figures 1-11-7 and 1-11-8]
Hilar or perihilar mass

Mediastinal adenopathy
Primary tumor
Rarely evident
Cavitation
Extremely rare
Small cell tends to spread along the

peribronchovascular lymphatics without endobronchial
invasion
Cushing Syndrome
SIADH
Eaton Lambert
Most common cause
SVC Syndrome
Figure 1-11-8
Small Cell Lung Cancer: Therapy
Response to chemotherapy and radiotherapy

Untreated: median survival 2-4 months
Treated: median survival 9-18 months
Limited stage 15-25% survive 2 years
Large Cell Carcinoma
Rapid growth
Location
Segmental
Subsegmental
Early metastases
Poor prognosis
Strong association with cigarette smoking
Large Cell Carcinoma: Microscopic

Features
Small cell most commonly presents as a mediastinal mass
Large cells
Prominent nucleoli
Poorly differentiated
Diagnosis of exclusion
Neuroendocrine features
Large Cell Carcinoma: Gross Features
Large and bulky

Greater that 3 cm
Soft
Large areas of necrosis
114
Chest Radiology
Large Cell Carcinoma: Radiologic Features
Usually peripheral
70% of tumors
> 4 cm at presentation
Figure 1-11-9
Large Cell Carcinoma [Figure 1-11-9]

Adenocarcinoma: Etiology
Cigarette smoke causatively linked to lung

cancer
1950
Squamous cell 18Xs Adeno
Squamous cell: central
Adenocarcinoma: Etiology
[Figure 1-11-10]
Cigarette smoke causatively linked to lung

cancer
Adenocarcinoma most common
Peripheral
Filtered low-yield cigarettes
Smaller particles
Reduced nicotine
Greater depth of puffs
Increased number of puffs
N-nitrosamines
Other factors - 10%
Passive smoke
Particulates
Cooking practices
Large cell cancers are commonly necrotic but rarely cavitate
Figure 1-11-10
Adenocarcinoma: Microscopic Features
Glands
Papillary structures
Mucin
Intracellular
Extracellular
Prominent nucleoli
Moderate cytoplasm
Desmoplastic reaction
Scar carcinoma
Rare!
Adenocarcinoma: Radiologic Features
Peripheral (75%)
Solitary mass or nodule
Upper lobes 3:2
Right lung 3:2
Lobulated
Borders
Ill-defined
Well-defined
Spiculated
Obstructive pneumonitis (25%)
Chest Radiology
Adenocarinoma, the most common lung

cancer is predominantly
a peripheral lesion
115
Spiculation and Retraction [Figure 1-11-11]
Figure 1-11-11
Scar Carcinoma [Figure 1-11-12]

Necrosis
Air Bronchogram [Figure 1-11-13]
Adenocarinomas are commonly spiculated peripheral

nodules
Figure 1-11-12
In scar carcinomas the scar is usually a reaction to the

malignancy
Figure 1-11-13
Air bronchograms are commonly seen in adenocarcinomas

116
Chest Radiology
Slow Growth
Atypical Adenomatous Hyperplasia: Preinvasive lesion
[Figures 1-11-14 and 1-11-15]
Figure 1-11-14
Atypical cuboidal epithelium

Lining alveoli
Lining bronchioles
Found in lung cancer resection specimens
Probable precursor
BAC
Invasive adenocarcinoma
Patchy ground glass
5mm or less
Kitamura, AJCP, 1999
AAH
Bronchioloalveolar Carcinoma: Microscopic Features
Lepidic growth pattern

No evidence
Stromal invasion
Vascular invasion
Pleural invasion
Diagnosis cannot be made on a small biopsy
Requires thorough sampling of resected specimen
WHO, 1999
Bronchioloalveolar Carcinoma: Mucinous Type
Alveolar spaces distended with mucin

Aerogenous spread is common
Multifocal consolidation
Bronchioloalveolar Carcinoma: Non-mucinous Type
The diagnosis of AAH may be difficult and

the differentiation form a small BAC may
be problematic
Figure 1-11-15
Alveoli lined with

Clara cells
Type II cells
Central alveolar fibrosis
Common
Close association
AAH
In AAH the architecture of the lung is not

disturbed
Chest Radiology
117
Bronchioloalveolar Carcinoma: Gross Features

[Figure 1-11-16]
Figure 1-11-16
Consolidation
Focal
Multifocal
Architecture
Preserved
Bronchioloalveolar Carcinoma: Radiologic Features

[Figure 1-11-17]
Solitary nodule
Excellent prognosis
Resection
Consolidation
May be multifocal
Ground glass
Multiple nodules
May cavitate?
Noguchi, Cancer 1995
Bronchioloalveolar Carcinoma
BAC Recurrence
BAC often presents as as area of

consolidation
BAC vs Adenocarcinoma
BAC - Adenocarcinoma: CT, Histology and Doubling Time
Type A
Ground glass
Localized BAC
Doubling time
Mean: 880 days
Range: 662-1486 days
Figure 1-11-17
Aoki et al, AJR, 2000
BAC - Adenocarcinoma: CT, Histology and Doubling

Time
Type B
Ground glass
Focal increased attenuation
Localized BAC
Doubling time
Mean: 880 days
Range: 662-1486 days
BAC - Adenocarcinoma: CT, Histology and Doubling

Time
BAC usually presents as an area of

consolidation
Type C
Solid attenuation
Focal ground glass
Spiculation
Pleural tag
Localized BAC
Active fibroblastic proliferation
Doubling time
Range: 42-1346 days
118
Chest Radiology
BAC - Adenocarcinoma: CT, Histology and Doubling Time
Type D
Solid attenuation only
Spiculation
Pleural tag
Poorly differentiated adenocarcinoma
Doubling time
Mean: 252 days
Range: 124-402 days
Figure 1-11-18
AdenoCa Appearance and Prognosis

Adenocarcinoma [Figure 1-11-18]
One form of adenocarcinoma begins as an invasive

process and presents with a solid nodule
BAC
AAH [Figure 1-11-19]
BAC [Figure 1-11-20]
Figure 1-11-20
Figure 1-11-19
The precursor lesion to BAC is AAH
BAC demonstrates lepidic growth and presents as an

area of ground glass and/or consolidation
Adenocarcinoma - BAC Prognosis [Figure 1-11-21]
Figure 1-11-21
Survival decreases with increasing amount of consolidation and less

ground glass opacity
Chest Radiology
119
References
General
1. Travis W, Colby T, Shimasato Y, Brambilla E. Histological Typing of Lung and Pleural Tumors., International
Classification of Tumors. Third ed. Berlin: Springer Verlag, 1999.
2. Colby T, Koss M, Travis W. Tumors of the Lower Respiratory Tract, Atlas of Tumor Pathology. Third ed. Washington,
DC: Armed Forces Institute of Pathology, 1999.
3. Travis WD, Brambilla E, et al: Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart (WHO
Classification of Tumours), IARC Press, 2004 (Oxford).
4. Patel AM, Peters SG. Clinical manifestations of lung cancer Mayo Clin Proc 1993; 68(3):273-7.
5. Davila DG, Williams DE. The etiology of lung cancer. Mayo Clin Proc 1993; 68(2): 170-82.
6. Travis WD, Lubin J, Ries L, Devesa S. United States lung carcinoma incidence trends: declining for most histologic
types among males, increasing among females. Cancer 1996; 77(12):2464-70.
7. Travis WD, Travis LB, Devesa SS. Lung cancer [published erratum appears in Cancer 1995 Jun 15;75(12):2979].
Cancer 1995; 75(1 Suppl):191-2O2.
8. Pisani RJ. Bronchogenic carcinoma: immunologic aspects. Mayo Clin Proc 1993; 68(4):386-92.
9. Whitesell PL, Drage CW. Occupational lung cancer Mayo Clin Proc 1993; 68(2):1 83-8.
10. Patel AM, Davila DG, Peters SG. Paraneoplastic syndromes associated with lung cancer [see comments]. Mayo Clin
Proc 1993; 68(3):278-87.
11. Morabia A, Wynder EL. Cigarette smoking and lung cancer cell types. Cancer 1991; 68(9):2074-8.
12. Ko YC, Lee CH, Chen MJ, Huang CC, Chang WY, Lin HJ, Wang HZ, Chang PY. Risk factors for primary lung cancer
among non-smoking women in Taiwan. Int J Epidemiol 1997; 26(1):24-31.
13. Kitamura H, Kameda Y, Ito T, Hayashi H. Atypical adenomatous hyperplasia of the lung. Implications for the
pathogenesis of peripheral lung adenocarcinoma [see comments]. Am J CIin Pathol 1999; 111(5):610-22.
14. Karsell PR, McDougall JC. Diagnostic tests for lung cancer. Mayo Clin Proc 1993; 68(3):288-96.
15. Dalager NA, Pickle LW, Mason TJ, Correa P, Fontham E, Stemhagen A, Buffler PA, Ziegler RG, Fraumeni JF, Jr. The
relation of passive smoking to lung cancer Cancer Res 1986; 46(9):4808-11.
16. Charloux A, Hedelin G, Dietemann A, Ifoundza T, Roeslin N, Pauli G, Quoix E. Prognostic value of histology in
patients with non-small cell lung cancer. Lung Cancer 1997; 17(1):123-34.
17. Charloux A, Ouoix E, Wolkove N, Small D, Pauli G, Kreisman H. The increasing incidence of lung adenocarcinoma:
reality or artefact? A review of the epidemiology of lung adenocarcinoma. Int J Epidemiol 1997; 26(1 ):14-23.
18. Muller NL, Miller RR. Neuroendocrine carcinomas of the lung. Semin Roentgenol 1990; 25(1 ):96-1 04.
19. Travis WD, Rush W, Flieder DB, Falk E=R, Fleming MV, Gal AA, Koss MN. Survival analysis of 200 pulmonary
neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid.
Am J Surg Pathol 1998; 22(8):934-44.
20. Hardy J, Smith I, Cherryman G, Vincent M, Judson I, Perren T, Williams M. The value of computed tomographic
(CT) scan surveillance in the detection and management of brain metastases in patients with small cell lung cancer
Br J Cancer 1990; 62(4):684-6.
21. Sone S, Takashima S, Li F, Yang Z, Honda T, Maruyama Y, Hasegawa M, Yamanda T, Kubo K, Hanamura K, Asakura
K. Mass screening for lung cancer with mobile spiral computed tomography scanner [see comments]. Lancet 1998;
351 (9111):1242-5.
120
Chest Radiology
Chest Seminar 1
Case 1: This 57 year old male with a long history of smoking

cigarettes.
He now complains of a chronic cough
Chest Radiology
121
Chest Seminar 1
Case 2: This 20 year old Caucasian female presented 7 years

prior to the current admission with sudden onset of shortness
of breath. The original chest radiograph revealed a
pneumothorax.
The patient now presents with increasing shortness of breath.
Chest Seminar 1
122
Chest Radiology
Case 3: This 58 year old Caucasion female presented with a one

month history of hemoptysis
Chest Radiology
123
Chest Seminar 1
Case 4: This 38 year old African American female presented

with a history of chronic asthma and increasing cough and
shortness of breath
Chest Seminar 1
124
Chest Radiology
Case 5: This 72 year old Caucasian female

presented with cough and occasional fever.
She was treated intermittently with antibiotics for 6 months.
Open biopsy was obtained because of progressive symptoms.
Chest Radiology
125
Chest Seminar 1
Chest Seminar 2
Case 1: 15 year old female was admitted to the ER with an
overdose of Nefazodone and other unknown pills. Activated
charcoal was administered after which she developed vomiting
and gagging. Respiratory distress required intubation.
Bronchial lavage was performed and immunosuppressants
were started. The patient developed progressive dyspnea and
obstructive pulmonary functions over the next 6 months.
Bilateral lung transplantation was done 19 months later.
Chest Seminar 2
126
Chest Radiology
Case 2: 59 year old Caucasian female with history of breast

cancer 12 years prior to admission and local recurrence treated
with radiation 7 years later. She presented with a right lung
mass.
Chest Radiology
127
Chest Seminar 2
Case 3: 46 year old Caucasian male with long standing year

history of shortness of breath. He presents with a 3 months of
worsening dyspnea.
He demonstrated a mild leukocytosis and an increase in serum
LDH.
Chest Seminar 2
128
Chest Radiology
Case 4: 57 year old Caucasian male with a new history of cough

and a new abnormality on chest radiograph.
A chest CT was done based on the abnormalities found on the
chest radiograph.
A bronchoscopy was performed.
Chest Radiology
129
Chest Seminar 2
Case 5: 61 year old female worked as a hospital storage room

manager.
She presented to the ER complaining of fatigue, increasing
shortness of breath, chest pain, and cough productive of blood
tinged sputum.
Chest Seminar 2
130
Chest Radiology
Pulmonary Hypertension
Aletta Ann Frazier, MD
Key Points
Radiologic findings distinguish precapillary (arterial) from postcapillary

(venous) pulmonary hypertension
Idiopathic and secondary conditions are included in the differential diagnosis
Vascular histopathology and secondary cardiac changes are often reflected in
the radiology of pulmonary hypertension
Precapillary Pulmonary Circulation
[Figure 1-14-1]
Figure 1-14-1
Precapillary (arterial) circulation and vascular anatomy. Arterial vessels

accompany the dichotomously branching airways of the lung
Postcapillary Pulmonary Circulation [Figure 1-14-2]

Figure 1-14-2
Postcapillary (venous) circulation drains the capillary beds of the alveoli. Veins and
venules course back to the left atrium within interlobular septa
Normal Pulmonary Circulation
Low pressure system with high degree of capacitance (recruitment and

distension)
Less than one tenth the resistance to flow in comparison to systemic
circulation (low vasomotor tone)
Right ventricle expends minimal energy to perfuse the pulmonary vascular bed
Chest Radiology
131
Precapillary (Arterial) Pulmonary Hypertension
Insidious: dyspnea, chest pain, syncope

Right heart pressure overload develops late
Imaging reveals pulmonary HTN & clues to etiology
NIH Criteria (cardiac cath): Mean PA pressure > 25 mm Hg at rest (normal 10)
Cor Pulmonale the best predictor of clinical outcome
Figure 1-14-3
PV regurgitation
RV hypertrophy & enlargement
TV regurgitation & RAE
Dilated IVC, hepatic veins
Normal Heart vs. Cor Pulmonale

American College of Chest Physicians: Venice
Classification
PAH
PH with left-sided heart dz
PH with lung dz, hypoxemia
PH due to thromboembolic dz
Miscellaneous
Simonneau G. et al. J Am Coll Cardiol. 2004 Jun 16;43 (12 Suppl S):5S-12S.
Pulmonary Hypertension: Precapillary Etiologies
Idiopathic
Secondary
Chronic thromboembolic disease
Sickle cell disease
Eisenmenger physiology
Chronic hypoxia (COPD, IPF)
Photomicrograph demonstrates a
muscular artery (adjacent to airway)
narrowed by medial hypertrophy and
obstructed by intravascular thrombus
Figure 1-14-4
Pulmonary Arterial Hypertension: Histology [Figure 1-14-3]
Medial hypertrophy
Intimal proliferation
Thrombosis
Arteritis
Precapillary Hypertension: Imaging
Dilated central arteries

Pruning of peripheral vessels
Mosaic perfusion
Cor pulmonale
PA atherosclerosis
Radiographic features of pulmonary

hypertension: enlarged main
pulmonary artery, dilated central hilar
vessels, and peripheral oligemia
Idiopathic Pulmonary Hypertension [Figures 1-14-4 to 1-14-6]
Figure 1-14-6
Figure 1-14-5
CT criteria for enlarged

main PA in precapillary
pulmonary
hypertension:
transverse diameter
exceeds 29 mm
132
CT manifestations of cor pulmonale:

dilated RA and RV, thickened anterior
RV wall, and flattened interventricular
septum
Chest Radiology
Cor Pulmonale
Figure 1-14-7
Axial White Blood Cine

Sagittal White Blood Cine
Courtesy Laura Heyneman, MD
Idiopathic Pulmonary Hypertension
Mean age 45, F>M (3:1)

6% familial
Autosomal dominant, incomplete penetrance
Associations
HIV infection
Appetite suppressants
Cocaine abuse
Chronic liver disease
Mean survival 2.8 years without treatment
Longstanding uncorrected ASD with acquired

Eisenmenger physiology in 35 y.o. female
[Courtesy of Melissa Rosado de Christenson, MD]
Atrial Septal Defect [Figures 1-14-7 to 1-14-9]

Figure 1-14-9
Figure 1-14-8
Lung specimen radiograph

demonstrates calcified atherosclerotic
plaques in the main PA
Vascular pruning pattern

Eisenmenger Physiology
Congenital L-to-R shunt

VSD, ASD, PDA
Endocardial Cushion Defect
Shunt reversal (R-to-L) follows sustained elevation in PVR
PAH irreversible & requires lung transplantation
Chronic Thromboembolic Disease
Figure 1-14-10
[Figures 1-14-10 to 1-14-12]
Approx 4% of cases acute PE; presents within 2 years

Symptomatic with >60% vascular bed occlusion
5-year survival rate <35% (without surgery)
V/Q scan - high probability (helps to exclude IPH)
CT
Enlarged central PA
Eccentric & linear filling defects +/- calcification
Abrupt cut-offs (pruning)
Bronchial arteries (50% of cases)
Mosaic perfusion
Pleural tags (healed infarcts)
Chest Radiology
133
Broad-based intravascular soft tissue

density in the right PA, combined with
multiple bronchial arterial collateral
vessels, suggests CTEPH
Figure 1-14-11
CT Angio
94-100% sensitivity, 96-98% specificity for CTEPH
More sensitive than PA angio for proximal disease
Non-invasive modality for pre- and post-op assessment
Multiplanar & curved multiplanar reconstructions further
characterize disease extent
Cardiac MR
Cine imaging
Right heart function
Phase-contrast imaging
low velocities in L & R PAs
shunt vol from bronchial arteries to pulmonary venous
circulation
R-to-L shunt via patent foramen ovale
Recanalized Chronic Thrombus

Organizing Chronic Thrombus
Pulmonary Thromboendarterectomy
15-30% CTEPH patients are candidates

Thrombi from main to segmental or subsegmental level
Intima & superficial media removed w/ thrombi
Operative mortality 8-23%
Improved long term survival - 75% at six years
Other thromboembolic materials
Chronic thromboembolic disease:

mural-based soft tissue masses and
calcium in the lumen of right main PA
Intravenous Talcosis
Chronic IV injection of crushed tablets (Methadone, amphetamines)

Thrombogenic pharmaceutical binding agent: magnesium silicate
Granulomas coalesce into birefringent particles
Figure 1-14-12
Intravenous Talcosis: Imaging
EARLY
Diffuse micronodular opacities
LATE
Fibrosis
High density perihilar masses
Emphysema
Mosaic attenuation reflects

geographic variations in blood flow
Postcapillary Pulmonary Circulation

Pulmonary Hypertension: Postcapillary Etiologies
Idiopathic
Pulmonary veno-occlusive disease (PVOD)
Pulmonary capillary hemangiomatosis (PCH)
Secondary
Mitral valve stenosis
Left ventricular failure
Left atrial mass / thrombus
Venous constriction / invasion by tumor
134
Chest Radiology
Pulmonary Venous Hypertension
Figure 1-14-13
Acute or chronic onset

Elevated wedge pressures (with exceptions)
PAH - secondary
Venous dilatation
Venous arterialization
Septal edema, fibrosis
+ Interlobular septa
Subpleural thickening
Ground glass opacities
Pleural effusion
PCH/PVOD
Capillary proliferation & dilatation

Venous medial hypertrophy & intimal proliferation
Recanalized thrombus in veins & venules
PCH/PVOD
Young adults (M:F is 2:1)

Pediatric in 1/3 of cases
Fatal within 3-5 years
Associations
Chemotherapy
Prior viremia
HIV
? Toxic exposure
Current Debate
Separate entities?
Contiguous spectrum of injury?
Cause-effect scenario?
PVOD, a rare cause of postcapillary

pulmonary hypertension: prominent
interlobular septa and subpleural edema
PCH/PVOD [Figure 1-14-13]
Difficult to discern from primary PAH clinically

Vasodilators contraindicated: severe pulmonary edema
Figure 1-4-14
PVOD/PCH
Difficult to discern from primary PAH clinically

Vasodilators contraindicated: severe pulmonary edema
CT clues
Septal lines
GG nodules (+/-)
Normal left atrium
Mediastinal Fibrosis [Figure 1-14-14]

Mediastinal Fibrosis: Imaging
Mediastinal contours abnormal

Coarse calcium
Soft tissue replaces mediastinal fat
Constriction, encasement of mediastinal structures
Two coronal CT reconstructions show
mediastinal fibrosis constricting
pulmonary venous drainage at their
entrance to left atrium, thereby
creating unilateral postcapillary
pulmonary hypertension. (Note that
precapiillary hypertension is also
evident: the main PA is dilated and
there is extrinsic compression of the
right PA by mediastinal fibrosis)
Chest Radiology
135
Mitral Stenosis
Diagnostic Strategy - Recommended Imaging Studies by ACCP
CXR
Echocardiography with Doppler
V/Q (if CTEPH suspected)
PA gram if positive (resectability)
ACCP Evidence-based Clinical Practice Guidelines. Chest 2004;126:14S-34S
Diagnostic Strategy - Imaging Studies
From the ACCP:

CXR
Echocardiography with Doppler
V/Q
We also suggest:
Chest CT/CTA
Precapillary vs. postcapillary origin
Clues to underlying etiology
MRI
Cardiac anatomy/function
Future: hemodynamics of lung perfusion
Go With the Flow .
Pulmonary Arterial Hypertension

Enlarged central and hilar vessels
Pruned peripheral vessels
Mosaic perfusion
Por pulmonale
PA atherosclerosis
Pulmonary Venous Hypertension
Septal lines
Smooth pleural thickening
Pleural effusion
References
1.
Bergin CJ, Rios G, King MA, Belezzuoli E, Luna J, Auger WR. Accuracy of high-resolution CT in identifying
chronic pulmonary thromboembolic disease. AJR Am J Roentgenol 1996; 166:1371-1377.
2. Benjamin MS, Drucker EA, Mcloud TC, Shepard JO. Small pulmonary nodules: Detection at chest CT and
outcome. Radiology 2003; 226:489-493.
3. Botticelli JT, Schlueter DP, Lange RL. Pulmonary venous and arterial hypertension due to chronic fibrous
mediastinitis. Hemodynamics and pulmonary function. Circulation 1966; 33:862-871.
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5. Jones AT, Hansell DM, Evans TW. Quantifying pulmonary perfusion in primary pulmonary hypertension using
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6. King MA, Ysrael M, Bergin CJ. Chronic thromboembolic pulmonary hypertension: CT findings. AJR Am J
Roentgenol 1998; 170:955-960.
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13. Remy-Jardin M, Remy J, Louvegny S, Artaud D, Deschildre F, Duhamel A. Airway changes in chronic pulmonary
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Chest Radiology
137
Pulmonary Metastases
Aletta Ann Frazier, MD
Key Points
The pathogenesis of pulmonary metastases is complex

The spectrum of radiological manifestations reflects pathways of spread
Many extrathoracic malignancies produce characteristic radiologic patterns of
pulmonary metastases
Metastatic Disease to the Lung
Most common lung neoplasm

Incidence: 20-55% of patients dying from extrathoracic malignancy
Lung is the ONLY site of metastatic disease in 15-25% of these patients
Metastatic Disease to the Lung:Routes of Spread
Pulmonary & bronchial arteries

Pulmonary & pleural lymphatics
Thoracic duct
Airways
Transdiaphragmatic lymphatics
What are the most likely primary extrathoracic malignancies?
Breast
Colon
Uterus
Kidney
Prostate
Oropharynx
Stomach
Pancreas
Which malignancies are the most highly predisposed?
Choriocarcinoma
Osteosarcoma
Testicular tumors
Melanoma
Ewings sarcoma
Thyroid carcinoma
Kaposis sarcoma
Principle: Generalizing Sites
Certain tumors seed the lung directly, others first drain via another filtration
organ (bone or liver)
Systemic venous drainage directly to lung
Melanoma
Sarcomas
Choriocarcinoma
Thyroid
Kidney, Testes, Adrenal Gland
Oropharynx
Venous drainage via liver
Colon
Pancreas
Stomach
Venous drainage via bone
Prostate
Dual venous drainage (simultaneous seeding)
Kidney, Bladder, Ureters
Uterus, Cervix
138
Chest Radiology
Anus, Rectum
Complex venous (and lymphatic) drainage
Breast
Pathogenesis of Hematogenous Metastases [Figure 1-15-1]
Tumor cells penetrate draining venules

Enter systemic venous circulation
Filtered by pulmonary arterial circulation
Figure 1-15-1
Pathogenesis of Hematogenous Metastases

[Figure 1-15-2]
Adherence, extravasation in distal arterioles

Expansile growth in interstitium and alveoli
Vascularization
Pulmonary arteries
Bronchial circulation
Transpleural collaterals
Parenchymal Nodules: Histology
Well-defined
Homogeneous cell population
Adjacent to arteries and arterioles
Alveolar septa compressed or obliterated
Nodular Metastases
Rounded, coalescent or multilobulated

Multiple
Peripheral, basilar
Variable sizes
Mixed areas of viability, necrosis, hemorrhage
Hematogenous metastases arise from tumor cells

which penetrate vessels and lymphatics at the
primary site, and are transported to the right
heart via the systemic venous circulation
Parenchymal Nodules:
Multidetector Chest CT
High sensitivity
95% for nodules >1cm
91% for nodules .5-1cm
Low specificity (60% in 40-65 y.o. adults)
intrapulmonary lymph nodes
granulomatous diseases
sarcoidosis
silicosis
amyloidosis
infection
Figure 1-15-2
Bloodborne tumor cells arrest in distal arterioles

of the pulmonary circulation, extravasate into the
interstitium, and establish nodules by expansile
growth
Chest Radiology
139
Parenchymal Nodular Metastases: [Figure 1-15-3]

HRCT-Pathologic Correlation: Nodules <1cm
Peribronchovascular (12%)
Periseptal (28%)
Intermediate (68%)
Angiocentric (76%)
Directly-centered on feeding vessel (18%)
Eccentric to feeding vessel (58%)
Figure 1-15-3
Hirakata et al. AJR 1993;161:37-43
Parenchymal Nodules: [Figure 1-15-4]

Imaging Features, Chest CT
Multiple
Peripheral, basilar
Variable in size
Random - eccentrically located between BVB & interlobular
septa
Occasionally angiocentric
Less commonly - cannonball or miliary
Rarely
cavitary
calcified
solitary
ground glass halo (hemorrhagic)
angiosarcoma
choriocarcinoma
post therapy
Figure 1-15-4
Secondary pulmonary lobule:

hematogenous metastases may be
angiocentric but are random with
respect to the secondary pulmonary
lobular architecture
Figure 1-15-5
Metastatic colon carcinoma in middle aged male:

variable-sized nodules are random, peripheral,
occasionally angiocentric in location
Cannonball Metastases [Figures 1-15-5 and 1-15-6]
Colorectal carcinoma
Renal cell carcinoma
Sarcomas
Melanoma
Cannonball metastases (gross lung)

in young adult male with soft tissue
sarcoma
140
Chest Radiology
Micronodular (Miliary) Metastases [Figure 1-15-7]
Malignancies
Thyroid CA (papillary)
Choriocarcinoma
Opacities may persist post-treatment (sterile)
DDX
Miliary tuberculosis
Viral pneumonia
Sarcoidosis
Figure 1-15-6
Pulmonary Metastases:
Unusual Manifestations
Cavitary, calcified or solitary pulmonary nodules

Lymphangitic carcinomatosis
Tumor thromboembolism
Endobronchial metastases
Pleural-based metastases
Cavitation in Metastases [Figure 1-15-8]
Incidence 4% (vs. 9% of lung primaries)

Malignancies
Squamous cell neoplasms (head and neck; cervix) - 69%
Adenocarcinomas (colon, breast) - 31%
Sarcomas (bone) - spontaneous ptx
Wall thickness NOT indicative of benignity
DDX
septic emboli
vasculitis
collagen vascular disease
Figure 1-15-7
Cannonball metastases in a young
adult male with a soft tissue sarcoma
(scout; axial lung and mediastinal CT
images)
Figure 1-15-8
Micronodular metastases in middle aged

female with thyroid cancer
Cavitary metastases in elderly male

with oropharyngeal cancer
Chest Radiology
141
Calcification in Metastases [Figure 1-15-9]
Figure 1-15-9
Malignancies
Osteosarcoma, chondrosarcoma, and synovial sarcomas
Papillary/mucinous adenocarcinomas (ovary, thyroid, GI)
Post-chemotherapy or post-radiation
Variable content
Osteoid matrix
Dystrophic calcification
Psammoma body formation
Solitary Metastasis
Unusual: 1-28% of all metastatic lesions

3-10% of all SPNs are solitary metastases
Variable margins
well-defined
multilobulated
spiculated
Calcified metastases in middle-aged

female with ovarian cancer
Solitary Metastasis vs. Lung Primary
In patients with known primary malignancies and single parenchymal nodules,

the overall incidence of second primary lung carcinoma is greater than that of
solitary metastases
Coppage et al: J Thorac Imaging 1987; 2(4):24-37
The likelihood of a primary lung cancer versus a metastasis depends on the

histologic characteristics of the extrapulmonary neoplasm and the patients
smoking history
Quint et al: Radiology 2000; 217: 257-61
A SPN is more likely to be bronchogenic CA than a solitary met if the patient

has carcinoma of:
Head and neck
Bladder
Esophagus
Breast
Cervix
Bile Ducts
Ovary
Prostate
Stomach
The incidence is fairly equal in patients with carcinoma of:

Kidney
Colon
Adrenal gland
Uterus
Salivary or parotid gland
Thyroid gland
SPN is more likely solitary metastasis in:
Melanoma
Sarcoma (soft tissue, bone)
Testicular carcinoma
Solitary Metastasis
142
Chest Radiology
Lymphangitic Carcinomatosis
Figure 1-15-10
Adenocarcinomas in 80%:
Lung
Breast
Stomach
Pancreas
Prostate
Colon
Incidence 6-55%
Symptoms: gradual onset dyspnea, cough
PFTs: reduced lung compliance & diffusing capacity
Diagnosis: bronchial lavage or TBB
Lymphangitic Carcinomatosis [Figure 1-15-10]
Blood-borne tumor cells extravasate and invade lymphatic

channels
Tumor also enters lymphatics retrograde via mediastinal, hilar
lymph nodes (25%)
Lymphatics expand with tumorlets and edema
Clusters or cords of tumor in lymphatics of the interlobular septa
and peribronchovascular interstitium
Edema and desmoplastic reaction accentuate interstitial
thickening
Pleural involvement: 2/3
Nodal involvement: 1/3
Secondary pulmonary lobule:

lymphangitic carcinomatosis
produces smooth and nodular
expansion of bronchovascular bundle
sheaths and interlobular septa
Lymphangitic Carcinomatosis:
Imaging Features - Chest radiograph
Normal (50%)
Kerley B lines
Subpleural edema
Pleural effusion (30-50%)
Hilar,mediastinal lymphadenopathy (20-40%)
Bilateral or unilateral findings
Figure 1-15-11
Lymphangitic Carcinomatosis
Imaging Features: Chest CT [Figure 1-15-11]
Smooth or nodular thickening of

Bronchovascular bundles
Interlobular septa (Kerleys lines; polygonal
arcades)
Lobar fissures (subpleural edema)
Focal or unilateral distribution (50%)
lung or breast CA
Pleural effusion
Lymphadenopathy (up to 50%)
Lymphangitic carcinomatosis in a middle aged

female with breast cancer
Tumor Embolism
Lodges in distal arterioles (100-200 micron diameter)

26% cancer pts (at autopsy)
<1% clinically significant
Complications
Cor pulmonale (PAH)
Lung infarction
Lung hemorrhage
Parenchymal or lymphatic mets if extravasation
Chest Radiology
143
Tumor Embolism
Figure 1-15-12
Malignancies
Stomach
Lung (esp. adenoCA)
Breast
ChorioCA
Ovary
Prostate
Liver
Kidney
Lymphoma
Right atrial myxoma
Tumor Embolism [Figure 1-15-12]
CXR
typically normal
if widespread: nodules, airspace opacities
CT
beading of peripheral pulmonary arteries
mosaic perfusion
wedge-shaped peripheral opacities
if extravasation: nodules, lymphangitic carcinomatosis
Endobronchial Metastases
Tumor thromboembolism may produce

beading along peripheral
bronchovascular bundles, as well as
pulmonary infarction
[Image courtesy of Mark Gosselin, MD]
Tumor arrives via bronchial arteries & peribronchial lymphatics

Rarely, via airways (BAC)
2-5% incidence in pts dying from metastases
Malignancies
Kidney
Colon, Rectum
Breast
Melanoma
Pancreas
Mean interval from diagnosis of primary: 65 months
Mean survival after discovery: 15.5 months
Treatment options
Radiation,
Chemotherapy
Surgery
Interventional bronchoscopy (stenting, laser or mechanical
resection, brachytherapy, photodynamic therapy)
Figure 1-15-13
Endobronchial metastases with left

upper lobe collapse (renal cell
carcinoma)
Chan et al. Curr Opin Pulm Med 2003;9:301-308
Endobronchial Metastases: Imaging Features [Figure 1-15-13]
Intraluminal soft tissue mass

Atelectasis or post-obstructive pneumonia
Serpiginous or nodular opacities (distal mucoid
impaction)
Hilar mass (if adjacent mediastinal invasion)
DDX: bronchogenic carcinoma
Figure 1-15-14
Pleural Metastases [Figures 1-15-14 to 1-15-16]
Malignancies
Lung
Breast (50% of patients)
Ovary
Stomach
Lymphoma
Arise from lymphangitic or vascular invasion
Large pleural effusion and pleural-based nodule in
patient with breast cancer
144
Chest Radiology
Figure 1-15-16
Typically manifest as exudative pleural effusion

Pleural nodules less common
Scattered nodules on pleural surface
Visceral & parietal pleura typically both involved
Radiologic DDX
asbestos exposure
splenosis
Rind-like or sheet-like pattern
Radiologic DDX
mesothelioma
post-inflammatory fibrothorax
Figure 1-15-15
Pleural rind-like metastases in elderly female with

NSCLC
Large pleural effusion and pleural-based nodule in

patient with breast cancer
Do we impact the management of cancer

patients with metastatic disease to the lung?
Statement from the Fleischner Society:
Guidelines for Management of Pulmonary Nodules
MacMahon H et al. Radiology
2005;237:395-400
Chest Radiology
145
Parenchymal Nodules
Assessing Therapeutic Response
Tumor doubling time

Definition: 25% increase in tumor diameter separated by time (Tdt)
Nodule growth rates vary widely
according to histologic tumor cell type
within the same patient
Proposed reasons for differences
Not all nodules are tumor (benign or inflammatory)
Smaller mets grow at faster rates than larger mets
Nodules represent different monoclonal cell populations (with variable
responses to treatment)
Document changes in several nodules in the same patient for optimal
therapeutic assessment - impacts treatment strategy & participation in clinical
trials
Chojniak et al. Am J Clin Oncol 2003;26(4):374-377
Parenchymal Nodules
Indications for Metastasectomy
To cure, but only if

Complete resection possible
No extrathoracic metastases (EXCEPT colon CA with liver mets)
No therapeutic alternative (chemotherapy-insensitive tumors)
Multiple nodules NOT necessarily a contraindication
To prolong 5-year survival
Colorectal cancer: up to 20-50%
Osteogenic & soft tissue sarcomas: up to 40%
Melanoma, renal cell, head & neck, female GU: up to 30%
Thyroid, parathyroid: up to 61%
Yoneda et al. Curr Opin Pulm Med 2000;6:356-363.
A Sarcoid-like Reaction
Rare but well-documented

Follows resection or treatment
Lymphoma (Hodgkin & Non-Hodgkin)
AML
Lung CA
Testicular CA
Gastric CA
Renal CA
Radiologic manifestations
Mediastinal, hilar lymphadenopathy
Pulmonary nodules or consolidations
Systemic sarcoidosis absent
Positive on FDG-PET: mimics recurrence
Biopsy required for confirmation
? Local immunologic response to tumor cells
Patterns of Metastatic Disease to the Lung

Overview
Parenchymal nodules
Well-circumscribed, random or angiocentric, basilar>apical
Unusual: cavitary, calcified, solitary
Lymphangitic carcinomatosis
Septal lines, nodular/thickened fissures, GGO
Pleural effusion
Lymphadenopathy
Tumor thromboembolism
Beading of peripheral arteries
Mosaic perfusion
Pleural-based opacity (infarction)
146
Chest Radiology
Endobronchial nodule
Rounded defect in airway, or cut-off of airway lumen
Post-obstructive atelectasis, pneumonia, mucoid impaction
Pleural-based metastases
Pleural effusion
Nodules on pleural surface
Variation: rind-like pattern mimics mesothelioma
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Hirakata K, Nakata H, Haratake J. Appearance of pulmonary metastases on high-resolution CT scans: comparison
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Chest Radiology
147
Differential Diagnosis of Mediastinal Masses

Learning Objectives:
To define the mediastinum and describe the mediastinal compartments

To provide a classification for a practical approach to the imaging diagnosis of
mediastinal masses
To list clinical and cross-sectional imaging features that allow a focused differential
diagnosis
To describe lesions with pathognomonic imaging features
To differentiate neoplastic from non-neoplastic conditions with emphasis on
management
Figure 1-16-1
Mediastinal Compartments [Figure 1-16-1]
Mediastinum - space between pleural surfaces and lungs

Bound by sternum and vertebrae
From thoracic inlet to diaphragm
Thymus, lymph nodes, heart, great vessels,trachea, esophagus,
nerves and other soft tissues
Arbitrary division into compartments - no anatomic boundaries
The Mediastinal Compartments
[Figures 1-16-2 and 1-16-3]
Anatomic - Superior, anterior, middle, posterior

Excludes paravertebral areas
Surgical - Superior, anterior, middle, posterior
Includes paravertebral areas
Radiographic (Felson) - Anterior, middle, posterior
Radiographic (Fraser, Mller, Colman, Par) - Anterior, middle-posterior,
paravertebral
Figure 1-16-2
The mediastinum
Figure 1-16-3
Anatomic and Surgical Mediastinal Compartments

Radiographic Mediastinal
Compartments
Mediastinal Masses
148
Chest Radiology
Mediastinal Masses
Patients are often asymptomatic

83% of asymptomatic masses are benign
57% of symptomatic masses are malignant
Approximately 1/3 are malignant
Approximately 1/10 are vascular
Mediastinal Masses Mayo Clinic (N=1,064)
Neurogenic tumor
Thymoma
Cysts
Lymphoma
Teratoma
Granuloma
Mediastinal goiter
20%
19%
18%
30%
Wychulis et al. J. Thorac Cardiovasc Surg 1971
Approach to Mediastinal Masses
Clinical
Demographics (age, gender) / Symptoms
Radiography
Mediastinal compartment / Adjacent structures
Focal mass vs. diffuse mediastinal enlargement
Lesion contours / Density
Cross-sectional Imaging
Location / Relationship to normal structures
Morphologic features / Associated findings
Mediastinal Masses
Neoplasia
Malignant (secondary / diffuse)
Benign and malignant (primary / focal)
Congenital cysts
Glandular enlargement
Vascular lesions
Herniations / Esophageal abnormalities
Miscellaneous conditions
Figure 1-16-4
Malignant Neoplasia Lung Cancer
Small cell / poorly differentiated lung cancer

Elderly smokers; males and females
Cough, dyspnea, SVC syndrome, weight loss
Mediastinal mass, lymphadenopathy, local invasion
Primary neoplasm may not be evident
Non-surgical lesion
Malignant Neoplasia - Metastases [Figure 1-16-4]
Known malignancy
Renal cell carcinoma
Testicular carcinoma
Head and neck cancer
Breast carcinoma
Melanoma
Chest Radiology
Metastatic renal cell carcinoma
149
Mediastinal Masses
Malignant Neoplasia Lymphoma
Figure 1-16-5
Hodgkin disease / Non-Hodgkin lymphoma

All age groups (young patients); Males and females
Palpable lymphadenopathy, constitutional symptoms
Lobular / diffuse mediastinal enlargement
Prevascular, paratracheal lymphadenopathy
Nodal coalescence, local invasion
Primary mediastinal lymphoma
Non-surgical lesion
Lymphoma
Non-Hodgkin lymphoma - 75% of all cases

50-70% of mediastinal lymphoma is Hodgkin disease
15-21% is non-Hodgkin lymphoma
Hodgkin 66% intrathoracic at presentation
Non-Hodgkin 37% intrathoracic at presentation
Treatment: Radiotherapy, chemotherapy
Hodgkin Disease: Microscopic Features
Lymphoma: Clinical Features
Hodgkin Disease
Males = Females (NSHD, 2 X more common in females)
Bimodal distribution: 2nd to 3rd and > 5th decades
Lymphadenopathy: cervical, supraclavicular
20-30%; fever, night sweats, wt. loss
Non-Hodgkin lymphoma
Systemic disease with constitutional symptoms: lymphadenopathy,
local invasion
Lymphoblastic - male children / adolescents
Diffuse large-B cell - young adult females
Figure 1-16-6
Lymphoma: Pathologic Features [Figures 1-16-5 and 1-16-6]
Hodgkin Disease
Nodal cellular infiltrate, collagenous connective tissue (NS), ReedSternberg cell
Lymphadenopathy, nodal coalescence, primary thymic
involvement, cystic change, hemorrhage, necrosis
Local invasion (including chest wall), hemorrhage, necrosis
Non-Hodgkin Lymphoma
Lymphoblastic (precursor T-lymphoblastic) -lymphoblasts
Diffuse large B-cell (primary mediastinal [thymic] large B-cell);
large cells, vesicular nuclei, prominent nucleoli
Large, infiltrative, locally invasive mass, necrosis
Lymphoma: Imaging Features [Figures 1-16-7 to 1-16-9]
Hodgkin Disease: Gross Features
Figure 1-16-7
Lobulated unilateral/bilateraldiffuse mediastinal enlargement

Hodgkin Disease
Intrathoracic involvement in 85%
Lymphadenopathy; prevascular, paratracheal
Nodal coalescence (homogeneous or heterogeneous)
Ca++; 1% - 1 year post-therapy, rare pre-therapy
Non-Hodgkin
Prevascular, paratracheal adenopathy
Isolated involvement of other mediastinal lymph nodes
Local invasion
Primary mediastinal lymphoma
Hodgkin Disease: Imaging Features
Mediastinal Masses
150
Chest Radiology
Figure 1-16-8
Figure 1-16-9
Non-Hodgkin lymphoma nodal coalescence with low

attenuation corresponding to necrosis
Hodgkin disease prevascular and paratracheal
lymphadenopathy and left pleural effusion
Secondary Neoplasia
Diffuse, bilateral mediastinal enlargement

Lymphadenopathy
Local invasion
Metastases
Other imaging features of malignancy
Non-Neoplastic Lymphadenopathy
Infection
Fungal: Mediastinal fibrosis; Calcification
Other granulomatous infections
Sarcoidosis
Bilateral symmetric hilar lymphadenopathy
Typical lung parenchymal involvement
Castleman disease
Enhancement / calcification (10%)
Mediastinal Fibrosis
Granulomatous lymphadenopathy
Young patients with signs and symptoms of obstruction
Trachea, bronchi, esophagus, vessels
Mediastinal mass, circumscribed or locally invasive,
calcification
Systemic antifungal agents, excision, dilatation,
bypass graft
30% mortality
Figure 1-16-10
Castleman Disease [Figure 1-16-10]
Angiofollicular or giant lymph node hyperplasia

Hyaline vascular type (> 90%) vs. plasma cell variant
Localized vs. systemic
Adult females (M:F - 4:1) may be asymptomatic
Middle mediastinal / hilar mass
Solitary mass
Dominant mass with lymphadenopathy
Multiple enlarged lymph nodes
Enhancement, calcification (10%)
Castleman Disease: Enhancing mediastinal
lymphadenopathy
Chest Radiology
151
Mediastinal Masses
Primary Neoplasia
Figure 1-16-11
Thymus
Thymoma
Thymic malignancy
Thymolipoma
Germ cell neoplasm
Neurogenic neoplasms
Thymoma
Epithelial neoplasm, most common primary thymic neoplasm

Slow growth, benign behavior
M=F; 70% in the 5th and 6th decades
Most patients asymptomatic
25-30% with symptoms of compression/invasion
Associated parathymic syndromes:
Myasthenia gravis
Pure red cell aplasia
Hypogammaglobulinemia
Thymoma and Myasthenia Gravis
Myasthenia gravis (MG) autoimmune neurological disorder

85% of patients with MG have follicular thymic hyperplasia
15% of patients with MG have a thymoma
Of all patients with thymoma, 30-50% have MG
Thymoma: Pathologic Features [Figures 1-16-11 to 1-16-13]
Lymphocytes and epithelial cells in varying proportions

WHO 1999 classification (morphology and lymphocyte-to-epithelial
cell ratios)
Types A, AB, B1, B2, B3
Tumor lobules compartmentalized by fibrous septa
Encapsulated vs. Invasive
Spherical mass, variable size, lobular contours, typically encapsulated
Hemorrhage, necrosis, cystic change (mural nodules)
Invasive thymoma - microscopic documentation of capsular invasion,
local invasion, tumor implants, metastases
Figure 1-16-13
Thymoma: Microscopic Features
Figure 1-16-12
Thymoma: pathologic features

tumor lobules compartmentalized by
fibrous bands
Thymoma: Gross Features

Mediastinal Masses
152
Chest Radiology
Thymoma: Imaging Features [Figures 1-16-14 to 1-16-18]
Anterior mediastinal mass; lobular, unilateral,

variable size
Normal radiographs in 25% (occult thymoma)
Focal, spherical, homogeneous or heterogeneous
Necrosis, cystic (mural nodules), calcification
(typically curvilinear and peripheral)
No lymphadenopathy
Exclude local invasion of fat, cardiovascular
structures, lung
Pleural implants (may cause diffuse pleural
thickening)
Figure 1-16-14
Thymoma: Unilateral, lobular, left anterior

mediastinal mass
Figure 1-16-15
Figure 1-16-16
Occult Thymoma: Well-defined, lobular, unilateral,

prevascular mass
Thymoma: Well-defined right cardiophrenic angle mass

with irregular low attenuation corresponding to necrosis
Figure 1-16-18
Figure 1-16-17
Cystic thymoma: Unilateral spherical cystic mass with

peripheral curvilinear calcification and mural nodules
Invasive thymoma: Direct invasion of left
brachiocephalic vein
Chest Radiology
153
Mediastinal Masses
Thymoma: Staging (Masaoka)

(10 - year survival)
Figure 1-16-19
I
II
Encapsulated / no microscopic capsular invasion (86-100%)

Microscopic invasion into surrounding fat / mediastinal pleura,
microscopic capsular invasion (55-100%)
III Macroscopic invasion of adjacent organs, pericardium, heart,
great vessels, lung (47-60%)
IVa Pleural / pericardial dissemination (0-11%)
IVb Lymphatic / hematogenous dissemination
Thymoma: Therapy / Prognosis
Encapsulated; complete excision

Best prognosis
Occasional local recurrence, distant metastases
Post-operative radiation for invasive thymoma to decrease local
Thymic Carcinoid: Mediastinal mass with
recurrence
adjacent lymphadenopathy in a patient
Chemotherapy for for progression after surgery and unresectable
with ACTH production and MEN 1
lesions
Thymic Malignancy: Carcinoid / Carcinoma
Rare malignant epithelial neoplasms

Symptomatic patients
Poor prognosis
Thymic Carcinoid
Neuroendocrine neoplasm; atypical carcinoid (necrosis / mitoses / invasion)

Males > Females; 3:1; wide age range (average, 43 yrs)
50% functionally active
ACTH Cushing syndrome (33-40%)
MEN type 1 (Wermer syndrome) (19-25%)
Hyperparathyroidism (90%), islet cell tumor of pancreas
(80%) pituitary adenoma (65%)
Figure 1-16-20
Thymic Carcinoma
Male > Female; wide age range (mean: 5th decade)

Several cell types identical to primary lung cancer; R/O
metastases
WHO Type C thymoma
Thymic Carcinoid / Carcinoma: Imaging Features

[Figure 1-16-19]
Large anterior mediastinal mass (R/O thymoma)

R/O metastatic lung malignancy (histology)
Lymphadenopathy
Local invasion, pleural or pericardial effusion/implantation,
metastases
Carcinoid
Octreotide imaging for diagnosis for occult (non-specific metastases and other neoplasms)
Thymolipoma
Rare benign thymic neoplasm

M=F; wide age range (average age, 28 yrs)
Asymptomatic patients: 50%
Symptoms with large tumors
Thymolipoma, microscopic features:

Thymic tissue admixed with mature
adipose tissue
Thymolipoma: Pathologic Features [Figure 1-16-20]
Encapsulated, soft, lobular, yellow

Mature adipose tissue and thymic tissue in variable proportions
Mediastinal Masses
154
Chest Radiology
Thymolipoma: Imaging Features [Figure 1-16-21]
Well-defined anterior / inferior mediastinal mass

Unilateral or bilateral, slow growth
May conform to shape of structures
R/O cardiac enlargement / diaphragmatic
elevation
Positional change in shape
Anatomic connection to the thymus (pedicle)
Mixed fat and soft tissue attenuation/signal
Figure 1-16-21
Germ Cell Neoplasms
Most common in the gonad

Extragonadal germ cell neoplasms; midline
locations, most commonly the mediastinum
Postulated origin in multipotential primitive germ
cells misplaced during embryogenesis
Cell types:
Teratoma (mature, immature [immature
neuroectoderm], malignant [mixed malignant
germ cell neoplasm])
Seminoma
Non-seminomatous germ cell neoplasms
Thymolipoma, imaging features anterior mediastinal

mass with anatomic connection with the thymus and
mixture of fat and soft tissue attenuation
Figure 1-16-22
Mature Teratoma
60-75% of mediastinal germ cell neoplasms

Males=Females
Children and young adults (< 40 yrs)
Often asymptomatic
Symptoms of compression or rupture
Mature Teratoma: Pathologic Features

[Figures 1-16-22 and 1-16-23]
More than one embryonic germ cell layer

Ectoderm skin, dermal appendages
Mesoderm bone, cartilage, muscle
Endoderm GI, respiratory tissue, mucus glands
Spherical, encapsulated, lobulated
Multilocular or unilocular cyst
Oily, sebaceous, gelatinous material (lipid)
Focal solid areas: hair, teeth, bone
Teratoma, microscopic features:
cystic neoplasm with ectodermal,
mesodermal and endodermal
components
Figure 1-16-23
Teratoma, gross features: multilocular

cystic mass
Chest Radiology
155
Mediastinal Masses
Mature Teratoma: Imaging Features [Figure 1-16-24]
Figure 1-16-24
Unilateral anterior mediastinal mass

Spherical, lobular contours, well-defined
Multilocular cystic - 85%
Attenuation:
Fluid 89%, Fat 76%, Ca++ 53%
Fat fluid level - 11%
ST/FL/FAT/Ca++
39%
ST/FL/FAT
24%
ST/FL
15%
Mature Teratoma: Therapy and Prognosis
Complete excision is curative

Excellent prognosis
Near 100% five-year survival
Teratoma, imaging features: Unilateral,

anterior mediastinal, multilocular cystic
mass with intrinsic fluid, soft tissue, fat
and calcium
Germ Cell Neoplasms
Most common in the gonad

Extragonadal germ cell neoplasms; midline locations, most
commonly the mediastinum
Postulated origin in multipotential primitive germ cells misplaced during
embryogenenesis
Cell types:
Teratoma (mature, immature, malignant)
Seminoma
Non-seminomatous germ cell neoplasms
Figure 1-16-25
Seminoma
40% of malignant germ cell neoplasms of a single histology

Caucasian males, third to fourth decades
Most patients are symptomatic
Rounded cells with sharp borders, clear cytoplasm, fibrous
bands, lymphocytes, plasma cells, granulomas
Homogeneous soft tissue mass
Radiation therapy / Cisplatin-based chemotherapy
60-80% long-term survival
Seminoma: Imaging Features [Figure 1-16-25]
Anterior mediastinal mass (both sides of midline)

Large, bulky, well-defined, lobulated, locally invasive
CT:
Homogeneous soft tissue mass
Mimics nodal coalescence
Slight homogeneous contrast-enhancement
Rarely necrosis / cystic change (8%)
Seminoma, imaging features: Diffuse

homogeneous anterior mediastinal mass
with mass effect
Figure 1-16-26
Non-Seminomatous
Malignant Germ Cell Neoplasms
Yolk sac (endodermal sinus) tumor

Embryonal carcinoma
Choriocarcinoma
Mixed germ cell neoplasm
Males, 90% symptomatic
Klinefelter syndrome (20%); hematologic malignancy
Alpha-fetoprotein (EST, EC)
B-human chorionic gonadotropin (choriocarcinoma)
LDH (60%) tumor burden
Large, unencapsulated
Hemorrhage, necrosis, cyst formation
Cisplatin-based chemotherapy; excision of residual tumor
Mediastinal Masses
156
Non-seminomatous malignant germ cell

neoplasm, imaging features: Large
anterior mediastinal locally invasive
heterogeneous mass
Chest Radiology
Non-Seminomatous GCN: Imaging Features
Figure 1-16-27
[Figure 1-16-26]
Large, well or poorly-defined anterior mediastinal mass

Extends to both sides of midline
Heterogeneous
Large areas of central low attenuation
Frond-like peripheral soft tissue
Loss of tissue planes
Local invasion, lymphadenopathy
Neurogenic Neoplasms [Figure 1-16-27]
20% of primary mediastinal neoplasms

35% in children
7080% benign
Peripheral nerves
Schwannoma
Neurofibroma
Malignant peripheral nerve sheath tumor
Sympathetic ganglia
Ganglioneuroma
Ganglioneuroblastoma
Neuroblastoma
Neurogenic neoplasms may arise from

peripheral nerves or sympathetic
ganglia
Figure 1-16-28
Schwannoma / Neurofibroma [Figure 1-16-28]
Schwannoma Most common mediastinal neurogenic neoplasm

Spherical, encapsulated
Cellular and less cellular areas (Antoni A / B)
Neurofibroma second most common mediastinal neurogenic
neoplasm
Spherical/fusiform, unencapsulated
Calcification, cystic change, hemorrhage
Young adults; 3rd and 4th decades
Most (65%) asymptomatic
Symptoms and signs of compression
Schwannoma / Neurofibroma: Imaging Features

[Figures 1-16-29 and 1-16-30]
Spherical, smooth / lobular, well-defined paravertebral mass

Osseous findings (50%): pressure erosion/deformity of ribs or vertebrae;
expanded neuroforamen
Homogeneous/heterogeneous
Heterogeneous enhancement; Ca++ in 10%
Growth into spinal canal in 10%
MR Imaging R/O spinal involvement
T1 Low-to-intermediate signal
T2 Foci of high signal
Figure 1-16-30
Figure 1-16-29
Schwannoma, imaging features: Unilateral paravertebral

spherical mass
Chest Radiology
Schwannoma, gross features:

Heterogeneous spherical mass
Schwannoma, imaging features: Intraspinal extension

157
Mediastinal Masses
Neurofibromatosis (NF1)
Multiple neoplasms (including ganglioneuroma)

Plexiform neurofibroma
Vagus nerve, sympathetic chain, phrenic nerve
Diffuse enlargement of peripheral nerve
Multiple masses along a nerve
Malignant Peripheral Nerve Sheath Tumor
Most frequent in the paravertebral region

Rare among neurogenic neoplasms
Large (> 5 cm) spherical mass
Central low attenuation necrosis
Calcification
May exhibit local invasion
Peripheral Nerve Neoplasms: Therapy and Prognosis
Excision
Schwannoma/Neurofibroma
Excellent prognosis
Malignant peripheral nerve sheath tumor
Solitary 75% five-year survival
Neurofibromatosis 30% five-year survival
Thoracic Meningocele
Intrathoracic extrusion of meninges and their fluid content

Well-defined spherical paravertebral mass
Enlarged neuroforamen, pressure erosion, sclerosis
Homogeneous, fluid attenuation / signal
Figure 1-16-31
Ganglioneuroma [Figure 1-16-31]
Children, adolescents, young adults

Asymptomatic patients
De novo; maturation of neuroblastoma
Benign paravertebral neoplasm
Mature ganglion cells, Schwann cells, nerve fibers
Encapsulated, elongate mass
Gray / yellow with lobular surface
Ganglioneuroma: Imaging Features
Well-defined, oblong paravertebral mass

Osseous erosion / displacement
Homogeneous or heterogeneous
Calcification in 25%
MR: Homogeneous intermediate signal on T1 / T2
R/O intraspinal extension
Ganglioneuroma, gross features:

Elongate paravertebral mass
Ganglioneuroblastoma/Neuroblastoma
Infants and young children

Asymptomatic; chest wall pain, paraplegia, Horner syndrome, diarrhea,
hemothorax
Elevation of urine catecholamines
Elevation of urine/serum VMA (screening)
Neuroblastoma Elongate paravertebral mass
50% < 2 years
90% < 5 years
May be congenital
Mediastinal Masses
158
Chest Radiology
Ganglioneuroblastoma/Neuroblastoma: Pathologic Features
Adrenal most common location

Paravertebral second most common location
Ganglioneuroblastoma: Neuroblasts and ganglion cells
Well / poorly differentiated
Neuroblastoma: Neuroblasts, Homer-Wright pseudorosettes
Well / poorly differentiated
Neuroblastoma: Imaging Features
Figure 1-16-32
[Figure 1-16-32]
Well-defined large elongate paravertebral mass

Radiographic evidence of Ca++ in 10%
Osseous erosion
R/O intraspinal growth
Local soft tissue invasion
Sympathetic Ganglia Tumors
Ganglioneuroma
Excision is curative
Five-year survival near 90%
Neuroblastoma
Five-year survival 30%
More favorable course with: age < 2 yrs, mediastinal
Spontaneous maturation to ganglioneuroma
Paraganglioma
Middle mediastinum: Aortopulmonary paraganglia

Paravertebral: Aortico sympathetic paraganglia
Heart
Adults (average age 30-40 yrs)
Males > Females; 2:1
Asymptomatic; excess catecholamines
Well-defined spherical mass
Homogeneous/heterogeneous
Marked contrast enhancement
90% uptake of I131 or I123 MIBG
Neuroblastoma, imaging features:

Unilateral calcified paravertebral mass in
a neonate with intraspinal extension
Primary Neoplasia
Benign
Focal, unilateral mass
No lymphadenopathy
No local invasion
Malignant (invasive)
Focal, unilateral mass
Lymphadenopathy
Local invasion
Figure1-16-33
Bronchogenic Cyst [Figure 1-16-33]
Most common congenital cyst of the mediastinum

Abnormal ventral foregut bud
Failure to induce mesenchymal development to lung
parenchyma
Mediastinum (85%), pericardium, diaphragm, pleura and
lung
Bronchogenic Cyst: Clinical Features
Rare in infants, infrequent in children

Young adults
Asymptomatic incidental finding
Symptomatic chest pain, mass effect, obstruction,
infection
Excision, observation, drainage, sterile alcohol ablation
Chest Radiology
159
Bronchogenic cyst: Typical subcarinal location

Mediastinal Masses
Bronchogenic Cyst : Pathologic Features

[Figures 1-16-34 and 1-16-35]
Figure 1-16-34
Respiratory epithelium
Wall: bronchial glands, cartilage, smooth muscle
Closed foregut connection
Spherical, ovoid, unilocular
Thin wall
Fluid variable: clear, turbid, hemorrhagic, serous, viscous
Bronchogenic Cyst: Imaging Features

[Figures 1-16-36 and 1-16-37]
Well-defined, spherical, middle mediastinal mass

Near trachea, carina, stem bronchi
CT:
Thin smooth wall (enhancement)
Water (40%) or soft tissue (43%) attenuation
Homogeneous / heterogeneous, non-enhancing contents
MR:
T1 - variable (slightly hyperintense to muscle)
T2 - isointense or hyperintense to CSF
Thin-walled pulmonary cyst; air, fluid, air-fluid level
Other Congenital Cysts
Foregut cysts
Esophageal - within esophageal; ectopic gastric mucosa
Neuroenteric - Associated spinal anomaly
Pericardial - Cardiophrenic angle, imperceptible wall, fluid
attenuation; asymptomatic patients
Bronchogenic cyst, microscopic

features: respiratory epithelium with
cartilage and smooth muscle in wall
Figure 1-16-35
Figure 1-16-36
Bronchogenic cyst, imaging features: Subcarinal

spherical mass with extension to the right
Bronchogenic cyst, gross features: Thinwalled unilocular cyst
Figure 1-16-37
Bronchogenic cyst, imaging features: spherical subcarinal mass that may not exhibit
water attenuation
Mediastinal Masses
160
Chest Radiology
Thymic Cyst [Figures 1-16-38 and 1-16-39]
Uncommon (3% of mediastinal masses)

Acquired vs. Congenital
Children / young adults
Association with neoplasia, AIDS
Diffuse infiltrative lymphocytosis syndrome (DILS)
Epithelial lining and thymus in cyst wall
Multilocular / unilocular
R/O cystic neoplasm
Figure 1-16-38
Congenital Cysts
Focal, spherical
Unilocular
Thin-walled
No mural nodules
No lymphadenopathy
Along foregut-derived structures
Thymic Hyperplasia
Lymphoid hyperplasia (lymphofollicular / autoimmune thymitis) secondary follicles with germinal centers; may not produce
thymus enlargement
Myasthenia gravis, hyperthyroidism, lupus, scleroderma, RA,
cirrhosis
True hyperplasia - global increase in the size and weight of the
thymus
Rebound hyperplasia - following chemotherapy (2 weeks to 14
months), steroids or severe insult
Ant. mediastinal widening
Homogeneous soft tissue
Maximal thickness
Under 20 years 1.8 cm
Over 20 years 1.3 cm
Follicular thymic hyperplasia normal or mildly enlarged thymus
Thymic cyst, gross features: multilocular

cyst
Figure 1-16-39
Mediastinal Goiter [Figure 1-16-40]
20% of cervical goiters

Asymptomatic females: incidental finding
May produce symptoms by mass effect
Adenomatous goiter; rarely malignancy or thyroiditis
Fibrous capsule; nodules composed of thyroid follicles
Hemorrhage, calcification, cystic change
Thymic cyst, imaging features:

Multilocular cyst
Figure 1-16-40
Mediastinal goiter, pathologic features: iodine content, well-defined

lobular soft tissue mass
Chest Radiology
161
Mediastinal Masses
Mediastinal Goiter: Imaging Features
Figure 1-16-41
[Figures 1-16-41 and 1-16-42]
Unilateral anterior mediastinal mass (80%)

Other compartments also affected, R > L
Well-defined lobular borders
Cervico-thoracic sign
Continuity with cervical thyroid
Calcification - punctate, coarse, curvilinear
Cystic change
High attenuation
Intense, sustained contrast enhancement
Figure 1-16-42
Mediastinal goiter, imaging features:

Large calcified unilateral mass with
cervicothoracic sign
Figure 1-16-43
Mediastinal goiter, imaging features: Continuity between cervical and
mediastinal portions of the mass, high attenuation and calcification
Parathyroid Adenoma
Ectopic parathyroid glands: superior pole of thymus (39%),

mediastinum (2%), intrathyroid (0.2-3.5%)
Primary hyperparathyroidism post surgical parathyroidectomy
MEN I
Imaging
Tc99m / Tl201 subtraction imaging
T123 / Tl201
Tc99m - Sestamibi (mitochondria)
Single radionuclide/Dual radionuclide
CT/MRI correlation of mediastinal uptake
Glandular Enlargement
Anatomically related to normal gland

Continuity with normal gland
Function similar to that of normal gland
Lymphangioma, microscopic features:

Interconnecting endothelial lined
vascular channels
Lymphangioma
Benign mesenchymal mediastinal tumor

Proliferation of lymphatic vessels without communication with lymphatic tree
Developmental vs. neoplasm vs. hamartoma
Asymptomatic / symptoms of compression
Mediastinal extension of cystic hygroma (10%), soft palpable mass; 90%
diagnosed in infancy
Mediastinal mass in asymptomatic child / adult
Lymphangioma: Pathologic Features

[Figures 1-16-43 and 1-16-44]
Intercommunicating spaces of variable size lined by endothelial cells

Soft, cystic mass
Cystic hygroma large vascular spaces
Cavernous lymphangioma small vascular spaces
Mediastinal Masses
162
Chest Radiology
Lymphangioma: Imaging Features [Figures 1-16-45 and 1-16-46]
Anterosuperior mediastinum; other compartments affected

Cervical / axillary / chest wall mass; mediastinal extension
Spherical, lobular, well-defined borders
Circumscribed mass / infiltrative mass
Multilocular, cystic, heterogeneous
Solid components, tissue septa
Figure 1-16-44
Figure 1-16-45
Lymphangioma, imaging features: Multilocular cystic mediastinal mass with

extension into the axilla
Lymphangioma, gross
features: Multilocular
cystic appearance due to
enlargement of vascular
channels
Figure 1-16-46
Lymphangioma, imaging features: Infiltrative or localized multilocular cystic

mediastinal mass
Hemangioma [Figure 1-16-47]
Rare vascular mediastinal tumor

Neoplasm vs. developmental
Young patients; 75% < 35 yrs.
Asymptomatic; 1/3-1/2 with
symptoms of compression
Rendu-Osler-Weber
syndrome
Communicating vascular spaces
Endothelial lining, organized
thrombi, Ca++, phleboliths
Anterior mediastinal mass (also in
other compartments)
Spherical, well-defined, Ca++
28%, punctate, phleboliths
Heterogeneous intense
enhancement
Chest Radiology
Figure 1-16-47
Hemangioma, imaging features: Anterior mediastinal mass with intrinsic

phleboliths and intense heterogeneous enhancement
163
Mediastinal Masses
Vascular Lesion - Aneurysm
Abnormal mediastinal contour contiguous with vascular structures

Saccular aneurysms may resemble other primary mediastinal masses
Curvilinear peripheral calcification
Contrast enhancement
Continuity with vascular lumen
Vascular Lesion - Varices
Esophageal / paraesophageal
Severe liver disease and portal hypertension; Left gastric portosystemic
collaterals
Visible on radiography in 10%
Middle-posterior-paravertebral cluster of serpiginous vessels with intense
enhancement
Vascular Lesions
Figure 1-16-48
Focal vs. infiltrative

Lymphatic
Multilocular cystic
Extramediastinal involvement
Blood vessels
Intense, heterogeneous / serpiginous enhancement
Aneurysms
Focal vascular enlargement
Herniations Hiatus Hernia [Figure 1-16-48]
Gastric herniation through enlarged esophageal hiatus

Increased intra-abdominal pressure / Increased prevalence with
increasing age
Asymptomatic; Reflux / bleeding
Retrocardiac mass, homogeneous, air-filled, air-fluid
Identification of abdominal contents in hernia sac
Herniation - Morgagni
Hiatus hernia, imaging features:

herniation of abdominal contents through
esophageal hiatus
Developmental defect in right anteromedial hemidiaphragm

Asymptomatic / Abdominal pain
Right cardiophrenic angle mass
Demonstration of internal fat (omentum), bowel loops or abdominal organs
(liver)
Herniations
Intrathoracic extension of abdominal contents

Bowel
Omental fat
Esophageal hiatus
Morgagni hernias
Miscellaneous Achalasia
Absent peristalsis and incomplete relaxation of esophageal sphincter

Primary deficiency of ganglion cells in myenteric plexus
Secondary (pseudo achalasia) Chagas disease and primary or secondary
malignancy at the GE junction
Esophageal dilatation with air-fluid levels
Esophageal displacement to the right, mass effect on mediastinum,
pulmonary consolidation (aspiration)
Miscellaneous - EMH
Extramedullary hematopoiesis
Compensatory formation of blood elements outside osseous medulla
Hemolytic anemia
Unilateral or bilateral paravertebral mass; may exhibit internal fat attenuation
Adjacent medullary expansion
Mediastinal Masses
164
Chest Radiology
Miscellaneous Acute Mediastinitis
Surgery, instrumentation with esophageal perforation

Ill patients with fever, chills and chest pain
Focal or diffuse mediastinal widening, pneumomediastinum, pleural effusion,
pneumothorax
Abscess, abnormal mediastinal air, extraluminal ingested contrast, obliteration
of tissue planes
Mediastinal Masses: Pathognomonic
Lateral thoracic meningocele

Aneurysm
Esophageal varices
Teratoma
Lipomatosis
Congenital cyst (BC, PC)
Mediastinal goiter
Mediastinal Masses: Cystic
Thymoma (mural nodules)

Congenital cysts (unilocular, middle, posterior mediastinum)
Neurogenic neoplasm (associated osseous erosion)
Meningocele (NF1, continuity with spinal canal, homogeneous water
attenuation / signal)
Mature teratoma (multilocular cystic mass with internal fat)
Lymphoma (lymphadenopathy)
Lymphangioma (multilocular cysts - vascular channels)
Esophageal enlargement (achalasia)
Mediastinal goiter (high attenuation, continuity with thyroid)
Mediastinal Masses: Fat
Lipomatosis (diffuse, no mass effect)

Lipoma
Thymolipoma (fat / soft tissue connecting to thymus)
Mature teratoma (cystic)
Morgagni hernia (CPA, right, continuous with abdominal fat)
Mediastinal Masses: Intense Enhancement
Mediastinal goiter (continuity with cervical thyroid)

Hemangioma (phleboliths, follows vascular enhancement)
Castleman disease (enhancing lymphadenopathy)
Paraganglioma (catecholamine production)
Aneurysm / Varices
References
General
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Germ Cell Neoplasms
1. Choi S-J, Lee JS, Song KS, Lim T-H. Mediastinal teratoma: CT differentiation of ruptured and unruptured tumors.
AJR 1998; 171: 591-594.
2. Moeller KH, Rosado-de-Christenson ML, Templeton PA. Mediastinal mature teratoma: imaging features. AJR 1997;
169: 985-990.
3. Strollo DC, Rosado-de-Christenson ML. Primary mediastinal malignant germ cell neoplasms: imaging features.
Chest Surg Clin N Am 2003; 12: 645-658.
Lymphoma
1. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal
from the international lymphoma study group. Blood 1994; 84: 1361-1392.
2. Fraser RS, Mller NL, Colman N, Par PD. Lymphoproliferative disorders and leukemia. In: Fraser RS, Mller NL,
Colman N, Par PD, eds. Fraser and Pars Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia:
Saunders, 1999; 1269-1330.
Non-Neoplastic Lymphadenopathy
1. Atasoy C, Fitoz S, Erguvan B, Akyar S. Tuberculous fibrosing mediastinitis: CT and MRI findings. J Thorac Imag
2001; 16: 191-193.
2. McAdams HP, Rosado de Christenson ML, Fishback NF, Templeton PA. Castleman disease of the thorax: radiologic
features with clinical and histopathologic correlation. Radiology 1998; 209: 221-228.
3. Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis.
Mediastinal Masses
166
Chest Radiology
Mediastinal Cysts
1. Choi YW, McAdams HP, Jeon SC, et al. Idiopathic multilocular thymic cyst: CT features with clinical and
histopathologic correlation. AJR 2001; 177: 881-885.
2. Jeung M-Y, Gasser B, Gangi A, et al. Imaging of cystic masses of the mediastinum. RadioGraphics 2002; 22:
S79-S93.
3. McAdams HP, Kirejczyk WM, Rosado-de-Christenson ML, Matsumoto S. Bronchogenic cyst: imaging features
with clinical and histopathologic correlation. Radiology 2000; 217: 441-446
Neurogenic Neoplasms
1. Ichikawa T, Ohtomo K, Araki T, et al. Ganglioneuroma: computed tomography and magnetic resonance features.
Br J Radiol 1996; 69: 114-121.
2. Marchevsky AM. Mediastinal tumors of peripheral nervous system origin. Semin Diagn Pathol 1999; 16: 65-78.
3. Moon WK, Im J-G, Han MC. Malignant schwannomas of the thorax: CT findings. J Comput Assist Tomogr 1993;
17: 274-276.
4. Rossi SE, Erasmus JJ, McAdams HP, Donnelly LF. Thoracic manifestations of neurofibromatosis-I. AJR 1999;
173: 1631-1638.
Endocrine Lesions
1. Buckley JA, Stark P. Intrathoracic mediastinal thyroid goiter: imaging manifestations. AJR 1999; 173: 471-475.
2. Fraser RS, Mller NL, Colman N, Par PD. Masses situated predominantly in the anterior mediastinal
compartment. In: Fraser RS, Mller NL, Colman N, Par PD, eds.
3. Fraser and Pars Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2875-2937.
4. Hopkins CR, Reading CC. Thyroid and parathyroid imaging. Semin US CT MRI 1995; 16: 279-295.
Vascular Lesions
1. Charruau L, Parrens M, Jougon J, et al. Mediastinal lymphangioma in adults: CT and MR imaging features. Eur
Radiol 2000; 10: 1310-1314.
2. Miyake H, Shiga M, Takaki H, Hata H, Osini R, Mori H. Mediastinal lymphangiomas in adults: CT findings. J
Thorac Imaging 1996; 11: 83-85.
3. McAdams HP, Rosado-de-Christenson ML, Moran CA. Mediastinal hemangioma: radiographic and CT features
in 14 patients. Radiology 1994; 193: 399-402.
4. Shaffer K, Rosado-de-Christenson ML, Patz EF Jr, Young S, Farver CF. Thoracic lymphangioma in adults: CT
and MR imaging features. AJR 1994; 162:283-29-89.
5. Henseler KP, Pozniak MA, Lee FT Jr, Winter TC III. Three-dimensional CT angiography of spontaneous
portosystemic shunts. RadioGraphics 2001; 21: 691-704.
6. Ibukuro K, Tsukiyama T, Mori K, Inoue Y. Preaortic esophageal veins: CT appearance. AJR 1998; 170: 15351538.
7. Ibukuro K, Tsukiyama T, Mori K, Inoue Y. Precaval draining vein from paraesophageal varices: Radiologicanatomic correlation. AJR 1999; 172: 651-654.
8. Kim M-J, Mitchell DG, Ito K. Portosystemic collaterals of the upper abdomen: Review of anatomy and
demonstration on MR imaging. Abdom Imaging 2000; 25: 462-470.
9. Lee SJ, Lee KS, Kim SA, Kim TS, Hwang JH, Lim JH. Computed radiography of the chest in patients with
paraesophageal varices: Diagnostic accuracy and characteristic findings. AJR 1998; 170: 1527-1531.
Miscellaneous Lesions
1. Fraser RS, Mller NL, Colman N, Par PD. The diaphragm. In: Fraser RS, Mller NL, Colman N, Par PD, eds.
Fraser and Pars Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2987-3010.
2. Mueller CF, Klecker RJ, King MA. Case 3. Achalasia. AJR 2000; 175: 867; 870-871
3. Woodfield CA, Levine MS, Rubesin SE, Langlotz CP, Laufer I. Diagnosis of primary versus secondary achalasia.
Reassessment of clinical and radiographic criteria. AJR 2000; 175: 727-731.
4. Dunnick NR. Image interpretation session: 1999. Extramedullary hematopoiesis in a patient with beta
thalassemia. RadioGraphics 2000; 20: 266-268.
5. Gilkeson RC, Basile V, Sands MJ, Hsu JT. Chest case of the day. Extramedullary hematopoiesis (EMH). AJR
1997; 169: 267, 270-273.
6. Moellers M-C, Bader JB, Alexander C, Samnick S, Kirsch C-M. Localization of extramedullary hematopoiesis
with Tc-99m-labeled monoclonal antibodies (BW 250/183). Clin Nuc Med 2002; 27: 354-357.
Chest Radiology
167
Mediastinal Masses
Chest Seminar: Where is the Lesion?

Learning Objectives
To review the radiologic features of thoracic radiologic abnormalities based on

location
To enumerate the radiologic characteristics that allow lesion localization and
the formulation of a focused radiologic differential diagnosis
Case 1: 38-year-old woman with cough
Location
Next best study
Diagnosis:
Solitary Lung Mass
Lung cancer
Size / frequency
Stage ?
Carcinoid tumor
Borders / bronchus
Solitary metastasis
Lower lobe location / shape
Hamartoma / Infection
Borders
Solitary Lung Mass
Young, relatively asymptomatic woman

Mass with well-defined lobular borders
Lower lobe location
Abutting bronchus
Chest Seminar: Where is the Lesion
168
Chest Radiology
Case 2: 16-year-old girl with cough
Location
Next best study
Diagnosis:
Anterior Mediastinal Mass
Young girl, relatively asymptomatic

Well-defined, unilateral mass with peripheral calcification and lobular borders
No lymphadenopathy
Central water attenuation
Low attenuation mural nodule
Anterior Mediastinal Mass
Mature teratoma
Fluid / fat / Ca++
Thymic cyst
Fluid / Ca++
Lymphoma
Age group but no lymphadenopathy
Thymoma
Fluid, calcium, mural nodule but fat
Chest Radiology
169
Case 3: 58-year-old man with chest pain and hemoptysis
Location
Next best study
Diagnosis:
Lung Mass with Cavitation and Chest Wall Involvement
Symptomatic older male

Chest wall invasion (rib destruction)
Upper lobe location
Cavitation
Chest Wall Mass / Cavitation
Bronchogenic carcinoma
Chest wall invasion
Stage?
Infection
Actinomycosis, tuberculosis, fungus
Primary chest wall tumor / Metastasis
Other
170
Chest Radiology
Case 4: Asymptomatic 40-year-old male; pre-operative

radiograph
Location
Next best study
Diagnosis:
Multifocal Pleural Nodules
Asymptomatic patient
No known malignancy
Well-defined peripheral pleural-based nodules
Associated findings - Abdominal abnormalities?
Multifocal Pleural Nodules
Splenosis
Where is the spleen?
Metastases
Malignant pleural mesothelioma
Other
Splenosis
Auto-transplantation of splenic tissue typically following splenic rupture

Most common manifestation: Multiple peritoneal nodules
Thoracic splenosis:
Multiple pleural-based nodules
May be missed on radiography
99mTC-tagged heated RBC scintigraphy
Liver-spleen scan
Chest Radiology
171
Case 5: 34-year-old man with left chest pain for many years
Location
Next best study
Diagnosis:
Multifocal Chest Wall and Mediastinal Masses
Chronic lesions with minimal symptoms

Unilateral or bilateral?
Benign pressure erosion
Pulmonary involvement
Other chest wall / mediastinal involvement
Multifocal Chest Wall and Mediastinal Masses
Neurofibromatosis
Malignant potential
Vascular lesions
Metastases
Other
172
Chest Radiology
Chest Seminar: Differential Diagnosis of

Mediastinal Masses
Learning Objectives
To review concepts of differential diagnosis of mediastinal masses

To emphasize importance of demographics, location and morphology in the
formulation of a focused differential diagnosis
Case 1: Elderly man with chest pain
Location
Characterization
Next study
Biopsy
Heterogeneous Middle Mediastinal Mass; Rim CA++
Neoplasia
Carcinoma
Lymphoma
Congenital Cyst but heterogeneous
Vascular lesion
Aneurysm rim CA++
Other
Chest Radiology
173
Chest Seminar: Differential Diagnosis of Mediastinal Masses
Case 2: Asymptomatic 52-year-old man
Next best study
Spherical Paravertebral Mass with Pressure Erosion
Neurogenic Neoplasm
Next study?
Lateral Thoracic Meningocele
History?
Other
174
Chest Radiology
Case 3: 40-year-old woman with difficulty swallowing
Next best study
Unilateral Cystic Anterior Mediastinal Mass; Mural CA++
Cystic Thymoma
Mural nodule
Cystic Teratoma
No fat no calcium
Thymic Cyst / Pericardial Cyst
but mural nodule
Cystic Lymphoma
but no lymphadenopathy
Unilateral Cystic Anterior Mediastinal Mass; Mural CA++ and

mural nodule
Symptomatic woman
Symptoms related to function of voluntary musculature
Age over 40
Pattern of enhancement
Mural nodule
Chest Radiology
175
Case 4: 24-year-old man with chronic abdominal discomfort
Diagnosis
Should the lesion be excised?
Right Cardiophrenic Angle Mass of Fat and Soft Tissue

Attenuation
Thymolipoma
Fat / soft tissue
Does not conform to adjacent structures / thymus?
Lipoma
Fat / soft tissue
Mature Teratoma
But...No fluid
Morgagni Hernia
Continuity with abdominal fat
176
Chest Radiology
Case 5: 29-year-old woman with fatigue and cough
Diagnosis
Should the lesion be excised?
Biopsied?
Anterior Mediastinal Mass (Cystic change, Ca++)
Lymphoma:
Age, local invasion, lymphadenopathy
Cystic change, Ca++ ?
Thymoma
Cystic change, Ca++,local invasion
Butlymphadenopathy
Teratoma
Butlymphadenopathy, local invasion and soft tissue predominant
Malignant GCN
Butwrong gender
Chest Radiology
177
Pneumonia: Usual and Unusual

Organisms
Rosita M. Shah, MD
Classification of Pulmonary Infection
Community-acquired Pneumonia
S. pneumoniae LOBAR
Mycoplasma LOBULAR
Influenzae INTERSTITIAL
Nosocomial Pneumonia
Unusual Pulmonary Infections
Pulmonary Infection: Classification: Morphology
3 radiographic and pathologic patterns

Lobar
Lobular (bronchopneumonia)
Interstitial
Pulmonary Infection: Classification
Lobar and lobular pneumonias both produce air space filling

Significant differences include:
Site of initial inflammation
Degree of lobular opacification
Radiographic pattern
Etiologic agents
Alveolar Filling Pneumonias
Site of initial infection varies

Alveolar level in lobar pneumonia
Bronchiolar level in bronchopneumonia
Degree of opacification of secondary lobule is different

Complete in lobar pneumonia
Incomplete in bronchopneumonia
Radiographic pattern will vary

Lobar pattern
Bronchopneumonia pattern

Etiologic agent may vary
Lobar Pneumonia
S.pneumoniae
K.pneumoniae
also seen with
Legionella
Mycoplasma
H.influenzae
Pneumonia
Bronchopneumonia
Gram s, anaerobes
Legionella
Actinomycosis
Nocardia
Mycoplasma
Typical, atypical TB
Parasites
178
Chest Radiology
Accurate pattern recognition depends on:

Early imaging
Normal lung structure
Organisms may produce more than one pattern
Basic pattern differentiation may be difficult
Interstitial vs bronchopneumonia
Community-acquired Pneumonia: Epidemiology
210 /1000 annual incidence

22-50% hospitalization rate
Outpatient mortality 1-5%
Inpatient mortality 25%
Community-acquired Pneumonia: Etiology
In up to 50%, no definitive organism isolated

Most common isolates:
S. pneumoniae
M. pneumoniae
K. pneumoniae
H. influenzae
L. pneumophila
Respiratory viruses
S. pneumoniae: Demographics
S. pneumoniae most frequent isolate in CAP

8-76% incidence
Recognized risk factors
alcoholism, splenic dysfunction, viral pneumonia, congenital and acquired
immune deficiencies
S. pneumoniae: Demographics
25% incidence of bacteremia

25-40% mortality, unchanged >30y
Age >65
CHF,DM
Alcoholism
Thrombocytopenia
Renal dysfunction
Number of lobes
Chest 1993; 103:1152-56
S. pneumoniae: Pathology
Aspiration to peripheral air spaces

Alveolus represents site of initial inflammatory lesion
Spread occurs by contignous involvement of adjacent alveoli
3 pathologic stages
S. pneumoniae: Pathology
ACUTE RESPONSE
Increased capillary permeability
Protein rich edema
Contiguous alveolar filling via Pores of Kohn and Canals of Lambert
RED HEPATIZATION
PMN infiltration and intra-alveolar hemorrhage
GRAY HEPATIZATION
Macrophage infiltration and uptake of blood products
Chest Radiology
179
Pneumonia
S. pneumoniae: Radiology [Figure 1-19-1]
LOBAR pattern
Homogeneous, confluent density
Nonsegmental distributions
S. pneumoniae: Radiology
Spread at alveolar level results in nonsegmental distributions characteristic of

early lobar pneumonia
Round pneumonia
Manifestation of nonsegmental distribution
Most common in pediatric infection with S.pneumoniae
Figure 1-19-1
LOBAR pattern
Prominent air bronchograms
Preserved volume
48% of consecutive hospitalized pts demonstrated focal lobar

patterns
33%, multifocal lobar patterns
16% lobular pattern
Dominant pattern did not vary with immune status or disease
severity
AJR 2000;175:1533
Lobar pattern consolidation due to S.

pneumoniae
Small pleural effusions up to 60%

Infrequent cavitation
Associated with serotype 3
Most frequent organism in pulmonary gangrene
Vascular thrombosis from severe necrosis
Intracavitary mass (sloughed lung)
M. pneumoniae: Demographics
15-35% of CAP
50% of CAP during summer months
Peak age 5-25 yo
Self limited
Few fatal cases associated with ARDS
Increased severity in sickle cell anemia
Most frequent etiology in Atypical Pneumonia Syndrome
Atypical radiographic features
Prominent extrapulmonary complaints
M. pneumoniae: Pathology
Eaton agent-1944
Gram -- filamentous rod
Absent cell wall
Acute cellular bronchiolitis
Superficial inflammation involving luminal surface of bronchi, bronchioles
Associated interstitial infiltrates
Pneumonia
180
Chest Radiology
M. pneumoniae: Radiology [Figure 1-19-2]
Figure 1-19-2
LOBULAR pattern Bronchopneumonia

Heterogeneous, patchy consolidation
Minimal exudate into centrilobular alveoli
Segmental distribution
Spread at bronchiolar level
Volume loss
Minimal air bronchograms
Peribronchial thickening
M. pneumoniae: Radiology
CT Findings [Figure 1-19-3]

86% centrilobular nodules
82% bronchovascular thickening
59% consolidation with lobular distribution
Reittner, AJR 2000; 174:37
Bronchopneumonia pattern due to

M. pneumoniae
Respiratory Viruses
Influenzae A,B,C
Para-influenzae
Respiratory syncytial virus
Adenovirus
Herpes viruses
SARS
Figure 1-19-3
Influenzae A: Demographics
10-20% CAP
10,000-40,000 deaths/ influenzae epidemic
Peak incidence
Pediatric population
Highest mortality-adult and aged
Superinfection
S.aureus
S.pneumoniae
Influenzae A: Pathology
St 1 infection of epithelial cells, proliferation and necrosis

St 2 bronchial and alveolar wall edema,hemorrhage
Ulceration, bacterial infection
HRCT of M. pneumoniae
Influenzae A: Radiology
INTERSTITIAL pattern
Reticular
Nodular
Peribronchial thickening
Subpleural edema
Hilar haze
Figure 1-19-4
[Figure 1-19-4]
Bilateral, parahilar, lower

lobe
Air trapping
Prominent GGO
(left) CXR
(right) HRCT Influenzae pneumonia mimicking edema
Chest Radiology
181
Pneumonia
Pleural effusions, cavitation uncommon without bacterial superinfection

Rapid deterioration should suggest superinfection
Adenovirus [Figure 1-19-5]
Interstitial pneumonia with prominent necrotizing bronchiolitis

Potential infection in immune competent and suppressed hosts with high
mortality
Military epidemics
Transplant recipients
Swyer James, Macleods syndrome
Bronchiolitis obliterans following viral infection in early
childhood
Figure 1-19-5
Respiratory Herpesviruses
HSV-1, HSV-2, VZV, EBV, CMV

Primary infection, latency, reactivation
Up to 40% mortality
Risk factors
Immune-suppression, lung transplantation, airway
management, pregnancy
Swyer James Syndrome due to

pediatric viral pneumonia
Varicella Pneumonia
Figure 1-19-6
Complication of adult chickenpox

5-50% incidence
Prominent acinar opacities
5-10mm nodules, coalescence
Patchy GGO
Kim AJR 1999;172:113

May heal with miliary calcifications
Varicella Pneumonia [Figure 1-19-6]
Prominent acinar opacities
Severe Acute Respiratory Syndrome
SARS-CoV (corona virus)

Initial cases Nov 2002-June 2003, rapid spread from
Asia
20-50% require mechanical ventilation
10% mortality, age dependant
Severe DAD
Severe Acute Respiratory Syndrome
Acinar nodules in varicella pneumonia
Predominant consolidation 1-2weeks

Focal (39%), multifocal (28%), diffuse (14%)
Reticulation
Bronchiolar dilation
Residual changes in 50% at 4wks
Ooi GC. Radiology 2004;230:836; Paul NS. AJR 2003;182:493
Severe Community-acquired Pneumonia: Definition
Impending respiratory failure

Hemodynamic instability
Radiographic assessment
Bilateral or multilobar involvemnt
50% increase in size of opacity within 48hr
Pneumonia
182
Chest Radiology
Severe Community-acquired Pneumonia: Etiology
S. pneumoniae
L. pneumophila
S. aureus
P. aeruginosa in patients with bronchiectasis
L. pneumophila: Demographics
15% of CAP
Epidemic and sporadic forms
Legionnaires disease= pneumonic form
Peak summer
Aerobic Gram -- bacillus
Proliferates in warm, humid environments
Figure 1-19-7
L. pneumophila: Pathology
Bronchocentric inflammation
L. pneumophila: Demographics
Acute onset
Prominent extrapulmonary symptoms
Neurologic manifestations, diarrhea, renal insufficiency
10% mechanical ventilation
15% mortality in cases requiring hospitalization
L. pneumophila: Radiology [Figure 1-19-7]
Pleural effusions in 2/3
Bilateral and multifocal in 50%
May produce lobar or mass-like consolidation
Cavitation uncommon without immunosupression
Delayed resolution
K. pneumoniae: Demographics
Nosocomial or community acquired

5-10% lobar pneumonias
25% bacteremic, 50% mortality
Males, >60yo
Risk factors: alcoholism, COPD, DM
K. pneumoniae: Pathology
Gram -- bacillus
Abundant PMN infiltration of alveoli, edema
Lobar expansion - Friedlanders pneumonia
Massive necrosis
Common association with gangrene
HRCT in Legionella pneumonia

demonstrating bronchocentric
nodules and pleural effusion
K. pneumoniae: Radiology
Lobar pattern
Bulging fissures
Abscess 30-50%
Necrotizing pneumonia at CT
Low density areas with small cavities
Moon JCAT 1995;19:176
Chest Radiology
183
Pneumonia
S.aureus: Demographics
Figure 1-19-8
30-50% colonization rates in healthy adults

DM
IVDA
HIV
Surgical pts
Methicillin resistance 1944
Increasing incidence of resistant community-acq infection
Antecedant viral pneumonia
Frequent cause of nosocomial infection
Extremes of age
Nursing home population
Risk factors
Debilitated states, mechanical ventilation, burns, indwelling
catheters, IVDA
S. aureus: Radiology
Aerogenous infection [Figures 1-19-8 and 1-19-9]

Multifocal Broncho-pneumonia
Hematogenous infection [Figure 1-19-10]
Multifocal, discrete nodular or wedge shaped abnormality with
normal intervening lung
Cavitation / abscess (25-75%)
Pneumatoceles (60% ped infection)
Pleural effusions / empyema (50%)
CXR and HRCT bronchopneumonia

pattern due to S. aureus
Figure 1-19-9
Necrotizing bronchopneumonia due to S. aureus
Pneumonia
184
Chest Radiology
P. aeruginosa in Cystic Fibrosis
Figure 1-19-10
Chronic colonization with P.aeruginosa

Mucoid variant
ABX resistance
Elastase production
Bronchiectasis
The Practical Points
S.pneumoniae and K.pneumoniae most commonly associated

with lobar pattern and pulmonary gangrene
M.pneumoniae, L.pneumophilus most commonly associated with
broncho-pneumonia pattern and atypical pneumonia syndrome
Viral pneumonias associated with interstitial pattern
Pathologic in immune-competent and suppressed hosts
Prominent bronchiolitis seen with mycoplasma, adeno and other
respiratory viruses
Septic emboli
Nosocomial Pneumonia
Rising incidence parallels usage of antibiotics

Gram negative infections
40-50% increase 1950-60
55-65% nosocomial infections
50% nosocomial pneumonia
75% ICU pneumonia
Nosocomial Pneumonia: Definition
Pneumonia developing >48hr sp admission, intubation or discharge
Diagnosis
Quantitative cultures
Tracheal aspirate
10 5-6 cfu/ml
BAL
10 4 cfu/ml
Protected specimen brush
10 3 cfu/ml
False negative and false positive rates 20-30%
Baughman Chest 2000
Pathophysiology
Direct inhalation
Hematogenous spread
Aspiration
45% incidence in sleep
Altered gag reflex, consciousness, GI motility
NG / ET
Pathophysiology
Abnormal gram negative airway colonization

25% 24hr
40% 7d
Gastric alkalinization
Serious illness
Antibiotic TX
Johanson Ann Intern Med 1972
Pathophysiology
Repetitive aspiration leads to

Bronchiolitis
Lobular (broncho) pneumonia
Peribronchiolar neutrophilic infiltrate (104 cfu/g)
Chest Radiology
185
Pneumonia
Nosocomial Pneumonia in the ICU
Ventilator-associated Pneumonia (VAP)

Most common nosocomial infection in ICU
3-21x greater incidence in intubated patients
> 1 intubation, >3 days
10-65% ICU patients acquire VAP
1/3-3/4 ARDS patients acquire VAP
20-80% mortality rate
Figure 1-19-11
Ventilator-associated Pneumonia
Prognosis depends:
Organism
Highest mortality: P.aeruginosa, MRSA
Population
Highest mortality: medical ICU
10-20% mortality: trauma ICU
Late onset
MDR
Nosocomial Pneumonia and Aspiration [Figure 1-19-11]
Pneumonitis (Mendelson Syndrome)

pH < 2.5, >0.3 ml/kg
Biphasic inflammatory response
1-2 hr permeability edema
4-6 hr
acute inflammation
30% mortality
Rapid clearing
Nosocomial Pneumonia and Aspiration
Sterile
vs
Normal flora (<5d)
vs
Gram bacilli(>5d)
Anaerobes seen in late aspiration
Aspiration accounts for upto 15% of CAP
Dependent aspiration pneumonia

complicated by ARDS
Marik NEJM 2001
Microbiology
Normal flora
Gram bacilli
S. aureus
Anaerobes
Legionella
Respiratory viruses
Microbiology
Early <5d
H. influenzae
S. pneumoniae
S. aureus
Late >5d
S. aureus
P. aeruginosa
Enterobacteriaceae
Acinetobacter spp.
Stenotrophomonas maltophilia
Pneumonia
186
Chest Radiology
P. aeruginosa
Most common ICU isolate

70% TX failure rate
Distal airway colonization, hematogenous dissemination
Increased severity in neutropenia, bacteremia
P. aeruginosa: Pathology
Micro-abscesses
Necrotic vasculits
Sm-med pulmonary arteries
Hemorrhage
P. aeruginosa: Radiology
Discrete nodules may be indicative of vasculitis
Frequent cavitation
Pleural effusions/empyema
Nosocomial Viral Pneumonia
Rate of infection assoc with length of hospitalization

Hospital worker as carrier
Frequently unsuspected
High mortality rates
RSV 30-100%
Parainfluenzae 15-30%
Nodular or Mass-like Consolidations
Nonsegmental distribution
round pneumonia
Granulomatous infection
M. tuberculosis
Fungi
Actinomycosis
Nocardia
A. Israelii; Demographics
Figure 1-19-12
Normal oral flora

Sites of infection:
Cervicofacial
55%
Abdomen
20%
Pulmonary
25%
Risk factors: poor oral hygiene, aspiration
Smego RA. Clin Infec Dis 1998;26:1255
A. Israelii: Pathology
Multifocal abscesses
Interconnecting sinus tracts
Sulphur granule
Spoke-wheel arrangement of neutrophils surrounding
filamentous organism
Pneumonia with chest wall

involvement due to A. israelli
A. Israelii: Radiology [Figure 1-19-12]
Consolidation
Mass-like
Cavitary
Pleural, chest wall and osseous involvement
Up to 50%
Chest Radiology
187
Pneumonia
N. Asteroides: Demographics
Ubiquitous distribution
50% of patients are immunocompetent
Risk factors:
Neutropenia
Steroids, late HIV, hemetologic malignancy, alveolar proteinosis
N. Asteroides: Pathology
Peribronchial abscesses, granulomatous inflammation

Extensive necrosis
May mimic M.TB or fungal infection
N. Asteroides: Radiology
Extrapulmonary disease 50% with 40-90% mortality

CNS 25%
Skin and subcutaneous abscesses
N. Asteroides: Radiology
Consolidation
Mass-like
Cavitary
Pleural and chest wall involvement 30-50%
Adenopathy 40%
Cavitary Pneumonia in AIDS
N. asteroides
Alveolar Proteinosis and N. asteroides

Parasitic Infection
Pulmonary involvement due to hypersensitivity or direct invasion

Echinococcosis
Paragonimiasis
Ascariasis
Strongyloidiasis
Figure 1-19-13
Parasitic Infection
Radiographic findings may overlap with other

infections
Fleeting, patchy infiltrates
Bronchopneumonia
Atelectasis
Echinococcus granulosus
Cestode (tapeworm), endemic to S.America,

Australia, Middle East, Africa and Mediterranean
Definitive host - dog,wolf
Intermediate host - sheep, cow, deer, moose
Duodenum - portal venous system liver

45-75% isolated liver involvement
15-35% pulmonary involvement
Intact (right lung) and ruptured (left lung)

echinococcal cysts
Echinococcus granulosus [Figure 1-19-13]
Pulmonary cysts acquired in childhood

Diagnosis 30-40yo
Intact cyst - asymptomatic
Eosinophilia 25-40%
Pneumonia
188
Chest Radiology
Echinococcus granulosus: Pathology
Hydatid cyst consists of 3 layers

Pericyst host inflammatory cells
Exocyst acellular laminated membrane
Endocyst fluid-filled germinal center, daughter cysts
Echinococcus granulosus: Radiology
Intact cyst
Well demarcated, homogeneous mass
Spherical when central, ovoid when peripheral
Multiple 20-30%
Lower lobes 60%
Echinococcus granulosus: Radiology
Impending Rupture
Crescent sign - air between pericyst and laminated membrane
Ruptured cyst
Water lily sign rupture of endocyst
Paragonimiasis westermani
Trematode (lung fluke)

endemic to Asia
Contaminated freshwater crab
Jejunum peritoneal cavity diaphragm pleura lung
Chronic granulomatous reaction
Paragonimiasis westermani: Radiology
Pulmonary findings dependant on stage of infection

PTX and pleural infection during pleural penetration by juvenile worms
Transient, patchy consolidation and linear tracts during larval migration
Peribronchial cysts associated with mature worm
Ascariasis lumbricoides
Roundworm infection
Most common parasitic infection
Endemic worldwide
25-95% prevalence
Highest incidence in children
Large iingestion associated with pneumonitis
Small bowel systemic circulation alveoli - trachea small bowel
Strongyloides stercoralis
Round worm
Skin systemic circulation alveoli trachea small bowel
Ascariasis Strongyloides: Radiology
Bronchopneumonia
Patchy, transient consolidation
Eosinophilic pneumonia
B. Anthracis: Anthrax
Gram+ spore forming rod

Dormant spores are virulent
Infection typical in livestock
Exotoxin production associated with hemorrhagic mediastinitis, edema and
pleuritis
Earls Radiology:222:305, 2001
Chest Radiology
189
Pneumonia
Complications of Pneumonia [Figure 1-19-14]
Figure 1-19-14
Pleural Infection
Empyema
Purulent exudate
WBC>25,000
pH<7.0
+ organisms
S.mitis Empyema in 51yo male with IVDA hx

Complications of Pneumonia
Right empyema and LUL septic

embolus due to S. aureus
Cavitation
Cavitary pneumonia
Lung abscess
Pneumatocele
Gangrene
DDX bronchopleural fitula
Complications of Pneumonia
Pneumatocele
Ball-valve mechanism
Rapid evolution
No lung destruction
Most common with S.aureus
60% of peds infection
Figure 1-19-15
Complications of Pneumonia [Figure 1-19-15]
Pulmonary Gangrene
Lung necrosis due to vascular thrombosis
Most common with S.pneumoniae K.pneumoniae
Bronchiectasis
Irreversible dilation
Should not be diagnosed < 4 m of acute
infection
Colonization with atypical TB, aspergillus
Advanced course in HIV
+/- antecedant infection
CXR and CT pulmonary gangrene due to

K.pneumoniae
A.fumigatus complicating post-infectious bronchiectasis

The Role of Imaging in Pneumonia
Diagnosis of infection
Presence of centrilobular nodules in acute parenchymal disease favors
pneumonia
Tomiyama N. AJR 2000;174:1745
Thin section CT allows earlier diagnosis of pneumonia in
immunosuppressed pts (5 days)
Heussel CP. AJR 1997;169:1347

Recognition of complications
Decreased enhancement in pneumonia indicates severe necrosis
Donnelly LF. Radiology 1997;205:817
The Practical Points
Organisms may produce more than one pattern

Bacterial, viral and fungal pneumonia have similar CT findings post lung
transplantation
Collins AJR 2000;175:811
Consider clinical setting
Pneumonia
190
Chest Radiology
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Moon WK, Im JG, Yeon KM, Han MC. Complications of Klebsiella pneumonia: CT evaluation. J Comput Assist
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Paul NS, Roberts H, Butany J, Chung T, Gold W, Mehta S, Konen E, Rao A, Provost Y, Hong HH, Zelovitsky L,
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Jan;174(1):37-41.
Shah RM, Gupta S, Angeid-Backman E, O'Donnell J. Pneumococcal pneumonia in patients requiring
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Watanakunakorn C, Greifenstein A, Stroh K, Jarjoura DG, Blend D, Cugino A, Ognibene. AJ. Pneumococcal
bacteremia in three community teaching hospitals from 1980 to 1989.Chest. 1993 Apr;103(4):1152-6.
Collins J, Muller NL, Kazerooni EA, Paciocco G. CT findings of pneumonia after lung transplantation. AJR Am J
Roentgenol. 2000 Sep;175(3):811-8.
Donnelly LF, Klosterman LA. Pneumonia in children: decreased parenchymal contrast enhancement--CT sign of
intense illness and impending cavitary necrosis. Radiology. 1997 Dec;205(3):817-20.
Earls JP, Cerva D Jr, Berman E, Rosenthal J, Fatteh N, Wolfe PP, Clayton R, Murphy C, Pauze D, Mayer T,
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Chest Radiology
191
Pneumonia
Uncommon Malignant Tumors of the Lung

Uncommon Malignant Tumors of the Lung:
Bronchial Carcinoid - most common Uncommon

Adenoid Cystic Carcinoma ----> Salivary Gland Tumors
Mucoepidermoid Carcinoma----> Salivary Gland Tumors
Carcinosarcoma
----> Mixed Tumors
Pulmonary Blastoma
----> Mixed Tumors
Bronchial Adenoma: History Lesson
Term formerly referred to:

Bronchial Carcinoid
Adenoid Cystic Carcinoma
Mucoepidermoid Carcinoma
A misnomer
These tumors are not benign
Carcinoids
Gastrointestinal tract 90%

Lung
Thymus
Biliary tract
Ovarian teratomas
Bronchial Carcinoid
Typical carcinoid
Low-grade malignancy
Atypical carcinoid
Moderate-grade malignancy
Typical Carcinoid
0.6-2.4% of all pulmonary neoplasms

Low grade malignancy
Good prognosis
95% five-year survival
Not associated with smoking
Typical Carcinoid: Demographics
Males = Females
Wide age range. Median age: 50 years
Symptoms: cough, hemoptysis, dyspnea
Typical Carcinoid: Microscopy
Uniform cells
Forming nests, ribbons, rosettes, trabeculae
Stroma highly vascular
May exhibit calcification or osseous metaplasia
Polygonal cells, pale cytoplasm, stippled nuclear chromatin
Rare mitoses
Ultrastructure: Neurosecretory granules
Neuroendocrine Cells and Tumors: Electron microscopy
Cytoplasmic neurosecretory granules

Central or eccentric dense core
Thin lucent halo
May contain biologically active peptides
Uncommon Malignant Tumors
192
Chest Radiology
Neuroendocrine Markers: Immunohistochemistry
Chromogranin
Synaptophysin
Neural cell adhesion molecules (NCAM)
Carcinoid: Relationship to Small Cell Carcinoma
Similarities:
Neurosecretory granules
Rosette and trabecula formation
Differences:
Fewer granules in Small Cell Carcinoma
Carcinoid not associated with smoking
Tumors with NE Morphology
A spectrum by light micropscopy
Low-grade
Intermediate
High-grade
Typical Carcinoid
Atypical Carcinoid
Small cell lung carcinoma
Large cell neuroendocrine carcinoma
Neuroendocrine Tumors:
World Health Organization criteria (1999) [Figures1-20-1]
Typical carcinoid: <2 mitoses per 10 HPF

Atypical carcinoid: 2-10 mitoses per 10 HPF
Large cell neuroendocrine ca: 11 or more mitoses per 10 HPF (median 70)
Small cell ca: 11 or more mitoses per 10 HPF (median 80)
Figure 1-20-1 NE Tumors: Metastases
Atypical Carcinoid: Histopathologic criteria
Poor architectural organization

Cellular pleomorphism
Focal necrosis
Increased mitotic activity
Arrigoni J Thorac Cardiovasc Surg 1972
Atypical Carcinoid
Morphology between Typical Carcinoid and Small Cell Ca

Tend to be larger, more invasive, peripheral
Age: Decade older than Typical
Symptoms: similar to Typical
Imaging: similar to Typical
Chest Radiology
193
Atypical Carcinoid
10% of bronchial carcinoid

Peripheral
Increased mitoses
Aggressive behavior; early metastases
Osteoblastic bone metastases
Pathology DDx: Small Cell Carcinoma
Bronchial Carcinoid: Gross Pathology

[Figure 1-20-2]
Usually seen at bronchoscopy

Soft, fleshy, endobronchial mass
Sessile. May be pedunculated
Often extend through wall
Figure 1-20-2
Growth patterns of bronchial carcinoid tumors. Partially endobronchial tumors

(iceberg configuration) most common
Bronchial Carcinoid: Central
Bronchiectasis
Mucoid impaction
Obstructive pneumonia
Bronchial Carcinoid: Peripheral
Late presentation
Discovered incidentally
194
Chest Radiology
Bronchial Carcinoid: Radiologic Findings
Figure 1-20-3
[Figures 1-20-3 and 1-20-4]
Central tumors in 80%

Lobar, segmental, subsegmental bronchi
Consolidation, atelectasis
Pleural effusion
Figure 1-20-4
Small endobronchial carcinoid tumor in distal left mainstem bronchus
Bronchial Carcinoid: CT Features [Figures 1-20-5 to 1-20-7]
Bronchial carcinoid manifesting as a

central, well-defined left perihilar mass
Figure 1-20-5
Bronchial relationship in 83%

Partially endobronchial
Completely endobronchial
Abutting a bronchus
Sharply marginated, lobulated mass
May enhance or demonstrate Ca++
Atelectasis, consolidation, bronchiectasis, mucoid
impaction
Lymphadenopathy
Figure 1-20-6
Bronchial carcinoid. Partially endobronchial (iceberg)
tumor in left upper lobe
Figure 1-20-7
Bronchial carcinoid obstructing left upper lobe bronchus

with distal atelectasis, pneumonitis and associated left
hilar lymphadenopathy
Bronchial carcinoid in a 34-year-old male. Unenhanced

CT (left) demonstrates central tumor and distal,
peripheral consolidation. Contrast enhanced CT (right)
shows diffuse contrast enhancement
Chest Radiology
195
Bronchial Gland Tumors
Figure 1-20-8

Mucoepidermoid Carcinoma
Equivalent to salivary gland tumors of same name
Adenoid Cystic Carcinoma [Figure 1-20-8]
Synonym - Cylindroma
80% of bronchial gland tumors
20 to 35% of all tracheal tumors
Second most common tracheal malignancy (after
Squamous cell carcinoma)
Guarded prognosis
Common local recurrence
Occasional metastases to regional nodes
Rarely extrathoracic spread
Adenoidcystic carcinoma partially occluding the trachea

and extending into the adjacent soft tissues of the
mediastinum
Adenoid Cystic Carcinoma: Demographics
Males = Females
Wide age range - Average age: 40 - 50
Symptomatic patients: cough, wheezing, dyspnea, hemoptysis
Adenoid Cystic Carcinoma: Microscopy
Figure 1-20-9
Mucin-containing cysts
Varying in caliber
Within larger tumor tubules
Surrounds, invades nerves
Encases vessels, infiltrates bronchi
Few mitoses
Adenoid Cystic Carcinoma: Pathologists

Pitfalls
Misdiagnosis
Adenocaracinoma
Pleomorphic adenoma
Metastatic salivary gland tumor
Solid pattern on small biopsy
Adenoid Cystic Carcinoma: Gross
Endobronchial mass
Trachea and main bronchi
Sessile, polypoid, annular growth
Proximal & distal spread
Extension into mediastinum
Adenoid Cystic Carcinoma: Radiology [Figure

1-20-9]
Adenoidcystic carcinoma. Coronal (left) and saggital
Central: trachea and main bronchi

(right) surface-rendered CT images of the trachea
Intraluminal nodule or mass
demonstrate both nodular and circumferential deformity
Constriction of tracheal/bronchial lumen
of the air-column in the lower trachea
10 - 15% in lung periphery
CT / MRI: length of involvement; mediastinal involvement
Mucoepidermoid Carcinoma: Demographics and Prognosis
Male=Female. Wide age range

Cough, fever, hemoptysis, pneumonia, atelectasis
Low-grade: excellent prognosis
High-grade: better prognosis than Bronchogenic Ca
196
Chest Radiology
Mucoepidermoid Carcinoma [Figure 1-20-10]
Figure 1-20-10
Mucoepidermoid Carcinoma: Microscopy
Low grade:
Mucinous cysts
Solid collections of squamous cells
High grade:
Solid sheets of tumor
Mitoses and necrosis
Mucoepidermoid Carcinoma: Gross
Submucosal, smooth surfaced

Endobronchial, exophytic, polypoid
High grade may have ragged invasive appearance
Mucoepidermoid Carcinoma:
Radiologic Findings [Figures 1-20-11 and 1-20-12]
Mucoepidermoid carcinoma arising in the right

mainstem bronchus at the origin of the right upper lobe
bronchus
Solitary nodule or mass

Most in main or lobar bronchi
Few in trachea
Distal effects:
Atelectasis, pneumonia
Central lesions
Figure 1-20-11
Mixed Tumors: Neoplasms with malignant epithelial

and mesenchymal components
Carcinosarcoma
Pulmonary Blastoma
Carcinosarcoma
Rare - 0.3% of all lung neoplasms

Middle aged and elderly males
Poor prognosis
Aggressive: local invasion, widespread metastases, and rapid
death
Carcinosarcoma: Microscopy
Mucoepidermoid carcinoma manifesting as a

solitary pulmonary nodule on chest
radiography
Epithelial component:
Adenocarcinoma
Undifferentiated Carcinoma
Mesenchymal component:
Usually dominant
Spindle cell (common)
Chondrosarcoma
Osteosarcoma
Rhabdomyosarcoma
Figure 1-20-12
Carcinosarcoma: Gross
Peripheral
Large mass
Average diameter 6 cms.
Frequent necrosis and hemorrhage
Central
Endobronchial growth
May extend to adjacent parenchyma
Tumor-distended bronchi may resemble mucus plugs
Central mucoepidermoid carcinoma manifesting as mild
prominence of the suprerior aspect of the right hilum
with associated atelectasis of the right upper lobe
Chest Radiology
197
Carcinosarcoma: Imaging [Figure 1-20-13]
Figure 1-20-13
Peripheral
Large
Well-circumscribed mass
Central
Tumor mucus plugs
Direct extension to pleura, chest wall, and
mediastinum
Pulmonary Blastoma
Primary lung tumor

Mix of epithelial and mesenchymal components
Both components blastomatous and immature
Morphologic mimic of embryonal lung
? a variant of carcinosarcoma
Pulmonary Blastoma: Demographics and

Prognosis
Predominantly males
Biphasic age distribution: first and seventh decades
Symptoms: cough, hemoptysis, dyspnea, chest pain
Poor survival
Carcinosarcoma. Contrast enhanced CT demonstrates

a peripheral mass with irregular and lobulated borders in
the right upper lobe
Figure 1-20-14
Pulmonary Blastoma: Microscopy
Mixture:
Epithelial-lined tubules
Primitive stroma
Resembles embryonal lung
Metastases: mesenchymal, epithelial, or mixed
Pulmonary Blastoma: Gross
Large mass
Unencapsulated and soft
Abundant central necrosis and hemorrhage
Pulmonary Blastoma: Radiology [Figure 1-20-14]
Large peripheral mass

Well-circumscribed
May show pleural invasion
May metastasize
Pulmonary blastoma manifesting as a

large, heterogeneous mass in the left
lower lobe
Endobronchial Tumors: Malignant
Squamous cell ca
Adenocarcinoma
Small cell ca (rare)
Carcinoid
Adenoid cystic ca
Mucoepidermoid ca
Carcinosarcoma
Pulmonary blastoma
Sarcoma (10%)
Endobronchial metastasis
Lymphoid malignancies (NHL>HD)
198
Chest Radiology
Benign Tumors of the Lung and

Tumor-like Lesions
Benign Tumors and Tumor-like lesions
Hamartoma
Papilloma / Papillomatosis
Inflammatory pseudotumor
Granuloma
Hamartoma
Albrecht, 1904
Tumor-like malformation
Tissues normal to location
In excess or disarray (disorganized)
Adult, Classic, Local hamartoma
Hamartoma
Acquired lesion
Disorganized growth of tissues normally found in lung
Benign neoplastic proliferation
Probably derived from bronchial wall mesenchymal cell
(benign mesenchymoma)
Hamartoma
Most common benign tumor of lung

77% of benign lung tumors
8% of SPNs
3% of all lung tumors
Hamartoma: Evidence of Acquired Lesion
Onset in adult life

Often adults with previously normal CXR
Almost never seen in infants
Histology: passive entrapement of epithelium
Cytogenetics: Chromosome 12: abnormal q13-q15 regions
(as in other benign soft-tissue neoplasms)
Hamartoma: Demographics
Age range: 30-70 years

Peak incidence: 6th decade
Female: Male = 3:2 (1:1 for endobronchial hamartoma)
Asymptomatic in 90%
< 8% obstructive symptoms
Hamartoma: Clinical
Most are peripheral and asymptomatic

If symptomatic: hemoptysis
If bronchial obstruction: pneumonitis
Fever, cough, expectoration, chest pain
Hamartoma: Microscopic
Cartilage nests (lobules) in 95%

Surrounded by fibrous tissue
Mature fat cells
Cleft-like invaginations of entrapped respiratory epithelium
Chest Radiology
199
Benign Tumors
Hamartoma: Gross
Figure 1-21-1
Solitary
1 3 cm (rarely Giant)
Rounded, well-circumscribed, lobulated
Firm lesions. Usually cartilaginous
May see areas of fat
Easily shelled-out
Hamartoma: Distribution
Peripheral > Central

80 90 % Peripheral
No lobar predilection
Hamartoma: Radiographic
Sharply defined, lobulated subpleural

Most < 3 cm
Calcification on CXR (10-15% )
Rarely see fat on CXR
May enlarge on serial CXRs
Up to 3 to 5 mm per year
Hamartoma. Unenhanced chest CT demonstrates a

peripheral solitary nodule with focal fat attenuation
Figure 1-21-2
Hamartoma: Calcification
10 15% speckled or Popcorn

Popcorn less frequent than once thought
Diagnostic when present
Nodular growths within lesion
Protrude in different directions
Hamartoma: Computed Tomography
Distinguishes fat and cartilage

Most are 2.5 cm or less
Smooth edge
No fat / Focal fat alone / Fat with areas of
calcification
Cavitation: rare
Hamartoma. Unenhanced chest CT demonstrates a

central mass in the left lower lobe with a lobular area of
popcorn calcification
Hamartoma: Computed Tomography [Figures 1-21-1 to 1-21-3]
Thin sections (2mm)

Smoothly contoured nodule
= or < 2.5 cm diameter
Focal fat in 8 voxels or more
Or fat with calcification
Figure 1-21-3
Siegelman. Radiology 1986; 160:313-317.
Hamartoma: Computed Tomography
no fat or calcification
36%
4%
diffuse calcification
38%
areas of fat
21%
calcium and fat
Occasionally: focal calcification, no fat
Carneys Triad
Gastric smooth muscle tumors

Extra-adrenal paraganglioma
Pulmonary chondroma
Association unclear
Young females < 20 years
May have only 2/3 of the triad
Hamartoma. Unenhanced chest CT demonstrates

speckled calcification in a central endobronchial tumor
with associated loss of volume in the left lung
Carney JA. Cancer 1979
Benign Tumors
200
Chest Radiology
Chondroma
Rare
Benign cartilaginous tissue
Parenchymal or endobronchial
Lack epithelial-lined clefts seen in hamartomas
In young female
Search for Carneys Triad
Hamartoma: Endobronchial
Morphologically identical to parenchymal

Often polypoid. Sessile or thin pedicle
Manifest by airway obstruction
Micro: more fat, lack clefts, cartilage scant or absent
Hamartoma: Treatment and Prognosis
Benign
Surgical excision = Cure
Exceptional cases: additional hamartomas
Papillomas
Branching or coarsely lobulated tumor

Arise from and project above an epithelial surface
Rare pulmonary tumors
Solitary (rare) or Multiple (papillomatosis)
Proximal or peripheral
Solitary Papillomas
Rare
Usually in adults
Papillary exophytic growth
Trachea, main or lobar bronchi
Males >40 years of age
Post-obstructive pneumonia, bronchiectasis
Juvenile Laryngeal Papillomatosis
Children 18 months to 3 years of age

Majority remain localized, disappear spontaneously
May spread distally and obstruct airways
5% Spread remains limited to trachea
1% Develop lung disease - 10 years after laryngeal disease
(extension to bronchi, bronchioles, alveolar airspaces)
Laryngeal Papillomatosis: Demographics and Etiology
Human papilloma virus - HPV types 6 and 11

0.1% of infants develop LP. Predilection for first-born infants
50% of their mothers have genital tract involvement
HPV spread transvaginally at birth
Infects oropharyngeal secretions of child
Papillomas: Microscopic
Non-keratinizing squamous cells

Fibrovascular core
Form papillomatous projections
Papillomatosis: Gross
Cauliflower-like excresences
Protrude into bronchial lumens
May extend into parcenchyma as nodules or cavities
Chest Radiology
201
Benign Tumors
Laryngeal Papillomatosis
Majority remain localized

5% spread to trachea and distal airways
Tracheobronchial Papillomatosis
Many remain limited to trachea

1% Develop lung disease
Patients with lung disease may develop
Squamous cell carcinoma
Tracheobronchial Papillomatosis: Pathogenesis of lower

respiratory tract involvement:
Implantation of inhaled fragments from larynx?

Multifocal viral infection?
Trauma-induced by tracheostomy?
In children, papillomas in bronchi and lung associated with multiple papillomas
of trachea or larynx
Figure 1-21-4
Papillomatosis: Imaging
Multiple nodules
Cavities, 2-3 mm thick walls
Air-fluid levels
Papillomatosis: Imaging [Figures 1-21-4 and 1-21-5]
Multiple, well-defined nodules

Perihilar, posterior thorax
Grow to several centimeters
Cavitate, 2-3 mm thick walls
Air-fluid levels may develop
Cavities may represent:
Papillomatosis
Abscess (obstructive pneumonitis)
Papillomatosis: Treatment and Prognosis
Multiple recurrences
Multiple excisions
Tracheostomy
37.5% mortality if spread to lungs
Worse if malignant degeneration occurs
Papillomatosis. Chest CT demonstrates nodular and

cystic opacities that predominantly involve the dorsal
aspects of both lungs
Figure 1-21-5
Inflammatory Pseudotumor: Synonyms
Plasma cell granuloma

Histiocytoma
Fibroxanthoma, Xanthoma
Myofibroblastic tumor
Mast cell granuloma
Inflammatory Pseudotumor
Uncommon. Reactive or neoplastic process?

May begin as organizing pneumonia
May have aggressive features:
Vascular invasion
Vertebral destruction
Recurrence
Papillomatosis and squamous cell
carcinoma. Contrast-enhanced Chest CT
demonstrates central squamous cell
carcinoma in the left lower lobe with distal
pneumonitis
Benign Tumors
202
Chest Radiology
WHO 1999: histologic spectrum of

fibroblastic and myofibroblastic proliferations
With varying infiltrate of inflammatory cells
Usually solitary, tumefactive lesion
Destroys underlying lung architecture
Reactive or Neoplastic ?
Inflammatory Pseudotumor: Demographics
Males = Females
Wide age range: 1 to 77 years. Average: 29.5 years
60% <40 years
Children: peak 6-7 years
o
Most common 1 lung mass in children
74% asymptomatic
Many patients have history of respiratory infection
Inflammatory Pseudotumor: Microscopic
Variable.
A continuum from plasma cell granuloma to fibrohistiocytic
Mixture of collagen, fibroblasts, myofibroblasts, and chronic inflammatory cells
Inflammatory Pseudotumor: Gross
SPN or Mass
Well-defined. Firm. Lobulated
Lack a capsule
Cut-surface: whorled, heterogeneous
1-10cm, 4.4 cm mean
Solitary nodule or mass in 70%

Well-defined
May manifest as consolidation
May mimic neoplasm
Endobronchial lesions occur in 10%
Figure 1-21-6
Inflammatory Pseudotumor: Radiographic
Solitary, well-defined nodule or mass

Endobronchial lesions occur
May extend into mediastinum
Calcification, cavitation infrequent
May mimic malignant neoplasm
Usually no or slow growth. May regress
70%
10%
5%
6%
Inflammatory Pseudotumor: CT [Figure 1-21-6]
Solitary nodule or mass

Sharply circumscribed
Lobulated
Heterogeneous or homogeneous
Enhancement: / calcification variable, nonspecific
Calcification: variable
Endobronchial lesions occur
Inflammatory pseudotumor. Contrast-enhanced chest

CT demonstrates an irregular, heterogeneous mass in
the left upper lobe
Inflammatory Pseudotumor: Therapy and Prognosis
Diagnosed and treated by surgical excision

Excellent prognosis after resection
Recurrence in 5%
Especially if mediastinal or chest wall involvement
DDx: fibrous histiocytoma, sarcomatoid carcinoma
Chest Radiology
203
Benign Tumors
Granulomas
Infectious
Sarcoid (necrotizing granuolomatosis)
Infectious Granulomas
Mycobacterial
Fungal
Parasitic
64%
30%
6%
Granuloma: Infectious
Tuberculoma or Histoplasmoma
Satellite lesions common
Usually small, smooth
Often calcified when healed
Granuloma Well-defined Pulmonary Nodule

Multiple Ill-defined Pulmonary Nodules
TB
Histoplasmosis
Coccidioidomycosis
Cryptococcosis
Aspergillosis
Granulomas Tiny nodules
<5 mm, micronodular, military

Histoplasmosis
Blastomycosis
Cryptococcosis
Coccidioidomycosis
Solitary Pulmonary Nodule

(n = 955)
Malignant
Primary carcinoma
Metastases
Other 1 malignancy
Benign
Non-neoplastic lesion
Tumor
( Hamartoma 8% )
49%
38%
9%
2%
51%
37%
14%
Toomes H. The coin lesion of the lung. Cancer 1983.
Benign Tumors
204
Chest Radiology
Pleural Disease I
Pleural Disease I and II: Objectives
Anatomy and physiology

Non-neoplastic and neoplastic pleural disease
Chest wall disease
Radiologic-Pathologic correlation
Figure 1-22-1
Pleural Disease I
Normal anatomy
Standard fissures
Accessory fissures
Non-neoplastic pleural disease
Effusions
Fibrosis
Pneumothoraces
Standard (solid lines)
and accessory (dashed lines) fissures
Pleural Anatomy
Parietal Pleura
Covers non-pulmonary surfaces
Systemic supply/drainage
Lymphatics communicate with pleural space
Pain fibers
5-15 ml of pleural fluid
Visceral Pleura [Figure 1-22-1]
Covers lung surface
Dual supply/drainage
Vagus nerve/sympathetic trunks
Lymphatics do not communicate with pleural
space
Figure 1-22-2
Pleural Imaging
Radiography / CT
Inconspicuous
Visceral + Parietal = 0.2 mm
Thin-collimation
1-2 mm thick line
Intercostal regions
Normal fluid
Endothoracic fascia
Innermost intercostal m.
Pneumothorax in a supine patient manifesting as a

deep sulcus and hyperlucency overlying the left
hemidiaphragm
Pleural Anatomy [Figure 1-22-2]
Caudal limit of pleura lower than lung

Costal and diaphragmatic pleura contact to form costophrenic recess
Pleural Anatomy
Junction Lines
Apposition of layers of pleura
Anterior
Posterior
Pleural Anatomy - Fissures
Visceral pleura
Variable depth into parenchyma
Complete
Incomplete
Chest Radiology
205
Pleural Disease I
Incomplete Major Fissure: CT
More frequent Right

RUL / RLL
70%
RML / RLL
47%
LUL / LLL
40%
Lingula / LLL
46%
Major Fissure: Radiography
Major (oblique) fissures

Best seen on lateral CXR
Origin: T4 Left, T5 Right
Right fissure more oblique
Figure 1-22-3
Major Fissure: CT
80-90% of standard CT
Lucent band
Line
Dense band
Major Fissure: CT
Propeller-like morphology [Figure 1-22-3]
Upper thorax
Anterior concave
Lateral-facing
Inferior thorax
Anterior convex
Medial-facing
Chest CT (lung window) demonstrates right upper

lobe loss-of-volume manifested by displacement of
the right major fissure. An endobronchial carcinoid
obstructs the origin of the right upper lobe
bronchus
Standard Fissures: Radiography
Minor fissure
Lights up in CHF
Interstitial edema
(Subpleural interstitium)
Figure 1-22-4
Minor Fissure: CT
Lucent area
Devoid of vasculature
44% triangular
8% round / ovoid
Ground glass attenuation
Incomplete Minor Fissure: HRCT
Curvilinear line or band

Increased attenuation
C-shaped
Fusion between RUL / RML (60-90%)
Accessory Fissures: Radiography [Figure 1-22-4]
10% CXR / 20% CT (50% of anatomic specimens)

Azygos, Superior, Inferior, Left minor
Accessory Fissures: Azygos
Abnormal migration of posterior cardinal vein

Four layers of pleural
1% population
2M : 1F
Pleural Disease I
Diagram illustrates position of

inferior (I), superior (S) and azygos
(az) accessory fissures
206
Chest Radiology
Accessory Fissures: CT Azygos [Figure 1-22-5]

Accessory Fissures: Inferior Accessory [Figures 1-22-6 and 1-22-7]
Separates medial basal segment from remaining basilar segments

Most common
30 - 45% anatomic specimens
CXR: 5 - 10%
80% Right-sided
CT: 15%
Figure 1-22-5
Indirect Signs of Atelectasis:

Juxtaphrenic Peak
Common in RUL or LUL atelectasis

Less common in RML
Seen post upper lobectomy
Small triangular opacity
Projects upward from diaphragm
Related to inferior accessory fissure
Accessory Fissures: Superior Accessory
Separates superior segment from basilar segments

6% anatomic specimens
Right > Left
Horizontal course
Inferior to minor fissure
Chest CT demonstrates an azygos fissure forming

the lateral margin of an azygos lobe
Figure 1-22-6
Accessory Fissures: Left Minor Fissure
Separates lingula from remainder of upper lobe

8-18% anatomic specimens
1.5% of chest radiographs
Oblique course
More cephalad
Pulmonary Ligament
Formed by Parietal & Visceral pleura

Courses inferiorly & posteriorly
Contains bronchial veins, lymphatics, nodes
Figure 1-22-7
Coned-down view of right lung demonstrates

an inferior accessory fissure separating the
medial basal segment from the remaining
basal segments of the right lower lobe
Chest CT demonstrates an
inferior accessory fissure
(arrow).
Chest Radiology
207
Pleural Disease I
Pulmonary Ligament: Imaging [Figure 1-22-8]
Figure 1-22-8
CXR: not visualized

CT: 60%-70%
Pleural Effusion
Cardiac Decompensation [Figure 1-22-9]
Most common cause

Increased hydrostatic pressure
Bilateral >80%
Unilateral = right-sided
Pseudotumor
Figure 1-22-9
Lateral chest
radiograph
demonstrates
lenticular opacity of
fluid accumulation in
the minor fissure
(pseudotumor)
Chest CT demonstrates inferior accessory fissure

(arrowhead) and right and left pulmonary
ligaments (arrows)
Figure 1-22-10
Pleural Effusion: Bacterial Pneumonia
Parapneumonic effusions 40%

Exudate
10% require drainage
Complications
Loculation
Empyema
Ultrasound demonstrates multiple septations

within a loculated fluid collection representing
empyema
Pleural Effusion: Empyema [Figure 1-22-10]
Figure 1-22-11
Three phases
Exudative
Fibrinopurulent
Organizing
Pleural Effusion: Empyema [Figure 1-22-11]
Lenticular shape
Obtuse margins
Compress lung
Split pleura sign
Pleural Effusion:
Lung abscess or Empyema ?
Pleural Effusion: Empyema
Lenticular shape
Obtuse margins
Compress lung
Split pleura sign
Disparity in length of air-fluid level
Pleural Disease I
Contrast
enhanced chest
CT demonstrates
smoothly
thickened
parietal and
visceral pleura
enclosing a fluid
collection of
empyema (split
pleura sign)
208
Chest Radiology
Pleural Effusion: Lung abscess
Round shape
Does not compress lung
Equal length of air-fluid level
Pleural Effusion: Empyema
Treatment
Tube thoracostomy
Fibrinolytics
Decortication
Pleural Effusion: Empyema necessitatis
Inadequate treatment
Drainage into chest wall
Tuberculosis 73%
Bacterial / Fungal
Malignancy
Immunocompromised patients
Pleural Effusion: Tuberculosis
Exudate
lymphocyte count
glucose level
Unilateral
Small to moderate
Pleural Effusion: Subpulmonic
Fluid accumulates between lung base and diaphragm

Shifts apex of diaphragm laterally
Usually transudate
Cardiac decompensation
Renal failure
Cirrhosis with ascites
Pleural Effusion: Subpulmonic
Imaging
Apparent elevation diaphragm
Ill-defined costophrenic angle
Diaphragmatic spur
Mobile fluid
Displace gastric bubble
Rock of Gibraltar on lateral
Pleural Effusion and Ascites
CT Features
Effusion = outside of hemidiaphragm
Ascites = inside of hemidiaphragm
Pleural Effusion: Connective Tissue Disease
Most common thoracic manifestation
Middle aged males
Antedates clinical disease
Exudate / chyliform / low glucose
Imaging
Unilateral
Chronic
Transient / relapse
Fibrothorax / decortication
Chest Radiology
209
Pleural Disease I
Pleural Effusion: Asbestos Exposure
Occupational exposure
No malignancy within 3 yrs
10 yrs post-exposure
Exudate
1/3 patients have chest pain
Recurrent 15 - 30%
Small (<500 ml)
Pleural Effusion - Asbestos Exposure
Associated with diffuse pleural thickening

Involves C-P angle
Implicated in formation of Rounded Atelectasis
Round Atelectasis: CXR
Peripheral mass
Abuts thickened pleura
3.5 to 7 cm
Posterior lower lobe most common
Other lobe, diaphragms
Bronchovascular bundles converge, forming comet
tail
Figure 1-22-12
Round Atelectasis: CT
Rounded subpleural mass

Broadly abuts contiguous pleural thickening
Air-bronchogram hilar aspect
Bronchovascular comet tail
Loss of volume in same lobe
Round Atelectasis: Required CT Findings

[Figure 1-22-12]
Chest CT (mediastinal and lung windows) demonstrates

the CT findings of round atelectasis
Subpleural mass
Thickened pleura
Loss of volume
Comet tail
Round Atelectasis: Pathogenesis
Asbestos exposure
Pleural effusion
Atelectasis
Pleural adherence
Effusion subsides
Lung re-expands
Pleural fibrosis
Contraction
Pleural Fibrosis
2nd most common pleural abnormality

Result of many primary diseases of the pleura
Complication of inflammatory disease
Most localized to single area
Less often diffuse
May have functional abnormalities
Pleural Fibrosis: Focal
Healed Pleuritis
Bacterial pleuritis/trauma
Imaging
Blunt posteriolateral CP sulci
Rule-out small effusion
Pleural Disease I
210
Chest Radiology
Pleural Fibrosis: Focal [Figure 1-22-13]
Figure 1-22-13
Pleural Plaques
Serpentine (chrysotile) asbestos
Dense hyalinized collagen
Parietal pleural surface
Asbestos exposure
Asymptomatic
Pleural Fibrosis: Focal
Pleural Plaques
50% of exposed individuals
Visible plaques
15 years non-calcified
20 years calcified
Pleural Fibrosis: Imaging
Bilateral (80%)
Lateral chest wall
4th to 8th ribs
Tendinous diaphragm
Spares apices and CPAs
En face Holly leaf
Chest CT (lung window) demonstrates multiple

bilateral pleural plaques
Figure 1-22-14
Pleural Fibrosis: Diffuse Fibrothorax Figure 1-22-14]
Fibrous obliteration of normal pleural space

Tuberculosis/bacterial empyema
Hemothorax
Asbestos-related pleural effusions
Rheumatoid effusions
Volume loss/restrictive disease
Pleural Fibrosis: Diffuse Fibrothorax
Radiographic definition
Smooth/uninterrupted
25% or more of chest wall
May obliterate c-p suclus
2.0 cm thickness
+/- calcification
Pleural Fibrosis: Diffuse Fibrothorax
Pleural fibrosis. Chest radiograph demonstrates

pleural thickening and calcification in the right
hemithorax
Imaging CT
Extends > 8.0 cm cranio-caudal
Pleura > 3 mm thick
Extrapleural fat hypertrophy
+/- Pleural calcification
Mediastinal pleura spared
Figure 1-22-15
Pneumothorax
Air within the pleural space

Spontaneous
Primary
Secondary
Traumatic
Pneumothorax: Primary Spontaneous [Figure 1-22-15]
M:F=5:1
3rd - 4th decade
Right-side predominance
30% ipsilateral recurrence
10% contralateral recurrence
Rupture of apical bleb/bulla
Chest Radiology
Chest CT demonstrates left pneumothorax

and a bleb along the visceral pleural
surface of the collapsed lung
211
Pleural Disease I
Pneumothorax: Secondary Spontaneous
COPD
Most common concurrent condition
0.5% per year
45-65 years of age
Peripheral emphysematous lung
Mortality rate ~3%
Pneumocystis Jiroveci Pneumonia (PCP)

Destruction of alveolar septa bulla
Subpleural necrosis cystic degeneration / bulla
Pneumocystis Jiroveci Pneumonia (PCP)

Complicates in 12%
Refractory air-leaks
Poor prognosis
Death in 8 weeks (<57%)
Lymphangioleiomyomatosis (LAM)
Women child-bearing age
Proliferation of immature smooth muscle
Bronchiolar obstruction
Cysts PTX
Recurrence ~40%

Smokers
Cysts rupture
Recurrent ptx (25%)
Pleural Disease I
212
Chest Radiology
Pleural Disease II and Chest Wall

Malignant Pleural Effusion
Most common manifestation of metastatic involvement

Exudative effusion
Lung Ca
36%
Breast Ca
25%
Lymphoma
10%
Ovarian
5%
Gastric Ca
2%
TNM Staging of Lung Cancer
T1
T2
T3
T4
Malignant Effusion = T4
N0
N1
N2
IA
IIA
IIIA
IB
IIB
IIIA
IIB
IIIA
IIIA
IIIB
IIIB
IIIB
N3
IIIB
IIIB
IIIB
IIIB
MI=IV
Malignant Pleural Effusion:

Diagnosis and Prognosis
Combined pleural cytology and pleural biopsy

Multiple thoracenteses / pleural biopsies
Poor prognosis
Lung Carcinoma - mean survival 2 to 3 months
Breast Carcinoma - mean survival 7 to 15 months
Pleural / Chest Wall Mass
Discrepant margins on orthogonal views

Elliptical shapes
Obtuse angles
Cross boundaries
Pleural Neoplasms
Primary
Localized fibrous tumor

Malignant mesothelioma
Secondary
Pleural metastases
Bronchogenic carcinoma
Other carcinomas
Lymphoma
Invasive thymoma
Localized Fibrous Tumor
Rare (< 5% of pleural neoplasms)

Not related to asbestos
M=F
Mean age: 50 years
Symptoms in 54%
Cough, chest pain, dyspnea
HPO 0 - 35%
Hypoglycemia 4%
Chest Radiology
213
Pleural Disease II
Localized Fibrous Tumor: Microscopic
Figure 1-23-1
Variable patterns
Disorderly arrangement
Spindle cells and collagen
High mitotic activity suggests malignancy 20%
Localized Fibrous Tumor: Gross
2 - 40 cm
80% visceral / 20% parietal
Lobular, encapsulated
Pedicle: good prognosis
Cut-surface: whorled, nodular, fibrous hemorrhage, necrosis,
cysts
Localized Fibrous Tumor: CXR [Figures 1-23-1 to 1-23-3]
Incidental finding
Solitary rounded, lobular mass
Mid to inferior thorax
Obtuse or acute angles at interfaces
Pedunculated tumors
Positional mobility
Pathognomonic
Localized fibrous tumor. PA chest radiograph

demonstrates a pleural mass a peripheral
mass in the right lower hemithorax with
incomplete borders
Figure 1-23-2
Figure 1-23-3
Localized fibrous tumor. Chest CT

(lung windows) demonstrates a
pleural mass that forms obtuse
angles at its interface with the chest
wall
Localized fibrous tumor. PA chest radiograph

demonstrates a large mass that opacifies most of
the right hemithorax and produces mass effect
with contralateral shift of the mediastinum
Localized Fibrous Tumor: CT [Figure 1-23-4]
Figure 1-23-4
Well-defined, smooth, lobular

Abutting pleural surface
Elongated, lenticular
Heterogeneity:
hemorrhage, necrosis, cysts
Localized fibrous tumor.

Contrast enhanced
chest CT demonstrates
a large heterogeneous
mass in the right lower
hemithorax with
lobulated contours
Pleural Disease II
214
Chest Radiology
Localized Fibrous Tumor

Treatment of choice: complete excision

90% cure rate
Symptoms usually resolve post-op
Recur with tumor recurrence
Recurrence in 10% of patients
Malignant Mesothelioma
Most common primary pleural neoplasm

2,000 to 3,000 cases / year in USA
10% of exposed individuals
Shipyards / asbestos plants
Sixth to eighth decades
Male : Female 3-6 : 1
Amphiboles most tumorigenic
Latency: 30-40 years
Malignant Mesothelioma: Clinical
Insidious onset of symptoms

6-8 months prior to Dx
Dyspnea, chest pain, cough, weight loss
Rarely: SVC Syndrome, Horner Syndrome, dysphagia,
vocal cord paralysis, HPO, clubbing, hypoglycemia
Figure 1-23-5
Malignant Mesothelioma - Microscopic
Epithelioid
Sarcomatous
Biphasic
Interobserver agreement
50 %
15 %
25 %
50%
Malignant Mesothelioma: Gross
Sheets, plaques, masses

Parietal > Visceral
Bulk in inferior hemithorax
Lung encasement
Fissural growth
Parenchymal involvement
Mediastinal, chest wall, diaphragmatic invasion
Malignant mesothelioma. PA chest

radiograph demonstrates circumferential,
nodular and contiguous pleural masses
throughout the left hemithorax
Malignant Mesothelioma: Radiographic
Figure 1-23-6
[Figure 1-23-5]
Pleural effusion
Pleural masses
Circumferential
Mediastinal shift
Pleural plaques 25%
Malignant Mesothelioma: Radiographic
Malignant mesothelioma cannot be reliably differentiated

from pleural metastases
Malignant Mesothelioma: CT [Figure 1-23-6]
Pleural thickening
Fissural thickening
Pleural effusion
Ipsilateral volume
Pleural calcification
Ipsilateral volume
92%
86%
74%
42%
20%
14%
Chest CT demonstrates circumferential,

nodular pleural thickening in the left
hemithorax that extends into the major
interlobar fissure. Calcified pleural plaques
are demonstrated bilaterally
Kawashima AJR 1990
Chest Radiology
215
Pleural Disease II
Malignant Mesothelioma: DX
Video-Assisted-Thoracoscopic-Surgery: Sensitivity 98%

Complication: tumor seeding along entry ports
Open biopsy: when adhesions preclude VATS
Cytology and FNA Bx of limited value
Malignant Mesothelioma: MR
Staging
Comparable / superior to CT
Tumor enhancement
Increased signal intensity
Malignant Mesothelioma: Treatment and Prognosis
Median survival: 10 months

Best prognosis:
25-30% 5-year survival
Negative margins
Epithelial cell type
No metastases
Extrapleural pneumonectomy
High mortality / morbidity
Pleural Metastases
Most common pleural neoplasm
Common
Adenocarcinoma
Lung, breast, ovary, stomach
Less common:
Lymphoma, Thymoma
Imaging
Pleural effusion
Pleural masses
Or both
Figure 1-23-7
Pleural Metastases
Hematogenous / Lymphatic
Direct extension
Lung ca, breast ca
Drop metastases
Invasive thymoma
May be bilateral
Pleural Thickening
Malignant Pleural Thickening [Figure 1-23-7]
Circumferential
Nodular
> 1 cm
Mediastinal pleura
Pleural metastases. Chest CT demonstrates

adenocarcinoma arising from a right upper lobe
bronchus and pleural metastases that are nodular,
circumferential, and involve the mediastinal pleura
Leung et al AJR 1990
Chest Wall
Congenital and developmental anomalies

Inflammatory and infectious diseases
Non-neoplastic conditions
Neoplasia: benign and malignant
Pleural Disease II
216
Chest Radiology
Chest Wall: Congenital / Developmental Anomalies

[Figures 1-23-8 and 1-23-9]
Figure 1-23-8
Pectus deformities
Anomalous ribs
Cleidocranial dysostosis
Poland syndrome
Chest Wall: Inflammatory / Infectious Diseases
Hematogenous
Direct extension
Pyogenic infection: S. aureus, P. aeruginosa
Imaging
Osseous destruction if advanced
CT / MR for better delineation
CT for biopsy and/or drainage
Chest Wall:Tuberculosis [Figure 1-23-10]
Uncommon
Hematogenous spread
Contiguous spread
Abscess / sinus tract 25%
Imaging
Bone / cartilage destruction
Soft-tissue mass
Calcification
Peripheral enhancement
Lateral chest radiograph

demonstrates pectus carinatum
Figure 1-23-9
Chest Wall: Inflammatory / Infectious

Diseases
Chest Wall: Actinomycosis [Figure 1-23-11]
Actinomyces israelii
Anaerobic gram-positive
Lung Pleura Chest Wall
Proteolysis Fistulas
Diagnosis: anaerobic cultures
Imaging: soft-tissue mass draining sinus, periostitis
Figure 1-23-11
Poland syndrome. Chest CT demonstrates

congenital absence of the right pectoralis muscles
Figure 1-23-10
Tuberculosis. Contrast enhanced chest CT

demonstrates an anterior chest wall
abscess with subtle peripheral
enhancement and right pleural effusion
and pleural thickening
Actinomycosis. Contrast enhanced chest CT

demonstrates peripheral consolidation in the left
upper lobe with contiguous soft-tissue density
extending into the adjacent mediastinum and
anterior chest wall
Chest Radiology
217
Pleural Disease II
Chest Wall: Neoplasms
Figure 1-23-12
Adults
Benign:
Lipoma
Other mesenchymal neoplasms
Malignant:
Fibrosarcoma
Malignant fibrous histiocytoma
Other mesenchymal neoplasms
Lymphoma
Chest Wall Neoplasms: Lipoma
Common
Subcutaneous
Intrathoracic
Both
Diagnostic CT number
Fibrosarcoma. Chest CT demonstrates postsurgical changes of right mastectomy and a softtissue mass in the left chest wall. The patient
received radiation therapy through ports that
included the left posterolateral chest wall.
Chest Wall Neoplasms:

Desmoid (Fibromatosis)
Aggressive fibromatosis
Most: second to fourth decades
Shoulder, chest wall
Soft tissue mass
Frequent recurrence
Chest Wall Neoplasms: Soft Tissue Involvement [Figure 1-23-12]
Adults
Malignant:
Fibrosarcoma
Chest Wall Neoplasms: Osseous Involvement [Figure 1-23-13]
Rib expansion
Fibrous Dysplasia
Enchondroma
Pressure erosion
Neurogenic (slow growth)
Rib destruction:
Metastatic or Primary
Inflammatory
Figure 1-23-13
Chest Wall Neoplasms: Osseous Destruction

Adult
Metastatic disease (Lung, Breast, Prostate, etc.)

Multiple myeloma
Chondrosarcoma
Child
Ewing sarcoma
Neuroblastoma
Lymphoma
Askin tumor (PPNET)
Chest Wall Neoplasms: Myeloma
Males > Females

5th - 7th decades
Axial skeleton
Multiple or solitary
Imaging:
Osseous destruction
Soft-tissue mass
Pleural Disease II
Fibrous dysplasia. Chest CT

demonstrates an expansile lesion
with intact cortical margins nvolving
a right rib
218
Chest Radiology
Chest Wall Neoplasms: Chondrosarcoma
Adults
Painful, palpable mass
Costochondral junction, rib, sternum
Imaging:
Expansile, destructive
Chondroid calcification
Soft-tissue mass
PNET Askin Tumor: Primitive Neuroectodermal Tumor
Malignant small round cell tumor

Children, adolescents
Female: Male = 3:1
Unilateral
Rib destruction 2/3
Pleural effusion
Poor prognosis
Chest Radiology
219
Pleural Disease II
Classic Breast Lesions

Figure 1-24-1
Most Lesions are Non-specific [Figure 1-24-1]
Differentials can be given

High likelihood diagnoses can be made
Is this a cyst or a solid mass?
Normal Variants
Pectoralis major
Lymph nodes
Pectoralis Major [Figure 1-24-2]

Figure 1-24-2
CC view showing a
rounded density medially,
the medial end of the
pectoralis major muscle
Non specific lobulated mass which could be a

cyst, benign solid mass or a carcinoma
Figure 1-24-3
Sternalis [Figure 1-24-3]
Sharply marginated medial density,

smaller than the pectoralis major,
is the sternalis muscle
Intramammary Lymph Nodes
Normal finding
28% of breasts
May enlarge and shrink in size
Circumscribed with hilar notch or fatty hilum
Usually less than 15 mm in size
Not related to the usual lymphatic drainage patterns
Usually upper outer quadrant
Figure 1-24-4
Intramammary Lymph Nodes

Figures 1-24-4 to 1-24-6]
Left: Medial lymph node Right: Typical lymph

node with fatty hilum
220
Chest Radiology
Figure 1-24-5
Figure 1-24-6
Left: Lymph node with hilar notch

Right: Core biopsy specimen of normal lymph
node
Six examples of benign lymph nodes.

The top three are with traditional real time scanning
and the bottom three with compound scanning
Congenital Anomalies
Polythelia
Accessory nipples
2.4% of neonates
Polymastia
2-3% of women
Axillary breast tissue most common
Inframammary fold and labia next
most common
Figure 1-24-7
Figure 1-24-8
Polythelia [Figures 1-24-7 and 1-24-8]

Polymastia [Figures 1-24-9 and 1-24-10]
Can be palpable or visible
Benign Abnormalities
Accessory nipple seen

medially
Fatty lesions
Gynecomastia
Fibrocystic changes
Foreign bodies
Fatty Lesions
Hamartoma
Lipoma
Fat necrosis
Galactocele
Accessory nipple in a patient at

the inframammary crease
Figure 1-24-9
All Lesions that Contain Fat are Benign Except
Very rare hamartomas

1 Phyllodes with liposarcoma
Figure 1-24-10
Palpable axillary
accessory breast
tissue
Chest Radiology
Visible bilateral axillary accessory

breast tissue
221
Hamartoma
[Figures 1-24-11 to 1-24-14]
Fibroadenolipoma
Palpable mass or mammographic finding
Can be large and not palpable
Encapsulated normal breast elements
Figure 1-24-14
Figure 1-24-11
Figure 1-24-12
Figure 1-24-13
Large hamartoma
containing fat, glandular
tissue and fibrous tissue
Small hamartoma
Hamartoma showing
mixed echogenicity
Lipoma
Hamartoma presenting
as an intermediate
density mass without
visible fat
Figure 1-24-15
[Figure 1-24-15]
Benign tumor
Usually not palpable because it is soft
Liposarcoma usually water density
Liposarcoma
Figure 1-24-16
[Figure 1-24-16]
Lipoma in the axillary

region
Left: Liposarcoma is water density, not fatty

Right: Water density liposarcoma
Figure 1-24-17
Figure 1-24-18
Figure 1-24-19
Fat Necrosis
50% have history of trauma

Including surgery & XRT
Oil cyst
Partially calcified lesion
Can be spiculated
Can progress from fatty to spiculated
Oil Cyst
[Figures 1-24-17 to 1-24-19]
Oil cyst
222
Oil cyst with scarring

and dystrophic
calcification
Heavily calcified oil

cyst
Chest Radiology
Fat Necrosis - Progression [Figure 1-24-20]
Figure 1-24-21
Figure 1-24-20
Left: Oil cyst with scarring

Right: Several years later the scarring
predominates and
the fatty part of the lesion disappears
Galactocele [Figure 1-24-21]
Pregnant or breast feeding

Cystic lesion
Fat fluid level on horizontal beam film
Aspiration usually curative
Two galactoceles with layering of

milk
(fat rising and calcium dropping)
Figure 1-24-22
Fibrocystic Changes [Figures 1-24-22 and 1-24-23]
Figure 1-24-23
Exaggerated physiologic phenomenon

Cysts
Apocrine metaplasia and hyperplasia
Stromal alterations
Mild epithelial hyperplasia
Mild adenosis
Apocrine Metaplasia [Figure 1-24-24]

Cyst [Figure 1-24-25]
Cystic lobular involution

Anechoic with sharp back wall
Enhanced thru-transmission of sound
10% atypical
Diagnosable on ultrasound or aspiration
Figure 1-24-24
Specimen showing
multiple small cysts with
a characteristic blue color
(blue domed cysts)
Multiple small
hypoechoic cysts and
textural irregularity of
fibrocystic change
Figure 1-24-25
Figure 1-24-26
Microcystic nodule of
apocrine metaplasia
Pneumocystography [Figure 1-24-26]
Anechoic mass with sharp

borders and enhanced
transmission of sound, a
classic cyst
Pneumocystogram of a
benign cyst
Chest Radiology
223
Foreign Body
Figure 1-24-27
Silicone or paraffin
Free injection
Leakage from implants
Surgical drain
Wire fragments
Figure 1-24-28
Free Silicone (Implant Rupture)

[Figure 1-24-27]
Free Silicone or Paraffin [Figure 1-24-28]

Free Silicone [Figure 1-24-29]
Silicone in the tissue

from implant rupture
Figure 1-24-29
Figure 1-24-30
Left: Multiple high density globules of injected

silicone for augmentation
Right: Low power view of holes in the tissue from
silicone
Penrose drain remaining

after biopsy
Diffuse fibrosis and

calcification from
injected silicone or
paraffin for augmentation
Figure 1-24-31
Wire fragment left

behind after breast
biopsy
Surgical Drain [Figure 1-24-30]

Wire Fragment [Figure 1-24-31]
Thickened Skin Pattern
Figure 1-24-32
Edema
Mastitis
Inflammatory carcinoma
Post-radiation change
Obstruction to lymphatic drainage in the axilla or
superior mediastinum
Lymphoma
Thickened Skin Pattern Mastitis

[Figure 1-24-32]
Prominent thickened skin pattern as compared to

the normal side
224
Chest Radiology
Thickened Skin Pattern Radiation Therapy

[Figure 1-24-33]
Figure 1-24-33
Inflammatory Carcinoma [Figure 1-24-34]
Clinical findings
Heavy firm breast
Red skin
Warm skin
Peau dorange
Can not differentiate from acute mastitis
Far advanced local disease
Usually poorly differentiated ductal carcinoma
Radiographic findings
Obstruction of dermal lymphatics
Can diagnose with a skin biopsy
Diffuse lymphatic invasion within the breast
Increased density
Trabecular thickening
50% five year survival
Pre-op chemo, mastectomy and radiation
Mammographic Findings
Skin thickening
Diffuse increased density
Adenopathy
Signs of carcinoma
Mass, calcification, asymmetry, distortion
Extensive edema and

increased density after
radiation therapy
Figure 1-24-34
Axillary nodes
Supraclavicular node
Figure 1-24-35
Far advanced inflammatory breast

cancer with skin necrosis
Left: Enlarged abnormal axillary nodes in

inflammatory breast cancer
Right: Abnormal supraclavicular node
Figure 1-24-36

Classically Benign Calcifications
Lobular
Sutural
Fibroadenoma
Skin
Vascular
Secretory
Lucent centered
Egg shell
Chest Radiology
Left: Extremely dense breast in inflammatory

breast cancer. Right: Inflammatory breast cancer
with enlargement, and extensive skin thickening
225
Lobular Calcifications [Figure 1-24-37]
Figure 1-24-37
Tightly clustered
Round
Fit together like a jigsaw puzzle
Sutural Calcifications [Figure 1-24-38]
Look like sutures

Usually post radiation therapy
Figure 1-24-38
Calcified Fibroadenoma
Coarse or popcorn-like
Calcification generally peripheral
Tight cluster of smooth

calcifications, a benign
lobular cluster
Peripheral Calcification
[Figure 1-24-39]
Figure 1-24-39
Dystrophic calcification with

calcified suture
Figure 1-24-40
Surface calcification in a
fibroadenoma
Calcified Fibroadenoma
[Figures 1-24-40 and 1-24-41]
Skin Calcifications
Faint peripheral clusters with lucent centers

Tangent view
Left: Dense calcification (popcorn like) in a

fibroadenoma
Right: Sclerotic fibroadenoma with calcification
Dermal Localization [Figure 1-24-42]
Figure 1-24-41
Figure 1-24-42
Left: Marker over cluster for dermal localization

Right: Tangential film showing cluster in skin
Extremely dense calcification in a

fibroadenoma
226
Chest Radiology
Vascular Calcifications
Figure 1-24-43
Parallel tracks associated with blood vessels

Calcifications are on the outside of the tube
Diabetes ?
Heart Disease ?
Vascular / Ductal [Figure 1-24-43]

Secretory Calcifications
Large rods
Luminal calcifications
Oriented toward nipple
Relatively smooth surface
May branch
Loa Loa [Figure 1-24-44
Also called eye worm

Found in rain forest and swamps of West
Africa, especially Cameroon
Transmitted by day biting Chrysops flies
Loa loa filarial nematode
Larvae develop over 1 year
Mature worms up to 3-6 cm x 0.5 cm
Left: Calcification in artery

Right: Calcification in duct
Figure 1-24-44
Figure 1-24-45
Osteosarcoma
Primary in the breast

27 to 89 years old
Median 64.5 years
Highly aggressive tumors
Calcified loa loa worm
Primary Osteosarcoma [Figure 1-24-45]

National Flower of the Radiologist is the Hedge
Mass with dense osteoid

matrix
Some Diagnoses Can be Made
Make them when you can
References
1.
2.
Adler DD, Jeffries DO, Helvie MA. Sonographic features of breast hamartomas. J Ultrasound Med 1990; 9:85-90.
Ahern V, Brennan M, Ung O, Kefford R. Locally advanced and inflammatory breast cancer. Aust Fam Physician
2005; 34:1027-1032.
3. Bassett LW, Gold RH, Cove HC. Mammographic spectrum of traumatic fat necrosis: the fallibility of
"pathognomonic" signs of carcinoma. AJR Am J Roentgenol 1978; 130:119-122.
4. Berg WA, Campassi CI, Ioffe OB. Cystic lesions of the breast: sonographic-pathologic correlation. Radiology
2003; 227:183-191.
5. Bosch X. Unique features of inflammatory breast carcinoma. Lancet Oncol 2005; 6:549.
6. Dershaw DD, Moore MP, Liberman L, Deutch BM. Inflammatory breast carcinoma: mammographic findings.
Radiology 1994; 190:831-834.
7. Ellis DL, Teitelbaum SL. Inflammatory carcinoma of the breast. A pathologic definition. Cancer 1974; 33:10451047.
8. Erguvan-Dogan B, Yang WT. Direct injection of paraffin into the breast: mammographic, sonographic, and MRI
features of early complications. AJR Am J Roentgenol 2006; 186:888-894.
9. Ganott MA, Harris KM, Ilkhanipour ZS, Costa-Greco MA. Augmentation mammoplasty: normal and abnormal
findings with mammography and US. Radiographics 1992; 12:281-295.
10. Hance KW, Anderson WF, Devesa SS, Young HA, Levine PH. Trends in inflammatory breast carcinoma incidence
and survival: the surveillance, epidemiology, and end results program at the National Cancer Institute. J Natl
Cancer Inst 2005; 97:966-975.
11. Helvie MA, Adler DD, Rebner M, Oberman HA. Breast hamartomas: variable mammographic appearance.
Radiology 1989; 170:417-421.
Chest Radiology
227
12. Hogge JP, Robinson RE, Magnant CM, Zuurbier RA. The mammographic spectrum of fat necrosis of the breast.
Radiographics 1995; 15:1347-1356.
13. Kushwaha AC, O'Toole M, Sneige N, Stelling CB, Dryden MJ. Mammographic-pathologic correlation of apocrine
metaplasia diagnosed using vacuum-assisted stereotactic core-needle biopsy: our 4-year experience. AJR Am J
Roentgenol 2003; 180:795-798.
14. Levitan LH, Witten DM, Harrison EG, Jr. Calcification In Breast Disease Mammographic-Pathologic Correlation.
Am J Roentgenol Radium Ther Nucl Med 1964; 92:29-39.
15. Mester J, Simmons RM, Vazquez MF, Rosenblatt R. In situ and infiltrating ductal carcinoma arising in a breast
hamartoma. AJR Am J Roentgenol 2000; 175:64-66.
16. Sheppard DG, Whitman GJ, Huynh PT, Sahin AA, Fornage BD, Stelling CB. Tubular carcinoma of the breast:
mammographic and sonographic features. AJR Am J Roentgenol 2000; 174:253-257.
17. Soo MS, Kornguth PJ, Hertzberg BS. Fat necrosis in the breast: sonographic features. Radiology 1998; 206:261269.
18. Stacey-Clear A, McCarthy KA, Hall DA, et al. Calcified suture material in the breast after radiation therapy.
Radiology 1992; 183:207-208.
19. Svane G, Franzen S. Radiologic appearance of nonpalpable intramammary lymph nodes. Acta Radiol 1993;
34:577-580.
228
Chest Radiology
Basic Breast Imaging

There are Two Diseases
Cancer and no cancer
Figure 1-25-1
Cancer has Two Predominant Signs
Mass and calcification
You Have the Opposite Side for Comparison
Anatomy is simple
Physiology is almost irrelevant
What You Need to Remember
90% of cancers present as calcification, mass or both

10% present as
Focal asymmetry, developing or neodensities
Dilated duct
Thickened skin pattern
Architectural distortion
Pagets disease
(left) Ductogram showing normal ducts;

(right) : Diffuse tubular parenchymal pattern
caused by periductal fibrosis not ductal
dilatation
Rad Path Correlation: What You Need to Remember
The mass edge interface with the surrounding tissue reflects the
aggressiveness of the underlying lesion
Benign masses tend to be less aggressive than malignant masses
The shape of the calcification represents a cast of an underling anatomic or
pathologic space
Benign processes often cause smooth spaces
Necrosis (benign or malignant) causes irregular spaces
Normal Anatomy
Figure 1-25-2
Skin
Nipple and areola
Subcutaneous fat
Premammary fascia
Normal Anatomy
Glandular cone
Breast disease occurs here
Retromammary fascia
Retromammary fat
Muscle
Ribs
(left) Normal ductal distribution from nipple to

lobules;
(right) : Normal duct with single cell epithelial
layer and clear lumen
Segmental Anatomy
15 - 20 lobes or segments
Figure 1-25-3
Normal Ducts [Figures 1-25-1 and 1-25-2]

Terminal Duct Lobular Unit (TDLU) [Figure 1-25-3]
Short segment of terminal duct and a cluster of

ductules (acini)
Functional unit of the breast
Ductal and lobular cancers begin here
Explains mixed ductal & lobular features in the same
neoplastic lesion
Chest Radiology
229
(left) Drawing of TDLU

(right) Microscopic view of TDLU
Embryology
Milk ridges
Ventral ectodermal thickenings from the axillary to the inguinal region
Usually limited to the pectoral regions by the ninth week of embryonic life
Athelia
Rarest anomaly of the breast
Absence of the nipple
Amastia
Agenesis of breast & nipple
Associated with hypoplasia of the ipsilateral pectoral muscles in 90%
Can be iatrogenic
Figure 1-25-4
Polythelia
Polymastia
Pregnancy Changes [Figure 1-25-4]
Increased estrogen & progesterone

Estrogen promotes ductal growth
Progesterone promotes lobular growth and breast secretion
Hyperplasia and hypertrophy
Extremely dense breast pattern
Can still see calcifications on mammography
Can see masses on sonography
Mastitis
3% of primary diagnoses at biopsy

Many different types
Infection
Systemic
Antigen-antibody reaction
Idiopathic
Very dense parenchyma in a

normal pregnant patient
Figure 1-25-5
Mastitis [Figures 1-25-5 and 1-25-6]
Acute mastitis
Usually in lactating women with a cracked nipple
Can go on to abscess
Chronic mastitis
Chronic Mastitis
Chronic infection
TB
Fungus
Immunologic
Diabetes
Amyloid
Wegener granulomatosis
Sarcoid
Churg Strauss
Idiopathic
Necrobiotic xanthogranulomatosis
Granulomatous mastitis
Focal parenchymal
density from acute
bacterial mastitis
Figure 1-25-6
Most Common Benign Neoplasms
Fibroadenoma
Biphasic tumor
Intraductal papilloma
Hamartoma
(left) Early abscess with accumulation of pus in

small spaces.
(right) Later stage with larger area of pus
230
Chest Radiology
Biphasic Tumors
Figure 1-25-7
Epithelial & stromal elements

Fibroadenoma
Benign epithelial and stromal elements
Phyllodes tumor
Benign epithelial & hyperplastic or
sarcomatous stroma
Carcinosarcoma
Both elements malignant
Fibroadenoma [Figure 1-25-7]
(left) Circumscribed mass (fibroadenoma) showing

Begins in TDLU
sharp border since there is no tissue invasion.
Response to unopposed estrogen in young women
(right)
Fibroadenoma showing surface devoid of
Oval or round circumscribed nodule
adherent surrounding tissue
May have coarse calcification, especially in periphery
Growth pushes surrounding tissue without invasion
Figure 1-25-9
7-16% of patients have multiple tumors
Polyclonal cell population
Begins as local fibroadenomatiod change which coalesces into a
fibroadenoma
Figure 1-25-8
Fibroadenoma [Figures 1-25-8 to 1-25-10]
Fibroadenoma
Carcinoma
Carcinoma Arising in a
Fibroadenoma
Rare
Most often lobular neoplasia (LCIS) or
DCIS
Invasive carcinoma very rare
Usually grows in from outside
Fibroadenoma Phyllodes
(left) Fibroadenoma showing pushing

Fibroadenoma showing
but not invasion of surrounding tissue.
sharp borders and
(right) Invasive carcinoma without the
sharp border because of invasion, not enhanced thru transmission
of sound
pushing.
Phyllodes Tumor [Figures 1-25-11 and 1-25-12]
Benign epithelial elements and cellular spindle cell stroma

Can act malignant
Local recurrence
Distant blood born metastases
Lymph node enlargement reactive usually
Well circumscribed lobulated mass
Similar appearance on sonography
May have cystic spaces
Figure 1-25-10
Figure 1-25-11
(left) Densely calcified fibroadenomas.
(right) Irregular calcification and ill defined density
from degenerated fibroadenoma
(left) Phyllodes tumor as a
macrolobulated
circumscribed mass similar
to a fibroadenoma.
(right) Phyllodes tumor
similar to a fibroadenoma
except note the small cystic
clefts.
Chest Radiology
231
Figure 1-25-12
Figure 1-25-13
(left) Mass with nonvascular cystic clefts in this

Phyllodes tumor.
(right) Phyllodes tumor showing clefts on low
power microscopy.
Ill defined subareolar

and UOQ mass in this
carcinosarcoma
Figure 1-25-14
Carcinosarcoma [Figure 1-25-13]

Papilloma [Figures 1-25-14 to 1-25-16]
Papillary growth pattern supported by a fibrovascular

stalk
Arises centrally
Usually solitary
Papillomatosis
Arises peripherally in the TDLU
Usually multiple
Figure 1-25-15
(left) Filling defect in duct from papilloma

(right) Specimen of papilloma in a dilated duct.
Figure 1-25-16
(left) Unusual presentation of a papilloma as a

discrete mass.
(right) Filling defect in a dilated duct from a
papilloma
(left) Lobulated mass representing a dilated duct.

(right) Dilated duct with papilloma showing
prominent blood flow
Lobular Neoplasia
No mammographic signs usually

Incidental finding on biopsy
Includes atypical lobular hyperplasia and LCIS
Lobular Neoplasia
High incidence of bilaterality and multifocality

Consider it a bilateral disease
High risk marker for the development of invasive carcinoma
Up to 15% in either breast equally within 20 years
Lobular or ductal features
If found on core biopsy 19% upgraded to carcinoma on excision
Usually LN2 or LN3
Pleomorphic and florid LCIS (subset of LN3) diagnosis carries highest risk
232
Chest Radiology
Pleomorphic type causes necrosis and can present as irregular

calcifications
Figure 1-25-17
Invasive Ductal Cancer [Figures 1-25-17 to 1-25-25]
NOS (not otherwise specified)

50 to 75% of invasive cancers
Medullary
Papillary
Colloid (Mucinous)
Tubular
Metaplastic
Cribriform
Adenoid cystic
Paget's disease
Inflammatory
Figure 1-25-18
Diffusely infiltrative invasive

ductal carcinoma
(left) Spiculated mass in this invasive ductal

carcinoma
(right) Specimen of spiculated invasive ductal
carcinoma
Figure 1-25-20
Figure 1-25-19
(left) Irregular calcification and spiculation in this

invasive ductal carcinoma
(right) Specimen of this case showing irregular
calcifications in irregular lumens and necrotic
tissue spaces
(left) Irregular calcifications in invasive ductal

carcinoma
(right) Specimen showing irregular
calcifications in irregular ductal lumens in this
Figure 1-25-22
Figure 1-25-21
(left) Mass with indistinct borders in invasive ductal

carcinoma
(right) Spiculated mass in this invasive ductal
carcinoma
MR showing enhancing
mass in invasive ductal
carcinoma
Chest Radiology
233
Figure 1-25-23
Figure 1-25-24
Irregular shaped mass with

lobulations and an acute angle on
the right side in this invasive
ductal carcinoma
(left) Invasive ductal carcinoma presenting as a

thick walled cyst (the cyst is central necrosis)
(right) Dense shadowing by invasive ductal
carcinoma
Figure 1-25-25
Pagets Disease [Figures 1-25-26 and 1-25-27]
Red nipple and areola

Scaling eczematoid reaction
50% have a palpable mass
Must have Pagets cells in skin
Usually has underlying carcinoma
Figure 1-25-26
Many irregular calcifications in this
Figure 1-25-27
(left) Pagets disease with red moist nipple
(right) Pagets disease with dry scaly nipple
Types of Invasive Ductal Carcinoma With

Rounded Expansile Periphery
Medullary
Papillary
Cribriform
Colloid
(left) Invasive ductal carcinoma calcifications

behind nipple in Paget's disease
(right) Specimen confirming calcifications in
invasive ductal carcinoma. Pagets cells in skin
Types of Invasive Ductal Carcinoma with

Improved Prognosis
Medullary
Papillary
Cribriform
Colloid
Tubular
Adenoid cystic
Figure 1-25-28
Medullary Carcinoma [Figure 1-25-28]

(left) Medullary carcinoma with slightly more
indistinct borders than fibroadenoma
(right) Minimal invasion of surrounding tissue
(bottom right) by medullary carcinoma
234
Chest Radiology
Papillary Carcinoma [Figure 1-25-29]
Figure 1-25-29
Tubular Carcinoma [Figures 1-25-30 and 1-25-31]
Typically spiculated
Must have 75 - 100% tubular formation
Less than 75% acts like usual invasive carcinoma
Figure 1-25-30
Circumscribed
macrolobulated mass
was a papillary
carcinoma
Figure 1-25-31
Very spiculated mass is typical

tubular carcinoma
(left) Tubule formation in tubular carcinoma

(right) Spiculations in tubular carcinoma
[Figure 1-25-32]
Invasive Lobular Cancer
Prognosis similar to invasive ductal cancer

Most commonly a spiculated mass
Some are more difficult to see as they are diffusely infiltrating
Present as asymmetric density
Figure 1-25-32
Invasive Lobular Cancer [Figures 1-25-33 to 1-25-35]
Invasive lobular
Invasive ductal
Figure 1-25-33
Figure 1-25-34
Typical adenoid cystic

carcinoma with
lobulations but no
spiculation
(left) Tumor cells of invasive lobular carcinoma in

single file
(right) Tumor cells of invasive ductal carcinoma in
enlarged thick walled ducts
Spiculkated mass is the
most common presentation
of invasive lobular
carcinoma
Chest Radiology
235
Sarcoma
1% of breast malignant tumors

Breast contains fat, fibrous tissue, blood vessels, etc.
Angiosarcoma, malignant fibrous hystiocytoma, chondrosarcoma,
rhabdomyosarcoma etc.
Metaplasia can occur
Malignant transformation can occur
Often after chest or breast irradiation
Figure 1-25-35
Fibrosarcoma [Figure 1-25-36]

Spindle Cell Sarcoma [Figure 1-25-37]
Angiosarcoma [Figure 1-25-38]
What You Need to Remember
The mass edge represents the aggressiveness of the underlying

abnormality
The shape of the calcification represents a cast of an underlying space
Figure 1-25-36
Focal asymmetric density in

invasive lobular carcinoma
often causes misdiagnosis
Figure 1-25-37
(left) Non specific mass in fibrosarcoma.

(right) Specimen of fibrosarcoma
(left) Irregular non specific mass in spindle cell

sarcoma.
(right) Specimen of spindle cell sarcoma
Figure 1-25-38
Angiosarcoma
236
Chest Radiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
24.
25.
26.
27.
28.
29.
30.
Alleva DQ, Smetherman DH, Farr GH, Jr., Cederbom GJ. Radial scar of the breast: radiologic-pathologic correlation
in 22 cases. Radiographics 1999; 19 Spec No:S27-35; discussion S36-37.
Bartella L, Liberman L, Morris EA, Dershaw DD. Nonpalpable mammographically occult invasive breast cancers
detected by MRI. AJR Am J Roentgenol 2006; 186:865-870.
Bassett LW. Imaging of breast masses. Radiol Clin North Am 2000; 38:669-691.
Chao TC, Lo YF, Chen SC, Chen MF. Phyllodes tumors of the breast. Eur Radiol 2003; 13:88-93.
Chen SC, Cheung YC, Su CH, Chen MF, Hwang TL, Hsueh S. Analysis of sonographic features for the differentiation
of benign and malignant breast tumors of different sizes. Ultrasound Obstet Gynecol 2004; 23:188-193.
Espinosa LA, Daniel BL, Vidarsson L, Zakhour M, Ikeda DM, Herfkens RJ. The lactating breast: contrast-enhanced
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Feder JM, de Paredes ES, Hogge JP, Wilken JJ. Unusual breast lesions: radiologic-pathologic correlation. Radiographics
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Goel NB, Knight TE, Pandey S, Riddick-Young M, de Paredes ES, Trivedi A. Fibrous lesions of the breast: imagingpathologic correlation. Radiographics 2005; 25:1547-1559.
Jackson VP, Bassett LW. Breast sonography. Breast Dis 1998; 10:55-66.
Kolb TM, Lichy J, Newhouse JH. Comparison of the performance of screening mammography, physical examination,
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Kriege M, Brekelmans CT, Boetes C, et al. Efficacy of MRI and mammography for breast-cancer screening in women
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Chest Radiology
237
Ductal Carcinoma in Situ (DCIS)

Ductal Carcinoma in Situ (DCIS)
Also called intraductal carcinoma

Not invasive ductal carcinoma
DCIS is benign
Disease is confined to the breast
Patients die from metastatic disease
secondary to invasive carcinoma
Figure 1-26-1
Relative Risk of Invasive Carcinoma

(Biopsy Findings)
Mild intraductal hyperplasia (IDH)

No increased risk
Moderate or florid IDH
Slight increased risk
Atypical hyperplasia (ductal or lobular) with no family
history and/or postmenopausal
Mild to moderate increased risk
Atypical hyperplasia (ductal or lobular) with positive
family history and/or premenopausal
High risk
Ductal carcinoma in situ
(left) Thickened duct walls with necrosis and comedo
High risk
secretion in the lumen.
Lobular carcinoma in situ
(top right) Stretching and thinning if duct lumen by early
High risk
DCIS
Confined to the Duct [Figure 1-26-1]
(bottom right) Dilated duct with wall irregularity in DCIS
No spread to blood or lymph nodes

Less than 1% positive axillary nodes
Probably unrecognized invasion
Most likely in large lesions and palpable lesions
The Problems
How big is the problem?

How do we classify the disease?
How do we diagnose it?
What is adequate treatment?
How Big is the Problem?
USA data
50% eligible women get screening annually after 40
240,000 new breast cancers annually
Includes invasive and intraductal
1980 5% of new breast cancers were DCIS
Usually a palpable lump or nipple discharge
2000 20% of new breast cancers
Usually microcalcifications on mammography
Age-adjusted incidence increasing
2.4 per 100,000 in 1973
15.8 per 100,000 in 1992
Is It Malignant?
30 to 60% of underdiagnosed DCIS becomes invasive cancer within 25 years

Usually in the same breast and near the biopsy site
Ductal Carcinoma in Situ
238
Chest Radiology
Invasive Cancer after DCIS
NASBP B17 (790 women) at 5 years

Women treated with breast conservation
115 recurrent cancers
80% same breast
15.7% opposite breast
4.3% regional or distant metastases
12.1% recurrence with lumpectomy + radiation (36% invasive)
26.8% recurrence with lumpectomy alone (51% invasive)
Indicators of Recurrence after Conservative Treatment
Tumor size
Nuclear grade
Necrosis
Margin status
Multifocality
Lymphocytic infiltrate
Epidemiology
Same risks as for invasive cancer

Increasing age
Early menarche
Family history
Previous breast biopsy
Nulliparity or late age at first birth
Pathologic Classification of DCIS
No uniform agreement on single scheme

Interobserver agreement between schemes is poor
Classifications of DCIS
DIN
European Commission Working Group
Lagios
Modified Lagios
Nottingham
UKNBCSP
Van Nuys
Architectural Classification of DCIS
Comedo
Needs comedonecrosis and high nuclear grade
Non-comedo
Cribriform
Micropapillary
Papillary
Solid
Special type
Apocrine
Clear cell
Signet ring cell
Small cell
Endocrine
Spindle cell
Intraductal Carcinoma (DCIS)
Histology may be able to predict recurrence risk

High grade, large cell, comedo have higher recurrence
Low grade, small cell, noncomedo (cribriform, micropapillary)
Poor correlation of calcification type and extent with grade and extent of tumor
Chest Radiology
239
Is DCIS One Disease?

Low Grade
ER
PR
HER-2/neu
p53
bcl-2
+++
+++
+
+
+
High Grade
+
+
+++
++
Is DCIS One Disease?
Associated invasive carcinoma shows marker phenotype like precursor DCIS

Low grade DCIS yields low grade invasive tumors
High grade DCIS yields high grade invasive tumors
Theory: Low grade and high grade DCIS are different from the start
Figure 1-26-2
Diagnosis of DCIS
Mass
Mammographic calcifications
Cant distinguish from invasive carcinoma
Associated mass usually invasive disease
Mass
Rare today
Usually small
Small indistinct mass

represents DCIS
DCIS Mass Close-up [Figure 1-26-2]
Figure 1-26-3
DCIS Mass [Figures 1-26-3 to 1-26-5]

Intraductal Carcinoma (DCIS)
Microcalcification usually without mass

Particles < 1 mm
Varying size shape and density
Clustered
May coexist with benign calcifications
Calcification
Size
Number
Distribution
Shape
Change over time
(left) Large obscured mass was entirely DCIS.

(right) Small vertically oriented solid mass
represents DCIS
Figure 1-26-5
Size of Particles
< 1 mm
Evaluate malignant potential by smallest particles in the
abnormality
Number
Cluster is 5 particles or more in 1 cubic cm.
Figure 1-26-4
Irregular filling defect in duct represents

DCIS
MRI with large area of enhancement was DCIS on
biopsy
240
Chest Radiology
Distribution [Figure 1-26-6]
Figure 1-26-6
Cluster
Linear
DCIS involves a duct
Linear distribution toward nipple
High grade is continuous involvement
Low grade has skip areas
Segmental
Involvement of an entire ductal system
Segmental Intraductal Carcinoma

[Figure 1-26-7]
(left) Cluster of irregular calcifications was DCIS

on biopsy
(right) Broken rod shaped calcification in a linear
distribution represents DCIS
Shape is Most Important
Irregular
Not smooth round or hollow
Heterogeneous or pleomorphic
Not all the same
Figure 1-26-7
Pleomorphic [Figure 1-26-8]

Calcifications represent the caste of a space
Irregular duct
Necrotic tissue space
Intraductal Carcinoma [Figure 1-26-9]

Figure 1-26-9
Segmental distribution of
granular calcifications was
DCIS
Figure 1-26-8
Left: Small calcifications filling the residual lumen

made irregular by wall thickening of DCIS
Right: Duct calcification shape is related to the
contour of the duct wall
Shapes of DCIS Calcification
Granular
Irregular rods
Casting
Irregular
Branching
Comma shaped
Arrow shaped or pointed
Chest Radiology
Multiple irregular calcifications in

biopsy proven DCIS
241
Granular [Figure 1-26-10]
Figure 1-26-10
Very small (<0.5 mm)

Need magnifying glass to evaluate
Too small to see true shape
Grains of sand
Irregular Rods
Made up of many tiny pieces on magnification

Not solid large rods
Secretory disease
Often branch
Extremely small calcifications
represent a finding of DCIS
Rods [Figure 1-26-11]
Regular
Irregular
Casting
Irregular
Figure 1-26-11
Granular calcifications filling the lumen of an irregular duct

Often branch
[Figure 1-26-12]
Castes of necrotic spaces

Branching
Comma shaped
Arrow shaped or pointed
(left) Smooth rods of secretory disease
(right) Broken rods of DCIS
Figure 1-26-12
Figure 1-26-13
(left) Diffuse microcalcifications of DCIS.

(right) Ultrasound shows calcifications and
parenchymal change in DCIS
Small irregular clustered calcifications were
biopsied yielding DCIS
Figure 1-26-14
Intraductal Carcinoma [Figures 1-26-13 to 1-26-15]

Figure 1-26-15
(left) Clustered irregular calcifications.

(right) Calcification in necrotic wall of duct involved
with DCIS
242
Diffuse amorphous
calcifications of DCIS
Chest Radiology
Change Over Time
Benign processes can change

Malignant processes almost always change within 3 years
Short interval follow-up
Probably benign findings
6 months unilateral, annual bilateral for 3 years
No scientific basis
Biopsy
Imaging guided biopsy with specimen radiography

Usually stereotactic
Wire guided excision with specimen radiography
Pathologic Findings Needed in Any Report
Nuclear grade
Low, intermediate or high
Necrosis
Comedo or punctate
Architectural pattern
Lesion size
Margin assessment
Specimen processing
Report should include
Presence of calcification
Correlation with specimen radiograph and/or mammogram
Extent of Calcification Does Not Correspond

to the Extent of the Tumor
Best correlation is with comedo type but not good enough

Poor correlation with cribriform and micropapillary
Must use histologic margins to define true extent
Treatment
Simple mastectomy without axillary dissection

25% of patients choose this option
Large lesions in small breasts
Multiple lesions
No radiation
Unavailability
Prior radiation
Patient preference
Reconstruction
Chest Radiology
243
Breast Conservation
Wide local excision without axillary dissection

Sentinel node when large or palpable lesions
Post -excision mammogram with magnification views of biopsy site
May be done 2 or 3 months after excision
Not necessary if specimen radiograph shows complete excision with
10 mm margin
Radiation is standard
Helps in all patients
Benefit may be small in a subset of patients
Small lesions
Low grade histology
Wide clear margins
Local excision alone without radiation
Controversial
Less than 2 - 3 cm lesion
Margins should be 10 mm or greater
Nuclear grade low or intermediate
Recurrence risk 1% per year
What is a Clear Margin?
Relative risk of recurrence after excision and radiation

10 mm or greater
1.14
1 9 mm
1.49
<1 mm
2.54
Radiation Therapy
1.8 to 2.0 Gy fractions Monday through Friday

45 to 50 Gy total dose
Boost 10 to 20 Gy to surgical bed
No axillary radiation
Chemotherapy
No cytotoxic drugs
Tamoxifen 20 mg daily for 5 years
Newer drugs possible with fewer side effects
Decreases invasive recurrences
No change in survival
Survival is over 90% without chemotherapy
Treatment of Recurrence
DCIS
Mastectomy if radiation given previously
Mastectomy or wide excision with radiation
Invasive carcinoma
Treat like any invasive cancer
Can not give radiation twice
Follow-up
Lifetime
Annual mammography
First exam 6 months after completion of treatment
Every 6 months for the first two years?
Use of magnification views common
Most common in first exam after treatment
Summary
DCIS is carcinoma without the ability to spread YET

It is detected on mammography as calcification
Adequate detection and treatment decreases the incidence of invasive cancer
and therefore death
244
Chest Radiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
Consensus Conference on the classification of ductal carcinoma in situ. The Consensus Conference Committee.
Cancer 1997; 80:1798-1802.
Cornfield DB, Palazzo JP, Schwartz GF, et al. The prognostic significance of multiple morphologic features and
biologic markers in ductal carcinoma in situ of the breast: a study of a large cohort of patients treated with surgery
alone. Cancer 2004; 100:2317-2327.
Hashimoto BE, Kramer DJ, Picozzi VJ. High detection rate of breast ductal carcinoma in situ calcifications on
mammographically directed high-resolution sonography. J Ultrasound Med 2001; 20:501-508.
Hermann G, Keller RJ, Drossman S, et al. Mammographic pattern of microcalcifications in the preoperative diagnosis
of comedo ductal carcinoma in situ: histopathologic correlation. Can Assoc Radiol J 1999; 50:235-240.
Hermann G, Keller RJ, Halton K, Schwartz I, Rabinowitz JG, Tartter P. Nonpalpable ductal carcinoma in situ versus
infiltrating carcinoma of the breast--can they be differentiated by mammography? Can Assoc Radiol J 1991; 42:219222.
Holland R, Hendriks JH. Microcalcifications associated with ductal carcinoma in situ: mammographic-pathologic
correlation. Semin Diagn Pathol 1994; 11:181-192.
Ikeda DM, Birdwell RL, Daniel BL. Potential role of magnetic resonance imaging and other modalities in ductal
carcinoma in situ detection. Magn Reson Imaging Clin N Am 2001; 9:345-356, vii.
Moon WK, Myung JS, Lee YJ, Park IA, Noh DY, Im JG. US of ductal carcinoma in situ. Radiographics 2002; 22:269280; discussion 280-281.
Morris EA, Liberman L, Ballon DJ, et al. MRI of occult breast carcinoma in a high-risk population. AJR Am J
Roentgenol 2003; 181:619-626.
Page DL, Lagios MD. Pathology and clinical evolution of ductal carcinoma in situ (DCIS) of the breast. Cancer Lett
1994; 86:1-4.
Schwartz GF, Solin LJ, Olivotto IA, Ernster VL, Pressman PI. Consensus Conference on the Treatment of In Situ
Ductal Carcinoma of the Breast, April 22-25, 1999. Cancer 2000; 88:946-954.
Sewell CW. Pathology of high-risk breast lesions and ductal carcinoma in situ. Radiol Clin North Am 2004; 42:821830.
Silverstein MJ, Lagios MD, Groshen S, et al. The influence of margin width on local control of ductal carcinoma in
situ of the breast. N Engl J Med 1999; 340:1455-1461.
Stomper PC, Connolly JL, Meyer JE, Harris JR. Clinically occult ductal carcinoma in situ detected with mammography:
analysis of 100 cases with radiologic-pathologic correlation. Radiology 1989; 172:235-241.
Waldman FM, DeVries S, Chew KL, Moore DH, 2nd, Kerlikowske K, Ljung BM. Chromosomal alterations in ductal
carcinomas in situ and their in situ recurrences. J Natl Cancer Inst 2000; 92:313-320.
Yen TW, Hunt KK, Mirza NQ, et al. Physician recommendations regarding tamoxifen and patient utilization of
tamoxifen after surgery for ductal carcinoma in situ. Cancer 2004; 100:942-949.
Chest Radiology
245
Breast Abnormalities in Young Women

Lesions in Young Women are Rare
Very few patients seen by the average radiologist

Women 21 years of age and younger
Fibroadenoma (up to 95%)
Juvenile hypertrophy
Abscess and mastitis
Phyllodes tumor
Malignancy
Primary
Metastatic
Cysts are rare
Lesions in Young Women are Rare
Women over 21 years of age

Fibroadenoma
Abscess and mastitis
Phyllodes tumor
Cysts
More common as age approaches 35
Malignancy
Diagnosis in Young Women
Ultrasound is primary modality

Breasts are dense after puberty
Radiation has a small risk
Mammography is used in select older patients
High risk screening
Masses in patients over 30
Malignant looking lesions
Breast Cancer Incidence in Young Women
% of all breast cancers

Age <20
0%
Age <30
0.3%
Age 20 34
2% Invasive
1% DCIS
Age 40 49
19%
High Risk Screening
Multiple first degree family members

Begin mammography 10 years before the earliest affected relative
Interval uncertain but every 1-2 years usual
BRCA 1 and 2
Begin at 25
Interval uncertain but annual is common
Ultrasound can be useful
MRI can be useful
More sensitive than mammography in high risk groups
246
Chest Radiology
Ultrasound in Young Women
Good test in dense breasts BUT

Many benign non palpable masses in young women
Very few cancers in young women
Breast MRI in Young Women
Diagnosis
Proven cancer
Multifocality
Difficult imaging
Is biopsy needed?
Screening
Strong family history
Gene positive BRCA 1 BRCA 2
Presenting Signs & Symptoms
Mass
Pain
Screening
BRCA 1
BRCA 2
Family history
Benign Lesions Occurring Multiple Times
Fibroadenoma
Juvenile
Giant
Phyllodes low grade
Granular cell tumor
Lactating adenoma
Hamartoma
Normal breast
Fibrocystic change
Juvenile papillomatosis
Vascular
Mastitis
Juvenile hypertrophy
Diabetic mastopathy
Benign Lesions Occurring Once
PASH
Granulomatous mastitis
Fibromatosis
Adenosis
Fibroadenomatoid hyperplasia
Fibrosis
Mondors disease
Varix
Rosai Dorfman disease (Sinus histiocytosis with lymphadenopathy)
32 Fibroadenoma
Age 0-35
3 Age 5-9
8 Age 10-14
3 Age 15-19
Chest Radiology
247
Fibroadenoma
Second most common breast lesion

Fibrocystic change is first
Begins in TDLU
Caused by unopposed estrogenic stimulation
Rare in men
Must have hyperestrogenic state
Figure 1-27-1
Fibroadenoma
Multiple in 16 25% of patients clinically

Found in 25% of breasts examined microscopically
Can undergo myxoid change or hyalinization
Gelatinous nodule
Calcification
Fibroadenoma
Originate in lobules (TDLU)

Stages of development
(left) Two intermediate density circumscribed
Proliferation of epithelial and stromal elements in
fibroadenomas.
multiple lobules
(right)
Sharply
bordered
horizontally oriented oval
Confluence of the hyperplastic lobules
solid
mass
representing
a fibroadenoma
Formation of fibroadenomatous nodules
Nodules coalesce to form FA
Fibroadenoma (32) [Figure 1-27-1]

Fibroadenoma vs Giant Fibroadenoma
vs Juvenile Fibroadenoma
Fibroadenoma
Giant fibroadenoma
Large lesion usually > 10 cm
Juvenile fibroadenoma
Age 20 years or younger
Typically rapid growth and large size
Usually pericanicular type with cellular stroma
Giant Fibroadenoma (32) [Figure 1-27-2]
Figure 1-27-2
Juvenile Fibroadenoma (14) [Figure 1-27-3]
Figure 1-27-3
(left) Large circumscribed intermediate density

mass which is a giant fibroadenoma.
(upper right) Same lesion on ultrasound. It is
sharply bordered and homogeneous
(lower right) Surgical specimen without adherent
surrounding tissue
(left) Large solid sharply bordered mass in a 14
year old, a juvenile fibroadenoma.
(right) Gross specimen of a juvenile fibroadenoma
248
Chest Radiology
Phyllodes Tumor
Benign epithelial elements and cellular spindle cell stroma

Can act malignant
Local recurrence
Distant blood born metastases
Lymph node enlargement reactive usually
Well circumscribed lobulated mass
Similar appearance on sonography to fibroadenoma
May have cystic spaces
Figure 1-27-4
Phyllodes Tumor
Low grade
Pushing margins
Mild atypia
May recur locally
Rare metastases
High grade
Invasive margin
Moderate to severe atypia
Common local recurrence
Hematogenous metastases
(left) CT scan showing left breast mass with areas

of liquefaction necrosis.
(right) Gross specimen of low grade Phyllodes
with areas of necrosis
Figure 1-27-5
Phyllodes Tumor
Treatment
Wide local excision
6 Phyllodes Low Grade
Age 25-35
3 Age 25-29
3 Age 30-35
Can occur in girls under 10 years old
Usually older than 10 years
Tendency to recur but not metastasize
Pushing margins without invasion
(left) Partially obscured large non calcified mass in

the upper breast.
(right) Specimen shows mass with only minimal
adherent tissue
Phyllodes Low Grade (34) [Figure 1-27-4]

Phyllodes Low Grade (31) [Figure 1-27-5]
6 Granular Cell Tumor
Age 15-35
1 Age 15-19
1 Age 20-24
2 Age 25-29
2 Age 30-35
Neural cell origin
First described in tongue
6% in the breast
1/1000 incidence of invasive ductal carcinoma
Wide age range (17-75 years)
Average age 30s
Discrete round mass or spiculated mass
Push or invasive margin
Rare metastasis to axillary nodes
One case in literature of lung metastases
Granular Cell Tumor (33) [Figure 1-27-6]
Chest Radiology
Figure 1-27-6
Circumscribed mass was

granular cell tumor on
biopsy
249
Granular Cell Tumor (35) [Figure 1-27-7]
Figure 1-27-7
Lactating Adenoma [Figures 1-27-8 and 1-27-9]
Young women
Pregnant or lactating women
Circumscribed lobulated masses
Figure 1-27-8
(left) Spiculated granular cell tumor.
(right) Irregular solid markedly hypoechoic mass
with microlobulations and spiculation in this
granular cell tumor
Figure 1-27-9
(left) Lobulated solid mass is a biopsy proven
lactating adenoma.
(right) Lactating adenoma presenting as a smooth
intermediate density non-calcified mass
Hamartoma
Fibroadenolipoma
Palpable mass or mammographic finding
Can be large and not palpable
Encapsulated normal breast elements
Hamartoma (34) [Figure 1-27-10]
Solid mass with an angular border on the right

side which was proven to be a lactating adenoma
Juvenile Papillomatosis
Firm discrete mass

Localized cystically dilated ducts with intraductal proliferation
2/3 less than 20 years old
Association with family history of breast carcinoma
10% develop carcinoma within 10 years
Treat with excisional biopsy
Figure 1-27-10
Juvenile Papillomatosis (14) [Figure 1-27-11]

Figure 1-27-11
(left) Hamartoma as a smooth large noncalcified mass with fat in it.
(right) Lobulated mildly inhomogeneous solid
mass corresponding to the hamartoma on the
last figure
(left) Multicystic mass with many small cystic
spaces represents classic juvenile papillomatosis
in a 14 year old.
(right) Pathologic specimen of juvenile
papillomatosis
250
Chest Radiology
Juvenile Hypertrophy [Figure 1-27-12]
Figure 1-27-12
Usually age 11-14

Usually coincides with first menses
Usually lasts 3-6 months
Unilateral or bilateral palpable mass
Iatrogenic amastia if removed
Diabetic Mastopathy
Focal fibrosis in the breast

Diabetes mellitus type 1 since childhood
Poorly controlled
Complications from vasculitis elsewhere
Occurs in young to middle age
Diabetic Mastopathy (33) [Figure 1-27-13]
Figure 1-27-13
Diabetic Mastopathy (28) [Figure 1-27-14]

Figure 1-27-14
Increased tissue behind
the nipple in this 12 year
old girl was juvenile
hypertrophy
Very irregular hypoechoic mass with

shadowing was also diabetic mastopathy in
a 28 year old
PASH (Pseudoangiomatous Stromal

Hyperplasia)
Dense mass in a 33 year old

Type 1 diabetic was diabetic
mastopathy
Wide age range

Focal lesion usually
Histologically shows slit-like separation of stromal cells
Exaggerated stromal response to hormone stimulation
Figure 1-27-15
PASH (35) [Figure 1-27-15]
(left) Circumscribed mass in PASH in a 35 year old.

(center) Large cystic spaces in PASH
(right) Specimen showing large cystic spaces in PASH
Chest Radiology
251
Granulomatous Mastitis [Figure 1-27-16]
Figure 1-27-16
Usually in reproductive age

Often within 3 years of pregnancy
Idiopathic
Specific causes must be excluded
TB or other bacteria
Sarcoid
Fat necrosis
Foreign body
Granulomatous Mastitis (26) [Figures 1-27-17 and 1-27-18]

Figure 1-27-17
Swelling and redness in
granulomatous mastitis
Figure 1-27-18
Spiculated mass in granulomatous

mastitis
Irregular hypoechoic mass was

granulomatous mastitis
Malignant
No malignant lesion under age 15 in our series

Invasive ductal carcinoma
DCIS
Sarcoma
Angiosarcoma most common
High grade phyllodes
Lymphoma
Metastasis
Figure 1-27-19
Ductal Carcinoma
Age 15-35
2 age 15-19
2 Age 20-24
4 Age 25-29
18 Age 30-35
2 Secretory carcinoma
Invasive Ductal Carcinoma [Figure 1-27-19]
Most common carcinoma

Youngest patient 6 years (not in this series)
Signs similar to older patients
Clustered amorphous calcifications

of invasive ductal carcinoma in a
30 year old
252
Chest Radiology
Invasive Ductal Carcinoma (28) [Figure 1-27-20]

Medullary Carcinoma (24)
Figure 1-27-20
Figure 1-27-21
[Figure 1-27-21]
Medullary Carcinoma (17)

[Figure 1-27-22]
Figure 1-27-22
Microlobulated mass with

spiculations in a 28 year old
represents invasive ductal
carcinoma
Seventeen year old patient with

medullary carcinoma
Secretory Carcinoma
Irregular shaped mass with

Previously called juvenile
spiculations in a 24 year old patient
carcinoma
with medullary carcinoma
Initial report age 3-15
Oldest patient 87
Limited aggressiveness in younger patients
Figure 1-27-23
Secretory Carcinoma (23) [Figure 1-27-23]

DCIS (26) [Figure 1-27-24]
Found on screening mammography in high risk

patients
Found as mass rarely or nipple discharge
Figure 1-27-24
Slightly irregular mass of secretory carcinoma in

a 23 year old
Amorphous calcifications of DCIS in a

26 year old
Sarcoma
Malignant mesenchymal tumors

1% of malignant tumors in all ages
Higher % in young women
After radiation therapy 2-15 years
Many histological subtypes
Chest Radiology
253
Sarcoma
Age 15-35
2 Age 15-19
2 Age 20-24
2 Age 25-29
4 Age 30-35
7 Angiosarcoma
2 Granulocytic sarcoma
1 Myosarcoma
Figure 1-27-25
Angiosarcoma
14 82 years
Mean of 35
Lobulated mass
Highly aggressive lesion
Axillary metastasis rare
Hematogenous metastasis
usual
Angiosarcoma (34) [Figure 1-27-
(left) Large mass replacing the entire breast in angiosarcoma

(right) Angiosarcoma
25]
Phyllodes High Grade
Figure 1-27-26
Age 20-35
2 Age 20-24
1 Age 25-30
2 Age 30-35
Usually older than 10 years
Tendency to recur and metastasize
Invasive margins
Axillary adenopathy usually reactive
Metastases hematogenous
Phyllodes High Grade (31) [Figure 1-27-26]

Lymphoma
(left) Lobulated mass representing a high grade

phyllodes tumor
(right) Lobulated edge of high grade phyllodes
tumor well seen on ultrasound
Primary or secondary
Focal mass or diffuse process
Lymphoma (27) [Figure 1-27-27]

Metastatic Disease
Neurofibrosarcoma
Medulloblastoma
In adults (male and female)
Melanoma
Lung
Prostate
Lymphoma
Figure 1-27-27
Irregular mass of primary lymphoma in a

27 year old
254
Chest Radiology
Metastatic Disease (29) [Figure 1-27-28]
Figure 1-27-28
Neurofibrosarcoma
Metastatic Disease (35) [Figure 1-27-29]
Medulloblastoma
Figure 1-27-29
(left) Well marginated oval solid mass in a

metastatic neurofibrosarcoma
(right) Specimen shows sharply marginated mass
without invasion of surrounding tissue
(left) Partially obscured noncalcified mass in

metastatic medulloblastoma
(right) Well marginated lobulated metastatic
medulloblastoma
Conclusions
Ultrasound is the primary modality in this age group

Mammography is reserved for screening, likely malignant lesions and the older
patients in this group
MRI indications are evolving
Cysts are rare especially in the younger age groups
Most solid lesions are benign
Fibroadenoma most common
Juvenile hypertrophy and juvenile papillomatosis are unique to this age group
and have specific appearances on imaging
Malignant lesions occur and look like malignant lesions in older women
Invasive ductal carcinoma most common
References
1.
Bock K, et.al. Pathologic Breast Conditions in Childhood and Adolescence. Evaluation by Sonographic Diagnosis.
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2. Chateil JF, Arboucalot F, Perel Y, Brun M, Boisserie-Lacroix M, Diard F. Breast metastases in adolescent girls: US
findings. Pediatr Radiol 1998; 28:832-835.
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J Pediatr Surg 1998; 8:67-70.
4. Elsheikh A, Keramopoulos A, Lazaris D, Ambela C, Louvrou N, Michalas S. Breast tumors during adolescence. Eur
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5. El-Tamer MB, Song M, Wait RB. Breast masses in African American teenage girls. J Pediatr Surg 1999; 34:14011404.
6. Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. Am
J Surg Pathol 1997; 21:664-668.
7. Greydanus DE, Parks DS, Farrell EG. Breast disorders in children and adolescents. Pediatr Clin North Am 1989;
36:601-638.
8. Harris VJ, Jackson VP. Indications for breast imaging in women under age 35 years. Radiology 1989; 172:445-448.
9. Karl SR, Ballantine TV, Zaino R. Juvenile secretory carcinoma of the breast. J Pediatr Surg 1985; 20:368-371.
10. Kronemer KA, Rhee K, Siegel MJ, Sievert L, Hildebolt CF. Gray scale sonography of breast masses in adolescent
girls. J Ultrasound Med 2001; 20:491-496; quiz 498.
11. Murphy JJ, Morzaria S, Gow KW, Magee JF. Breast cancer in a 6-year-old child. J Pediatr Surg 2000; 35:765-767.
12. Pettinato G, Manivel JC, Kelly DR, Wold LE, Dehner LP. Lesions of the breast in children exclusive of typical
fibroadenoma and gynecomastia. A clinicopathologic study of 113 cases. Pathol Annu 1989; 24 Pt 2:296-328.
Chest Radiology
255
13. Raganoonan C, Fairbairn JK, Williams S, Hughes LE. Giant breast tumours of adolescence. Aust N Z J Surg 1987;
57:243-247.
14. Raju GC, Jankey N, Naraynsingh V. Breast disease in young West Indian women: an analysis of 1051 consecutive
cases. Postgrad Med J 1985; 61:977-978.
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16. Rosen PP, Holmes G, Lesser ML, Kinne DW, Beattie EJ. Juvenile papillomatosis and breast carcinoma. Cancer 1985;
55:1345-1352.
17. Simmons PS. Diagnostic considerations in breast disorders of children and adolescents. Obstet Gynecol Clin North Am
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19. Squire R, Bianchi A, Jakate SM. Radiation-induced sarcoma of the breast in a female adolescent. Case report with
histologic and therapeutic considerations. Cancer 1988; 61:2444-2447.
20. Templeman C, Hertweck SP. Breast disorders in the pediatric and adolescent patient. Obstet Gynecol Clin North Am
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256
Chest Radiology
The Male Breast

Figure 1-28-1
Development
Birth to puberty same as female
Anatomy [Figure 1-28-1]
Major ducts with little branching

Connective tissue and fat
Almost no lobules
Imaging
Less than 1% of breast imaging

Mammography
Ultrasound
MRI
CT
Normal Male Mammogram [Figures 1-28-2 to 1-28-5]

Figure 1-28-2
Figure 1-28-3
Figure 1-28-4
Normal male with no

tissue seen except
fat
Figure 1-28-5
Normal male with

minimal subareolar
tissue
Normal male with

small focus of
subareolar tissue
Normal male with

intramammary node
Male Breast Disease
Presents as mass, swelling or pain

Presents as nipple discharge
Can be benign or malignant
Large amount of
subareolar tissue in
an asymptomatic
male
Benign Disease
Gynecomastia
Pseudogynecomastia
Papilloma
Adenoma
Myofibroblastoma
More common in men than women
Granular cell tumor
Fibrocystic change
Chest Radiology
257
The Male Breast
Benign Disease
Diabetic mastopathy
Epitheal inclusion cyst
Cystic Lymphangioma
Pleomorphic hyalinizing angioectatic tumor of soft parts
Varix
Leiomyoma
Lipoma
Benign Disease
No lactating adenomas
No pregnancy
Rare lobular tumors
No lobules without progesterone
Rare invasive lobular carcinomas reported
Benign Disease
Rare biphasic tumors

Fibroadenoma, phyllodes, carcinosarcoma
Lesions begin in TDLU (lobules)
Lobular development rare in men
Gynecomastia
Potentially reversible enlargement of the male breast

Presents as soft mobile tender subareolar mass
Simultaneous proliferation ducts and stroma without encapsulation
Florid (early) phase
Begins as increased number of ducts and epithelial proliferation with
edema and cellular fibroblastic stroma
Reversible phase
Fibrotic (late) stage
Progresses to dilated ducts, moderate epithelial proliferation and
fibrosis
Gynecomastia
2cm or more of subareolar tissue in non obese male

Common normal finding
55% of men at autopsy
Peak incidence 60 69 years
Significant if new or symptomatic
Palpable unilateral or bilateral subareolar mass
Often conical shape
65% of breast lesions in elderly males
25% Carcinoma
10% Other lesions
Gynecomastia
Response to hyperestrogenism or estrogen like response

Absolute increase in estrogen HCG or estrogen precursors
Secretion by tumors
Leydig cell tumor
Germ cell tumors
Hepatoma
Adrenal cortical tumors
Pituitary tumors
Gynecomastia
Absolute increase in estrogen HCG or estrogen precursors

Estrogen therapy
Prostate carcinoma
Topical estradiol to scalp
The Male Breast
258
Chest Radiology
Figure 1-28-6
Increase in estrogen precursors

Cirrhosis
Hyperthyroidism
Gynecomastia
Relative increase in estrogen

Testicular failure or atrophy
Idiopathic
Cytotoxic chemotherapy
Puberty and senescence
Transient in puberty (1 2 years)
Klinefelters syndrome (XXY)
Testicular feminization syndrome
Gynecomastia
Hyperthyroidism
Reverses when the patient is euthyroid
Refeeding after malnutrition or starvation
Onset of hemodialysis
Symptomatic male with prominent

subareolar tissue
Gynecomastia
Figure 1-28-7
Drugs (partial list)

Spironolactone
Reserpine
Digitalis
Ergot
Thyroid extract
Dilantin
Thiazide diuretics
Cimetadine
Marijuana
Figure 1-28-8
Gynecomastia
Mammographic patterns
Nodular glandular (florid phase)
Dendritic (fibrotic phase)
Diffuse glandular (very high estrogen levels)
Irregular dense
tissue behind the
nipple
Nodular Pattern [Figure 1-28-6]
Fan shaped density radiating from the nipple

May be more prominent in UOQ
Blends into surrounding fat
Dendritic Pattern [Figure 1-28-7]
Symptomatic male
with diffusely dense
pattern
Subareolar density with prominent extensions into fat

Density smaller than nodular pattern
Figure 1-28-9
Diffuse Pattern [Figure 1-28-8]
Small heterogeneously dense breast
Pseudogynecomastia [Figure 1-28-9]
Usually bilateral
No palpable mass
Excessive fat deposition in breast area
Normal variant
Obesity
Neurifibromatosis
Male with enlarged
fatty breast
Chest Radiology
259
The Male Breast
Myofibroblastoma [Figures 1-28-10 and 1-28-11]
Solitary palpable firm mass

Rarely bilateral
No calcifications
Freely moveable
Mean age late 50s
Circumscribed lobulated mass without calcification
Treated with local excision
Figure 1-28-10
Granular Cell Tumor [Figures 1-28-12 and 1-28-13]
Benign tumor of neural origin

6% in breast
Typical age is 30s
Bimodal appearance
Spiculated or circumscribed
Usually circumscribed in males
Figure 1-28-13
Figure 1-28-12
Figure 1-28-11
Typical appearance
of a
myofibroblastoma
Oval solid mass proven to be

myofibroblastoma
Granular cell tumor with smooth

margins
Granular cell tumor

with irregular
margins
Figure 1-28-14
Epidermal Inclusion Cyst [Figure 1-28-14]
Skin lesion
Round well circumscribed dense mass
Cystic Lymphangioma
[Figure 1-28-15]
Figure 1-28-15
Left: Smooth circumscribed superficial mass proven

to be an epidermal inclusion cyst.
Right: Ultrasound shows epidermal origin of the
mass
Non specific
lobulated mass
proven to be a
cystic
lymphangioma
The Male Breast
260
Chest Radiology
Granulomatous Mastitis
Figure 1-28-16
Idiopathic
Specific causes must be excluded
TB or other bacteria
Sarcoid
Fat necrosis
Foreign body
Pleomorphic Hyalinizing
Angioectatic Tumor of Soft Parts [Figure 1-28-16]
Varix [Figure 1-28-17]
Leiomyoma [Figure 1-28-18]
Complex mass on MRI with non

specific appearance proven to be a
pleomorphic hyalinizing angioectatic
tumor of soft parts
Circumscribed mass
No mitotic activity
>2 mitoses/hpf is leiomyosarcoma
Malignant Disease
Carcinoma
Metastasis
Lymphoma
Sarcoma
Figure 1-28-17
Male Breast Cancer
1690 new cases estimated in 2005 in USA

460 men will die of disease in 2005
1% of all invasive mammary cancers
Less than 0.1% of male cancers
Higher incidence in China and Africa
High incidence of hyperestrogenism secondary to
parasitic liver disease
Male Breast Cancer
Occurs in 60s (10 years after women)

Reported in ages 5 - 93
Unilateral painless subareolar mass
Bilateral in 2% of cases
Can present as bloody nipple discharge
Most invasive ductal cancer including special types
Invasive lobular cancer very rare
DCIS rare (no screening)
80% ER positive
Large vascular structure with venous

flow (not shown)
Figure 1-28-18
Male Breast Cancer
Frequently located subareolar

Most common presentation is a painless subareolar mass
Mass usually eccentric to the nipple
Mass round, oval or irregular
Calcifications rare and coarser than in women
Male Breast Cancer
Pagets disease and skin ulceration more common than in women

Axillary metastases similar to women at same stage
Found at later stage than women
Male Breast Cancer
Leiomyoma with
typical appearance
as a circumscribed
non specific mass
Infiltrating ductal carcinoma including special types

93.7% invasive ductal (usually NOS)
2.6 % papillary
1.8% colloid
1.5% lobular
Chest Radiology
261
The Male Breast
Male Breast Cancer
Figure 1-28-19
DCIS
10% of cases
Usually papillary type
Comedo type very rare
Liposarcoma
Lymphoblastic lymphoma
Metastasis
Carcinoma Risk Factors
Advanced age
Family history
Jewish heritage
Chest wall irradiation
Hyperestrogenism
Hyperthyroidism
Exposure to hepatotoxins
Occupational exposure to high heat
Irregular lobulations
in a subareolar
invasive ductal
carcinoma
Carcinoma Risk Factors
BRCA 2 in 4 16% of cancer patients

40% in Iceland
Undescended testes
Orchiectomy and orchitis
Klinefelters syndrome
47, XXY
6% of male breast cancer
3% lifetime risk
Figure 1-28-20
Invasive Ductal Carcinoma [Figures 1-28-19 to 1-28-21]

Papillary Carcinoma [Figure 1-28-22]
Figure 1-28-21
Irregular mass with spiculations is

proven invasive ductal carcinoma
Figure 1-28-22
Ultrasound of irregular lobulated
vascular mass typical of invasive
ductal carcinoma
Metastasis
Prostate most common in males

Hematogenous spread from primary
Usually in patients with widespread disease
Occasionally solitary
Usually round or oval circumscribed lobulated non-calcified mass
Ductal involvement by a papillary

carcinoma
The Male Breast
262
Chest Radiology
Metastasis Small Cell Carcinoma Lung [Figure 1-28-23]
Figure 1-28-23
Lymphoma
Usually a unilateral mass
Can be diffuse thickening rarely
No calcification or retraction
Liposarcoma [Figures 1-28-24 and 1-28-25]
Very rare sarcoma

Slowly enlarging painful mass
Gynecomastia
Age 60s
Soft
Mobile
Tender usually
Subareolar
Central
Unilateral or bilateral
Nodular, fibrotic or diffuse
Gynecomastia
Nodular
Fan shaped
Fibrotic
Subareolar density with
extensions into fat
Carcinoma
Age 60s
Soft or hard
Mobile or fixed
Tender or painless
Subareolar
Eccentric usually
Unilateral usually
Mass, large or small
Typical rounded
masses in
metastatic disease
Figure 1-28-24
Carcinoma
Large mass
Lobulated border
Small mass
Spiculations
Gynecomastia / Carcinoma [Figures 1-28-26]

Conclusion
Disease presents as mass pain or nipple discharge

Gynecomastia and invasive ductal cancer are the most common lesions in the
male breast
There are other rarer benign and malignant lesions
Gynecomastia and carcinoma can look similar
Biopsy is sometimes necessary to separate gynecomastia from carcinoma
All lesions eccentric to the nipple need biopsy unless they are
characteristically benign
Contain fat
Lymph node
Large water density

mass in a male
breast with a
preexisting lipoma
Figure 1-28-25
Figure 1-28-26
CT scan showing water density mass

in this male patient
Left: Gynecomastia.
Right: Carcinoma
Chest Radiology
263
The Male Breast
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
Appelbaum AH, Evans GF, Levy KR, Amirkhan RH, Schumpert TD. Mammographic appearances of male breast disease.
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Braunstein GD, Glassman HA. Gynecomastia. Curr Ther Endocrinol Metab 1997; 6:401-404.
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1993; 160:267-270.
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Fentiman IS, Fourquet A, Hortobagyi GN. Male breast cancer. Lancet 2006; 367:595-604.
Giordano SH. A review of the diagnosis and management of male breast cancer. Oncologist 2005; 10:471-479.
Giordano SH, Cohen DS, Buzdar AU, Perkins G, Hortobagyi GN. Breast carcinoma in men: a population-based study. Cancer
2004; 101:51-57.
Gunhan-Bilgen I, Bozkaya H, Ustun EE, Memis A. Male breast disease: clinical, mammographic, and ultrasonographic
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Haraldsson K, Loman N, Zhang QX, Johannsson O, Olsson H, Borg A. BRCA2 germ-line mutations are frequent in male breast
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Hill TD, Khamis HJ, Tyczynski JE, Berkel HJ. Comparison of male and female breast cancer incidence trends, tumor
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Hodgson NC, Button JH, Franceschi D, Moffat FL, Livingstone AS. Male breast cancer: is the incidence increasing? Ann Surg
Oncol 2004; 11:751-755.
Iredale R, Brain K, Williams B, France E, Gray J. The experiences of men with breast cancer in the United Kingdom. Eur J
Cancer 2006; 42:334-341.
Jellici E, Malago R, Remo A, Bonetti F, Pozzi Mucelli R. Imaging of the male breast. Radiol Med (Torino) 2005; 110:574-588.
Michels LG, Gold RH, Arndt RD. Radiography of gynecomastia and other disorders of the male breast. Radiology 1977;
122:117-122.
Pappo I, Wasserman I, Halevy A. Ductal carcinoma in situ of the breast in men: a review. Clin Breast Cancer 2005; 6:310-314.
Shi AA, Georgian-Smith D, Cornell LD, et al. Radiological reasoning: male breast mass with calcifications. AJR Am J
Roentgenol 2005; 185:S205-210.
The Male Breast
264
Chest Radiology
266
Benign Hepatic Neoplasms

AFIP Classification - Tumors of the Liver and Intrahepatic Bile Ducts
Hepatocellular origin
Hepatocellular adenoma, focal nodular hyperplasia, nodular regenerative
hyperplasia
Hepatocellular carcinoma, fibrolamellar carcinoma, hepatoblastoma
Cholangiocellular origin
Bile duct cyst, biliary cystadenoma, bile duct adenoma
Cholangiocarcinoma, biliary cystadenocarcinoma
Mesenchymal origin
Hemangioma, angiomyolipoma, myelolipoma, mesenchymal hamartoma
Angiosarcoma, epithelioid hemangioendothelioma
Benign Hepatic Neoplasms - Objectives
Benign neoplasms
Hemangioma
Focal nodular hyperplasia (FNH)
Hepatocellular adenoma
Bile duct cyst
Biliary cystadenoma/cystadenocarcinoma
Lipomatous tumors
Surgical vs. nonsurgical neoplasms
Hemangioma
Most common benign hepatic tumor

Likely a hamartoma rather than true neoplasm
1% to 7% of the population
Most common in adult women
Least common in pediatric population
More common in women, 5:1
Estrogen influences
May enlarge during pregnancy
Symptoms
85% asymptomatic
Pain
Palpable mass
Rupture
Hemangioma
Kasabach-Merritt syndrome
Hemolytic anemia and consumptive coagulopathy
Erythropoietin secretion
Erythrocytosis
Associations
Focal nodular hyperplasia
Tuberous sclerosis
Hemangioma - Pathology
Peripheral feeding vessels

Blood filled spaces
Endothelial lining
Fibrosis from
Slow flowing blood
Thrombosis
Hyalinization
Scar formation
267
Hemangioma - Sonography [Figure 2-1-1]
Figure 2-1-1
Homogeneous, hyperechoic
Minimal posterior acoustic
enhancement
Atypical features
Hypoechoic center
Echogenic border
Scalloped borders
Heterogeneous hypoechoic
Hemangioma - Hypoechoic
Foci
Hemangioma - Scalloped,
echogenic border
Hepatic hemangioma on sonography shows a well-defined mass that

is homogenously hyperechoic. Histologically, the tumor is composed
of multiple blood filled spaces that provide interfaces to produce an
echogenic mass on sonography
Figure 2-1-2
Hemangioma Heterogeneous, hypoechoic

Hemangioma - CT and MR
Peripheral globular enhancement

in arterial phase
Slow centripetal filling during
portal venous/equilibrium
Rapid enhancement pattern
Capillary hemangiomas
Classic appearance of hemangioma on CT.
"Flash fill" phenomenon
There
is
discontinuous,
nodular, peripheral enhancement and gradual
MR
contrast
filling in the lesion
Homogenous hyperintense
T2
Figure 2-1-3
Progressive hyperintensity
as TE increases
"Light bulb" phenomenon
Hemangioma - CT
[Figure 2-1-2]
Hemangioma - MR
Hemangioma - Pedunculated
Hemangioma - Edematous
scar
Atypical hemangioma on MR due to large size and central

hyalinization. Tagged-RBC nuclear medicine imaging is positive
confirming the diagnosis of hemangioma
Hemangioma Heterogeneous with Fibrosis [Figure 2-1-3]
Figure 2-1-4
Hemangioma - Multiplicity
[Figure 2-1-4]
Focal Nodular Hyperplasia
Second most common benign liver

neoplasm
80% to 95% occur in women
Peak age, 20 to 40 years
80% asymptomatic
Associations
Hepatic hemangiomas
Intracranial aneurysms
Dysplastic system arteries
Intracranial neoplasms:
meningioma, astrocytoma
Multiple hemangiomas shown on MR

268
Pathogenesis
Hyperplastic response to a vascular malformation
Central artery
Central scar
Gross Pathology
Central scar
Nodular with fibrous septa
No hemorrhage or necrosis
No capsule
Figure 2-1-5
Histology
Fibrous septa
FNH is often isoechoic to normal liver on sonography and may show
Large arteries
marked flow on color doppler of power doppler imaging
Normal hepatocytes
Kupffer cells
No portal tracts or central veins
Focal Nodular Hyperplasia - Sonography [Figure 2-1-5]
Subtle
Similar texture to normal liver
Stealth lesion
Scar is hypoechoic
Doppler
Peripheral and central vessels
Figure 2-1-6
Focal Nodular Hyperplasia - CT
Noncontrast
Iso- or hypodense
Hypodense scar
Arterial
Rapid enhancement
Hypodense scar
Portal venous
Iso- or hypo- or hyperdense
Delayed enhancement of
scar
Peripheral capsule-like
vessels
FNH shows contrast enhancement during the arterial phase and near
isoattenuation during the portal venous phase
FNH [Figures 2-1-6 and 2-1-7]
Figure 2-1-7
FNH - Sulfur Colloid
Normal uptake 60%

Defect 30%
Increased uptake 10%
FNH - MR
T1 isointense
Low signal scar
T2 iso or slightly hyperintense
High signal scar
FNH on CT showing late enhancement of the central scar and
Gd-DTPA
peripheral vessels
Rapid homogeneous
enhancement
May have flash enhancement
Delayed enhancement of the scar
Rim-like enhancement late
T2 with ferumoxide
Lesion decreases signal
Except scar
269
FNH [Figure 2-1-8]

FNH - Flash Enhancement
FNH - Ferumoxide-enhanced MR
Paley MR, et al. AJR 2000; 175:1: 159-63
Figure 2-1-8
FNH - Atypical Imaging Features
Multiplicity
Absent scar
Very large scar
Fat
Hemorrhage
Calcification (very rare)
Atypical FNH - Absent Scar

Atypical FNH - Hemorrhage
Typical appearance of FNH on MR
Hepatocellular Adenoma
Third most common benign liver tumor

Composed of benign hepatocytes
Almost always occur in women
Mean age, 30 years
History of oral contraceptive use
Declining incidence
Surgical resection
Risk of hemorrhage
Small risk of malignant transformation to HCC
Hepatocyte proliferation
Exogenous estrogens
Ovarian tumors
Anabolic steroids
Antiestrogens
Glycogenosis, type Ia and III
Hurler syndrome
Hepatocellular Adenoma - Clinical Features
Acute abdominal pain 40%

Hemorrhage within tumor
Intraperitoneal hemorrhage
Palpable mass 35%
Incidental 10%
Hepatocellular Adenoma - Pathologic Features
Histology
Benign hepatocytes
Rich in glycogen
Kupffer cells
Gross
Solitary
Multiple (up to 50%)
Capsule (25%)
Peripheral vessels
Central fat
Necrosis, infarcts, hemorrhage
270
Hepatocellular Adenoma - CT and MR
Capsule
Heterogeneous
Hemorrhage (25% to 50%)
Acute, high density on
unenhanced CT
Chronic, hemosiderin rings
on MR
Focal fat
Enhancement
Variable
Intracellular glycogen/fat
Diffuse low attenuation on CT
Loss of signal on out-ofphase MR
Figure 2-1-9
Hemorrhagic hepatocellular adenoma
Figure 2-1-10
Hepatocellular Adenoma Acute Hemorrhage

[Figure 2-1-9]
Hemosiderin Rings
Focal Fat and Capsule
[Figure 2-1-10]
Diffuse Low Attenuation
Hepatocellular adenoma with focal fat and a capsule on CT with the

corresponding gross specimen
[Figure 2-1-11]
Hepatocellular Adenoma - Out-of-Phase MR
Figure 2-1-11
Hepatocellular Adenoma Fat Suppression

Hepatocellular Adenoma Imaging Difficulties
Nonhemorrhagic
Fibrosis/scar formation
Multiple
Glycogenosis
Hepatocellular adenomatosis Diffuse low attenuation in hepatocellular adenoma due to intracellular
glycogen
Hepatocellular Adenoma Multifocality
Multiple estrogen-associated adenomas

Hepatocellular adenomatosis
Hepatocellular Adenomatosis [Figure 2-1-12]
Affects men and women

Unrelated to estrogens
Abnormal LFT's
Biopsy for diagnosis
Treated symptomatically
Figure 2-1-12
Hepatocellular adenomatosis
271
Bile Duct (Hepatic) Cyst
Common
Congenital/developmental origin
Lined by a single layer of columnar cells
Affect all age groups
Majority occur in 4th to 6th decades of life
Rare in children
Asymptomatic
Majority of cases
Incidental discovery
Symptomatic
Large size
Secondary hemorrhage or infection
Treated with drainage, sclerotherapy, or excision
Imaging
Unilocular, simple cyst
Septations, debris when complicated by infection or hemorrhage
Complex cyst differential

Echinococcal cyst
Simple cyst with hemorrhage/infection
Post-traumatic cyst
Abscess
Ciliated hepatic foregut cyst
Peliosis
Biliary cystadenoma
Biliary cystadenocarcinoma
Cystic metastasis
Teratoma
Biliary Cystadenoma
Benign tumor, but

May recur after excision
May develop into cystadenocarcinoma
Middle-aged women
42 - 55 years
Ovarian stroma histologically
Cystic neoplasms
Unilocular or multilocular
Septations
Mural nodules
Calcification
Biliary Cystadenoma - Imaging Features
Cystic neoplasms
Unilocular or multilocular
Cyst fluid variable composition
Septations
Mural nodules
May enhance
Calcification
Punctate or linear
May communicate or extend into biliary system
272
Biliary Cystadenoma [Figure 2- 1-13]
Figure 2-1-13
Lipomatous Tumors
Angiomyolipoma
Benign
Composed of adipose, smooth muscle, and blood vessels
Most cases sporadic
Tuberous sclerosis in 6%
Myelolipoma
Rare
Benign
Composed of myeloid, adipose, and blood vessels
Angiomyolipoma [Figures 2-1-14 and 2-1-15]

Myelolipoma
Summary - Benign Hepatic Neoplasms
Nonsurgical lesions
Hemangioma
Surgical lesions
Biliary cystadenoma
Biliary cystadenoma
Figure 2-1-14
Summary - Hemangioma
Sonography
Homogenous
Hyperechoic
CT/MR
Peripheral nodular enhancement
Tagged-RBC
Summary - FNH
CT/MR
Rapid enhancement
Homogenous tumor
Hypodense/intense scar
Delayed scar enhancement
Delayed peripheral enhancement
Sulfur colloid
Summary - HCA
Echogenic angiomyolipomas on
sonography
For imaging diagnosis

Female patient
Oral contraceptive use
Evidence of hemorrhage
Suggest HCA
Diffuse low attenuation
Diffuse fat on MR
Appropriate patient
BIOPSY !
Figure 2-1-15
Summary - Biliary
Cystadenoma
Cystic neoplasm
Septations
Nodules
Calcification
Most common in middle-aged
women
Multiple hepatic angiomyolipomas in a patient with tuberous sclerosis

who has angiomyolipomas in the right kidney and a history of left
nephrectomy for a hemorrhagic angiomyolipoma
273
References
Hemangioma
1. Freeny PC, Marks WM. Patterns of contrast enhancement of benign and malignant hepatic neoplasms during bolus
dynamic and delayed CT. Radiology 1986; 160:613-618.
2. Birnbaum BA, Noz ME, Chapnick J, et al. Hepatic hemangiomas: diagnosis with fusion of MR, CT, and Tc-99mlabeled red blood cell SPECT images. Radiology 1991; 181:469-474.
3. Quinn SF, Benjamin GG. Hepatic cavernous hemangiomas: simple diagnostic sign with dynamic bolus CT.
Radiology 1992; 182:545-548.
4. Yamashita Y, Ogata I, Urata J, Takahashi M. Cavernous hemangioma of the liver: pathologic correlation with
dynamic CT findings. Radiology 1997; 203:121-125.
5. Kim T, Federle MP, Baron RL, Peterson MS, Kawamori Y. Discrimination of small hepatic hemangiomas from
hypervascular malignant tumors smaller than 3 cm with three-phase helical CT. Radiology 2001; 219:699-706.
1. Mattison GR, Glazer GM, Quint LE, Francis IR, Bree RL, Ensminger WD. MR imaging of hepatic focal nodular
hyperplasia: characterization and distinction from primary malignant hepatic tumors. AJR Am J Roentgenol 1987;
148:711-715.
2. Rummeny E, Weissleder R, Sironi S, et al. Central scars in primary liver tumors: MR features, specificity, and
pathologic correlation. Radiology 1989; 171:323-326.
3. Buetow PC, Pantongrag-Brown L, Buck JL, Ros PR, Goodman ZD. Focal nodular hyperplasia of the liver:
radiologic-pathologic correlation. RadioGraphics 1996; 16:369-388.
4. Paley MR, Mergo PJ, Torres GM, Ros PR. Characterization of focal hepatic lesions with ferumoxides-enhanced
T2-weighted MR imaging. AJR Am J Roentgenol 2000; 175:159-163.
5. Brancatelli G, Federle MP, Grazioli L, Blachar A, Peterson MS, Thaete L. Focal nodular hyperplasia: CT findings
with emphasis on multiphasic helical CT in 78 patients. Radiology 2001; 219:61-68.
6. Ruppert-Kohlmayr AJ, Uggowitzer MM, Kugler C, Zebedin D, Schaffler G, Ruppert GS. Focal nodular
hyperplasia and hepatocellular adenoma of the liver: differentiation with multiphasic helical CT. AJR Am J
Roentgenol 2001; 176:1493-1498.
7. Hussain SM, Terkivatan T, Zondervan PE, et al. Focal nodular hyperplasia: findings at state-of-the-art MR
imaging, US, CT, and pathologic analysis. Radiographics 2004; 24:3-17; discussion 18-19.
1. al-Otaibi L, Whitman GJ, Chew FS. Hepatocellular adenoma. AJR Am J Roentgenol 1995; 165:1426.
2. Casillas VJ, Amendola MA, Gascue A, Pinnar N, Levi JU, Perez JM. Imaging of nontraumatic hemorrhagic
hepatic lesions. Radiographics 2000; 20:367-378.
3. Grazioli L, Federle MP, Ichikawa T, Balzano E, Nalesnik M, Madariaga J. Liver adenomatosis: clinical,
histopathologic, and imaging findings in 15 patients. Radiology 2000; 216:395-402.
4. Ichikawa T, Federle MP, Grazioli L, Nalesnik M. Hepatocellular adenoma: multiphasic CT and histopathologic
findings in 25 patients. Radiology 2000; 214:861-868.
Biliary Cystadenoma
1. Palacios E, Shannon M, Solomon C, Guzman M. Biliary cystadenoma: ultrasound, CT, and MRI. Gastrointest
Radiol 1990; 15:313-316.
2. Buetow PC, Buck JL, Pantongrag-Brown L, et al. Biliary cystadenoma and cystadenocarcinoma: clinical-imagingpathologic correlations with emphasis on the importance of ovarian stroma. Radiology 1995; 196:805-810.
3. Levy AD, Murakata LA, Abbott RM, Rohrmann CA, Jr. From the archives of the AFIP. Benign tumors and
tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces
Institute of Pathology. Radiographics 2002; 22:387-413.
274
Malignant Hepatic Neoplasms

Malignant Hepatic Neoplasms - Objectives
Malignant neoplasms
Hepatocellular carcinoma (HCC)
Fibrolamellar carcinoma (FLC)
Intrahepatic cholangiocarcinoma
Angiosarcoma
Epithelioid hemangioendothelioma
Approach to the incidentally discovered liver mass
Hepatocellular Carcinoma
Neoplasm composed of malignant hepatocytes

Fifth most common cancer worldwide
Hepatocellular Carcinoma - Geographic Variation
High incidence areas

Sub-Saharan Africa, Asia
30 to 45 years old
Hepatitis B and C, aflatoxins
Aggressive
Low incidence areas
Western hemisphere
70 to 80 years old
Alcoholic cirrhosis, hepatitis C, hemochromatosis
Insidious
Hepatocellular Carcinoma - Etiology
Strong association with chronic liver disease

Cirrhosis
Hepatitis B
Hepatitis C
Hepatocellular Carcinoma - Other Etiologies
Aflatoxin B1
Metabolic Diseases
Hemochromatosis (25%)
Hereditary tyrosinemia (20%)
Alpha-1-antitrypsin deficiency (15%)
Anabolic steroids
Hepatocellular Carcinoma - Clinical Features
More common in men

2:1 to 5:1
Elevated alpha-fetoprotein (AFP)
Elevated in 70%-90%
Paraneoplastic syndromes
Hypoglycemia
Erythrocytosis
Hypercholesterolemia
Rare, hypercalcemia, precocious puberty, gynecomastia, carcinoid
syndrome, osteoporosis, hypertrophic pulmonary osteoarthropathy
275
Hepatocellular Carcinoma - Pathophysiology
Key feature relevant to imaging

Angiogenesis
Normal liver blood supply
~80% portal venous
~20% hepatic artery
HCC blood supply
~100% hepatic artery
Rarely, hypovascular
Figure 2-2-1
Gross Pathology
Key features relevant to imaging

Capsule
Necrosis/hemorrhage/fibrosis
Vascular invasion
Macroscopic fat
No calcification when HCC occurs in chronic liver disease
Hepatocellular Carcinoma - Gross Pathology
Solitary and Encapsulated

Macroscopic fat
Hemorrhage and necrosis
Multifocal
Vascular invasion
Sonographic appearance of a small

HCC. The lesion is well defined and
hypoechoic. On CT, the mass is
hypervascular in the arterial phase of
contrast enhancement
Hepatocellular Carcinoma - Histologic Features
Trabecular growth
Occasional Kupffer cells
Vascular invasion
Figure 2-2-2
Hepatocellular Carcinoma Sonographic Features
Variable and nonspecific

Small lesions, hypoechoic
and uniform
Large lesions, focal and
heterogeneous
Diffuse, multinodular pattern
Suggestive features
High velocity arterial flow
Peripheral hypoechoic rim
Mosaic appearance of HCC on sonography, CT, and gross pathology
Figure 2-2-3
Hepatocellular Carcinoma Hypoechoic [Figure 2-2-1]

Hepatocellular Carcinoma - Peripheral Hypoechoic
Rim
Hepatocellular Carcinoma - Mosaic Pattern [Figure 2-2-2]
Hepatocellular Carcinoma - Multifocal with Portal Vein
Invasion [Figure 2-2-3]
Multifocal HCC with portal vein

invasion on sonography
276
Hepatocellular Carcinoma - CT and MR Features
Arterial phase
Rapid enhancement in
small HCC
Late arterial phase
better than early
arterial phase
Portal venous phase
Heterogeneous,
"mosaic pattern"
Suggestive features
Capsular
enhancement
Central fibrosis
Fatty change
Vascular invasion
Arterioportal shunting
Figure 2-2-4
Hypervascular HCC in cirrhosis
Figure 2-2-5
Hepatocellular
Carcinoma
Small HCC in Cirrhosis
[Figure 2-2-4]
Hepatocellular
Carcinoma
Capsule and
Macroscopic Fat [Figure 2-2-5]
HCC with capsule and macroscopic fat
Figure 2-2-6
Hepatocellular Carcinoma - Mosaic Pattern

Mosaic Pattern with Capsular Enhancement [Figure 2-2-6]
Mosaic Pattern
Fibrosis
Multifocal with Portal Vein Invasion
Solitary and Portal Vein Invasion
Hepatocellular Carcinoma Hepatic Vein/IVC Invasion
[Figure 2-2-7]
Figure 2-2-7
HCC with IVC

invasion on MDCT
277
HCC with a mosaic pattern and

capsular enhancement
Hepatocellular Carcinoma - MR Imaging in Cirrhosis
Figure 2-2-8
Hepatocellular Carcinoma - Noncirrhotic Liver
Large, solitary masses

Heterogeneous
Capsule
Fat (10%)
Calcification (25%)
Hepatocellular Carcinoma - Noncirrhotic Liver

Fibrolamellar Carcinoma [Figure 2-2-8]
Variant of HCC
Bands of fibrous lamellae
Tumor cells have "oncocytic" cytoplasm
Young patients
Mean age, 23 years
No cirrhosis
AFP usually normal
Fibrolamellar Carcinoma - Gross Pathology
Histology and gross pathology of

fibrolamellar carcinoma
Central scar
Radiating septa
Calcification
Lobulated contour
Bile staining
Fibrolamellar Carcinoma - CT Features[Figures 2-2-9 and 2-2-10]
Lobulated, well defined margins

Heterogeneous mass
Arterial phase enhancement
Central scar
Hypodense in all phases of enhancement
Calcification in 40%
Figure 2-2-9
Fibrolamellar Carcinoma MR Features
Lobulated margins
Heterogeneous signal mass
Dark T1
Bright T2
Hypointense central scar
Dark T1
Dark T2
No enhancement
Fibrolamellar carcinoma
Fibrolamellar Carcinoma
How can I differentiate FLC from FNH?
Figure 2-2-10
Tumor heterogeneous in FLC

Homogeneous in FNH
Scar nonenhancing in FLC
Delayed enhancement in
FNH
Scar dark T2 signal in FLC
Scar bright T2 in FNH
278
Intrahepatic Cholangiocarcinoma (ICC)
Adenocarcinoma arising from intrahepatic bile ducts

10% of bile duct adenocarcinomas
Synonyms
Peripheral cholangiocarcinoma, cholangiocellular
carcinoma,
intrahepatic bile duct carcinoma
Geographic incidence variation
10 times more common in Japan compared to
U.S.
Figure 2-2-11
Intrahepatic Cholangiocarcinoma - Etiology
Majority of cases
Unknown etiology
Noncirrhotic liver
Intrahepatic Cholangiocarcinoma - Etiologic

Associations
Chronic cholestatic disease

Primary sclerosing cholangitis
Primary biliary cirrhosis
Caroli disease/congenital hepatic fibrosis
Chronic biliary inflammation
Recurrent pyogenic cholangitis
Parasitic infection
Hepatolithiasis
Hepatitis B and C
ETOH abuse
Radiation
Figure 2-2-12
Intrahepatic Cholangiocarcinoma Pathologic Features [Figure 2-2-11]
Morphology
Solitary
Multifocal
Diffuse
Satellite nodules
Marked fibrosis
No capsule
Rare
Hemorrhage and necrosis
Calcification
Intrahepatic cholangiocarcinoma showing

capsular contraction and
biliary dilatation peripheral to the mass
Figure 2-2-13
Intrahepatic
Cholangiocarcinoma
CT and MR Features
Irregular borders
Infiltrative
Enhancement pattern
Due to
fibrosis/hypovascularity
Delayed peripheral to central Intrahepatic cholangiocarcinoma on MR showing central to peripheral
enhancement on gadolinium enhanced T1-weighted images.
Biliary dilatation peripheral to the
The gross photograph shows
tumor
characteristic
fibrosis within the tumor
Capsular contraction
Vascular invasion
Intrahepatic Cholangiocarcinoma
[Figures 2-2-12 and 2-2-13]
279
How can I differentiate ICC from HCC?
Difficult
HCC has variable morphology
HCC occurs more commonly
HCC associated with cirrhosis and hepatitis
But, HCC may occur in normal livers
Ultimately
Biopsy is needed for diagnosis
How can I differentiate ICC from HCC?
Enhancement
Delayed, peripheral to central favors ICC
Rapid filling favors HCC
Marked heterogeneity (mosaic) favors HCC
Tumor margins
Lobulated, irregular favors ICC
Capsule favors HCC
More common in ICC
Biliary dilatation peripheral to the
tumor
More common in ICC
Figure 2-2-14
Angiosarcoma
Malignant neoplasm of
endothelial cells
Rare
But, most common hepatic
sarcoma
Etiologic associations
Vinyl chloride
Arsenical compounds
Radiation therapy
Anabolic steroids
Angiosarcoma on CT and MR showing central hemorrhage that is

fluid attenuation on CT and high signal on T1-and T2-weighted MR
Angiosarcoma
More common in men, 3:1

Variable
Hemoperitoneum
Metastasis in 60%, spleen, lung
Figure 2-2-15
Angiosarcoma - Imaging Features
Solitary or multifocal
Enhancement
Peripheral or heterogeneous
Metastatic disease
Spleen and lung
Angiosarcoma [Figure 2- 2-14]

Epithelioid Hemangioendothelioma
[Figure 2- 2-15]
Rare malignancy of endothelial origin

Contains dense fibrous stroma
Imaging
Multifocal, lesions coalesce over time
Peripheral enhancement
Central fibrous stroma
Retracted liver capsule
May calcify
showing multifocality and capsular
contraction
280
Approach to the incidentally discovered liver mass?
Does the mass meet the criteria for a benign, nonsurgical lesion?
Bile duct cyst
Hemangioma
FNH
Are there equivocal features of hemangioma or FNH on CT and/or MR?
Consider scintigraphy
Is there clinical history that will suggest the etiology?
History of primary malignancy
History of chronic liver disease
History or exogenous estrogens
Are there features that suggest HCC?
Capsule
Fat
Vascular invasion
Mosaic pattern
Are there features that suggest hepatocellular adenoma?
Clinical/demographic history
Capsule
Fat
Diffuse low attenuation
Hemorrhage
Are there features that suggest cholangiocarcinoma?
No capsule
Ill-defined margins
Biliary dilatation
If the answer is NO to all the above, and the finding is a small, focal area of
arterial enhancment on MDCT, the possibilities are:
Hemangioma
FNH
Small adenoma
Small HCC
Hypervascular met
AVM
THAD
Summary Hepatocellular Carcinoma
Most common primary hepatic malignancy

Strong association with chronic liver disease
Variable imaging features
Rapid enhancement
Capsule
Mosaic pattern
Focal fat
Vascular invasion
Summary Fibrolamellar Carcinoma
Variant of HCC
Young patients
Otherwise normal liver
Key features
Lobular tumor
Central scar
Heterogeneous mass
Summary Intrahepatic Cholangiocarcinoma
Arise from bile duct epithelium

Uncommon
Key features
Delayed central enhancement
281
Biliary dilatation peripheral to tumor

Summary Angiosarcoma
Rare
Key features
Splenic metastasis at presentation
References
1. Winter TC, 3rd, Takayasu K, Muramatsu Y, et al. Early advanced hepatocellular carcinoma: evaluation of CT and
MR appearance with pathologic correlation. Radiology 1994; 192:379-387.
2. Takayasu K, Furukawa H, Wakao F, et al. CT diagnosis of early hepatocellular carcinoma: sensitivity, findings, and
CT-pathologic correlation. AJR Am J Roentgenol 1995; 164:885-890.
3. Baron RL, Oliver JH, 3rd, Dodd GD, 3rd, Nalesnik M, Holbert BL, Carr B. Hepatocellular carcinoma: evaluation
with biphasic, contrast-enhanced, helical CT. Radiology 1996; 199:505-511.
4. Kelekis NL, Semelka RC, Worawattanakul S, et al. Hepatocellular carcinoma in North America: a
multiinstitutional study of appearance on T1-weighted, T2-weighted, and serial gadolinium-enhanced gradientecho images. AJR Am J Roentgenol 1998; 170:1005-1013.
5. Loyer EM, Chin H, DuBrow RA, David CL, Eftekhari F, Charnsangavej C. Hepatocellular carcinoma and
intrahepatic peripheral cholangiocarcinoma: enhancement patterns with quadruple phase helical CT--a comparative
study. Radiology 1999; 212:866-875.
6. Winston CB, Schwartz LH, Fong Y, Blumgart LH, Panicek DM. Hepatocellular carcinoma: MR imaging findings
in cirrhotic livers and noncirrhotic livers. Radiology 1999; 210:75-79.
7. Ward J, Guthrie JA, Scott DJ, et al. Hepatocellular carcinoma in the cirrhotic liver: double-contrast MR imaging
for diagnosis. Radiology 2000; 216:154-162.
8. Murakami T, Kim T, Takamura M, et al. Hypervascular hepatocellular carcinoma: detection with double arterial
phase multi-detector row helical CT. Radiology 2001; 218:763-767.
9. Brancatelli G, Federle MP, Grazioli L, Carr BI. Hepatocellular carcinoma in noncirrhotic liver: CT, clinical, and
pathologic findings in 39 U.S. residents. Radiology 2002; 222:89-94.
10. Iannaccone R, Laghi A, Catalano C, et al. Hepatocellular carcinoma: role of unenhanced and delayed phase multidetector row helical CT in patients with cirrhosis. Radiology 2005; 234:460-467.
1. Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma:
imaging and pathologic findings in 31 recent cases. Radiology 1999; 213:352-361.
2. McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR. Fibrolamellar carcinoma of the liver:
3. Soyer P, Roche A, Levesque M, Legmann P. CT of fibrolamellar hepatocellular carcinoma. J Comput Assist
Tomogr 1991; 15:533-538.
4. Titelbaum DS, Hatabu H, Schiebler ML, Kressel HY, Burke DR, Saul SH. Fibrolamellar hepatocellular carcinoma:
MR appearance. J Comput Assist Tomogr 1988; 12:588-591.
5. Titelbaum DS, Burke DR, Meranze SG, Saul SH. Fibrolamellar hepatocellular carcinoma: pitfalls in nonoperative
diagnosis. Radiology 1988; 167:25-30.
6. Blachar A, Federle MP, Ferris JV, et al. Radiologists' performance in the diagnosis of liver tumors with central
scars by using specific CT criteria. Radiology 2002; 223:532-539.
1. Choi BI, Park JH, Kim YI, et al. Peripheral cholangiocarcinoma and clonorchiasis: CT findings. Radiology 1988;
169:149-153.
2. Tani K, Kubota Y, Yamaguchi T, et al. MR imaging of peripheral cholangiocarcinoma. J Comput Assist Tomogr
1991; 15:975-978.
3. Kim TK, Choi BI, Han JK, Jang HJ, Cho SG, Han MC. Peripheral cholangiocarcinoma of the liver: two-phase
spiral CT findings. Radiology 1997; 204:539-543.
4. Loyer EM, Chin H, DuBrow RA, David CL, Eftekhari F, Charnsangavej C. Hepatocellular carcinoma and
intrahepatic peripheral cholangiocarcinoma: enhancement patterns with quadruple phase helical CT--a comparative
study. Radiology 1999; 212:866-875.
5. Zhang Y, Uchida M, Abe T, Nishimura H, Hayabuchi N, Nakashima Y. Intrahepatic peripheral
cholangiocarcinoma: comparison of dynamic CT and dynamic MRI. J Comput Assist Tomogr 1999; 23:670-677.
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MR cholangiopancreatography. RadioGraphics 2000; 20:959-975; quiz 1108-1109, 1112.
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Levy AD. Malignant liver tumors. Clin Liver Dis 2002; 6:147-164.
Angiosarcoma
1. Peterson MS, Baron RL, Rankin SC. Hepatic angiosarcoma: findings on multiphasic contrast-enhanced helical CT
do not mimic hepatic hemangioma. AJR Am J Roentgenol 2000; 175:165-170.
2. Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at
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Characteristics in 12 Patients. Radiology 2005. In Press
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a clinicopathologic and follow-up study of 32 cases. Hum Pathol 1984; 15:839-852.
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with pathologic correlation [see comments]. AJR Am J Roentgenol 1992; 159:53-57.
3. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study
of 137 cases. Cancer 1999; 85:562-582.
4. Mermuys K, Vanhoenacker PK, Roskams T, D'Haenens P, Van Hoe L. Epithelioid hemangioendothelioma of the
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283
Hepatic Infections
Hepatic Infections
Pyogenic Abscess
Amebic Abscess
Echinococcal Infections
Schistosomiasis
Clonorchiasis
Infections in the Immunocompromised host
Candidasis
Pneumocystis
Pyogenic Hepatic Abscess
Polymicrobial infections
Variable clinical presentation
Septicemia, pain, fever, indolent symptoms
Tender hepatomegaly
Pyogenic Hepatic Abscess: Pathogenesis
Biliary
MOST COMMON ETIOLOGY
Cholangitis, biliary obstruction
Multiple and bilateral
Portal vein
Pylephlebitis
Solitary, 65% right lobe
Hepatic artery
Direct extension
Traumatic-blunt or penetrating trauma
Necrotic tumor
Mortality rate <10%

Effectively treated with percutaneous drainage
8% failure rate
8% recurrence rate
Pyogenic Hepatic Abscess: Sonography
Variable echogenicity
Anechoic (50%)
Hyperechoic (25%)
Hypoechoic (25%)
Ill-defined margins
Internal character
Irregular wall
Septations
Fluid-fluid levels
Debris
Reverberation artifact if gas is present
Posterior acoustic enhancement
Hepatic Infection
284
Pyogenic Hepatic Abscess: CT
Figure 2-3-1
Singe best imaging method

Sensitivity 97%
Intravenous contrast essential
Hypoattenuating
0 to 45 H.U.
Helpful CT features
Rim-enhancement
Transition zone
Cluster sign
Gas (<20%)
Air/fluid or debris/fluid level
Suspect GI communication
Pyogenic hepatic abscess shows

cluster sign and transition zone
Pyogenic Hepatic Abscess CT

Pyogenic Hepatic Abscess: Cluster Sign
Pyogenic Hepatic Abscess: Cluster Sign/ Transition zone
[Figure 2-3-1]
Pyogenic Hepatic Abscess: Intrahepatic Gas

Pyogenic Hepatic Abscess: Imaging Guided Drainage
Unilocular and liquefied

Multilocular or multiple
Multiple catheters
Multiple, small (<1 cm)
Aspiration for diagnosis
Treatment: antibiotics or aspiration + antibiotics
Amebic Liver Abscess
Most common extra-intestinal manifestation of amebiasis

3%-7% of patients with amebic infection
Fever, RUQ pain
Route of spread
Portal venous (most common)
Lymphatic
Direct extension from colon
Amebic Abscess: Sonography
Round or oval shape

Absent wall echoes
Homogenous low level internal echoes
Location
Near or touching the liver capsule
85% solitary
72% right lobe
Enhanced through transmission
Figure 2-3-2
Amebic Abscess [Figure 2-3-2]

Amebic Abscess: CT
Enhancing wall (3-15 mm)

Round
Smooth or irregular
Peripheral zone of edema
Low attenuation/complex fluid
Septations
Fluid/debris level
Extrahepatic extension
Amebic abscess
285
Hepatic Infection
Amebic Abscess
Figure 2-3-3
Amebic vs. Pyogenic Abscess
Cannot reliably differentiate by imaging

Patients with amebic abscess
More likely to have hepatomegaly
and diarrhea
History of recent travel or inhabitant
of high prevalence areas
Serologic tests positive in >90%
Amebic Abscess: Therapy
Medical therapy
Percutaneous biopsy of abscess wall
If serology does not confirm
diagnosis and clinical suspicion is high
Percutaneous drainage if
Large, >5 cm abscess
Left lobe
Biliary communication
Pregnancy
Perforation
Poor response to drug therapy
Worldwide distribution of E. granulosus
Figure 2-3-4
Amebic Abscess / Pyogenic

Abscess
Echinococcus: E. granulosus and
E. multilocularis
Worldwide distribution of E. multilocularis

Nomenclature
Hydatidosis is the infection by the larval tapeworm of the genus
Echinococcus
Endemic worldwide
Humans accidental host
Infection usually acquired during childhood
E. granulosus [Figure 2-3-3]
Figure 2-3-5
E. multilocularis [Figure 2-3-4]

Echinococcus
E. granulosus and
E. multilocularis
Symptoms occur during adulthood

Cyst enlargement
Erosion of cyst into peritoneal or
pleural cavity
Development of biliary
communication
Serology confirms diagnosis
Positive >80% of cases
Echinococcus [Figure 2-3-5]

Echinococcus: E. granulosus
Echinococcus: E. multilocularis
Hepatic Infection
Echinococcus lifecycle
286
E. granulosus: Imaging Features
Figure 2-3-6
Unilocular or multilocular cyst

Calcification in cyst wall
Internal debris (hydatid sand)
Complex cyst
Internal daughter cysts
Undulating membrane
(water lily sign)
Fibrous and avascular walls and
membranes
Low MR signal
No enhancement
Daughter cysts of E. granulosus
E. granulosus
Figure 2-3-7
E. granulosus:
Daughter cysts [Figure 2-3-6]
E. granulosus:
Water Lily Sign [Figures 2-3-7 and 2-3-8]
E. granulosus:
Complications and Treatment
Cyst rupture
Anaphylaxis
Biliary tract, peritoneal cavity
Pleural, pericardial cavity
Treatment
Surgical excision
Laparoscopic excision
Percutaneous drainage
+ sclerosing scolicidal agents
Laminated membranes and water lily sign of E. granulosus
Figure 2-3-8
E. multilocularis:
Pathologic Features
Alveolar hydatid disease

Propagation by external budding
Invade surrounding tissue
Infiltrative mass
No limiting host tissue
Resembles neoplasm
E. multilocularis:
Imaging Features
Ultrasound
Echogenic
Single or multiple
Ill-defined walls
Partially calcified
CT
Geographic
Infiltrating lesions
Amorphous calcification
Water lily sign of E. granulosus
Figure 2-3-9
E. multilocularis [Figure 2-3-9]
E. multilocularis
287
Hepatic Infection
Schistosomasis (Bilharziasis)
Figure 2-3-10
Trematode (fluke)
S. Japonicum, S. mansoni,
S. hematobium
Humans are definitive host
Mature in the portal venules
Migrate to deposit eggs
Intestine (S. japonicum, S.
mansoni)
Bladder (S. hematobium)
Schistosomasis: S. japonicum
Schistosomasis: S. mansoni
Schistosomasis: S. hematobium
Lifecycle of Schistosomiasis
Schistosomasis [Figure 2-3-10]
Figure 2-3-11
Schistosomasis [Figure 2-3-11]
Fibrosis
Symmers' fibrosis
Turtle back liver
Progressive portal vein occlusion
Presinusoidal portal hypertension
Schistosomasis: Imaging
Features
S. japonicum
Hepatic calcification
Turtle back configuration
S. mansoni
Low attenuation, rounded foci
Low attenuation, linear branching
bands
Symmers' fibrosis
Figure 2-3-12
Schistosomiasis japonicum
[Figure 2-3-12]
Biliary Parasites
Parasites that invade bile ducts

Trematodes
Clonorchis sinensis
Fasciola gigantica, Fasciola
hepatica
Opisthorchis viverrini
Opisthorchis felineus
Nematodes
Ascariasis lumbricoides
Cestodes
Taenia saginata
Hepatic Infection
Schistosomiasis japonicum on CT
288
Clonorchis sinensis [Figures 2-3-13 and 2-3-14]
Figure 2-3-13
Peripheral intrahepatic bile ducts

Dilatation of small intrahepatic ducts
Periductal fibrosis
Complications
Cholangitis
Cholangiohepatitis
Liver abscess
Cholangiocarcinoma
Figure 2-3-14
Lifecycle of Clonorchis sinensis
Cholangiogram shows a
filling defect, peripheral
intrahepatic strictures,
and dilatation due to
infestation of Clonorchis
sinensis
Fasciola Hepatica
Hepatic Infections in the Immunocompromised Host
Candidiasis
Pneumocystis Carinii
Herpes Simplex Virus
Liver Abscess
Pyogenic
Multiorganism
Disseminated Candidiasis
Synonym: hepatosplenic candidiasis

Pathogenesis
Prolonged neutropenia
Mucosal damage to the GI tract
Local invasion of candida with entry into the hepatosplenic circulation
Clinical manifestations
Neutropenic with fever
Return of neutrophil count
Organ Involvement
Spleen 94%, liver 75%, kidney 69%
Hepatosplenic Candidiasis: Pathology
Necrosis with minimal inflammation

Microabscesses with severe inflammation
Collagen formation/fibrosis
Granuloma formation
289
Hepatic Infection
Hepatosplenic Candidiasis: Sonography [Figures 2-3-15]
Type 1 lesion
wheel-within-a-wheel
Type 2 lesion
bulls-eye
Type 3 lesion-most common
hypoechoic nodule
Type 4 lesion
hyperechoic nodule
Figure 2-3-15
I
II
III
IV
Figure 2-3-16
Hepatosplenic Candidiasis:
CT Features [Figure 2-3-16]
Concentric rings
Hypodense nodules
Punctate calcification
Hepatosplenic Candidiasis:
MR Features
Low T1, high T2

Fat-suppressed T2 improves detection
Gd-FLASH most sensitive
Splenic gamna-gandy bodies false positive T1
Hepatic Infection
Hepatic candidiasis on MDCT
290
Hepatosplenic Candidiasis: Imaging Management
High index of suspicion

Imaging during neutropenia is often negative
Follow up studies if clinical suspicion high and prophylactic therapy
contraindicated
Prophylactic therapy
Biopsy
Lesions change morphology with healing
Pneumocystis jiroveci
Previously classified as Pneumocystis carinii

Now considered a fungus
Opportunistic infection
AIDS
Organ transplant recipients
Pneumocystis jiroveci (carinii): Imaging Features Figure 2-3-17]
Sonography
Nonshadowing hyperechoic nodules
Shadowing echogenic clumps of calcification
CT scan
Hypodense nodules with progressive calcification
Renal and lymph node calcification
Figure 2-3-17
Summary: Pyogenic Abscess
Transition zone
Cluster sign
Summary: Amebic Abscess
Cannot reliably distinguish from

pyogenic abscess on imaging
Percutaneous biopsy if necessary
Viable organisms in wall
Drainage if necessary
Summary: Echinococcus
E. granulosus
Daughter cysts
Water-lily sign
Rim-like calcification
E. multilocularis
Infiltrating mass
Calcification
Sonogram and CT of disseminated pneumocystis
Summary: Immunocompromised Hosts
Candidiasis
Neutropenics
Imaging negative during neutropenia
Imaging positive during WBC rebound
Pneumocystis carinii
Hyperechoic nodules
+/- shadowing
Hypodense on CT with progressive calcification
Renal and lymph node calcification
291
Hepatic Infection
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1994; 193:539-542.
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6. Cheung H, Lai YM, Loke TK, et al. The imaging diagnosis of hepatic schistosomiasis japonicum sequelae. Clin
Radiol 1996; 51:51-55.
7. Mortele KJ, Ros PR. Imaging of diffuse liver disease. Semin Liver Dis 2001; 21:195-212.
Hepatosplenic Candidiasis
1. Ho B, Cooperberg PL, Li DK, Mack L, Naiman SC, Grossman L. Ultrasonography and computed tomography of
hepatic candidiasis in immunosuppressed patients. J Ultrasound Med 1982; 1:157-159.
2. Shirkhoda A. CT findings in hepatosplenic and renal candidiasis. J Comput Assist Tomogr 1987; 11:795-798.
3. Pastakia B, Shawker TH, Thaler M, O'Leary T, Pizzo PA. Hepatosplenic candidiasis: wheels within wheels.
Radiology 1988; 166:417-421.
4. Thaler M, Pastakia B, Shawker TH, O'Leary T, Pizzo PA. Hepatic candidiasis in cancer patients: the evolving
picture of the syndrome. Ann Intern Med 1988; 108:88-100.
5. Gorg C, Weide R, Schwerk WB, Koppler H, Havemann K. Ultrasound evaluation of hepatic and splenic
microabscesses in the immunocompromised patient: sonographic patterns, differential diagnosis, and follow-up. J
Clin Ultrasound 1994; 22:525-529.
6. Rudolph J, Rodenwaldt J, Ruhnke M, Hamm B, Kopka L. Unusual enhancement pattern of liver lesions in
hepatosplenic candidiasis. Acta Radiol 2004; 45:499-503.
Hepatic Infection
292
Imaging of Chronic Liver Disease

Chronic Liver Disease: Objectives
Cirrhosis
Steatosis and steatohepatitis
Budd-Chiari
Disorders of Iron Deposition
Hemosiderosis
Hemochromatosis
Cirrhosis: Definition
Endpoint of chronic liver disease
Cirrhosis: Pathology
Hepatocyte injury and loss

Fibrosis
Nodule formation
Architectural reorganization
Nodules
Micronodular (<3mm)
Macronodular (>3mm)
Mixed
Liver volume
Early, hepatomegaly from inflammation
Late, small liver from fibrosis
Cirrhosis: Segmental Alterations in Volume
Common feature
Not fully understood
Alteration in blood flow
Atrophy
Segments IV, VI, VIII
Hypertrophy
Segments I, II, III
Cirrhosis: Imaging
Cannot assess severity

Role of imaging
Assess disease complications
Evaluation of portal hypertension
HCC surveillance
Cirrhosis: Sonography
Fibrosis
Increased parenchymal echogenicity
Decreased penetration of the ultrasound beam
Poor visualization of hepatic vasculature
Loss of triphasic hepatic vein doppler
Increased pulsatility of portal vein doppler
Nodules
Volume redistribution
Portal hypertension
293
Cirrhosis: CT
Figure 2-4-1
Morphologic changes
Volume redistribution
Nodules
Fibrosis
Prominent porta and fissures
Focal confluent fibrosis
Decreased parenchymal
enhancement
Mesenteric changes
Lymphadenopathy
Cirrhosis with fibrosis, altered enhancement and nodules on CT
Increased mesenteric attenuation
Cirrhosis: Volume redistribution and nodules
Figure 2-4-2
Cirrhosis: Volume redistribution

Cirrhosis: Fibrosis, altered
enhancement, nodules [Figure 2-4-1]
Cirrhosis: Mesenteric Changes,
Adenopathy [Figure 2-4-2]
Cirrhosis: Nodules
Chronic hepatitis B cirrhosis with nodules, mesenteric changes,

and adenopathy
Regenerative nodule
Benign
Proliferation of hepatocytes
Precursor to dysplastic nodule and HCC
Dysplastic nodule
Premalignant
Hepatocellular carcinoma
Figure 2-4-4
Cirrhosis: Regenerative Nodule
Benign proliferation of hepatocytes

Hemosiderin deposition
"Siderotic nodule"
Noncontrast scans helpful for detection
CT
Isodense with and without contrast
Hyperdense on noncontrast (siderotic nodule)
MR
Dark T1, T2, gradient echo
Bright T1 (rare), Dark T2
Best seen on GRE and T2 images
Cirrhosis: Regenerative Nodules on CT [Figures 2-4-3 and

2-4-4]
Figure 2-4-3
Cirrhosis with high attenuation regenerating nodules (siderotic

nodules) on noncontrast CT
294
Cirrhosis with regenerating nodules

on CT
Cirrhosis: Regenerative Nodules and HCC on MR
Figure 2-4-5
Cirrhosis: Regenerative Nodules on MR [Figure 2-4-5]

Cirrhosis: Dysplastic Nodule
Premalignant nodule
Nodule with histologic evidence of dysplasia
Very common
Most undetectable on CT and MR
Rarely seen on preoperative imaging
Imaging appearance variable
Detection of malignant transformation depends upon evidence
of angiogenesis
Arterial enhancment
Nodule in a nodule
MR bright T1 with central dark signal
MR dark T2 with central high signal
Cirrhosis: Dysplastic Nodule on MR

Cirrhosis: Hepatocellular Carcinoma [Figure 2-4-6]
Incidence in cirrhosis
20% of hepatitis B and C cirrhosis
10% of alcoholic cirrhosis
CT and MR equally accurate
70%-75% of patients
35%-40% of lesions
Late arterial phase imaging is key
Maximum contrast volume
4 to 5 ml/sec injection rate
Cirrhosis with regenerating nodules

on T2 MR and gross photography
Figure 2-4-6
Cirrhosis: HCC Detection
False positives
Transient hepatic attenuation (intensity) difference
(THAD/THID)
Focal confluent fibrosis (look for associated atrophy)
Enhancing regenerative nodule
Flash filling hemangioma
Early enhancing pseudolesions (arterioportal shunting)
Cirrhosis: Transient Hepatic Attenuation (Intensity)

Difference
Causes
Portal vein obstruction
Hepatic venous outflow obstruction
Adjacent benign or malignant mass
Arterioportal shunting
Aberrant venous drainage
Imaging features
Typical locations
Subcapsular
Adjacent to falciform ligament
No mass effect
Straight margins
Wedge shape
HCC in cirrhosis
295
Cirrhosis: Focal Confluent Fibrosis
Massive areas of fibrosis

Present in up to 30% of cirrhotic livers
Typical location
Anterior segment right lobe
Medial segment left lobe
Imaging
Focal mass
Wedge shape, radiating from
porta hepatis
Capsular retraction
Low density on noncontrast
CT
Isodense with contrast or
irregular enhancement
MR: low signal T1, high
signal T2
Figure 2-4-7
Cirrhosis: Hemangioma
Primary Biliary Cirrhosis
Primary biliary cirrhosis
Chronic cholestasis
Unknown etiology
Probably immune mediated
Middle-aged women
Median age 50
Female to male ratio 9:1
Primary Biliary Cirrhosis: CT [Figure 2-4-7]
Global or segmental atrophy

Nodules
Fibrosis
Lace-like pattern
Segmental
Focal confluent
Portal hypertension
Often present before morphologic changes
At risk for HCC
Fatty Liver Diseases
Steatosis
Alcohol-associated
Nonalcoholic fatty liver disease (NAFLD)
Steatohepatitis
Alcoholic steatohepatitis
Nonalcoholic steatohepatitis (NASH)
Steatosis
Nomenclature
Fatty infiltration, fatty change, nonalcoholic fatty liver disease (NAFLD)
Very common
Pathogenesis
Abnormal fatty acid metabolism
Insulin/glucagon imbalance
Shift to lipogenesis
296
Steatosis: Etiology
Figure 2-4-8
ETOH
Obesity, diabetes
Malnutrition
Parenteral nutrition
Hepatitis, hepatotoxins,
chemotherapy, hyperlipidemia, drugs
Malabsorption syndromes
Idiopathic
Sonography of diffuse fatty infiltration
Steatosis and Steatohepatitis:

Clinical Features
Asymptomatic
Mild RUQ pain
Mild hepatomegaly and/or tenderness on exam
Mild transaminase elevation
Figure 2-4-9
Steatosis: Sonography
Diffuse
Echogenic parenchyma
Poor visualization hepatic
vasculature
Absorption of sound
Focal
Focal fat
Focal sparing
Diffuse Steatosis: Sonography

[Figure 2-4-8]
Focal Steatosis: Sonography

Focal Fatty Sparing: Sonography
Focal fatty infiltration
Steatosis: CT
Normal liver noncontrast CT

30 to 60 HU
8 to 10 HU > spleen
Fatty liver noncontrast CT
10 HU < spleen
Fatty liver contrast CT
25 HU < spleen
Steatosis in Celiac Disease

Nonalcoholic Steatohepatitis (NASH): CT
NASH with Cirrhosis: CT
Focal Steatosis: CT [Figure 2-4-9]
Features of focal fat

No mass effect
Straight line margin
No contour abnormality
Often transient
Common locations
Falciform ligament
Subcapsular
Adjacent to porta hepatis
Adjacent to gallbladder fossa
297
Focal Steatosis: Common Locations
Figure 2-4-10
Focal Steatosis
vs. Focal Sparing
Focal Sparing [Figure 2-4-10]
Focal Steatosis: Transient
Steatosis: MR
Conventional spin echo typically

insensitive to fat deposition
Chemical shift imaging
Fat and water signal additive in-phase
Fat signal subtracted from water signal out-of-phase
Steatosis: 1.5T MR [Figure 2-4-11]
Focal fatty sparing
Figure 2-4-11
Steatosis: MR [Figure 2-4-12]

Multifocal Steatosis: Pseudometastatic Disease
Budd-Chiari Syndrome

Primary
Membranous (web) obstruction of hepatic veins
Secondary
Hypercoaguable states, infections, neoplasms, trauma
1.5 T MR fat and water proton signal

intensity
Budd-Chiari Syndrome: Clinical
Acute fulminant disease

Total obstruction
Rare
Subacute/Chronic
Vague illness, 6 months duration
Ascites
Triad of hepatomegaly, ascites, pain
Figure 2-4-12
Budd-Chiari Syndrome:
Pathophysiology
Sinusoidal dilatation
Increase sinusoidal pressure
Centrolobular hepatocyte necrosis
Centrolobular fibrosis
Lobular collapse
Nodular regeneration
Focal fat on in-phase images and out-of-phase images
Budd-Chiari Syndrome: Pathology
Acute
Hepatomegaly
Sinusoidal dilatation
Hemorrhagic necrosis
Chronic
Fibrosis
Cirrhosis
298
Budd Chiari Syndrome: Imaging
Figure 2-4-13
Vascular changes
Hepatic vein stenosis
Intravascular thrombus
Web-like stenosis or narrowed IVC
Intrahepatic collaterals
Parenchymal changes
Nonvisible hepatic veins
Cirrhosis
Nodular regenerative hyperplasia
Hepatic Venous Waveforms [Figure 2-4-13]

Budd-Chiari Syndrome:
Enhancement Patterns
Noncontrast
Heterogeneous hypodensity
Hepatic parenchymal congestion
Hyperdense thrombi
Patchy enhancement
Normal central hepatic, left lobe, and caudate lobe
enhancement
Late peripheral enhancement
Normal hepatic vein (upper image)

and Budd Chiari (lower image)
Figure 2-4-14
[Figure 2-4-14]
Budd Chiari Syndrome

[Figure 2-4-15]
Budd Chiari Syndrome: MR

[Figure 2-4-16]
Narrowed veins
Intraluminal thrombus
Collaterals
Budd Chiari
Figure 2-4-15
Figure 2-4-16
Budd Chiari
Budd-Chiari
299
Budd Chiari from IVC Web

Budd Chiari and Nodular Regenerative Hyperplasia
Functional Classification
Hemosiderosis
Iron accumulation in the reticuloendothelial system
Iron in the liver with no organ damage
Hemochromatosis
Iron in hepatocytes with eventual fibrosis and cirrhosis
Two types
Hereditary hemochromatosis
Secondary hemochromatosis
High risk for HCC
Hemosiderosis in Sickle Cell Anemia [Figure 2-4-17]

Hemochromatosis
Hereditary hemochromatosis
Increased intestinal absorption of iron
Iron predominantly within hepatocytes
Highest incidence of cirrhosis and HCC (14%)
Secondary hemochromatosis
Multiple transfusions
Iron predominantly in the reticuloendothelial system
Hereditary Hemochromatosis (HHC): Clinical Features
Hyperpigmentation
Diabetes mellitus (bronze diabetes)
Hepatomegaly
Chondrocalcinosis/osteoarthritis
Cardiomyopathy
Figure 2-4-17
Hereditary Hemochromatosis
(HHC): Pathology
Hemosiderosis
Hemochromatosis: Increased CT
Attenuation (75-135 HU)
Figure 2-4-18
Increased Hepatic CT
Attenuation
Differential Diagnosis
Iron deposition
Glycogen storage disease
Amiodarone
Wilsons disease
Chronic arsenic poisoning
Hemochromatosis: MR
T2*-gradient echo imaging is most sensitive

Quantitate with liver:muscle ratio
Decrease signal on T2-weighted images
Hereditary = iron in liver and pancreas
Secondary = iron in liver and spleen
Hereditary Hemochromatosis [Figure 2-4-18]
300
Secondary Hemochromatosis
Summary: Cirrhosis
Endpoint of chronic liver disease

Nodules
Regenerative
Dysplastic
HCC
HCC false positives
Summary: Fatty Infiltration
Steatosis
Steatohepatitis
Summary: Focal Steatosis
No mass effect
Straight line margins
Typical Locations
Subcapsular
Falciform ligament
Chemical shift MR
Signal loss on out-of-phase images
Summary: Budd-Chiari Syndrome

Sonography
CT enhancement
Early central
Late peripheral
MR
Summary: Disorders of Iron Overload
Hemosiderosis
Hemochromatosis
Hereditary
Secondary
References
Cirrhosis
1. Baron RL, Peterson MS. From the RSNA refresher courses: screening the cirrhotic liver for hepatocellular carcinoma
with CT and MR imaging: opportunities and pitfalls. Radiographics 2001; 21 Spec No:S117-132.
2. Brancatelli G, Baron RL, Peterson MS, Marsh W. Helical CT screening for hepatocellular carcinoma in patients with
cirrhosis: frequency and causes of false-positive interpretation. AJR Am J Roentgenol 2003; 180(4):1007-1014.
3. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, Federle MP. Spectrum of imaging findings of the liver in end-stage cirrhosis:
Part II, focal abnormalities. AJR Am J Roentgenol 1999; 173(5):1185-1192.
4. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, Federle MP. Spectrum of imaging findings of the liver in end-stage cirrhosis:
part I, gross morphology and diffuse abnormalities. AJR Am J Roentgenol 1999; 173(4):1031-1036.
5. Hussain HK, Syed I, Nghiem HV, et al. T2-weighted MR imaging in the assessment of cirrhotic liver. Radiology
2004; 230(3):637-644.
6. Ohtomo K, Baron RL, Dodd GD, 3rd, et al. Confluent hepatic fibrosis in advanced cirrhosis: appearance at CT.
Radiology 1993; 188(1):31-35.
7. Ohtomo K, Itai Y, Ohtomo Y, Shiga J, Iio M. Regenerating nodules of liver cirrhosis: MR imaging with pathologic
correlation. AJR Am J Roentgenol 1990; 154(3):505-507.
8. Shimizu A, Ito K, Koike S, Fujita T, Shimizu K, Matsunaga N. Cirrhosis or chronic hepatitis: evaluation of small
(<or=2-cm) early-enhancing hepatic lesions with serial contrast-enhanced dynamic MR imaging. Radiology 2003;
226(2):550-555.
301
Steatosis/Steatohepatitis
1. Yoshikawa J, Matsui O, Takashima T, et al. Focal fatty change of the liver adjacent to the falciform ligament: CT and
sonographic findings in five surgically confirmed cases. AJR Am J Roentgenol 1987; 149:491-494.
2. Jain KA, McGahan JP. Spectrum of CT and sonographic appearance of fatty infiltration of the liver. Clin Imaging
1993; 17:162-168.
3. Siegelman ES. MR imaging of diffuse liver disease. Hepatic fat and iron. Magn Reson Imaging Clin N Am 1997;
5:347-365.
4. Jacobs JE, Birnbaum BA, Shapiro MA, et al. Diagnostic criteria for fatty infiltration of the liver on contrast-enhanced
helical CT. AJR Am J Roentgenol 1998; 171:659-664.
5. Kroncke TJ, Taupitz M, Kivelitz D, et al. Multifocal nodular fatty infiltration of the liver mimicking metastatic disease
on CT: imaging findings and diagnosis using MR imaging. Eur Radiol 2000; 10:1095-1100.
6. Brunt EM. Nonalcoholic steatohepatitis: definition and pathology. Semin Liver Dis 2001; 21:3-16.
7. Siegelman ES, Rosen MA. Imaging of hepatic steatosis. Semin Liver Dis 2001; 21:71-80.
8. Kemper J, Jung G, Poll LW, Jonkmanns C, Luthen R, Moedder U. CT and MRI findings of multifocal hepatic steatosis
mimicking malignancy. Abdom Imaging 2002; 27:708-710.
1. Noone TC, Semelka RC, Siegelman ES, et al. Budd-Chiari syndrome: spectrum of appearances of acute, subacute,
and chronic disease with magnetic resonance imaging. J Magn Reson Imaging 2000; 11:44-50.
4. Brancatelli G, Federle MP, Grazioli L, Golfieri R, Lencioni R. Large regenerative nodules in Budd-Chiari syndrome
and other vascular disorders of the liver: CT and MR imaging findings with clinicopathologic correlation. AJR Am
J Roentgenol 2002; 178:877-883.
5. Maetani Y, Itoh K, Egawa H, et al. Benign hepatic nodules in Budd-Chiari syndrome: radiologic-pathologic correlation
with emphasis on the central scar. AJR Am J Roentgenol 2002; 178:869-875.
1. Bonkovsky HL: Disorders of iron overload. In Bloomer JR, Goodman ZD, Ishak KG (eds): Clinical and pathologoical
correlations in liver disease: approaching the next millennium. Washington, DC: Armed Forces Institute of Pathology,
1998
2. Gandon Y: Iron, liver, and MRI. http://www.radio.univ-rennes1.fr/Sources/EN/Hemo.html, 2001
3. Siegelman ES, Mitchell DG, Semelka RC: Abdominal iron deposition: metabolism, MR findings, and clinical
importance. Radiology 199:13, 1996
302
Benign Biliary Disease

Objectives
Congenital Disorders
Caroli disease
Choledochal cyst
Polycystic Liver Disease
Inflammatory Disorders
AIDS-related cholangiopathy
Recurrent Pyogenic Cholangitis
Acute Pyogenic Cholangitis
Obstructive biliary dilatation

Caroli disease
Choledochal cyst
Polycystic liver disease
Cholangitis
RPC, Pyogenic
Figure 2-5-1
41-year-old male with history of renal

stones and diagnosis of medullary
sponge kidney presents with abdominal
pain, sepsis, elevated LFTs [Figure 2-5-1]
Obstructive Biliary Dilatation
Tubular dilatation
Diffuse dilatation proximal to the obstruction
Abrupt termination at level of obstruction
Congenital Disorders
Caroli disease
Choledochal cyst
Saccular and fusiform biliary dilatation in

Caroli disease
Caroli Disease
Autosomal recessive
Secondary to ductal plate malformation (DPM)
Associated with renal disorders
ARPCKD, ADPCKD
Medullary sponge kidney
Medullary cystic disease
Ductal Plate
Embryologic precursor of intrahepatic bile ducts
Ductal Plate Malformation
Abnormal development of intrahepatic bile ducts

Lack of ductal plate remodeling
Persistence of embryonic structures (DPM)
Segmental dilatation
Destructive inflammation/fibrosis
Spectrum of diseases
Small interlobular ducts: Congenital hepatic fibrosis (CHF)
Large intrahepatic ducts: Caroli disease
All ducts: Caroli syndrome (CHF and Caroli disease)
303
Intrahepatic Duct Embryology: Ductal Plate

Figure 2-5-2
Figure 2-5-3
Fusion and
absorption of ductal
plate cells
Normal ductal plate development of the intrahepatic bile ducts
Single layer of ductal Double layer of

ductal plate cells
plate cells
Caroli Disease: Clinical Features
Presentation at any age (mean age 37 years)

Pain, fever, jaundice
Recurrent cholangitis
Stone formation
Liver failure
Fibrosis
Caroli Disease: Complications
Recurrent biliary stones

Recurrent cholangitis
Hepatic abscess
Fibrosis/cirrhosis
Cholangiocarcinoma (7%)
Caroli Disease: Radiologic Features
Intrahepatic duct dilatation

Distribution: segmental (83%) or diffuse (17%)
Shape: saccular (76%) or fusiform (24%)
Central dot sign
Extrahepatic duct dilatation
53% of cases
Secondary to cholangitis and stone passage
Cirrhosis
Normal ductal plate remodeling

results in intercommunicating
intrahepatic bile ducts
surrounding a normal portal
tract.
Ductal plate malformation
results in biliary duct ectasia
and fibrosis surrounding the
portal tract
Figure 2-5-4
Caroli Disease: Central Dot Sign [Figure 2-5-4]
Caroli disease showing saccular

biliary dilatation and
the central dot sign
304
Caroli Syndrome Cirrhosis, Portal Hypertension

[Figure 2-5-5]
Figure 2-5-5
Caroli Disease Cholangiography

Caroli Disease: Segmental
Caroli Disease: Hepatic Abscess
Caroli Disease: Intrahepatic Lithiasis
Caroli Disease: Intraductal Cholangiocarcinoma
Caroli Disease: Cholangiocarcinoma
Enhancing intraductal masses

Focal strictures
Irregular margins
Shoulders
Irregular tapering
Infiltrating masses
Choledochal Cyst
Congenital dilatation of the bile ducts

Association with anomalous pancreatico-biliary junction (APBJ)
Figure 2-5-6
Choledochal Cyst:
Clinical Features
Presentation at any age

(mean, 17 years)
F>M
Pain, jaundice, palpable
mass
Complications
Stones
Cholangitis
Malignancy
Choledochal Cyst:
Etiology [Figures 2-5-6 and 2-5-7]
Caroli disease in two different patients

showing diffuse fusiform and saccular
dilatation
Normal pancreaticobiliary junction showing union of the ducts in the

sphincter complex, which is located in the duodenal wall. There may be a
common channel (ampulla) or not
Normal Pancreaticobiliary
Junction
Sphincter complex encircles distal CBD and PD
80% to 90% have a common channel (4-5 mm
Anomalous Junction (APBJ)
Union of CBD and PD outside of duodenum and sphincter
complex
Reflux of pancreatic enzymes into CBD)
Figure 2-5-7
Choledochal Cyst: Pathologic Features
Extrahepatic duct dilatation

Mural thickening
Normal epithelium
Anomalous pancreaticobiliary
junction showing union of the
common bile duct and pancreatic
duct proximal to the duodenal wall
and sphincter complex
305
Choledochal Cyst: Todani Classification [Figure 2-5-8]
Figure 2-5-8
Todani Type I
Todani Type II: Diverticulum
Todani Type III:
Choledochocele
Tubulovillous Adenoma
Todani Type IV [Figure 2-5-9]
Todani Type V: Caroli Disease
Is Todani Type V Caroli
Disease?
Choledochal cyst
Congenital, not inherited
Extrahepatic bile duct
dilatation with varying degrees
Todani classification of choledochal cysts
of proximal dilatation
Surgical therapy with biliary reconstruction
Caroli disease
Figure
Congenital, inherited
Intrahepatic +/- extrahepatic dilatation
Liver biopsy shows DPM
Medical therapy (surgery for complications)
2-5-9
Malignancy and Choledochal Cyst
Adenocarcinoma most common

Locations
Within the choledochal cyst
Gallbladder
Biliary tract
Malignancy and Choledochal Cyst

Todani Type IV choledochal cyst showing central

Two forms
intrahepatic and extrahepatic duct dilatation on CT.
Isolated to the liver
The cholangiogram shows an anomalous
Occurring with ADPCKD
pancreaticobiliary junction as well as the extent of
Bile duct cysts
duct dilatation
Secondary to von Meyenberg complexes
Von Meyenberg complex is DPM at the lowest level
Polycystic Liver Disease - Radiologic Features
Multiple liver cysts

May have internal hemorrhage
Normal bile ducts
Associated feature of ADPCKD
Renal cysts
Pancreatic cysts
Thyroid cysts
Seminal vesicle cysts in males
306
Figure 2-5-10
Polycystic Liver Disease in ADPCKD [Figure 2-5-10]

Polycystic Liver Disease without ADPCKD
Bile ducts
Displaced, but normal
Rare, mucosal irregularity
41-year-old male with history of renal stones and

diagnosis of medullary sponge kidney presents with
abdominal pain, sepsis, elevated LFTs
Obstructive biliary dilatation

Caroli disease
Choledochal cyst
Cholangitis
RPC, Pyogenic
Polycystic liver disease occurring in

ADPCKD
Figure 2-5-11
Caroli Disease
Summary: Congenital
Disorders
Exclude obstructive
dilatation
Congenital disorders
Caroli disease
Intrahepatic
Choledochal cyst
Extrahepatic
Noncommunicating
cysts
40-year-old woman with elevated LFTs

[Figure 2-5-11]
Figure 2-5-12
Cholangitis
Primary sclerosing
AIDS-related
Recurrent pyogenic
Acute pyogenic cholangitis
Neoplasm
Inflammatory Disorders

AIDS-related cholangiopathy
Primary Sclerosing Cholangitis [Figure 2-5-12]
Cholestatic liver disease

Unknown etiology
Fibrosing inflammation
Diagnosis
Liver biopsy
Cholangiogram
Illustration showing disease distribution, mural

thickening, and inflammatory changes of primary
sclerosing cholangitis
307
PSC - Imaging
Thickened duct wall

Asymmetric or circumferential
2 to 5 mm
Hepatic parenchymal changes
Cirrhosis
Periportal fibrosis
Discontinuous duct dilatation
Portal hypertension
Distribution
Intrahepatic 100%
Extrahepatic 70%
Cystic duct 30%
Pancreatic duct (rare)
Other features
Gallbladder disease (40%)
Ductal stones (8%)
Adenopathy
Neoplasm
Figure 2-5-13
CT of primary sclerosing cholangitis showing discontinuous bile

duct dilatation, mural thickening of the common hepatic duct,
and hepatoduodenal ligament adenopathy
Figure 2-5-14
PSC: Sonographic Features

PSC: CT Features [Figure 2-5-13]
PSC: Cholangiography
Beading
Pruned-tree
Mural irregularity
Diverticula
[Figure 2-5-14]
MRCP
PSC: Cholangiocarcinoma
Stricture (90%)
Long strictures (>1cm)
Completely obstructing strictures
Associated mass
Multicentric (10%)
Polypoid mass
ERCP of primary sclerosing cholangitis showing beading and

pruning of the intrahepatic bile ducts. The extrahepatic bile
duct shows mural irregularity with focal stricture formation
Figure 2-5-15
AIDS Cholangiopathy [Figure 2-5-15]
Group of disorders
Sclerosing cholangitis
Papillary stenosis
Acalculous cholecystitis
Cryptosporidium
Cytomegalovirus
Declining incidence
HAART therapy
AIDS Cholangiopathy
Cholangiographic features
Beading
Pruning
Mural irregularity
Filling defects (granulation tissue)
Papillary stenosis (papillitis)
No EHD stenosis or diverticula
Illustration of AIDS cholangiopathy showing
disease distribution
308
AIDS Cholangiopathy [Figure 2-5-16]
Figure 2-5-16
AIDS Cholangiopathy
Sonographic features
Gallbladder wall thickening
Bile duct wall thickening
Hyperechoic nodule in distal
CBD (papillitis)
AIDS Cholangiopathy
[Figure 2-5-17]
Recurrent Pyogenic
Cholangitis (RPC) [Figure 2-5-18]
Clinical syndrome
Pigmented stones
Recurrent infection
Unknown etiology
Biliary parasites
Malnutrition
Portal bacteremia
Complications
Biliary cirrhosis
Cholangiocarcinoma
RPC: Imaging
AIDS cholangiopathy with papillary stenosis
Figure 2-5-17
[Figure 2-5-19]
Stones, sludge
Duct dilatation
Left lobe predominant
Parenchymal changes
Atrophy
Fatty change
Altered enhancement
Abscess
Acalculous cholecystitis in AIDS
Figure 2-5-18
RPC
Acute Pyogenic Cholangitis [Figure 2-5-20]
Almost always post-obstructive

Polymicrobial
Etiology
Stones
Anastomotic stricture
Papillary stenosis
Carcinoma
Underlying biliary disease
Acute Pyogenic Cholangitis
Imaging Features
Duct dilatation
Obstructive lesion
Echogenic bile
Mural irregularity
Hepatic abscess
Illustration of recurrent pyogenic cholangitis

showing typical disease distribution
309
Figure 2-5-19
Recurrent pyogenic cholangitis with intrahepatic lithiasis and duct dilatation
Figure 2-5-20
Acute Pyogenic Cholangitis with
Microabscesses
40-year-old woman with elevated LFTs
Cholangitis
Primary sclerosing
AIDS-related
Recurrent pyogenic
Neoplasm
Primary Sclerosing Cholangitis

Summary
PSC
Fibrosis
AIDS cholangiopathy
Papillary stenosis
RPC
Stones
Focal dilatation
Pyogenic cholangitis
Obstruction
Illustration showing typical manifestations of acute

pyogenic cholangitis
References
Caroli Disease
1. Choi BI, Yeon KM, Kim SH, et al: Caroli disease: central dot sign in CT. Radiology 174:161, 1990
2. Desmet VJ: Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo
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3. Krause D, Cercueil JP, Dranssart M, et al: MRI for evaluating congenital bile duct abnormalities. J Comput Assist
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4. Levy AD, Rohrmann CA, Jr., Murakata LA, et al: Caroli's disease: radiologic spectrum with pathologic correlation.
AJR 179:1053, 2002
5. Marchal GJ, Desmet VJ, Proesmans WC, et al: Caroli disease: high-frequency US and pathologic findings.
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Abdom Imaging 21:117, 1996
Pavone P, Laghi A, Catalano C, et al: Caroli's disease: evaluation with MR cholangiography. AJR 166:216, 1996
Choledochal Cyst
1. Babbitt DP, Starshak RJ, Clemett AR: Choledochal cyst: a concept of etiology. AJR 119:57, 1973
2. Govil S, Justus A, Korah I, et al: Choledochal cysts: evaluation with MR cholangiography. Abdom Imaging
23:616, 1998
3. Levy AD, Rohrmann CA, Jr.: Biliary cystic disease. Curr Probl Diagn Radiol 32:233, 2003
4. Liu CL, Fan ST, Lo CM, et al: Choledochal cysts in adults. Arch Surg 137:465, 2002
5. O'Neill JA, Jr.: Choledochal cyst. Curr Probl Surg 29:361, 1992
6. Savader SJ, Benenati JF, Venbrux AC, et al: Choledochal cysts: classification and cholangiographic appearance.
AJR 156:327, 1991
7. Savader SJ, Venbrux AC, Benenati JF, et al: Choledochal cysts: role of noninvasive imaging, percutaneous
transhepatic cholangiography, and percutaneous biliary drainage in diagnosis and treatment. J Vasc Interv Radiol
2:379, 1991
8. Todani T, Watanabe Y, Fujii T, et al: Anomalous arrangement of the pancreatobiliary ductal system in patients with
a choledochal cyst. Am J Surg 147:672, 1984
9. Todani T, Watanabe Y, Narusue M, et al: Congenital bile duct cysts: Classification, operative procedures, and
review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263, 1977
10. Wearn FG, Wiot JF: Choledochocele: not a form of choledochal cyst. J Can Assoc Radiol 33:110, 1982
1. Dranssart M, Cognet F, Mousson C, et al: MR cholangiography in the evaluation of hepatic and biliary
abnormalities in autosomal dominant polycystic kidney disease: study of 93 patients. J Comput Assist Tomogr
26:237, 2002
2. Grunfeld JP, Albouze G, Jungers P, et al: Liver changes and complications in adult polycystic kidney disease. Adv
Nephrol Necker Hosp 14:1, 1985
3. Gupta S, Seith A, Dhiman RK, et al: CT of liver cysts in patients with autosomal dominant polycystic kidney
disease. Acta Radiol 40:444, 1999
4. Itai Y, Ebihara R, Eguchi N, et al: Hepatobiliary cysts in patients with autosomal dominant polycystic kidney
disease: prevalence and CT findings. AJR 164:339, 1995
5. Pirson Y, Lannoy N, Peters D, et al: Isolated polycystic liver disease as a distinct genetic disease, unlinked to
polycystic kidney disease 1 and polycystic kidney disease 2. Hepatology 23:249, 1996
6. Segal AJ, Spataro RF: Computed tomography of adult polycystic disease. J Comput Assist Tomogr 6:777, 1982
Primary Sclerosing Cholangitis
1. Ament AE, Haaga JR, Wiedenmann SD, et al: Primary sclerosing cholangitis: CT findings. J Comput Assist
Tomogr 7:795, 1983
2. Brandt DJ, MacCarty RL, Charboneau JW, et al: Gallbladder disease in patients with primary sclerosing
cholangitis. AJR Am J Roentgenol 150:571, 1988
3. Campbell WL, Ferris JV, Holbert BL, et al: Biliary tract carcinoma complicating primary sclerosing cholangitis:
evaluation with CT, cholangiography, US, and MR imaging. Radiology 207:41, 1998
4. Campbell WL, Peterson MS, Federle MP, et al: Using CT and cholangiography to diagnose biliary tract carcinoma
complicating primary sclerosing cholangitis. AJR Am J Roentgenol 177:1095, 2001
5. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, et al: End-stage primary sclerosing cholangitis: CT findings of hepatic
morphology in 36 patients. Radiology 211:357, 1999
6. Fulcher AS, Turner MA, Franklin KJ, et al: Primary sclerosing cholangitis: evaluation with MR cholangiography-a
case-control study. Radiology 215:71, 2000
7. Gulliver DJ, Baker ME, Putnam W, et al: Bile duct diverticula and webs: nonspecific cholangiographic features of
primary sclerosing cholangitis. AJR Am J Roentgenol 157:281, 1991
8. Ito K, Mitchell DG, Outwater EK, et al: Primary sclerosing cholangitis: MR imaging features. AJR Am J
Roentgenol 172:1527, 1999
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Lumsden AB, Alspaugh JP: Cholangiocarcinoma complicating primary sclerosing cholangitis: cholangiographic
appearances. Radiology 158:856, 1986
MacCarty RL, LaRusso NF, Wiesner RH, et al: Primary sclerosing cholangitis: findings on cholangiography and
pancreatography. Radiology 149:39, 1983
Majoie CB, Smits NJ, Phoa SS, et al: Primary sclerosing cholangitis: sonographic findings. Abdom Imaging
20:109, 1995
May GR, Bender CE, LaRusso NF, et al: Nonoperative dilatation of dominant strictures in primary sclerosing
cholangitis. AJR Am J Roentgenol 145:1061, 1985
Olsson R, Danielsson A, Jarnerot G, et al: Prevalence of primary sclerosing cholangitis in patients with ulcerative
colitis. Gastroenterology 100:1319, 1991
Teefey SA, Baron RL, Rohrmann CA, et al: Sclerosing cholangitis: CT findings. Radiology 169:635, 1988
Teefey SA, Baron RL, Schulte SJ, et al: Patterns of intrahepatic bile duct dilatation at CT: correlation with
obstructive disease processes. Radiology 182:139, 1992
Williams SM, Harned RK: Hepatobiliary complications of inflammatory bowel disease. Radiol Clin North Am
25:175, 1987
AIDS Cholangiopathy
1. Chen XM, LaRusso NF: Cryptosporidiosis and the pathogenesis of AIDS-cholangiopathy. Semin Liver Dis 22:277,
2002
2. Collins CD, Forbes A, Harcourt-Webster JN, et al: Radiological and pathological features of AIDS-related
polypoid cholangitis. Clin Radiol 48:307, 1993
3. Da Silva F, Boudghene F, Lecomte I, et al: Sonography in AIDS-related cholangitis: prevalence and cause of an
echogenic nodule in the distal end of the common bile duct. AJR Am J Roentgenol 160:1205, 1993
4. Defalque D, Menu Y, Girard PM, et al: Sonographic diagnosis of cholangitis in AIDS patients. Gastrointest Radiol
14:143, 1989
5. Dolmatch BL, Laing FC, Ferderle MP, et al: AIDS-related cholangitis: radiographic findings in nine patients.
Radiology 163:313, 1987
Recurrent Pyogenic Cholangitis
1. Chan FL, Man SW, Leong LL, et al: Evaluation of recurrent pyogenic cholangitis with CT: analysis of 50 patients.
Radiology 170:165, 1989
2. Federle MP, Cello JP, Laing FC, et al: Recurrent pyogenic cholangitis in Asian immigrants. Use of
ultrasonography, computed tomography, and cholangiography. Radiology 143:151, 1982
3. Jeyarajah DR: Recurrent Pyogenic Cholangitis. Curr Treat Options Gastroenterol 7:91, 2004
4. Kim MJ, Cha SW, Mitchell DG, et al: MR imaging findings in recurrent pyogenic cholangitis. AJR Am J
5. Okuno WT, Whitman GJ, Chew FS: Recurrent pyogenic cholangiohepatitis. AJR Am J Roentgenol 167:484, 1996
6. Park MS, Yu JS, Kim KW, et al: Recurrent pyogenic cholangitis: comparison between MR cholangiography and
direct cholangiography. Radiology 220:677, 2001
312
Biliary Neoplasms
Objectives
Biliary adenocarcinoma
Intraductal cholangiocarcinoma
Hilar (Klatskin) cholangiocarcinoma
Extrahepatic duct adenocarcinoma
Benign strictures
Other neoplasms
Biliary Adenocarcinoma
Incidence in U.S.
~2000 to 2500 cases per year
Incidence worldwide
Up to 10 times greater in Asian countries
More common in men
2:1
High risk groups

Autoimmune diseases
PSC, ulcerative colitis, primary biliary cirrhosis
Congenital anatomic anomalies
Caroli, choledochal cyst, anomalous pancreaticobiliary junction
Abnormal tumor suppressor genes, FAP, NF1
Infection
Biliary parasites, recurrent pyogenic cholangitis
Jaundice
Pain
Fever if secondary cholangitis
Moderately to well differentiated

Desmoplastic stroma
Infiltrative margins
NO CAPSULE
Dismal 5-year survival

< 20% resectable at diagnosis
Curative resection
Tumor free margins
No touch technique
Intraductal Cholangiocarcinoma
Hilar Cholangiocarcinoma
313
Biliary Neoplasms
Figure 2-6-1
Extrahepatic bile duct adenocarcinoma
Intrahepatic Cholangiocarcinoma:
Pathologic Features
Solitary, large mass

No capsule
Dense fibrous stroma
No necrosis or hemorrhage
Multifocal mass
Satellite lesions
Intrahepatic metastasis
Intrahepatic Cholangiocarcinoma [Figure 2-6-1]
Delayed enhancement
Peripheral biliary dilatation
Metastasis
HCC
Gallbladder adenocarcinoma
Rare, sarcoma
Identifying key features of ICC
Evidence of fibrous stroma
Contrast enhancement pattern
Intraductal Cholangiocarcinoma [Figure 2-6-2]
Rare
Intrabiliary mass
Biliary diliatation peripheral to the mass
Intrahepatic cholangiocarcinoma with

delayed enhancement and capsular
contraction
Hilar Cholangiocarcinoma: Klatskin Tumor
Tumors arising at or near the confluence of the right and left hepatic ducts
Most common site of biliary adenocarcioma
Aggressive biologic behavior
Figure 2-6-2
Imaging features
Anatomic location
Pathologic features
Imaging features
Duct dilatation
Ill-defined mass
Lobar atrophy
Vascular invasion
Hilar Cholangiocarcinoma: Imaging Features [Figure 2-6-3]
Dilated ducts
Discontinuous ducts
Poorly defined mass
Poor visibility of tumor mass
Minimal tumor enhancement on CT (50% of cases)
More likely to enhance on MR
Parenchymal invasion (segment IV) 30% of cases
Lobar or segmental atrophy
Secondary to vascular compromise
Biliary Neoplasms
314
Intraductal cholangiocarcinoma.
There is an intrabiliary mass on the
CT and corresponding gross
photograph
Hilar Cholangiocarcinoma [Figure 2-6-4]
Figure 2-6-3
Hilar Cholangiocarcinoma [Figure 2-6-5]
Cholangiographic features of malignant strictures

Evidence of mass effect
Irregular margins
Irregular or abrupt tapering at obstruction
Limitations of MRCP
Spatial resolution
Evaluation of secondary ducts
Role of Preoperative Imaging
Determination of resectablility
Surgical planning
Bismuth-Corlette classification1
Define extent of duct involvement
1Bismuth H, Corlette MB. Surg Gynecol Obstet 1975, 140: 170-178.
Hilar Cholangiocarcinoma: Unresectability
Bilateral tumor extension

Into secondary ducts
Into hepatic parenchyma
Hepatic artery or portal vein
Occlusion main portal vein
N2 nodes
Distant mets
Medically unfit patients
Classic sonographic appearance

of hilar cholangiocarcinoma
showing biliary dilatation and an
ill-defined hilar mass
Figure 2-6-4
Bismuth-Corlette: Type I
Tumor below confluence
Bismuth-Corlette: Type II
[Figure 2-6-8]
Tumor at confluence
Hilar cholangiocarcinoma. Ill-defined mass adjacent to bile duct stent

and extending into hepatoduodenal ligament
Bismuth-Corlette: Type IIIa and IIIb [Figure 2-6-9]
Tumor extends to right or left

hepatic ducts
Hemiliver resection
Preoperative PV embolism
Figure 2-6-5
Bismuth-Corlette: Type IIIa

Bismuth-Corlette: Type IV
[Figures 2-6-10 and 2-6-11]
Tumor in bilateral ducts
Hilar cholangiocarcinoma on MRCP and percutaneous transhepatic

cholangiography
315
Biliary Neoplasms
Figure 2-6-6
Figure 2-6-7
Figure 2-6-8
Bismuth Corlette Type II hilar

cholangiocarcinoma. Tumor involves
the confluence
Bismuth Corlette Type I hilar

cholangiocarcinoma. Tumor is
below the confluence of the right
and left hepatic ducts
Bismuth Corlette Type I
Figure 2-6-10
Figure 2-6-9
Bismuth Colette Type IV hilar

bilateral intrahepatic ducts
Figure 2-6-11
Bismuth Colette Type IIIa (upper)

and IIIb (lower) hilar
the right (IIIa) or left (IIIb) hepatic
duct
Bismuth Corlette Type IV

Biliary Neoplasms
316
EHBD Adenocarcinoma [Figure 2-6-12]
Figure 2-6-12
Variable morphology
Diffusely infiltrating
Polypoid
Nodular
Constricting (scirrhous)
EHBD Adenocarcinoma: Imaging
Biliary obstruction
Tumor
Intraluminal mass
Stenosis
Complete obstruction
Papillary Adenocarcinoma [Figure 2-6-13]

EHBD Adenocarcinoma vs. Benign Stricture
Malignant
Duct abruptly terminates at stricture
Benign
Duct tapers to stricture
Baron, RL. Radiol Clin N Am

1991. 29:1237.
Figure 2-6-13
Morphologic types of extrahepatic

duct adenocarcinoma
Papillary adenocarcinoma of the extrahepatic bile duct
Malignant Stricture [Figures 2-6-14 and 2-6-15]

EHBD Adenocarcinoma
Figure 2-6-14
Figure 2-6-15
Cholangiographic and CT features of a malignant

stricture
Malignant stricture
317
Biliary Neoplasms
Benign Stricture [Figures 2-6-16 and 2-6-17]
Figure 2-6-16
Pancreatitis with Stricture

Post-inflammatory benign strictures

Pancreatitis
Post radiation or chemotherapy
Inflammatory strictures
AIDS cholangiopathy
Biliary parasites
Other neoplasms
Pancreatic adenocarcinoma
Metastasis
Granular cell tumor
Biliary papillomatosis
Granular Cell Tumor
[Figure 2-6-18]
Benign tumors of Schwann cell origin

90% of patients are females, mean age 34 years
Location:
CBD (50%)
Cystic duct (37%)
CHD (11%)
Gallbladder (4%)
Intrahepatic ducts (4%)
Infiltrative lesions that produce short annular
strictures
Benign stricture
Figure 2-6-17
Figure 2-6-18
Cholangiographic and CT features of a benign

stricture
Granular cell tumor

Biliary Neoplasms
318
Biliary Papillomatosis
Multiple and recurrent adenomas of the biliary tract

Men and women in the 6th and 7th decades
Clinical presentation: biliary obstruction, cholangitis
Local recurrence and malignant transformation common
Summary
Biliary Adenocarcinomas
Uncommon
Peripheral intrahepatic cholangiocarcinoma
Mass forming tumors
Delayed, patchy enhancement
Look for imaging evidence of fibrosis
Intraductal cholangiocarcinoma
Rare
Intraductal masses
Biliary obstruction
Hilar cholangiocarcinoma
Most common subtype
Look for discontinuous biliary dilatation
Determination of resectablility
Extrahepatic duct adenocarcinoma
Must differentiate from a benign stricture
Summary: Benign vs. Malignant
Malignant stricture
Abrupt change
Benign stricture
Tapering
Klatskin / PSC
Summary: Granular Cell Tumor
Benign Neoplasm
Young, women
True mimic for carcinoma
References
1. Choi BI, Lee JM, Han JK: Imaging of intrahepatic and hilar cholangiocarcinoma. Abdom Imaging 29:548, 2004
2. Ishak KG, Goodman ZD, Stocker JT: Tumors of the Liver and Intrahepatic Bile Ducts. Washington, D.C.: Armed
Forces Institute of Pathology under the auspices of Universities Associated for Research and Education in
Pathology For sale by the Armed Forces Institute of Pathology, 2001
3. Kim TK, Choi BI, Han JK, et al: Peripheral cholangiocarcinoma of the liver: two-phase spiral CT findings.
Radiology 204:539, 1997
4. Lim JH: Cholangiocarcinoma: morphologic classification according to growth pattern and imaging findings. AJR
Am J Roentgenol 181:819, 2003
5. Tani K, Kubota Y, Yamaguchi T, et al: MR imaging of peripheral cholangiocarcinoma. J Comput Assist Tomogr
15:975, 1991
6. Vilgrain V, Van Beers BE, Flejou JF, et al: Intrahepatic cholangiocarcinoma: MRI and pathologic correlation in 14
patients. J Comput Assist Tomogr 21:59, 1997
7. Worawattanakul S, Semelka RC, Noone TC, et al: Cholangiocarcinoma: spectrum of appearances on MR images
using current techniques. Magn Reson Imaging 16:993, 1998
8. Yalcin S: Diagnosis and management of cholangiocarcinomas: a comprehensive review. Hepatogastroenterology
51:43, 2004
9. Zhang Y, Uchida M, Abe T, et al: Intrahepatic peripheral cholangiocarcinoma: comparison of dynamic CT and
dynamic MRI. J Comput Assist Tomogr 23:670, 1999
319
Biliary Neoplasms
1. Arepally A, Georgiades C, Hofmann LV, et al: Hilar cholangiocarcinoma: staging with intrabiliary MRI. AJR Am J
2. Bold RJ, Goodnight JE, Jr.: Hilar cholangiocarcinoma: surgical and endoscopic approaches. Surg Clin North Am
84:525, 2004
3. Koea J, Holden A, Chau K, et al: Differential diagnosis of stenosing lesions at the hepatic hilus. World J Surg
28:466, 2004
4. Manfredi R, Masselli G, Maresca G, et al: MR imaging and MRCP of hilar cholangiocarcinoma. Abdom Imaging
28:319, 2003
5. Principe A, Ercolani G, Bassi F, et al: Diagnostic dilemmas in biliary strictures mimicking cholangiocarcinoma.
Hepatogastroenterology 50:1246, 2003
6. Soyer P, Bluemke DA, Reichle R, et al: Imaging of intrahepatic cholangiocarcinoma: 2. Hilar cholangiocarcinoma.
AJR Am J Roentgenol 165:1433, 1995
Extrahepatic Bile Duct Adenocarcinoma
1. Albores-Saavedra J, Henson DE, Klimstra DS: Tumors of the gallbladder and extrahepatic bile ducts, and ampulla
of vater. Washington, D.C.: Armed Forces Institute of Pathology under the auspices of Universities Associated for
Research and Education in Pathology For sale by the Armed Forces Institute of Pathology, 2000
2. Park MS, Kim TK, Kim KW, et al: Differentiation of extrahepatic bile duct cholangiocarcinoma from benign
stricture: findings at MRCP versus ERCP. Radiology 233:234, 2004
3. Stroszczynski C, Hunerbein M: Malignant biliary obstruction: value of imaging findings. Abdom Imaging 30:314,
2005
4. Uchida M, Ishibashi M, Tomita N, et al: Hilar and suprapancreatic cholangiocarcinoma: value of 3D angiography
and multiphase fusion images using MDCT. AJR Am J Roentgenol 184:1572, 2005
Biliary Neoplasms
320
Pancreatic Neoplasms
Classification of Pancreatic Tumors
Tumors of the Exocrine Pancreas

Ductal adenocarcinoma
Acinar cell carcinoma
Solid-pseudopapillary neoplasm
Intraductal papillary mucinous neoplasm
Mucinous cystic neoplasm
Microcystic adenoma
Pancreatoblastoma
Mature cystic teratoma
Tumors of the Endocrine Pancreas
Islet cell tumors (neuroendocrine tumors)
Non-epithelial tumors
Soft tissue tumors
Lymphoma
Secondary Tumors
Tumor like lesions
Pancreatitis
Lymphoepithelial cyst
Pseudocyst
Objectives
Adenocarcinoma
Mucinous noncystic adenocarcinoma
Cystic neoplasms
Solid and pseudopapillary epithelial neoplasm
Microcystic adenoma
Endocrine neoplasms
Metastasis
Pancreatic Adenocarcinoma: Epidemiology
85% to 90% of pancreatic neoplasms

Second most common GI tract malignancy in the U.S.
US: colorectal, pancreas, stomach, liver, esophagus, gallbladder
Worldwide: stomach, colorectal, liver, esophagus, pancreas, gallbladder
Pancreatic Adenocarcinoma: Epidemiology
Death:incidence ratio of .99

5-year survival 4%, overall
5-year survival 17%, early stage
80% of cases occur between 60 to 80 years of age
Pancreatic Adenocarcinoma: Risk Factors
Cigarette smoking (2-3 fold relative risk)

Hereditary risk factors
Hereditary nonpolyposis colon cancer (HNPCC)
Familial breast cancer (BRCA2)
Familial adenomatous polyposis
Peutz-Jeghers
321
Peutz-Jeghers
Ataxia telangiectasia
Familial atypical multiple mole-melanoma
Familial pancreatitis
Figure 2-7-1
Pancreatic Adenocarcinoma:
Clinical Features
Symptoms
Pain most common
Unexplained weight loss
Jaundice in 50% tumors in the head of
the pancreas
Diabetes in 25% to 50%
Distribution
60% head
20% body
10% tail
5% to 10% entire gland
Pancreatic Adenocarcinoma:
Microscopy
Moderately to well differentiated
Pathology of ductal adenocarcinoma of the pancreas
Desmoplastic stromal reaction
Gross Pathology
Fibrotic
Infiltration and invasion of adjacent structures
Hemorrhage and necrosis uncommon
Pancreatic Adenocarcinoma: MDCT
Dual Phase Technique

Rapid bolus, 3 ml/sec
Pancreatic phase, 40 sec after injection
Portal venous phase
Thin reformations with overlap, 1.25 mm
Fletcher JG, Wiersema MJ, Farrell MA, et al. Pancreatic malignancy: value of arterial,
pancreatic, and hepatic phase imaging with multi-detector row CT. Radiology 2003;
229(1):81-90.
Pancreatic Adenocarcinoma: MDCT
Volume rendering
Maximum intensity projection (MIP)
Curved planar reformations
Additional information for local extension
Secondary signs in iso-attenuating carcinomas
Pancreatic Adenocarcinoma: CT Features
Pancreatic and CBD duct dilatation

"Double duct sign
Abrupt tapering of the CBD
Atrophy of the distal gland
Focal soft tissue density in a fatty gland
Alterations in morphology
Spherical enlargement of the pancreatic head
Rounded borders of the uncinate process
Extension to adjacent organs
Vascular encasement
322
Double Duct Sign [Figure 2-7-2]
Figure 2-7-2
Spherical Enlargement of the

Pancreatic Head
Rounded Borders of the Uncinate
Process [Figure 2-7-3]
Atrophic Changes in the Distal
Pancreas [Figure 2-7-4]
Double duct sign of ductal adenocarcinoma of the pancreas
Pancreatic Adenocarcinoma: MR
Figure 2-7-3
Problem solving
Equivocal CT
Small tumors
T1-weighted images
Low signal tumor on
unenhanced images
Subtraction images from in
and out of phase images
Nonresectability
Ductal adenocarcinoma of the pancreas showing a rounded contour

Invasion of adjacent organs,
to the uncinate process
except duodenum
Tumor diameter > 5 cm
Encasement or occlusion of vessels
Figure 2-7-4
SMA, SMV, portal vein
Celiac trunk and major
branches
+/- isolated focal involvement
of PV or SMV
Accuracy of CT 88%-90%
3D CT angiography
Distant nodal metastasis
Liver metastasis
Resectable? No, stomach

and vascular invasion
Ductal adenocarcinoma of the pancreas showing atrophy of the distal

pancreas
Figure 2-7-5
Resectable? No, SMA Encasement

Resectable? No, SMA
Encasement and Liver Mets
[Figure 2-7-5]
Resectable? YES
Nonresectable adenocarcinoma of the pancreas
Mucinous Noncystic
Adenocarcinoma
(Infiltrating Colloid Carcinoma)[Figure 2-7-6]
Rare variant of adenocarcinoma

Marked extracellular mucin
Signet rings cells
Imaging
Large tumors
Well-defined hypoattenuating
mass
May have calcification
Figure 2-7-6
Mucinous noncystic adenocarcinoma of the pancreas

323
Mucinous Noncystic Adenocarcinoma

(Infiltrating Colloid Carcinoma)
Microcystic adenoma
Intraductal papillary mucinous
neoplasm
Microcystic (Oligocystic) adenoma
Pancreatic pseudocyst
Figure 2-7-7

neoplasm, diffuse main duct
pattern
Intraductal Papillary Mucinous

Neoplasm (IPMN)
Intraductal papillary neoplasm

Produces mucin
All have malignant potential
May or may not have an invasive
component
Synonyms
Intraductal mucin-hypersecreting neoplasm
Ductectatic type of pancreatic ductal carcinoma
Mucinous ductal ectasia
Mucin-producing tumor (or carcinoma)
Mucin-hypersecreting tumor
Figure 2-7-8
Intraductal Papillary Mucinous Neoplasm (IPMN)
Most common in men, 7th decade

Clinical symptoms similar to chronic pancreatitis
Pain
Malabsorption
Diabetes
Prior episodes of pancreatitis
IPMN: Histopathology
Neoplastic papillary epithelium

Mucin production
Duct dilatation
IPMN: Imaging Features

neoplasm, focal main duct pattern
Duct dilatation
Main duct or side branch
Diffuse or focal (cystic appearing)
Pancreatic glandular atrophy
Calcification
Bulging duodenal papilla
Figure 2-7-9
IPMN: Diffuse Involvement of Main Pancreatic Duct

[Figure 2-7-7]
IPMN: Focal Involvement of Main Pancreatic Duct

[Figure 2-7-8]
IPMN: Focal Involvement of Main Pancreatic Duct
Show communication with duct structures

MRCP
ERCP/EUS
Show intraductal masses
IPMN: Focal Involvement of a Side Branch Duct

[Figure 2-7-9]

neoplasm, focal side branch pattern
324
Side Branch IPMN
Figure 2-7-10
IPMN: Diffuse Involvement of a Side Branch Duct [Figure 2-7-10]

Bulging Papilla [Figure 2-7-11]
IPMN: Diagnostic Difficulties
MR/MRCP
Target imaging for ductal communication
IPMN: MR/MRCP
IPMN: Diagnostic Difficulties
Must differentiate from chronic pancreatitis

Side branch variant may mimic
Pseudocyst
Microcystic adenoma
ERCP
Definitive imaging technique

neoplasms, diffuse side branch
pattern
Solid and Pseudopapillary Tumor (SPT)
Neoplasm of young women

Mean age, 24 years
Benign or low-grade malignancy
Good prognosis
Synonyms
Solid-cystic tumor
Papillary cystic tumor
Solid and pseudopapillary epithelial neoplasm
Solid and Pseudopapillary Tumor (SPT)
Clinical features
Usually incidentally discovered
Abdominal discomfort, pain
Jaundice or duct obstruction is rare
SPT: Pathology Features
Pathologic features
Capsule
Hemorrhage, necrosis, cystic
areas
Solid areas
May calcify
Histopathology
Highly cellular areas
Pseudopapillary areas
Hemorrhage
Sclerosis
Figure 2-7-11
Bulging papilla in IPMN
SPEN: Imaging Features
Circumscribed
Capsule
Early peripheral enhancement
Cystic change
Hemorrhage
Fluid-fluid levels
Calcification
Rare features
Biliary/pancreatic duct dilatation
Adjacent organ invasion
325
SPT [Figures 2-7-12 and 2-7-13]
Figure 2-7-12
SPT: Diagnostic Difficulties
Older patient age

Male patient
May mimic other cystic lesions
Oligocystic adenoma
Supportive CT and MR features for diagnosis
Evidence of capsule
Evidence of blood products
Mucinous Cystic Neoplasm (MCN)
Mucin-secreting cystic neoplasm

Mucinous cystadenoma
Mucinous cystadenocarcinoma
Most common in women
80% occur in women
Mean age 49 years
Less common in men
Older age, mean 70 years
Solid and pseudopapillary tumor
Figure 2-7-13
Mucinous Cystic Neoplasm (MCN)
Variable
Dependent upon tumor size
Jaundice and CBD obstruction are rare
MCN: Histopathology
Columnar cell lining

May have ovarian-type stroma
Mucin
Hemorrhage
Calcification
MR showing early capsular enhancement in SPT
MCN: Gross Pathology
Majority in tail of pancreas

70% to 95%
Large size at diagnosis
Mean diameter, 6 to 10 cm
Multilocular cysts
Septations
Solid mural nodules
Occasional calcifications
Unilocular, rare
MCN: Imaging
Well-circumscribed cystic mass

Cannot differentiate benign from malignant
Enhancement
Cyst wall, septations, mural nodules
Calcifications
Cyst wall, septations, mural nodules
Cyst fluid
Variable CT attenuation/MR signal intensity
Mucin
Hemorrhage
Proteinaceous fluid
326
Mucinous Cystic Neoplasm [Figure 2-7-14]
Figure 2-7-14
MCN: Diagnostic Difficulties
Small lesions
Lesions in the head of the pancreas
Lesions without septations and mural nodules
Pseudocyst
Oligocystic adenoma
Solid and pseudopapillary tumor
Rare, congenital cysts
Microcystic Adenoma
Benign
Synonyms
Serous cystadenoma
Glycogen-rich cystadenoma
Variants
Oligocystic adenoma
Microcystic Adenoma

70% in women
Mean age, 66 years
Incidental
Pain, nausea, vomiting, weight loss
Jaundice is unusual
Figure 2-7-15
Microcystic Adenoma: Gross Pathology
Small cysts arranged around a central scar

Honeycomb or sponge appearance
Thin fibrous septae
Central stellate scar
Occasional hemorrhage
Microcystic Adenoma: Histopathology
Histology
Small cysts
Cuboidal cells
High glycogen content
Microcystic Adenoma: Pathology

Microcystic Adenoma: Imaging features
[Figures 2-7-15 and 2-7-16]
Sharp, circumscribed margins

Lobular margins
Honeycomb appearance
Multiple small cysts
Central scar
+/- calcification
Microcystic adenoma
Figure 2-7-16
Microcystic adenoma
327
Oligocystic Adenoma [Figure 2-7-17]
Figure 2-7-17
Uncommon variant of microcystic

adenoma
Few, very large cysts
? association with von Hippel
Lindau syndrome
Synonyms
Macrocystic serous
cystadenoma
Serous oligocystic adenoma
Microcystic Adenoma: Diagnostic Difficulties
Oligocystic adenoma
Oligocystic variant
Pseudocyst
Small lesions
Difficult to identify central scar/septations
Islet Cell Tumors (Neuroendocrine Tumors)
Tumors of the pancreatic islets

Occur in all age groups
Variable biologic behavior
Benign or malignant
Clinical evidence of hormone production
65% to 85%
Clinically silent
15% to 35%
Figure 2-7-18
Insulinoma [Figure 2-7-18]
Somatostatin receptor
scintigraphy
Positive in only 60-70% of
cases
Zollinger-Ellison Syndrome - Pancreatic Gastrinoma
Insulinoma
Somatostatin receptor scintigraphy

Sensitivity for primary tumor, 58%-75%
Sensitivity for metastatic disease, 100%
Glucagonoma (DM-Dermatitis Syndrome)

MEN I Syndrome (Wermers Syndrome)
3Ps
Pituitary
Pancreas
Parathyroid
Other: thymus, thyroid, adrenal gland, GI tract
Autosomal dominant
Long arm chromosome 11
Clinically-Silent Islet Cell Tumors [Figures
Occur at any age

Typically large at time of diagnosis
Range, 6 to 20 cm
Nonspecific imaging appearance
Necrosis and cystic degeneration common
25% calcify
Liver metastases are common
2-7-19 and 2-7-20]
328
Metastatic Disease
Figure 2-7-19
Mets to pancreas
Lung, breast
Melanoma
Renal cell
Lymphoma-adjacent nodal
disease
Mimic primary pancreatic
neoplasms
Islet cell
Biliary obstruction in 30%
Clinically-silent islet cell tumor
Figure 2-7-20
Summary: Adenocarcinoma
Most common pancreatic

neoplasm
MDCT
Dual phase
Thin reformations
Overlapping images
RESECTABLILITY
Vascular encasement
Liver mets
Summary: IMPN

Imaging
Duct dilatation
Intraductal masses
Bulging papilla
Summary: Solid
Pseudopapillary Tumor
Renal cell metastatic to the pancreas
Young women
Imaging features
Capsule
Solid and cystic
Hemorrhage
Summary: Mucinous Cystic Neoplasm
Mucinous cystadenocarcinoma
Complex cyst
Middle-aged women
Tail of the pancreas
Septations, nodules, calcification
Summary: Microcystic Adenoma
Older women
Benign neoplasm
Central scar
Honeycomb pattern of cysts
Lobulated margins
329
References
Adenocarcinoma
1. Vargas R, Nino-Murcia M, Trueblood W, Jeffrey RB, Jr. MDCT in Pancreatic adenocarcinoma: prediction of
vascular invasion and resectability using a multiphasic technique with curved planar reformations. AJR Am J
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2. Bronstein YL, Loyer EM, Kaur H, et al. Detection of small pancreatic tumors with multiphasic helical CT. AJR
Am J Roentgenol 2004; 182:619-623.
3. Roche CJ, Hughes ML, Garvey CJ, et al. CT and pathologic assessment of prospective nodal staging in patients
with ductal adenocarcinoma of the head of the pancreas. AJR Am J Roentgenol 2003; 180:475-480.
4. Fletcher JG, Wiersema MJ, Farrell MA, et al. Pancreatic malignancy: value of arterial, pancreatic, and hepatic
phase imaging with multi-detector row CT. Radiology 2003; 229:81-90.
5. Prokesch RW, Chow LC, Beaulieu CF, et al. Local staging of pancreatic carcinoma with multi-detector row CT:
use of curved planar reformations initial experience. Radiology 2002; 225:759-765.
6. Prokesch RW, Chow LC, Beaulieu CF, Bammer R, Jeffrey RB, Jr. Isoattenuating pancreatic adenocarcinoma at
multi-detector row CT: secondary signs. Radiology 2002; 224:764-768.
7. Imbriaco M, Megibow AJ, Camera L, et al. Dual-phase versus single-phase helical CT to detect and assess
resectability of pancreatic carcinoma. AJR Am J Roentgenol 2002; 178:1473-1479.
8. Horton KM, Fishman EK. Adenocarcinoma of the pancreas: CT imaging. Radiol Clin North Am 2002; 40:12631272.
9. McNulty NJ, Francis IR, Platt JF, Cohan RH, Korobkin M, Gebremariam A. Multi--detector row helical CT of the
pancreas: effect of contrast-enhanced multiphasic imaging on enhancement of the pancreas, peripancreatic
vasculature, and pancreatic adenocarcinoma. Radiology 2001; 220:97-102.
10. Tabuchi T, Itoh K, Ohshio G, et al. Tumor staging of pancreatic adenocarcinoma using early- and late-phase helical
CT. AJR Am J Roentgenol 1999; 173:375-380.
11. O'Malley ME, Boland GW, Wood BJ, Fernandez-del Castillo C, Warshaw AL, Mueller PR. Adenocarcinoma of the
head of the pancreas: determination of surgical unresectability with thin-section pancreatic-phase helical CT. AJR
Am J Roentgenol 1999; 173:1513-1518.
12. Hough TJ, Raptopoulos V, Siewert B, Matthews JB. Teardrop superior mesenteric vein: CT sign for unresectable
carcinoma of the pancreas. AJR Am J Roentgenol 1999; 173:1509-1512.
13. Keogan MT, Tyler D, Clark L, et al. Diagnosis of pancreatic carcinoma: role of FDG PET. AJR Am J Roentgenol
1998; 171:1565-1570.
14. Zeman RK, Cooper C, Zeiberg AS, et al. TNM staging of pancreatic carcinoma using helical CT. AJR Am J
Roentgenol 1997; 169:459-464.
15. Raptopoulos V, Steer ML, Sheiman RG, Vrachliotis TG, Gougoutas CA, Movson JS. The use of helical CT and CT
angiography to predict vascular involvement from pancreatic cancer: correlation with findings at surgery. AJR Am
J Roentgenol 1997; 168:971-977.
16. Lu DS, Reber HA, Krasny RM, Kadell BM, Sayre J. Local staging of pancreatic cancer: criteria for unresectability
of major vessels as revealed by pancreatic-phase, thin-section helical CT. AJR Am J Roentgenol 1997; 168:14391443.
17. Lu DS, Vedantham S, Krasny RM, Kadell B, Berger WL, Reber HA. Two-phase helical CT for pancreatic tumors:
pancreatic versus hepatic phase enhancement of tumor, pancreas, and vascular structures. Radiology 1996;
199:697-701.
18. Megibow AJ, Zhou XH, Rotterdam H, et al. Pancreatic adenocarcinoma: CT versus MR imaging in the evaluation
of resectability--report of the Radiology Diagnostic Oncology Group. Radiology 1995; 195:327-332.
19. Megibow AJ. Pancreatic adenocarcinoma: designing the examination to evaluate the clinical questions. Radiology
1992; 183:297-303.
Intraductal Papillary Mucinous Neoplasm
1. Cellier C, Cuillerier E, Palazzo L, et al: Intraductal papillary and mucinous tumors of the pancreas: accuracy of
preoperative computed tomography, endoscopic retrograde pancreatography and endoscopic ultrasonography, and
long-term outcome in a large surgical series. Gastrointest Endosc 47:42, 1998
2. Fukukura Y, Fujiyoshi F, Sasaki M, et al: Intraductal papillary mucinous tumors of the pancreas: thin-section
helical CT findings. AJR Am J Roentgenol 174:441, 2000
3. Itai Y, Kokubo T, Atomi Y, et al: Mucin-hypersecreting carcinoma of the pancreas. Radiology 165:51, 1987
4. Lim JH, Lee G, Oh YL: Radiologic spectrum of intraductal papillary mucinous tumor of the pancreas.
Radiographics 21:323, 2001
5. Prasad SR, Sahani D, Nasser S, et al: Intraductal papillary mucinous tumors of the pancreas. Abdom Imaging
28:357, 2003
6. Procacci C, Graziani R, Bicego E, et al: Intraductal mucin-producing tumors of the pancreas: imaging findings.
Radiology 198:249, 1996
330
7.
8.
Procacci C, Megibow AJ, Carbognin G, et al: Intraductal papillary mucinous tumor of the pancreas: a pictorial
essay. Radiographics 19:1447, 1999
Taouli B, Vilgrain V, Vullierme MP, et al: Intraductal papillary mucinous tumors of the pancreas: helical CT with
histopathologic correlation. Radiology 217:757, 2000
Mucinous Cystic Neoplasm

1. Buetow PC, Rao P, Thompson LD: From the Archives of the AFIP. Mucinous cystic neoplasms of the pancreas:
radiologic-pathologic correlation. Radiographics 18:433, 1998
2. Procacci C, Carbognin G, Accordini S, et al: CT features of malignant mucinous cystic tumors of the pancreas. Eur
Radiol 11:1626, 2001
3. Thompson LD, Becker RC, Przygodzki RM, et al: Mucinous cystic neoplasm (mucinous cystadenocarcinoma of
low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases. Am J Surg Pathol 23:1,
1999
Solid and Pseudopapillary Tumor
1. Buetow PC, Buck JL, Pantongrag-Brown L, et al: Solid and papillary epithelial neoplasm of the pancreas:
imaging-pathologic correlation on 56 cases. Radiology 199:707, 1996
2. Cantisani V, Mortele KJ, Levy AD, et al: MR imaging features of solid pseudopapillary tumor of the pancreas in
adult and pediatric patients. AJR Am J Roentgenol 181:395, 2003
3. Coleman KM, Doherty MC, Bigler SA: Solid-pseudopapillary tumor of the pancreas. Radiographics 23:1644, 2003
4. Friedman AC, Lichtenstein JE, Fishman EK, et al: Solid and papillary epithelial neoplasm of the pancreas.
Radiology 154:333, 1985
Microcystic Adenoma
1. Buck JL, Hayes WS: From the Archives of the AFIP. Microcystic adenoma of the pancreas. Radiographics 10:313,
1990
2. Healy JC, Davies SE, Reznek RH: CT of microcystic (serous) pancreatic adenoma. J Comput Assist Tomogr
18:146, 1994
3. Hough DM, Stephens DH, Johnson CD, et al: Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical
significance, and CT findings. AJR Am J Roentgenol 162:1091, 1994
4. Itai Y, Ohhashi K, Furui S, et al: Microcystic adenoma of the pancreas: spectrum of computed tomographic
findings. J Comput Assist Tomogr 12:797, 1988
5. Khurana B, Mortele KJ, Glickman J, et al: Macrocystic serous adenoma of the pancreas: radiologic-pathologic
correlation. AJR Am J Roentgenol 181:119, 2003
6. Minami M, Itai Y, Ohtomo K, et al: Cystic neoplasms of the pancreas: comparison of MR imaging with CT.
Radiology 171:53, 1989
7. Yeh HC, Stancato-Pasik A, Shapiro RS: Microcystic features at US: a nonspecific sign for microcystic adenomas
of the pancreas. Radiographics 21:1455, 2001
Islet Cell Tumors
1. Buetow PC, Miller DL, Parrino TV, et al: Islet cell tumors of the pancreas: clinical, radiologic, and pathologic
correlation in diagnosis and localization. Radiographics 17:453, 1997
2. Buetow PC, Parrino TV, Buck JL, et al: Islet cell tumors of the pancreas: pathologic-imaging correlation among
size, necrosis and cysts, calcification, malignant behavior, and functional status. AJR Am J Roentgenol 165:1175,
1995
3. Ichikawa T, Peterson MS, Federle MP, et al: Islet cell tumor of the pancreas: biphasic CT versus MR imaging in
tumor detection. Radiology 216:163, 2000
4. Semelka RC, Cumming MJ, Shoenut JP, et al: Islet cell tumors: comparison of dynamic contrast-enhanced CT and
MR imaging with dynamic gadolinium enhancement and fat suppression. Radiology 186:799, 1993
5. Sheth S, Hruban RK, Fishman EK: Helical CT of islet cell tumors of the pancreas: typical and atypical
manifestations. AJR Am J Roentgenol 179:725, 2002
6. Stafford Johnson DB, Francis IR, Eckhauser FE, et al: Dual-phase helical CT of nonfunctioning islet cell tumors. J
Comput Assist Tomogr 22:59, 1998
7. Van Hoe L, Gryspeerdt S, Marchal G, et al: Helical CT for the preoperative localization of islet cell tumors of the
pancreas: value of arterial and parenchymal phase images. AJR Am J Roentgenol 165:1437, 1995
Metastases
1. Klein KA, Stephens DH, Welch TJ: CT characteristics of metastatic disease of the pancreas. Radiographics 18:369,
1998
2. Ng CS, Loyer EM, Iyer RB, et al: Metastases to the pancreas from renal cell carcinoma: findings on three-phase
contrast-enhanced helical CT. AJR Am J Roentgenol 172:1555, 1999
331
Gastric Malignancies
Adenocarcinoma
Lymphoma
Gastrointestinal Stromal Tumors
Carcinoid
Kaposi Sarcoma
Metastases
Gastric Adenocarcinoma
Fourth most common cancer worldwide1

Lung, breast, colorectum, stomach, liver
1Steward BW and Kleihues P (eds). World Cancer Report. IARC Press. Lyon
2003.
Gastric Adenocarcinoma: Geographic Variation
High incidence areas

China, Japan, South America, Eastern Europe
Low incidence areas
North America and Northern Africa
Gastric Adenocarcinoma: Clinical
Peak incidence 50 to 70 years of age

More common in men
2:1
Early disease
Asymptomatic
Advanced disease
Epigastric pain, bloating, nausea
Early satiety, anorexia, vomiting
Upper GI bleeding
Figure 2-8-1
Gastric Adenocarcinoma:
Etiology [Figure 2-8-1]
Atrophic Gastritis
Helicobacter pylori
(80% of cases)
Pernicious Anemia
Partial Gastrectomy
Adenomatous Polyps
Polyposis syndromes
HNPCC-Hereditary
Nonpolyposis Colon
Cancer Syndromes
(Lynch Syndromes)
Gastritis
Normal
Dysplasia
Atrophic Gastritis
Intramucosal Carcinoma
Intestinal Metaplasia
Invasive Carcinoma
Pathogenesis of gastric adenocarcinoma

332
WHO Classification of Gastric Adenocarcinoma
Figure 2-8-2
Signet Ring [Figure 2-8-2]

Papillary [Figure 2-8-3]
Mucinous [Figure 2-8-4]
Figure 2-8-3
Signet ring cell adenocarcinoma
produces "linitis plastica"
Figure 2-8-4
Papillary adenocarcinoma produces

intraluminal polypoid masses
Early Gastric Carcinoma
Japanese Research Society for

Gastric Carcinoma
Carcinoma limited to the mucosa and
submucosa, irrespective of nodal
metastases
Mucinous adenocarcinoma produces tumor calcifications
Advanced Gastric Carcinoma [Figure 2-8-5]
Figure 2-8-5
Borrmann Classification
Most common gastric cancer in the U.S.
Tumor penetrating the muscularis propria
Advanced Gastric Carcinoma
Morphology
Polypoid
Ulcerating
Infiltrating
Scirrhous
Gastric Carcinoma: Polypoid [Figure 2-8-6]
Figure 2-8-6
Advanced gastric carcinoma

(Borrmann) classification
Polypoid gastric adenocarcinoma
333
Figure 2-8-7
Ulcerated - Bulk of tumor mass
has been replaced by ulceration
Ulcerated Carcinoma
Lesser Curvature
Carmen Meniscus Sign
[Figure 2-8-7]
Ulcerated gastric
adenocarcinoma showing the
Carmen meniscus sign
Gastric Carcinoma: Ulcerated

Gastric Carcinoma: Infiltrating
[Figure 2-8-8]
Scirrhous Carcinoma [Figure 2-8-9]
Infiltrating tumors with desmoplasia

Signet ring cell carcinomas
Radiologic Features
Irregular narrowing
Decreased gastric capacity
Rigidity, linitis plastica
Most common in the antrum
Rarely-nodular,distorted folds
when the tumor is proximal
Figure 2-8-8
Carcinoma of the Cardia
One-third of all gastric

carcinomas in the U.S.
Compared to other gastric
carcinomas:
Male predominance, 6:1
40% associated with hiatal hernia
Atrophic gastritis and signet
ring cell types are uncommon
Association with smoking and
alcohol
Early lesions are difficult to detect
Difficult to differentiate from
Barretts adenocarcinoma
Pseudoachalasia
Pitfalls on CT:
GE junction pseudomass
Hiatal hernias
Infiltrating gastric adenocarcinoma
Figure 2-8-9
Scirrhous carcinoma
Figure 2-8-10
Normal Gastric Cardia

Carcinoma of the Cardia
[Figure 2-8-10]
Carcinoma of the cardia

334
Staging
Figure 2-8-11
Endoscopic Ultrasound
Depth of tumor invasion
T stage accuracy 85%
Perigastric nodes
Sensitivity 55%-80%
CT
Presence and extent of extragastric spread
Peritoneal
Lymph nodes
Distant metastasis
Staging - Extragastric Spread [Figure 2-8-11 and 2-8-12]
Anatomic Pathways
Lesser omentum
Greater omentum
Transverse mesocolon
Lesser sac
Lower esophagus
CT features
Soft tissue stranding
Soft tissue nodules
Routes of extragastric spread of

carcinoma
Figure 2-8-12
Staging - Lymphatic Spread

Staging - Krukenberg Metastasis
Staging - Adjacent Organ Invasion
Contiguous tumor
Loss of fat planes
Focal enlargement of the adjacent organ
Direct Extension and Adjacent Organ Invasion

Gastric Lymphoma
Increasing incidence
Up to 10% of gastric malignancies
Most common site of extranodal lymphoma
Most common site of GI lymphomas
Gastric Lymphoma: Clinical
Low grade
Dyspepsia, nausea, vomiting
High grade:
Bleeding, pain, early satiety, weight loss
Mucosa-Associated Lymphoid Tissue (MALT)
Gastric adenocarcinoma extending into

Organized lymphoid tissue located in mucosal sites
the greater omentum and lesser sac
Native MALT
Intraepithelial lymphocytes
Plasma cells, B and T lymphocytes in the lamina propria
Mesenteric lymph nodes
Peyers patches
Acquired MALT
Arises as a result of antigenic stimulation (H. pylori infection)
Accumulates before the development of B cell lymphomas
335
Primary Gastric Lymphoma
Figure 2-8-13
MALT lymphoma
Arises from acquired MALT
H. pylori is invariably present
Good clinical prognosis
High grade B cell lymphoma
Probably arises from MALT lymphoma
Normal mucosa
Primary Gastric Lymphoma: Etiology and

Pathogenesis Figure 2-8-13]
H. pylori infection
H. pylori infection
H. pylori gastritis
formation of acquired MALT
low-grade MALT lymphoma
high-grade B-cell lymphoma
Evolution of gastric lymphoma
336
Low Grade Malt Lymphoma [Figure 2-8-14]
Figure 2-8-14
Clinical
Dyspepsia, nausea, vomiting
Good prognosis
Imaging
Nodules
Ulcers
Erosions
Thick rugal folds
Low Grade Malt Lymphoma: CT Features
Homogeneous mural thickening

Perigastric adenopathy
High Grade B Cell Lymphoma
Clinical
Bleeding, pain,
Early satiety, weight loss
Imaging
Mural thickening
Adenopathy
High Grade B Cell Lymphoma:Typical CT Features

[Figure 2-8-15]

Perigastric adenopathy
High Grade B Cell Lymphoma:Typical CT Features
Cavitation
High Grade B Cell Lymphoma: Atypical CT Features
Heterogeneous attenuation
Enhancement
Low attenuation necrosis
Low grade MALT lymphoma
Figure 2-8-15
CT features of Gastric Lymphoma
Wall thickening1
Tends to be greater (mean, 4 cm) than that of
adenocarcinoma
Tends to be homogeneous attenuation
Ulceration
Polypoid masses
Regional adenopathy
1Buy J, Moss A. AJR 138:859-865, 1982
CT features differentiating Gastric Adenocarcinoma

and Lymphoma1
Gastric wall thickening in lymphoma (mean 4 cm) is typically more

impressive than adenocarcinoma (mean 1.8 cm)
Wall thickening is more homogeneous in lymphoma
Perigastric fat is more likely to be preserved in lymphoma
Regional adenopathy is common in both
Adenopathy in lymphoma tends to be bulky and may extend
below the level of the renal veins
High grade B cell lymphoma
1Buy J, Moss A. AJR 138:859-865, 1982
Fork FT, Haglund U, Hogstrom H. Endoscopy 17:5-7, 1985
337
Gastric Lymphoma
Mesenchymal Neoplasm of the Stomach
Gastrointestinal Stromal Tumor

Leiomyoma
Leiomyosarcoma
Schwannoma
Neurofibroma
Ganglioneuroma
Paraganglioma
Granular cell tumor
Lipoma, liposarcoma
Fibrous lesions
Tumors of blood vessels
Gastrointestinal Stromal Tumors (GIST)
Most common mesenchymal neoplasm of the GI tract

Arise from the muscularis propria
Cellular origin
Primitive "stem cell like cell
Interstitial cell of Cajal (gut pacemaker cell)
GIST: Clinical Features
Uncommon tumors
Prevalence in the U.S.
5000 to 6000 new cases per year1
Increased incidence
Neurofibromatosis (NF-1)
KIT germline mutations
1Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal

tumors: A consensus approach. Hum Pathol 2002. 33:459-465
GIST: Clinical Features
Median age 50-60 years

60% occur in the stomach
Benign and malignant
Defined by KIT expression
Initial diagnosis
Therapy (Gleevac)
What is KIT?
KIT-tyrosine kinase growth factor

KIT-tyrosine kinase growth factor receptor
CD117 binds to KIT receptors
Normally expressed
Hematopoietic stem cells
Germ cells
Interstitial cell of Cajal (gut pacemaker cell)
KIT-inhibitor therapy
STI-571, Imatinib [Gleevac]
What Happened to Leiomyomas and Leiomyosarcomas?
Very rare
Except,
Leiomyomas are the most common benign tumor of the ESOPHAGUS
Leiomyosarcomas of the RETROPERITONEUM
GIST
Most common sites

Stomach
Small bowel
338
Figure 2-8-16
Anorectum
Unusual sites
Esophagus
Colon
Mesentery/omentum
GIST
Spindle Cell GIST

Epithelioid GIST
GIST: CD117 (KIT) Positive

GIST [Figure 2-8-16]
Mural Origin
Mural mass
Exophytic mass
Mural and exophytic
GIST: Mural Origin [Figure 2-8-17]

Figure 2-8-17
Imaging and morphologic patterns of

gastrointestinal stromal tumors: intramural
polypoid mass, exophytic mass, or intramural and
exophytic mass
Gastrointestinal stromal tumor

339
GIST: Internal Hemorrhage and Necrosis
Figure 2-8-18
[Figure 2-8-18]
GIST: Cyst Formation

GIST: Cavity Formation
GIST: Calcification
GIST: Metastatic Spread
Direct invasion
Peritoneal
Hematogenous
Liver
Management of Metastatic and Recurrent Disease
Kit-inhibitor therapy
Gleevac
Clinical trials of other kit-inhibitors
Imaging features of treated metastasis
Cystic transformation
Pet important to determine residual functional
tumor
Figure 2-8-19
Imaging of Recurrent Disease

Choi H, Charnsangavej C, de Castro Faria S, et al. CT
evaluation of the response of gastrointestinal stromal
tumors after imatinib mesylate treatment: a quantitative
analysis correlated with FDG PET findings. AJR Am J
Roentgenol 2004; 183:1619-1628.
Gastric GIST vs. Adenocarcinoma
[Figures 2- 7-19 and 2-7-20]
Gastric Carcinoid
Type I: autoimmune chronic atrophic gastritis

Hypergastrinemia
Multiple, small
Benign biologic behavior
Type II: MEN I and Zollinger Ellison syndrome
Hypergastrinemia
Multiple, small
Benign biologic behavior
Type III: sporadic
Single
GIST vs. adenocarcinoma

340
Carcinoid: Imaging Features
Figure 2-8-20
Submucosal mass
Central ulceration-bulls eye
Pedunculated polypoid lesions, rarely
Large ulcerative masses
Thick, rugal folds if hypergastrinemia is present
Carcinoid: Bulls Eye Lesion

Carcinoid: Pedunculated Polyps
Kaposi Sarcoma [Figure 2-8-21]
AIDS patients
Cutaneous KS usually
Stomach, duodenum, and small bowel most
common GI locations
Radiologic features
Submucosal masses
Bulls-eyeappearance
Polypoid masses
Infiltrating variant, rare
Metastases
Melanoma, breast, lung

Radiologic features
Ulcerating masses
Polyps
Infiltrating
Linitis Plastica
GIST vs. lymphoma
Figure 2-8-21
H. pylori
Chronic atrophic gastritis
Primary tumor morphology
Polypoid
Ulcerating
Infiltrating
Schirrous
CT: extragastric spread
Summary: Lymphoma
H. pylori
Low grade MALT to high grade B cell
Compared to adenocarcinoma
Greater wall thickening
Bulky, more extensive adenopathy
Summary: GIST
Most common mesenchymal neoplasm

KIT reactivity
Diagnosis
Gleevac therapy
Classic mural masses on barium
May have extensive extragastric growth
Bulls eye lesions from Kaposi Sarcoma.
Endoscopy shows hemorrhagic masses
341
Summary: Bulls Eye Lesions
Carcinoid
Metastasis
(Breast, Lung, Melanoma)
Kaposis Sarcoma
Lymphoma
Adenocarcinoma
Ectopic Pancreas
References
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Gastric Lymphoma
1. An SK, Han JK, Kim YH, et al: Gastric mucosa-associated lymphoid tissue lymphoma: spectrum of findings at
double-contrast gastrointestinal examination with pathologic correlation. Radiographics 21:1491, 2001
2. Buy JN, Moss AA: Computed tomography of gastric lymphoma. AJR 138:859, 1982
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4. Jaffe ES, Harris NL, Stein H, et al (eds): World Health Organization Classification of Tumours: Pathology and
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5. Kim YH, Lim HK, Han JK, et al: Low-grade gastric mucosa-associated lymphoid tissue lymphoma: correlation of
radiographic and pathologic findings. Radiology 212:241, 1999
6. Levine MS, Elmas N, Furth EE, et al: Helicobacter pylori and gastric MALT lymphoma. AJR Am J Roentgenol
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7. Levine MS, Rubesin SE, Pantongrag-Brown L, et al: Non-Hodgkin's lymphoma of the gastrointestinal tract:
radiographic findings. AJR Am J Roentgenol 168:165, 1997
8. Megibow AJ, Balthazar EJ, Naidich DP, et al: Computed tomography of gastrointestinal lymphoma. AJR 141:541,
1983
9. Parsonnet J, Hansen S, Rodriguez L, et al: Helicobacter pylori infection and gastric lymphoma. N Engl J Med
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10. Wotherspoon AC, Doglioni C, de Boni M, et al: Antibiotic treatment for low-grade gastric MALT lymphoma.
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11. Yoo CC, Levine MS, Furth EE, et al: Gastric mucosa-associated lymphoid tissue lymphoma: radiographic findings
in six patients. Radiology 208:239, 1998
Gastrointestinal Stromal Tumor (GIST)
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4. Fletcher CD: Clinicopathologic correlations in gastrointestinal stromal tumors. Hum Pathol 33:455, 2002
342
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10. Sharp RM, Ansel HJ, Keel SB: Best cases from the AFIP: gastrointestinal stromal tumor. Armed Forces Institute of
Pathology. RadioGraphics 21:1557, 2001
5.
Gastric Carcinoid
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2. Berger MW, Stephens DH: Gastric carcinoid tumors associated with chronic hypergastrinemia in a patient with
Zollinger-Ellison syndrome. Radiology 201:371, 1996
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AJR 176:947, 2001
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atrophic gastritis. A prospective analysis of 11 cases. Am J Surg Pathol 11:435, 1987
5. Ho AC, Horton KM, Fishman EK: Gastric carcinoid tumors as a consequence of chronic hypergastrinemia: spiral
CT findings. Clin Imaging 24:200, 2000
343
Abdominal Non Hodgkin Lymphoma

Objectives
Definition
Patterns of disease
NHL Adenopathy
Gastrointestinal Lymphoma
Immunodeficiency-related lymphomas
Post-transplantation Lymphoproliferative Disorder (PTLD)
AIDS-related Lymphomas
Lymphoid Neoplams
2001 WHO classification of Hematological Malignancies

Three major categories
B cell, T and NK (natural killer) cell, Hodgkin lymphoma
NHL
Large group of diverse diseases
Indolent, aggressive, and very aggressive
Non-Hodgkin Lymphoma (NHL)
4% of all cancers
5th most common cancer
5th leading cause of cancer death
4 times more common than Hodgkin lymphoma
Male to female ratio: 1.3 to 1
Median age 55 years
Third most common cancer mortality in children under age 15
Rising incidence
True increase in incidence
Improved identification and understanding
HIV infection
Organ transplants
Immunodeficiency increases risk
Wiskott-Aldrich syndrome
Ataxia telangiectasia
Long-term immunosuppressive therapy
Role of Imaging in Newly Diagnosed NHL
Clinical Staging:
Ann Arbor Staging Classification
Tumor bulk has important prognostic significance in intermediate and high
grade NHL
Identification of nodal and extranodal sites
Mesenteric adenopathy
GI tract
Liver
Spleen
Abdominal Non-Hodgkin Lymphoma
344
Role of Imaging in Management of NHL
Figure 2-9-1
CT and PET complimentary

CT imaging
Define anatomy
Response to therapy
Limitations of CT
Decrease in node size is not a
reliable indicator of response
Cannot differentiate residual tumor
vs. fibrosis vs. necrosis
Well-defined mesenteric nodes in NHL

Raanani P, Shasha Y, Perry C, et al. Is CT
scan still necessary for staging in Hodgkin and non-Hodgkin lymphoma patients in
the PET/CT era? Ann Oncol 2005.
PET/CT in Management of NHL
Initial staging
Equivalent or better to CT alone
Prognosis
Persistent uptake after
chemotherapy predicts treatment
failure/early recurrence
Figure 2-9-2
Kumar R et al. Utility of fluorodeoxyglucosePET imaging in the management of patients

with Hodgkins and non-Hodgkins
lymphomas. RCNA 2004. 42:1083-1100
Sandwich sign of the mesentery in NHL
NHL: Abdomimal Adenopathy
Mesentery
Retroperitoneum
CT Patterns
Discrete rounded nodes
Confluent nodes
Ill-defined masses
Mesenteric caking
Stellate mesentery
CT attenuation at diagnosis
Homogeneous in most cases
Heterogeneous in cases with
aggressive histology
CT attenuation during treatment
Heterogeneous from necrosis
Calcification may occur
Figure 2-9-3
Confluent retroperitoneal nodes in NHL
NHL: Discrete Nodes [Figure 2-9-1]

NHL: Sandwich Sign [Figure 2-9-2]
Figure 2-9-4
NHL: Confluent Nodes [Figure 2-9-3]

NHL: Confluent Nodes in AIDSAssociated NHL [Figure 2-9-4]
NHL: Mesenteric Caking in AIDSAssociated NHL
Confluent nodes in AIDS-associated NHL. There is extensive

necrosis and heterogeneous enhancement
345
Differential Diagnosis: Mesenteric Masses
Lymphoma
Metastasis
Carcinoid
Castleman disease
Mesenteric fibromatosis
Granulomatous disease
Tuberculosis
Histoplasmosis
Sarcoid
Sprue
Whipple disease
Castleman Disease
Hyperenhancing masses
May calcify
Mesenteric Fibromatosis (Desmoid Tumor)
Homogeneous attenuation
Soft tissue or low attenuation myxoid stroma
Foci of low attenuation myxoid stroma
May infiltrate adjacent bowel
Carcinoid
Primary lesion in bowel

Mesenteric metastasis
Spiculations/tethering of mesentery
May calcifiy
Mesenteric masses of GIST

Primary to small bowel or mesentery
Metastatic disease
Low attenuation hemorrhage/necrosis
Sarcoid
Small, discrete nodes

Retrocural nodes atypical in sarcoid
Gastrointestinal Lymphoma
Lymphoma that presents with GI disease and no other major site of

involvement
Most common extranodal site of NHL
4.4% of all lymphomas
25% of all extranodal lymphomas
Almost exclusively NHL
Stomach is the most common site in US and Western Europe
Small bowel is the most common site in the Mediterranean, Northern Africa,
Middle East
B-cell lymphomas
MALT lymphomas
Immunoproliferative small intestinal disease, alpha-heavy chain
disease
Mantle cell lymphoma (multiple lymphomatous polyposis)
Burkitt and Burkitt-type lymphoma
Nodal equivalents (diffuse large B-cell lymphomas, follicular, etc)
346
T-cell lymphoma
Enteropathy-type T-cell lymphoma (ETTL)
Figure 2-9-5
NHL: Small Intestine
Approximately 25% of all primary small bowel malignancies

Male predominance, mean age 60 years
Clinical presentation:
Weight loss, pain, bleeding
Intussusception, obstruction, perforation
Ileum most common location and duodenum least common
Multiple lesions in 10 to 25% of cases
NHL Small Bowel: Radiologic Patterns
Mural infiltration
Fold thickening
Circumferential wall thickening
Luminal dilatation
Polypoid nodules
Solitary
Multiple (lymphomatous polyposis)
Cavities
Mesenteric disease
NHL Small Intestine: Tumor Morphology [Figure 2-9-5]

NHL Small Intestine: Mural Infiltration
[Figures 2-9-6 to 2-9-8]
Figure 2-9-7
Lymphoma histology shows tumor

extension from mucosa to serosa
Figure 2-9-6
Ileal lymphoma showing mural infiltration, ulceration,

and nodularity
Ileal lymphoma extending

into small bowel mesentery
347
NHL Small Intestine: Cavitary Mass [Figure 2-9-9]
Figure 2-9-8
Figure 2-9-9
Cavitary mass
NHL Small Intestine: Adjacent Mesenteric Disease

[Figure 2-9-10]
Figure 2-9-10
Mesenteric mass engulfing small intestine
Burkitt Lymphoma
High grade B-cell lymphoma

More common in males
Endemic
African Burkitt, related to EBV
Head and neck disease
Sporadic
Western countries, not related to EBV
ileocecal region of children
Intestinal obstruction
Intussusception
Mural infiltration with luminal

dilatation
Mantle Cell Lymphoma [Figure 2-9-11]

Mantle Cell Lymphoma (Multiple Lymphomatous Polyposis)
Histologically resembles the mantle zone of the lymph follicle

Median age 65, male predominance
Abdominal pain and bloody stools
Imaging
Multiple polyps, 0.5 to 2.0 cm
Solitary polyp
Most common in the ileocecal
region
Figure 2-9-11
Polypoid masses of mantle cell lymphoma

348
Enteropathy-Type T-cell Lymphoma (ETTL) [Figure 2-9-12]
Associated with celiac disease (Sprue)

Sixth to seventh decade of life
Most common site jejunum
Gross pathology
Ulcerated plaques
Strictures
Poor prognosis
Figure 2-9-12
NHL Small Intestine:

Adenocarcinoma
GIST
Carcinoid
Metastases
Crohn disease
Tuberculosis
Causes of fold thickening
Sprue
Hemorrhage
Edema
Ischemia
Enteropathy type T-cell lymphoma
Figure 2-9-13
Malignant Melanoma Metastases

Gastrointestinal Stromal Tumor
Jejunal Adenocarcinoma
Tuberculosis / Lymphoma
Post-transplantation Lymphoproliferative
Disorder (PTLD)
Spectrum of benign and malignant disorders

Variable incidence
1% renal transplants
10% combined heart/lung
10% of patients on cyclosporine and OKT3
Association with EBV infection
Lung, GI tract
PTLD [Figures 2- 9-13 and 2-9-14]
Pathologic Features
Driven by Epstein-Barr Virus infection
Pathogenesis of post-transplantation
lymphoproliferative disorder
Clinical
May respond to reducing immunosuppression, anti-virals, surgery
Figure 2-9-14
AIDS-Related Lymphoma
Second most common neoplasm in HIV

infection
AIDS defining illness
Incidence is 4% to 10% in the AIDS
population
Three categories
Systemic (nodal and/or extranodal)
Primary CNS
Colonic lymphoma in a patient with a renal transplant

349
Body cavity-based (primary effusion) lymphomas

Major histologic subtypes
Burkitt lymphoma
Burkitt-like lymphoma
Large cell lymphoma
Large cell immunoblastic lymphoma
AIDS-Related Lymphoma
25% have GI tract disease

Higher incidence of mesenteric disease than non-AIDS lymphomas
Aggressive histology and biologic behavior
Atypical radiologic features
Hemorrhage
Necrosis
Unique subtypes
Body cavity-based lymphoma (Kaposis sarcoma-associated herpes virus
(KSHV))
Anorectal lymphoma
Figure 2-9-15
Primary Peritoneal Lymphoma

[Figure 2-9-15]
Colonic Lymphoma
Anorectal Lymphoma [Figure 2-9-16]
Summary
Primary peritoneal lymphoma in AIDS

Spectrum of Adenopathy
GI lymphomas are predominantly
NHL
Unique subtypes involve the bowel
Various patterns: infiltrating masses, luminal dilatation, polyps, cavitary
masses, mesenteric masses
AIDS-related
Aggressive behavior
Unusual sites, unusual manifestations
PTLD
Figure
Colon, liver
2-9-16
Patterns of Adenopathy
Patterns of Small Bowel Disease
1-Mural Infiltration
2-Polyps
Anorectal lymphoma in AIDS
3-Cavitary Masses
4-Mesenteric Masses
AIDS-Related Lymphomas
Post Transplantation Lymphoproliferative Disorder (PTLD)
350
References
Ann Arbor Staging of Gastrointestinal Lymphomas
Stage IE:
Confined to the wall of the stomach or bowel
Stage II1E:
Regional lymph nodes contiguous to primary site
Regional lymph nodes not contiguous to primary site
Stage II2E:
Stage III:
Lymph nodes on both sides of the diaphragm, spleen (IIIS), or both (IIIE&S)
Stage IV:
Bone marrow or other non-hematolymphoid organ
World Health Organization Classification of B-Lymphoid Neoplasms
Precursor B-cell Neoplasms
Precursor B-lymphoblastic leukemia/lymphoma
Mature (peripheral) B-cell Neoplasms
B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma
B-cell prolymphocytic leukemia
Lymphoplasmacytic lymphoma
Splenic marginal zone B-cell lymphoma
Hairy cell leukemia
Plasma cell myeloma/plasmacytoma
Extranodal marginal zone B-cell lymphoma of MALT type
Nodal marginal zone B-cell lymphoma
Follicular lymphoma
Mantle-cell lymphoma
Diffuse large B-cell lymphoma
Burkitt lymphoma
World Health Organization Classification of T- and NK-Lymphoid Neoplasms
Precursor T-cell Neoplasms
Precursor T-lymphoblastic lymphoma/leukemia
Mature (peripheral) T-cell Neoplasms
T-cell prolymphocytic leukemia
T-cell granular lymphocytic leukemia
Aggressive NK-cell leukemia
Adult t-cell lymphoma/leukemia
Extranodal NK/T-cell lymphoma
Enteropathy-type T-cell lymphoma
Hepatosplenic gamma-delta T-cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma
Mycosis fungoides/Sezary syndrome
Peripheral T-cell lymphoma, not otherwise characterized
Angioimmunoblastic T-cell lymphoma
Anaplastic large-cell lymphoma
Lymphoma Classification
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and lymphoid tissues: report of the Clinical Advisory Committee meeting--Airlie House, Virginia, November, 1997.
Hematol J 1:53, 2000
2. Jaffe ES, Harris NL, Stein H, et al (eds): World Health Organization Classification of Tumours: Pathology and
Genetics of Tumours of Haematopoietic and Lymphoid Tissues), Lyon: IARC Press, 2001
Imaging of non Hodgkin lymphoma
1. Byun JH, Ha HK, Kim AY, et al: CT Findings in Peripheral T-Cell Lymphoma Involving the Gastrointestinal Tract.
Radiology 227:59, 2003
2. Choi D, Lim HK, Lee SJ, et al: Gastric mucosa-associated lymphoid tissue lymphoma: helical CT findings and
pathologic correlation. AJR 178:1117, 2002
3. Crump M, Gospodarowicz M, Shepherd FA: Lymphoma of the gastrointestinal tract. Semin Oncol 26:324, 1999
4. Gossios K, Katsimbri P, Tsianos E: CT features of gastric lymphoma. Eur Radiol 10:425, 2000
5. Isaacson PG: Gastrointestinal lymphoma. Hum Pathol 25:1020, 1994
6. Isaacson PG: Gastrointestinal lymphomas of T- and B-cell types. Mod Pathol 12:151, 1999
7. Isaacson PG: Intestinal lymphoma and enteropathy. J Pathol 177:111, 1995
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Isaacson PG: Mucosa-associated lymphoid tissue lymphoma. Semin Hematol 36:139, 1999
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Histopathology 8:641, 1984
Kessar P, Norton A, Rohatiner AZ, et al: CT appearances of mucosa-associated lymphoid tissue (MALT) lymphoma.
Eur Radiol 9:693, 1999
Levine MS, Elmas N, Furth EE, et al: Helicobacter pylori and gastric MALT lymphoma. AJR Am J Roentgenol
166:85, 1996
Levine MS, Rubesin SE, Pantongrag-Brown L, et al: Non-Hodgkin's lymphoma of the gastrointestinal tract:
radiographic findings. AJR Am J Roentgenol 168:165, 1997
Megibow AJ, Balthazar EJ, Naidich DP, et al: Computed tomography of gastrointestinal lymphoma. AJR 141:541,
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high-grade malignancy in relation to the mucosa-associated lymphoid tissue concept. AJR 179:1297, 2002
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and Differential Diagnosis. Radiographics 23:457, 2003
AIDS-related lymphomas
1. Albin J, Lewis E, Eftekhari F, et al: Computed tomography of rectal and perirectal disease in AIDS patients. Gastrointest
Radiol 12:67, 1987
2. Brar HS, Gottesman L, Surawicz C: Anorectal pathology in AIDS. Gastrointest Endosc Clin N Am 8:913, 1998
3. Burkes RL, Meyer PR, Gill PS, et al: Rectal lymphoma in homosexual men. Arch Intern Med 146:913, 1986
4. Ferrozzi F, Tognini G, Mulonzia NW, et al: Primary effusion lymphomas in AIDS: CT findings in two cases. Eur
Radiol 11:623, 2001
5. Gottlieb CA, Meiri E, Maeda KM: Rectal non-Hodgkin's lymphoma: a clinicopathologic study and review. Henry
Ford Hosp Med J 38:255, 1990
6. Ioachimm HL, Antonescu C, Giancotti F, et al: EBV-associated anorectal lymphomas in patients with acquired immune
deficiency syndrome. Am J Surg Pathol 21:997, 1997?
7. Munn S: Imaging HIV/AIDS. Burkitt's lymphoma. AIDS Patient Care STDS 16:395, 2002
Post-transplantation lymphoproliferative disorder
1. Meador TL, Krebs TL, Cheong JJ, et al: Imaging features of posttransplantation lymphoproliferative disorder in
pancreas transplant recipients. AJR 174:121, 2000
2. Pickhardt PJ, Siegel MJ: Abdominal manifestations of posttransplantation lymphoproliferative disorder. AJR Am J
3. Pickhardt PJ, Siegel MJ: Posttransplantation lymphoproliferative disorder of the abdomen: CT evaluation in 51
patients. Radiology 213:73, 1999
4. Pickhardt PJ, Siegel MJ, Hayashi RJ, et al: Posttransplantation lymphoproliferative disorder in children: clinical,
histopathologic, and imaging features. Radiology 217:16, 2000
5. Tubman DE, Frick MP, Hanto DW: Lymphoma after organ transplantation: radiologic manifestations in the central
nervous system, thorax, and abdomen. Radiology 149:625, 1983
6. Vrachliotis TG, Vaswani KK, Davies EA, et al: CT findings in posttransplantation lymphoproliferative disorder of
renal transplants. AJR Am J Roentgenol 175:183, 2000
7. Wu L, Rappaport DC, Hanbidge A, et al: Lymphoproliferative disorders after liver transplantation: imaging features.
Abdom Imaging 26:200, 2001
352
Small Intestinal Neoplasms

Small Intestinal Neoplasms: Introduction
Small bowel neoplasms

Uncommon
Spectrum from hamartoma to benign to malignant
Majority of malignant small bowel tumors are metastatic
Primary small bowel tumors account for 1% to 2% of GI malignancies
Most common benign small bowel tumors
Adenoma
GIST (may be benign or malignant)
Lipoma
Location of benign tumors
Least common in duodenum
Most common in ileum
Primary malignant small bowel tumors
Strong associations with chronic inflammation: sprue, Crohn disease
Association with precursor conditions: FAP, MEN, NF1, Peutz-Jegher
Trend in distribution of malignant tumors is relative to histology
Adenocarcinoma most common in duodenum
Carcinoid most common in ileum
Lymphoma most common in ileum
GISTs, even distribution throughout small bowel
Clinical features
Many occult and asymtomatic until advanced disease
Symptoms dependent upon location and tumor morphology
Symptoms are similar in benign, malignant, primary or secondary
Diagnostic approach
Differential diagnosis based upon location, morphology, and associated
conditions
Small Intestinal Neoplasms: Objectives
Case based approach

Tumors of proximal duodenum
Periampullary tumors
Polypoid jejunal/ileal tumors
Annular tumors of jejunum and ileum
Tumors associated with adjacent disease in the mesentery
Tumors associated with NF1
Case based approach
Brunner gland lesions
Adenomas
Adenocarcinomas
Carcinoid
GISTs
Metastatic disease
38-year-old man with recent onset of abdominal pain
Figure 2-10-1
[Figure 2-10-1]
Brunner gland hamartoma

353
Differential Diagnosis: Proximal Duodenal Polyp
Nonneoplastic
Heterotopia
Prolapsed antral mucosa
Peutz-Jegher hamartoma
Neoplastic
Adenoma
Adenocarcinoma
GIST
Carcinoid
Prolapsed gastric neoplasm
Brunner Gland Hamartoma [Figure 2-10-1]
Solitary hamartoma
Brunner glands, muscular, and fatty elements
Heterotopic pancreatic acini and ducts
Synonym: Brunner gland adenoma
Most common in duodenal bulb
Clinical
Peak incidence, 4th to 6th decade
Asymptomatic or rarely, obstruction or bleeding
Treatment
Resection
Brunner Gland Hamartoma
Imaging features
Solitary
Sharply circumscribed polyp
Proximal duodenum
Homogenous CT attenuation
Composed of mostly glandular elements
Heterogeneous CT attenuation
Abundant fat, smooth muscle, and cystic change
Brunner Gland Hyperplasia
Smaller, multifocal version of Brunner hamartoma

Clinical associations
Duodenal ulcers
Gastric hypersecretory states
Treatment
None
Figure 2-10-2
Brunner Gland Hyperplasia [Figure 2-10-2]
Brunner gland hyperplasia
Lymphoid hyperplasia
Duodenitis
Adenomas in FAP
Hamartomas in Peutz-Jegher
Heterotopia
354
68-year-old woman with recurrent pancreatitis

[Figure 2-10-3]
Periampullary Duodenal Mass
Figure 2-10-3
Nonneoplastic
Choledochocele
Duplication cyst
Peutz-Jegher hamartoma
Neoplastic
Adenoma/adenocarcinoma
Carcinoid/NF1
GIST
Periampullary tubulovillous adenoma
Metastatic disease
Adjacent pancreatic or ampullary adenocarcioma
Figure 2-10-4
Tubulovillous Adenoma
Adenoma
Benign intraepithelial neoplasm composed of

dysplastic cells
Tubular, villous, or tubulovillous histology
May progress to adenocarcinoma
Locations
80% are periampullary
Increased incidence
Familial adenomatous polyposis, FAP
Hereditary nonpolyposis colon carcinoma,
HNPCC
Periampullary Adenocarcinoma
Imaging features
Biliary obstruction
Duodenal mural thickening or polypoid mass
May extend into adjacent pancreas and/ampulla
Periampullary adenocarcinoma
Figure 2-10-5
Adenocarcinoma Duodenum:
Ampullary/Periampullary
[Figures 2-10-4 and 2-10-5]
May arise from periampullary duodenal mucosa

May arise from ampulla
May be mixed location
Origin not clear
Small Bowel Adenocarcinoma
More common in proximal small intestine1

55% periampullary/ampullary
10% duodenum
25% jejunum
10% ileum
Association with colonic adenocarcinoma
APC gene
Mismatch repair gene
Ampullary adenocarcinoma
1Riddel RH, Petras RE, Williams GT, Sobin LH. Atlas of Tumor Pathology:Tumors
of the Intestines. AFIP 2003
355
Small Bowel Adenocarcinoma
Most patients between 50 and 60 years

Mean age, 55 years
Predisposing conditions
Inherited syndromes: FAP, HNPCC, Peutz-Jegher, NF1
Chronic inflammation: sprue, Crohn disease, ileostomies, ileal pouches,
bypassed bowel
Differential Diagnosis: Small Bowel, Intussuscepting Mass
Benign
Adenoma
Peutz Jegher polyp
Lipoma
Uncommon
Neurofibroma
Schwannoma
Inflammatory fibroid polyp
Heterotopia
Malignant
Metastasis
Adenocarcinoma
Lymphoma
GIST
Carcinoid
Figure 2-10-6
Jejunal Adenocarcinoma: Annular
Adenocarcinoma of the jejunum with annular morphology
[Figure 2-10-6]
CT features
Focal, annular mural thickening
Spiculated or irregular margins
+/- mesenteric adenopathy
Figure 2-10-7
Ileal Adenocarcinoma: Annular

and Infiltrating [Figure 2-10-7]
Differential Diagnosis: Jejunal or
Ileal Stricture
Neoplastic
Adenocarcinoma
Carcinoid
Lymphoma
Metastasis
Nonneoplastic
Crohn disease
Celiac disease
NSAID (tend to be web-like)
Ischemia
Tuberculosis
Heterotopia
Radiation
Adenocarcinoma of the ileum with annular and infiltrating

morphology
Figure 2-10-8
Ileal Adenocarcinoma:
Cavitary Mass [Figure 2-10-8]
Unusual presentation for

adenocarcinoma
More aggressive histology
Poorly differentiated
Endocrine features mixed
with adenocarcinoma
Adenocarcinoma of the ileum manifesting as a cavitary mass
356
Differential Diagnosis: Jejunal or Ileal Cavitary Mass
Figure 2-10-9
Metastatic disease
Lymphoma
Look for homogenous attenuation tumor
GIST
Adenocarcinoma
50-year-old man with abdominal pain and

diarrhea
[Figures 2-10-9 and 2-10-10]
CT findings
Fixed segment of ileum
Mural thickening
Adjacent spiculated mesenteric mass
Carcinoid
Well-differentiated endocrine neoplasms

Classification
Foregut, stomach and proximal duodenum
Midgut (60% to 80%), distal duodenum, jejunum, ileum, appendix,
ascending colon, proximal transverse colon
Hindgut, distal transverse colon,
descending colon, rectum
Spectrum of clinical/imaging features
Population and type of endocrine
cell changes throughout the bowel
Variety of hormones produced
Biologic behavior ranges from
benign to malignant
Figure 2-10-10
Carcinoid
Duodenal Carcinoid
Carcinoid
Most common in first and second portion

Low-grade malignancies
Gastrin or somatostatin production most common
Periampullary tumors = somatostatin producing and NF1 association
Associations
Zollinger-Ellison syndrome
Multiple endocrine neoplasia (MEN 1)
Neurofibromatosis type 1 (NF1)
Figure 2-10-11
Duodenal Carcinoid [Figure 2-10-11]
Imaging features
Solitary or multifocal polyps
Intramural mass
Jejunal and Ileal Carcinoid

Serotonin production
Desmoplasia
Kinking of bowel
Spiculation of mesentery
Ischemia from "elastic vascular sclerosis
May have carcinoid syndrome with liver mets
357
Duodenal carcinoid
Jejunal and Ileal Carcinoid: Imaging Features
Figure 2-10-12
Discrete mass in wall of bowel

Mural mass
Polypoid mass
Multiple masses, less
common
Extensive wall abnormalities
Luminal narrowing
Thick, spiculated folds
Local nodal mesenteric
metastasis often most prominent
feature
Spiculated, fibrotic mass
adjacent to bowel
Sunburst pattern of vessels
on angiogram
May calcify
Carcinoid Ileum [Figure 2-10-12]

Carcinoid Ileum
Elastic vascular sclerosis

Sunburst pattern of mesenteric
vessels
Multifocal nodules
Kinking of bowel
Rigid segment of bowel
Ileal carcinoid
Somatostatin Receptor Scintigraphy:

Octreotide Scans (111In-Pentetreotide) [Figure 2-10-13]
Carcinoid Syndrome
10% of patients with carcinoids

Most common with ileal
carcinoids
Hepatic metastasis are usually
present
Serotonin and metabolites in
systemic circulation
Classic syndrome
Paroxysms of sweating,
flushing, cyanosis, wheezing,
abdominal colic, right-sided
heart failure, diarrhea
Symptoms precipitated by
ETOH intake, stress, exercise
Carcinoid heart disease
Right sided valvular dysfunction
Congestive heart failure
Figure 2-10-13
Metastatic carcinoid shown on CT and 111In-pentetreotide

scintigraphy
Carcinoid: Differential Diagnosis
Figure 2-10-14
Metastatic disease
Lymphoma
Granulomatous infection
Sclerosing mesenteritis
77-year-old asymptomatic man

[Figure 2-10-14]

358
Differential Diagnosis:
Small Bowel Polypoid Mass
Figure 2-10-15
Benign
Adenoma
Peutz Jegher polyp
Inflammatory fibroid polyp
Malignant
Metastatic disease
Adenocarcinoma
Lymphoma
GIST
Carcinoid

Small bowel second most common site
Variable biologic behavior
Figure 2-10-16
GIST: Small Bowel, polypoid

GIST: Small Bowel, mural
[Figure 2-10-15]
GIST: Small Bowel, polypoid

and exophytic
GIST: Small Bowel, exophytic
[Figure 2-10-16]
GIST: Small Bowel, cavitary
52-year-old man with NF-1 complains of abdominal pain

Gastrointestinal Neoplasms in NF-1
Neurofibroma
Carcinoid
Duodenal
Somatostatinoma
Gastrointestinal stromal tumors
Small intestine, multiple
Ganglioneuroma
Leiomyoma, leiomyosarcoma
Adenocarcinoma
Metastatic Disease
Most common site for metastasis in GI tract

Metastasis are more common than primary malignancies in the small bowel
Widespread metastatic disease usually present
Hematogenous spread
Melanoma
Lung
Breast
Kidney
Intraperitoneal, direct extension, lymphatic spread
Tumors of GI origin
Ovarian and endometrial carcinoma
Imaging patterns
Identical to primary neoplasms
Polyps, mural masses, annular strictures, cavitary lesions, association with
mesenteric nodal masses
359
Metastatic Disease: Renal Cell Carcinoma [Figure 2-10-17]
Figure 2-10-17
Metastatic Disease:
Melanoma [Figure 2-10-18]
Summary: Brunner Gland
Lesions

Solitary mass
Proximal duodenum
Multiple nodules
Proximal duodenum
Renal cell carcinoma metastatic to the small bowel
Summary: Adenoma
Uncommon
Most periampullary
Association
FAP
HNPCC
Figure 2-10-18
Periampullary location most

common
Morphology
Polypoid
Annular
Infiltrating
Cavitary
Melanoma metastatic to the small bowel
Summary: Carcinoid
Endocrine neoplasms
Midgut most common
Serotonin production
Octreotide scintigraphy
Key imaging features
Mural wall thickening
Fixation of bowel
Mesenteric mass
Mesenteric retraction
Summary: GIST

KIT positive
Mural masses
Intraluminal polyp
Exophytic component
Hemorrhage
Cyst formation
Cavitation
Summary: Metastatic Disease
Most common malignancy in the small bowel
360
Colorectal Carcinoma
Colorectal Carcinoma: Objectives
Epidemiology/pathogenesis
Screening
Detection
Preoperative assessment
Staging
Rectal carcinoma
Third most frequent cancer in the U.S.1

~150,000 new cases per year
11% of cancers in men and women
10% of cancer deaths
1Jemal A et al. CA Cancer J Clin 2005; 55:10-30
Colorectal Carcinoma: Risk Factors
Lifetime risk 6%
Incidence increases after age 50
Familial risk
2 to 4 fold increase risk with a single first degree relative
3 to 6 fold increase risk with two first degree relatives
Increased risk
Familial adenomatous polyposis syndrome (FAP)
Inflammatory bowel disease
Colorectal Carcinoma: Pathogenesis
Adenoma-Carcinoma Sequence
Slow evolution to cancer, average 10 years
Adenoma detection and removal = cure
Exception to adenoma-carcinoma sequence
Carcinomas in inflammatory bowel disease
Adenoma-Carcinoma Sequence [Figures 2-11-1]

Colorectal Carcinoma: Role of Radiology
Screening
ACBE
CT colonography
Detection
Symptomatic patients
Preoperative screening
Primary disease complications
Preoperative staging
Recurrent disease
Figure 2-11-1
Adenoma to carcinoma sequence progressive from normal

mucosa, unicryptal adenoma, polypoid adenoma, dysplasia,
high-grade dysplasia, carcinoma in-situ, to invasive carcinoma
361
Colorectal Carcinoma: American Cancer Society Screening

Recommendations
Average risk adults begin screening at age 50

Annual fecal occult blood (FOBT) or fecal immunochemical test (FIT)
Sigmoidoscopy every 5 years
Annual FOBT or FIT + Sigmoidoscopy every 5 years
Colonoscopy every 10 years
DCBE every 5 years
Colorectal Carcinoma: American College of Gastroenterology

Polyp guidelines1
Colonoscopy every 3 years, high risk for metachronous adenomas

>2, >1cm, villous histology or high-grade dysplasia
Colonoscopy every 5 years, low risk for metachronous adenomas
1-2 tubular adenomas, no family history
1Bond JH. Am J Gastroenterology 2000. 95(11): 3053-3063
Colorectal Carcinoma: Screening
Air contrast barium enema

Accuracy 90% for polyps >1 cm
Pitfalls
Anatomic difficulties (overlapping segments)
Diverticular disease
Perceptive errors
Colonoscopy
Accuracy 90%
Invasive, requiring sedation
Perforation rate .1% to .5%
Pitfalls
Failure to reach cecum
Blind spots
Colorectal Carcinoma: Screening
Virtual colonography
Sensitivity 73% to 93% for >10mm polyps
Prone and supine imaging improves sensitivity
Difficult lesions
Poor bowel preparation
Flat adenomas
Adenomas on folds
Adenomas seen in only one position
Colorectal Polyps: Histologic Spectrum
Hyperplastic
Most common
Usually <5 mm, descending colon and rectum
NOT neoplastic
Adenoma
Tubular, 75% are <1 cm, most pedunculated
Villous, 60% are > 2 cm, most sessile
Mixed
Juvenile
Peutz-Jeghers
Inflammatory/post-inflammatory
Tubular Adenoma
Villous Adenoma
362
Adenoma
Figure 2-11-2
Size
< 5 mm, benign
5 mm to 1 cm, 1% are carcinoma
1 - 2 cm, 10% are carcinoma
> 2 cm, 30% to 50% are carcinoma
Synchronous adenomas
40% to 50%
Recurrence
20% to 60% recurrence rate
Majority recur within 2 years
Adenoma: Barium Features
Filling defect in barium pool

Protrusion into the lumen
Innies not Outies
Bowler hat sign
Sessile or pedunculated
Carpet lesions
Sessile lesions
Bubbly or nodular contour
Villous change
Bowler Hat Sign [Figure 2-11-2]

Virtual Colonography
Villous Adenoma
Higher rate of malignancy

Recurrence rate 9.3%
Three types
Flat, carpet-like
Sessile, lobulated
Pedunculated
Histology
Nonbranching finger-like fronds
Sessile adenomatous polyp showing

the Bowler Hat sign
Villous Adenoma: Pathology [Figure 2-11-3]

Figure 2-11-3
Villous adenoma of the cecum showing a bubbly, carpet-like

appearance
363
Villous Adenoma: CT Features [Figure 2-11-4]
Figure 2-11-4
Soft tissue mass

Sessile
Eccentric
Stalk
Expands lumen
Irregular luminal margin
Low attenuation luminal margin
High mucin content
Colonic Adenocarcinoma
Villous adenoma of the rectum showing low attenuation along

the luminal margin
Colorectal Carcinoma: Distribution
[Figure 2-11-5]
Colorectal Carcinoma: Clinical Presentation
Figure 2-11-5
Minimal or absent symptoms in up to 12% of patients

Bleeding
Initial complaint in 50%
Weight loss, malaise
Pain
Change in bowel habits
Right vs. left sided lesions
Symptoms from complications
Obstruction, ischemia, perforation, peritonitis,
fistula
Colorectal Carcinoma: Morphologic Patterns
Polypoid
Intraluminal masses
Bulky, fungating masses in cecum and ascending
colon
Infiltrating/annular constricting
Transverse, descending, and sigmoid colon
Encircle the bowel
Apple core
Diffuse infiltration (linitis plastica) uncommon
Ulcerating
Deeply invade colonic wall
Edge of tumor slightly elevated above normal mucosa
Flat plaques
Carcinomas from flat adenomas
Carcinomas in inflammatory bowel disease
Distribution of colorectal carcinoma
Colorectal Carcinoma: Computed Tomography
Primary Tumor
Discrete mass
Mural thickening
Extension beyond the bowel
Irregular outer margin
Soft-tissue stranding in pericolonic fat
Adjacent organ/muscle invasion
Loss of fat planes
Tumor mass in adjacent organ or muscle
Liver metastasis
Lymph node metastasis
364
Polypoid Adenocarcinoma
Figure 2-11-6
[Figure 2-11-6]
Annular Adenocarcinoma
[Figure 2-11-7]
Infiltrating Adenocarcinoma
[Figure 2-11-8]
Pericolonic Extension and

Adenopathy
Adjacent Organ Invasion:
Contiguous Soft Tissue
Attenuation [Figure 2-11-9]
Polypoid adenocarcinoma of the cecum showing pericolonic

extension
Figure 2-11-7
Adjacent Organ Invasion

Coloduodenal Fistula
Multiple Carcinomas
Synchronous carcinomas
Diagnosed within 6 months of each
other
Incidence 1.5% to 12%
Most are >5 cm away from each
other
Metachronous carcinomas
Incidence 0.6% to 9.1%
Time interval to second lesion
discovery
64% within 5 years
45% within 3 years
20% within 1 year
8% to 20% of patients with colorectal
carcinomas have malignancies in other
organs
Annular adenocarcinoma of the distal transverse colon
Figure 2-11-8
Infiltrating adenocarcinoma of the sigmoid colon with

pericolonic extension and pericolonic adenopathy
Figure 2-11-9
Synchronous Carcinomas
[Figure 2-11-10]
Rectal adenocarcinoma (T4) showing contiguous soft tissue

attenuation into the pelvic side walls consistent with adjacent
organ invasion
Figure 2-11-10
Synchronous adenocarcinomas of the hepatic flexure and

descending colon
365
Colonic Adenocarcinoma in Inflammatory Bowel Disease:

[Figure 2-11-11]
Figure 2-11-11
Ulcerative colitis
Highest incidence
Crohn disease
Large and small intestinal
adenocarcinoma
Features of carcinoma in IBD
Typically do not arise in pre-existing
adenomas
Arise in flat mucosa
Carcinomas may be long and flat
Adenocarcinoma in Ulcerative
Colitis
Colorectal Carcinoma:
Complications
CT of Obstructing Colon
Carcinomas
Adenocarcinoma in ulcerative colitis
Bleeding
Occult
Chronic anemia
Massive bleeding, unusual
Obstruction
Occlusion of the colonic lumen
Colocolic intussusception
Perforation
Abscess
Fistula
Differential diagnosis, diverticulitis
IV contrast
Identify obstructing lesion
Infiltration of adjacent fat
Evaluate bowel integrity
Obstructive colitis (1% to 7%)
Ischemic changes
Pneumatosis
Stage
Local extension
Lymph node mets
Liver mets
Figure 2-11-12
Colonic ischemia in an obstructing carcinoma of the

descending colon
Figure 2-11-13
Perforated adenocarcinoma of the transverse colon with

abscess formation
CT of Obstructing Colon Carcinomas

Ischemia in Obstructive Cancers [Figure 2-11-12]
Carcinoma with Perforation and Abscess [Figure 2-11-13]
Colorectal Carcinoma:Role of Preoperative Imaging
Tumors proximal to the rectum are staged surgically

Preoperatively image patients with clinical evidence of advanced disease
Preoperative imaging rectal tumors
EUS and CT
MR
366
Colorectal Carcinoma: Preoperative CT
Local tumor extension

Liver metastasis
Early rim enhancement, followed by hyperdensity
Hypodense in the portal venous phase
Isodense in the equilibrium phase
Lymphatic Spread
Rectal Adenocarcinoma: Preoperative EUS
Endoscopic Ultrasound (EUS)*

360 degree probe
Normal 5-layer rectal wall
T stage accuracy 69% to 97%
Nodal accuracy 70% to 80%
EUS limitations
EUS best at early stage tumors
Limited assessment because of location or bulk occurs
May overstage (fibrosis vs. tumor vs. inflammation)
Intraobserver variability
Wolfman NT, Ott DJ. Endoscopic Ultrasonography. Semin Roentgenol 1996. 31(2):
154-161.
Beets-Tan RGH, Beets GL. Rectal cancer: review with emphasis on MR imaging.
Radiology 2004. 232: 335-346
Rectal Adenocarcinoma: Preoperative MR
Endoluminal MR
Equal accuracy for early stage tumors to EUS
T stage accuracy, 71% to 91%
Phased array MR
High spatial resolution
Large field of view
Limitations differentiating T2 and T3 lesions
Colorectal Carcinoma: Role of PET
No role in screening/diagnosis
Highly sensitive for liver mets
Not sensitive for T staging and nodal mets
Detection of recurrent disease
Following liver met resection/treatment
Scar vs. recurrent tumor at resection margin
Hustinx R. PET imaging in assessing gastrointestinal tumors. RCNA 2004. 112 (6)
1123-1139.
Rectal Adenocarcinoma: Management
High T1 or T2 lesion
Lesions 5 to 6 cm above dentate line or at peritoneal reflection
Primary resection and anastomosis (LAR)
Low T1 or T2 lesion
APR (Miles procedure), LAR, coloanal anastomosis with J-pouch, local or
transanal excision, total mesorectal excision, posterior proctotomy
T3 or T4
Downstage with preoperative neoadjuvant chemoradiation
APR and post operative XRT, adjuvant chemotherapy
367
TNM Staging [Figure 2-11-14]
Figure 2-11-14
T-Primary tumor
T1 invades submucosa
T2 invades muscularis propria
T3 through muscularis propria or
into nonperitonealized pericolic fat
T4 perforates visceral peritoneum or
directly invades adjacent organs or
structures
N-Regional nodes
M-Distant metastasis
EUS Layer 1: Hyperechoic

superficial mucosa
EUS Layer 2: Hypoechoic deep
mucosa
EUS Layer 3: Hyperechoic
submucosa
EUS Layer 4: Hypoechoic
muscularis propria
TNM Staging for colorectal carcinoma
EUS Layer 5: Hyperechoic perirectal fat [Figure 2-11-15]

T1 N0 M0
Figure 2-11-15
T2 N0 M0
T3 N2 M0 [Figure 2-11-16]
T3 N10 M0
T3 N8 M1
T4
T4: Extension to pelvic side
wall
T4: Extension to labia
Normal anatomy of the colon wall

with endoscopic ultrasound
Figure 2-11-16
T3N2M0 rectal adenocarcinoma
368
Colorectal Carcinoma:
Lymphatic Spread [Figure 2-11-17]
Figure 2-11-17
Pericolonic nodes
Paracolic
Epiploic
Mesenteric Nodes
Intermediate nodes
Principal nodes
SMA
IMA
Pericolonic Nodes
Intermediate
Principal
Rectal Adenocarcinoma:
Lymphatic Drainage [Figures 2-11-18 and 2-1119]
Pararectal nodes
Internal iliac nodes
Tumors above dentate line
Inguinal nodes
Tumors below dentate line
Distribution of
lymphatic spread for
colon carcinoma
Figure 2-11-18
Distribution of lymphatic spread for rectal carcinoma
Figure 2-11-19
Inguinal lymph nodes in a rectal adenocarcinoma that

extended below the dental line
369
Summary: Adenoma
40% - 50% synchronous

20% - 60% recur
BE features
Filling defect
Bowler hat
Sessile
Pedunculated
Summary: Villous Adenoma
Carpet lesions
Bubbly appearance
Expand lumen
Low attenuation on luminal surface
Summary: Primary Tumor
Morphology
Polypoid
Infiltrating/annular
Ulcerating
Flat plaques
Synchronous carcinomas
CT
Local extent
Summary: Complications
Bleeding
Usually chronic blood loss
Massive GI bleed, unusual
Obstruction
CT
Identify lesion and bowel wall integrity
Perforation
Abscess
Fistula
Differential diagnosis inflammatory disorders
Summary: Role of Imaging
Preoperative CT
Local tumor extent
Liver metastasis
Lymphatic spread
Summary: Rectal Adenocarcinoma
EUS and CT, MR
T3 lesions
Through muscularis propria
Spiculated outer margin on CT
Perirectal adenopathy
370
References:
Virtual Colonography
1. Fidler JL, Johnson CD, MacCarty RL, et al: Detection of flat lesions in the colon with CT colonography. Abdom
Imaging 27:292, 2002
2. Fletcher JG, Johnson CD, MacCarty RL, et al: CT colonography: potential pitfalls and problem-solving techniques.
AJR Am J Roentgenol 172:1271, 1999
3. Fletcher JG, Johnson CD, Welch TJ, et al: Optimization of CT colonography technique: prospective trial in 180
patients. Radiology 216:704, 20
4. Gluecker TM, Fletcher JG, Welch TJ, et al: Characterization of Lesions Missed on Interpretation of CT Colonography
Using a 2D Search Method. AJR Am J Roentgenol 182:881, 2004
5. Gluecker TM, Johnson CD, Harmsen WS, et al: Colorectal cancer screening with CT colonography, colonoscopy,
and double-contrast barium enema examination: prospective assessment of patient perceptions and preferences.
Radiology 227:378, 2003
6. Johnson CD, Ahlquist DA: Computed tomography colonography (virtual colonoscopy): a new method for colorectal
screening. Gut 44:301, 1999
7. Johnson CD, Harmsen WS, Wilson LA, et al: Prospective blinded evaluation of computed tomographic colonography
for screen detection of colorectal polyps. Gastroenterology 125:311, 2003
8. Johnson CD, Toledano AY, Herman BA, et al: Computerized tomographic colonography: performance evaluation in
a retrospective multicenter setting. Gastroenterology 125:688, 2003
9. Macari M: Virtual colonoscopy: clinical results. Semin Ultrasound CT MR 22:432, 2001
10. Pescatore P, Glucker T, Delarive J, et al: Diagnostic accuracy and interobserver agreement of CT colonography (virtual
colonoscopy). Gut 47:126, 2000
11. Pickhardt PJ: Three-dimensional endoluminal CT colonography (virtual colonoscopy): comparison of three
commercially available systems. AJR Am J Roentgenol 181:1599, 2003
12. Pickhardt PJ, Choi JR, Hwang I, et al: Computed tomographic virtual colonoscopy to screen for colorectal neoplasia
in asymptomatic adults. N Engl J Med 349:2191, 2003
13. Royster AP, Fenlon HM, Clarke PD, et al: CT colonoscopy of colorectal neoplasms: two-dimensional and threedimensional virtual-reality techniques with colonoscopic correlation. AJR Am J Roentgenol 169:1237, 1997
14. Spinzi G, Belloni G, Martegani A, et al: Computed tomographic colonography and conventional colonoscopy for
colon diseases: a prospective, blinded study. Am J Gastroenterol 96:394, 2001
15. Taylor SA, Halligan S, Bartram CI: CT colonography: methods, pathology and pitfalls. Clin Radiol 58:179, 2003
16. Taylor SA, Halligan S, Bartram CI, et al: Multi-detector row CT colonography: effect of collimation, pitch, and
orientation on polyp detection in a human colectomy specimen. Radiology 229:109, 2003
17. Taylor SA, Halligan S, Goh V, et al: Optimizing bowel preparation for multidetector row CT colonography: effect of
Citramag and Picolax. Clin Radiol 58:723, 2003
18. Taylor SA, Halligan S, Goh V, et al: Optimizing colonic distention for multi-detector row CT colonography: effect
of hyoscine butylbromide and rectal balloon catheter. Radiology 229:99, 2003
371
Mesenteric Masses and Cysts

Mesenteric Masses and Cysts: Objectives
Definitions
Review mesenteric anatomy
Case based approach to differential diagnosis
Mesenteric and omental cysts
Mesothelioma
Mesenteric Fibromatosis
Inflammatory myofibroblastic pseudotumor
Extrapleural solitary fibrous tumor
Figure 2-12-1
MesentericAnatomy: Definitions
Mesentery
Double fold of peritoneum
Connects an organ to the
abdominal wall
Omentum
Specialized mesentery
extending from stomach
to an adjacent organ
Anatomy Mesentery
[Figure 2-12-1]
Transverse mesocolon
Small bowel mesentery
Sigmoid mesentery
Mesoappendix
Anatomy: Omentum
[Figure 2-12-2]
Greater omentum
Gastrocolic ligament
Gastrosplenic ligament
Gastrophrenic ligament
Lesser omentum
Gastrohepatic ligament
Hepatoduodenal ligament
Normal posterior attachments of the

mesentery in sagittal and AP planes
Figure 2-12-2
Mesenteric and Omental Cyst
Descriptive term
5 histologic subtypes
Defined by internal lining
Mesenteric and Omental Cyst
Lymphangioma
Endothelial lining
Enteric duplication cyst
Enteric lining with muscular wall
Enteric cyst
Enteric lining with a fibrous wall
Mesothelial cyst
Mesothelial lining
Nonpancreatic pseudocyst
No lining
Normal anatomy of greater and lesser omentum
372
35-year-old woman with increasing abdominal girth [Figure 2-12-3]

Differential Diagnosis: Cystic Mesenteric Mass
Mesenteric cyst
Lymphangioma
Enteric cyst
Mesothelial cyst
Cystic neoplasm
Teratoma
Cystic malignant mesothelioma
Benign multicystic mesothelioma
Cystic soft tissue primary
Pseudomyxoma peritonei
Complex ascites
Infectious, neoplastic
Pseudocyst
Internal hemorrhage, abscess
Figure 2-12-3
Lymphangioma
Lymphangioma
Figure 2-12-4
Benign
Vascular origin
Affect all ages
Many anatomic sites
95% neck, axilla
5% mesentery
Lymphangiomatosis
Abdominal Lymphangioma:
Pathology
Lymphangioma of the greater omentum
Interconnecting cysts
Endothelial lining
Dilated lymphatic spaces
Proteinaceous fluid
Chyle, low attenuation
Hemorrhage
Abdominal Lymphangioma: Imaging Features [Figures 2-12-4 and 2-12-5]
Mesenteric, omental or retroperitoneal location

Complex cyst
Multilocular
Enhancing septations
Internal debris
Closely associated with small bowel
Figure 2-12-5
Lack features of free fluid
Mass effect
Septations
No fluid in dependent spaces
peritoneum
Infiltration/insinuation
Within mesentery and bowel
Complications
Small bowel obstruction
Volvulus
Mesenteric lymphangioma showing low attenuation
Infection
and insinuating growth
373
71-year-old woman with abdominal pain [Figure 2-12-6]
Figure 2-12-6
Pancreatic cystic neoplasm

Oligocystic adenoma
Mesenteric cyst
Lymphangioma
Enteric cyst
Mesothelial cyst
Cystic mesenteric neoplasm
Metastatic disease
Cystic mesothelioma
Enteric Duplication Cyst

Enteric Cyst and Mesothelial Cyst
Enteric cyst
Variant of enteric duplication, does not contain muscular wall
Mesothelial cyst
Rare
Fusion failure of visceral/parietal peritoneum
Nonspecific imaging features
Similar appearance compared to enteric duplication cyst
Figure 2-12-7
Nonpancreatic Pseudocyst [Figure 2-12-7]
Old hematoma, abscess

No histologic lining
Imaging
Thick walled
Internal debris
55-year-old man, former shipyard

worker, with worsening abdominal
pain [Figure 2-12-8]
Metastatic disease
Primary neoplasms
Diffuse malignant mesothelioma
Serous papillary carcinoma
Intra-abdominal desmoplastic round
cell tumor
Leiomyomatosis peritonealis
disseminata
Diffuse Infection
Tuberculosis
Histoplasmosis
Figure 2-12-8
Diffuse Malignant Mesothelioma
Malignancy of mesothelial origin

Association with asbestos
Variants
Diffuse peritoneal malignant mesothelioma
374
Diffuse Malignant Mesothelioma
Gross Pathology
Nodules, masses, caking
Bowel encasement
Thick, nodular peritoneum
Ascites
Histopathologic variants
Desmoplastic
Lymphohistiocytoid
Small cell
Papillary
Diffuse Peritoneal Malignant Mesothelioma
Imaging features
Peritoneal soft tissue nodules
Omental and mesenteric masses, nodules
Ascites
Bowel wall thickening
Fixation of small bowel
Diffuse Peritoneal Malignant Mesothelioma: Peritoneal, Omental

Nodules and Masses
Figure 2-12-9
Diffuse Malignant Mesothelioma:

Small Bowel Fixation
Cystic Malignant Mesothelioma
[Figure 2-12-9]
Benign Multicystic Mesothelioma

[Figure 2-12-10]
Rare
Arises from pelvic peritoneum
Unrelated to asbestos
Unrelated to malignant mesothelioma
Synonym
Multilocular peritoneal inclusion cyst
Mean age, 37 years
Clinical symptoms
Chronic pelvic pain
Figure 2-12-10
Benign Multicystic Mesothelioma
Imaging features
Multicystic pelvic mass
Enhancing septa
Peritoneal surfaces of uterus, bladder
May extend into upper abdomen
Benign multicystic mesothelioma
Benign Multicystic Mesothelioma: Differential Diagnosis
Metastasis
Mucinous adenocarcinoma
Serous papillary carcinoma of ovary
Primary serous papillary carcinoma of peritoneum
Infection with complex ascites
Tuberculosis
375
31-year-old man complained of abdominal fullness and early

satiety. Physical exam revealed a palpable mass
Figure 2-12-11
[Figure 2-12-11]
Solid Mesenteric Mass
Malignant
Soft tissue sarcoma
Lymphoma
Metastatic disease
Benign
Figure 2-12-12
Mesenteric Fibromatosis:
(Intraabdominal Fibromatosis or
Abdominal Desmoid)
Classified as a deep fibromatosis

Mesenteric, pelvic, retroperitoneal
Abdominal wall
Extraabdominal
Benign proliferative process
Mesenteric fibromatosis with low CT attenuation, located in the
Locally aggressive
greater omentum
Recurs following excision
Does not metastasize
No gender predilection
Most cases sporadic
13% associated with FAP
Abdominal fibromatosis
Most common in young women, 20-30 years of age
Mesenteric Fibromatosis: Pathologic Features
Gross pathology
Well-defined or infiltrative margins
Histology
Melting insinuating and tentacular growth
Microscopic tumor infiltration into bowel
Collagenous and/or myxoid stroma
Figure 2-12-13
Mesenteric Fibromatosis: Imaging
Homogeneous
Collagenous stroma
Myxoid stroma (low attenuation
CT/high signal T2 MR)
Heterogeneous
Bands of myxoid stroma whorls
Homogeneous Attenuation
Mesenteric Fibromatosis: Low CT Attenuation Myxoid Stroma

[Figure 2-12-12]
Mesenteric Fibromatosis : High T2 Signal [Figure 2-12-13]
376
Mesenteric Fibromatosis: MR Enhancement

Mesenteric Fibromatosis in FAP
[Figure 2-12-14]
Figure 2-12-14
Complications
Fistula formation
Perforation
GI bleeding
Infiltrates small bowel wall
Mesenteric Fibromatosis in FAP
Mesenteric fibromatosis in a patient with FAP. The myxoid

stroma creates a whorled pattern in this example
Almost always post operative

Occurs at operative sites
Usually within 4 years of surgery
Unusual manifestations
Multiplicity
May occur with abdominal wall fibromatosis
Diffuse form may involve mesentery, pelvis, and retroperitoneum
Mesenteric, Pelvic, and Retroperitoneal: Fibromatosis in FAP

Differential Diagnosis: Solid Mesenteric Mass
Malignant
Soft tissue sarcoma
Lymphoma
Metastatic disease
Benign
Metastatic Disease: Metastatic Lung Carcinoma

Soft Tissue Sarcoma: Synovial Sarcoma
Lymphoma
Gastrointestinal Stromal Tumor: Small Bowel Primary
Gastrointestinal Stromal Tumor: Primary to the Mesentery
Gastrointestinal Stromal Tumor: Retroperitoneal
Mesenteric Fibromatosis: Management
Controversial
Wide excision, antiestrogens, chemotherapy, radiation therapy
Complications and recurrence common
Sporadic cases
Surgery often curative
FAP
Higher recurrence rate
Higher morbidity
Nonsurgical therapy more commonly used
377
Mesenteric Fibromatosis: Postoperative Recurrence

Sclerosing Mesenteritis
Rare
Idiopathic, nonneoplastic
Chronic inflammation
Synonyms represent histologic spectrum
Mesenteric panniculitis
Fibrosing mesenteritis
Mesenteric lipodystrophy
Sclerosing Mesenteritis: Clinical Features
Twice as common in men

Mean age, 60 years
Symptoms
Pain
Palpable mass
Bowel complications
Incidental
Figure 2-12-15
Sclerosing Mesenteritis:
Pathologic Features
Pathologic spectrum
Loose myxomatous to dense
sclerosis
Histologic features
Sclerosing fibrosis
Fat necrosis
Lipid-laden macrophages
Focal calcification
Sclerosing Mesenteritis: Imaging Features [Figure 2-12-15]
Mesenteric mass
Mixed fat and soft tissue
Radiating fibrosis
Fat-ring sign
Calcifications
Cystic appearance
Small Bowel
Kinking or fixation
Figure 2-12-16
Sclerosing mesenteritis with the fat ring sign on CT
[Figure 2-12-16]
Fat-ring Sign
Sclerosing Mesenteritis [Figure 2-12-17]

Sclerosing Mesenteritis: Differential Diagnosis
Figure 2-12-17
Carcinoid metastasis
Look for primary
Somatostatin scintigraphy
Metastatic disease
Sclerosing mesenteritis with low CT attenuation from loose

myxomatous stroma shown on the accompanying histology
image
378
Sclerosing Mesenteritis: Somatostatin Receptor Scintigraphy

Sclerosing Mesenteritis: Management
Biopsy may establish diagnosis

Many cases self-limiting
Asymptomatic or mild symptoms
Observation
Symptomatic
Immunosuppresive therapy
Surgical resection
Inflammatory Pseudotumor (Inflammatory Myofibroblastic

Tumors)
Unclear pathogenesis
Sequelae occult infection
Minor trauma
Post surgical
Variable nomenclature
Plasma cell pseudotumor
Inflammatory fibrosarcoma
Inflammatory Pseudotumor: Clinical Features
Most common in pediatrics and young adults

May occur in may anatomic locations
Symptoms
Fever
Malaise
Weight loss
Pain
Inflammatory Pseudotumor [Figure 2-12-18]

Figure 2-12-18
Extrapleural Solitary Fibrous Tumor
Rare neoplasms
Submesothelial origin
Most commonly pleural origin
Solitary Fibrous Tumor of the

Peritoneum
Few case reports

Natural history unknown
Pleural lesions may show
aggression
Long-term follow up
Extrapleural Solitary Fibrous Tumor

Summary
Mesenteric cysts
Solid mesenteric masses
Summary: Mesenteric Cyst
Lymphangioma
Most common
Imaging
Multilocular
Enhancing septa
Insinuating growth
379

Enteric cyst
Histologic differentiation
Identical imaging
Mesothelial cyst
Nonspecific imaging appearance
No histologic lining
Old trauma/abscess
Imaging
Thick wall
Internal debris
Summary: Mesothelioma

Asbestos
Nodules, masses
Bowel encasement
Bowel fixation
Variant of DMM
Cystic masses
Ascites
Summary: Benign Multicystic Mesothelioma
Controversial
AKA peritoneal inclusion cyst
Unrelated to DMM
Unrelated to asbestos
Pelvic peritoneum
Multicystic mass
Summary: Benign Fibrous Lesions
Solitary fibrous tumor of peritoneum
Summary: Mesenteric Fibromatosis
Benign
Locally aggressive
Association with FAP
Imaging
Homogeneous
Heterogeneous
Myxoid stroma
Low CT attenuation
High T2 signal
whorled pattern
Summary: Sclerosing Mesenteritis
Idiopathic inflammation
Imaging
Mixed attenuation
Bowel retraction
May calcify
Conservative treatment
380
Summary: Inflammatory Pseudotumor
Inflammatory/fibrotic infiltrate
Nonspecific imaging
Summary: Extrapleural Solitary Fibrous Tumor
Few case reports
References
Lymphangioma
1. Kempson RL, Fletcher CDM, Evans HL, Hendrickson MR, Sibley RK. Tumors of the soft tissues: atlas of tumor
pathology, third series, fascicle 30. Washington, DC: Armed Forces Institute of Pathology; 2001
2. Levy AD, Cantisani V, Miettinen M. Abdominal Lymphangiomas: Imaging Features with Pathologic Correlation.
AJR 2004. 182: 1485-1491
3. Ros PR, Olmsted WW, Moser RP, Jr., Dachman AH, Hjermstad BH, Sobin LH. Mesenteric and omental cysts:
histologic classification with imaging correlation. Radiology 1987;164:327-332
1. Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB. Intra-abdominal fibromatosis. A pathologic analysis
of 130 tumors with comparison of clinical subgroups. Am J Surg Pathol 1990; 14(4):335-341.
2. Levy AD, Rimola J, Mehrotra AK, Sobin LH. Benign Fibrous Tumors and Tumor-like Lesions of the Mesentery:
Radiologic Pathologic Correlation. RadioGraphics 2006; 26: 245- 264
3. Magid D, Fishman EK, Jones B, Hoover HC, Feinstein R, Siegelman SS. Desmoid tumors in Gardner syndrome: use
of computed tomography. AJR Am J Roentgenol 1984; 142(6):1141-1145.
1. Emory TS, Monihan JM, Carr NJ, Sobin LH. Sclerosing mesenteritis, mesenteric panniculitis and mesenteric
lipodystrophy: a single entity? Am J Surg Pathol 1997; 21(4):392-398.
2. Sabate JM, Torrubia S, Maideu J, Franquet T, Monill JM, Perez C. Sclerosing mesenteritis: imaging findings in 17
patients. AJR Am J Roentgenol 1999; 172(3):625-629.
3. Valls C. Fat-ring sign in sclerosing mesenteritis. AJR Am J Roentgenol 2000; 174(1):259-260.
381
Idiopathic Inflammatory Bowel Disease

Idiopathic Inflammatory Bowel Disease: Objectives
Ulcerative colitis (UC)

Crohn disease
Idiopathic Inflammatory Bowel Disease: General Features
Etiology unknown
? Genetic basis
? Immune related
? Infectious agent
Incidence of IBD
UC is more common than Crohn
IBD: Epidemiologic Comparison
Ulcerative colitis
35-100 cases/100,000
Age range, 15-25 years,
second peak, 50 to 80 years
Urban dwellers
Developed countries
Whites, 2 to 5 times risk
Jewish, 2 to 4 times risk
Family history, 30 to 100
times risk
Crohn disease
10-70 cases/100,000
Age range, 15-25 years,
second peak, 50-80 years
Urban dwellers
Whites
Jewish (8 fold increase)
Family history, 12 to 15 times
risk
IBD: Comparison of Clinical Features
Ulcerative colitis
Diarrhea
Obstruction rare
Rectal bleeding usually
Abdominal pain,
predefecatory urgency
Chronic, low grade illness in
most
Acute, fulminating in 15%
Crohn disease
Diarrhea
Obstruction common
Rectal bleeding, less
common
Abdominal pain, post
prandial, colicky
Abdominal mass
Vomiting
Perianal disease
Alternating attacks and
remissions
IBD: Comparison of Disease Distribution
Ulcerative colitis
Colon to anus, rarely TI

Continuous disease or
Ulcerative proctitis that may
extend proximally
Crohn disease
Entire GI tract, mouth to anus
Asymmetric, skip lesions
May extend beyond bowel
382
IBD: Pathologic Features
Ulcerative colitis
Mucosal and submucosal
inflammation
Minimal mural edema
Crohn disease
Transmural inflammation
Marked mural edema
IBD: Gross Pathologic Features [Figure 2-13-1]
Ulcerative colitis
Fine ulceration
Granular mucosa
Hyperemic
IBD: Gross Pathologic Features
Ulcerative colitis
Shallow ulcers, granularity
Loss of haustra
IBD: Histologic Features
Ulcerative colitis
Diffuse mucosal ulceration
Crypt abscesses
Inflammatory infiltrate
Pseudopolyps
Crohn disease
Linear ulcers
Cobblestones
Marked mural thickening
Crohn disease
Linear ulceration
Nodules
Crohn disease
Aphthous ulcer
Linear, serpiginous ulcers
Wide-based ulcers
Cobblestones
Fissures, fistulas, abscesses
Strictures
Pseudopolyps
IBD: Histologic Features of Active Disease
Ulcerative colitis
Crypt abscesses,
lymphoplasmacytic
lamina propria infiltrate
Ulcerative colitis
Crypt destruction
Crypt abscess
Hemorrhage
Ulcerative colitis
Atrophic, distorted
mucosa
Thick muscularis mucosa
Fat within submucosa
Crohn disease
Ulcers, fissures,
transmural lymphoid
aggregates, granulomas
Figure 2-13-1
Crohn disease
Aphthous ulcer
Lymphoid aggregates
Crohn disease
Fissures
Transmural lymphocytes
Gross pathologic specimens show

ulcerative colitis with a hemorrhagic
ulcerated mucosa and mural thinning.
Crohn disease has marked mural
thickening, mucosal cobblestones,
and proliferation of adjacent
mesenteric fat
383
Ulcerative Colitis: Imaging Features

Acute Changes [Figure 2-13-2]
Figure 2-13-2
Mucosal granularity
Mucosal stippling
Collar button ulcers
Haustral thickening or loss
Inflammatory polyps
Confluent, contiguous, circumferential disease
Ulcerative Colitis: Imaging Features

Chronic Changes
Haustra loss
Luminal narrowing
Loss of rectal valves
Widened presacral space
Backwash ileitis
Post-inflammatory pseudopolyps
Ulcerative Colitis: Role of CT
Early, acute disease

Low diagnostic sensitivity
Often normal
Complementary to endoscopy to assess for complications
Toxic megacolon
Pneumatosis
Perforation
Gore RM et al. AJR 1996: 167:3-15.
Ulcerative Colitis: CT Features [Figures 2-13-3 and 2-13-4]
Severe, acute disease

Mural thinning
Pneumatosis
Perforation
Subacute and chronic disease
Mural thickening
Luminal narrowing
Proliferation of
perirectal fat
Assessment/detection
of carcinoma
Figure 2-13-4
Acute ulcerative colitis with fine

mucosal ulceration producing
granularity and stippling on barium
evaluation
Figure 2-13-3
Gore RM et al. AJR 1996:

167:3-15.
Chronic ulcerative colitis

with mural stratification
(target sign), submucosal
fat in the rectum, and an
increase in the perirectal fat
Acute ulcerative colitis with mild

mural thickening and pericolonic
hyperemia on CT
384
Ulcerative Colitis
Mural stratification and luminal narrowing in chronic UC

Thickening of muscularis mucosa
Edema and fat in submucosa
Ulcerative Colitis: Perirectal Fat Proliferation

Toxic Megacolon and IBD
Clinical features
Fever, tachycardia, hypotension
Incidence
5% to 10% of UC
2% to 4% Crohn disease
May be initial manifestation of IBD
Other causes
Pseudomembranous colitis
Ischemia
Infection
Pathology
Transmural inflammation
Loss of normal tissue cohesion
Thinned wall and areas of denuded mucosa
Imaging
Abdominal radiograph establishes diagnosis
Marked colonic dilatation, 6 to 15 cm
Transverse colon most often involved
Nodular mucosa
Loss of haustra
Intraluminal fluid
Figure 2-13-5
Toxic Megacolon [Figure 2-13-5]
Ulcerative colitis
Colonic distension
Pseudopolyps
Ulcerative Colitis
Differential CT Features
CT features suggesting UC over Crohn disease

Mural stratification, 61% UC vs. 8% Crohn
Mural thickness less in UC compared to Crohn
Outer colonic contour, smooth in UC and irregular in Crohn
Gore RM et al. AJR 1996: 167:3-15
Crohn Disease
Early Imaging Features
Distribution
Asymmetric
Segmental
Skip lesions
Ulceration
Aphthous ulcerations
Linear ulcers
Deep ulcerations (fissuring)
Cobblestoning
Mural thickening
Inflammatory pseudopolyps
Ulcerative colitis with toxic

megacolon
385
Crohn Disease: Aphthous Ulcers [Figure 2-13-6]
Figure 2-13-6
Crohn Disease: Linear Ulceration and Nodules

Crohn Disease
Rose thorn ulcers
Crohn Disease: Cobblestoning [Figure 2-13-7]

Crohn Disease [Figure 2-13-8]
Figure 2-13-7
Figure 2-13-8
Crohn disease with aphthous ulcers
Crohn disease with cobblestoning
Crohn disease with terminal ileal

nodularity and ulceration
386
Crohn Disease: Acute/Early CT Features
Figure 2-13-9
Mural thickening
1 to 2 cm
Mural stratification
Mural enhancement
Target or double halo sign
Crohn Disease
Mesenteric hyperemia
Target sign
Acute inflammation
Crohn Disease
Linear ulceration
Mural thickening
Inflammatory polyps
Crohn Disease: Subacute to Chronic CT Features
Mural thickening
Homogeneous, nonenhancing = fibrosis or stricture
Enhancing wall = reversible inflammatory disease
Mesenteric changes
Fibrofatty proliferation
Lymphadenopathy
Hypervascularity
Inflammatory stranding
Phelgmon/abscess
Crohn Disease
Crohn disease with distal ileal

inflammation (target sign), fibrofatty
proliferation of the small bowel
mesentery, and mesenteric
lymphadenopathy

Nonenhancing = irreversible fibrosis
Loss of mural stratification
Crohn Disease
Fibrofatty proliferation
creeping fat
Mesenteric lymphadenopathy,
3 to 8 mm
Prominent, dilated vasa recta

comb sign
Figure 2-13-10
Crohn disease with prominent

and engorged vasa recta (comb
sign)
387
Crohn Disease: Chronic Features
Fissures, fistulas, and sinus tracts

Haustral loss
Strictures
Sacculations
Post-inflammatory pseudopolyps
Figure 2-13-11
Crohn Disease: Strictures

Sacculations and Strictures
[Figure 2-13-11]
Crohn Disease: Stricture

Crohn Disease
Complications
Sinus tracts
Fistula
Abscess
Carcinoma
Crohn Disease
Intramural Fistula
Crohn Disease
Fistulae [Figure 2-13-12]
Crohn Disease: Abscess
Chronic Crohn disease with structuring and sacculations
[Figure 2-13-13]
Secondary to deep penetrating ulcers

Sinus tracts
Fistulas
Perforation
15% to 20% of patients
Most frequently associated with small bowel or ileocolic disease
Figure 2-13-12
Crohn Disease: Perirectal Sinus Tract
Figure 2-13-13
Crohn disease with a ileal-ileal fistula
Crohn disease with a psoas abscess

388
Crohn Disease: Extraintestinal Complications
Hepatobiliary
Hepatic steatosis, 20% to 50%
Cholelithiasis, 30% to 50%
PSC, 1% to 4%
Hepatic abscess
Pancreatic
Pancreatitis
Musculoskeletal
Arthritis
Sacroileitis-spondylitis
GU tract
Nephrolithiasis, 2% to 10%
Neoplasia in IBD
Adenocarcinoma
Ulcerative colitis, highest incidence
Crohn disease, small and large intestine
Lymphoma
Increased incidence in Crohn disease
Features of carcinoma in IBD
Typically do not arise in pre-existing adenomas
Arise in flat mucosa
Carcinomas may be long and flat
May arise in bypassed segments of bowel
Adenocarcinoma in Ulcerative Colitis

Adenocarcinoma in Crohn Disease
Additional Imaging Modalities: Sonography UC vs. Crohn
Disease
Ulcerative colitis
Hypoechoic wall
Mural stratification
Loss of haustra
Loss of peristalsis
Crohn disease
Hypoechoic wall
Loss of mural stratification
Loss of haustration
Diminished compressibility
Absent peristalsis
Increased blood flow
Gore RM, Laufer I, Berlin, JW. Ulcerative and granulomatous colitis: idiopathic
inflammatory bowel disease. In: Gore RM, Levine MS (eds), Textbook of
Gastrointestinal Radiology. 2nd ed.
Sarrazin J, Wilson SR. Manifestations of crohn disease at US. RadioGraphics
1996. 16: 499-520.
Additional Imaging Modalities: MR Enterography of Crohn

Disease
Evolving technique
Assessment of active disease
Mural thickening > 4mm
Mural enhancement
Increase in mesenteric vascularity
Koh DM et al. MR imaging evaluation of the activity of crohns disease. AJR 2001:
177(6) 1325-1332.
389
Establishing Diagnosis: UC vs. Crohn
Clinical course
Disease distribution
Endoscopy findings
Biopsy
UC vs. Crohn Disease: CT Features
Mural thickening
Greatest with Crohn disease
Submucosal fat
More commonly seen in UC
Mesenteric fibrofatty proliferation
Crohn disease
But, perirectal fat may increase in UC
Sinus tracts, fistulas, abscess
Crohn disease
Differential Diagnosis of IBD
Infectious colitis
Ischemic colitis
Radiation enteropathy and colitis
Behet disease
Graft vs. host disease
Diverticular disease
Summary
UC vs. Crohn
Similar demographics
UC, contiguous colonic disease
Crohn, entire GI tract with skip areas
UC, mucosal and submucosal disease
Crohn, transmural disease with extension into the mesentery
390
Approach to Inflammatory Disease of the

Colon
Objectives
General approach
Differential diagnosis of idiopathic IBD
Neutropenic colitis (typhlitis)
Ischemic colitis
Diverticulitis
Infectious colitis
General Approach
Disease location
Small vs. large bowel
Focal vs. multifocal vs. diffuse
Ascites
Degree and pattern of mural thickening
How much mural thickening?
Mural enhancement?
Fat attenuation in the submucosa?
Associated mesenteric disease
Inflammation
Phelgmon/abscess
Mesenteric fat proliferation
Clinical history
Helpful Features for Differential Diagnosis
Location of disease
Small bowel involvement
Diffuse vs. focal vs. multifocal
Degree of mural thickening
Marked mural thickening favors PMC
Clinical history
Antibiotics (PMC)
Radiation therapy
Neutropenia
Travel history
Colitis Differential Diagnosis
Right-sided disease
Campylobacter
Yersinia
Salmonella
Typhlitis
Crohn disease
TB
Amebiasis
Histoplasmosis
Diffuse disease
Ischemia
PMC
E. Coli
Shigella, campylobacter,
salmonella, amebiasis
CMV
Inflammatory bowel
disease
Behet syndrome
Graft vs. host disease
(GVDH)
Radiation
391
Inflammatory Disease of the Colon
41-year-old man who developed diarrhea one month after

hospitalization for pneumonia [Figure 2-14-1]
Figure 2-14-1
Pseudomembranous Colitis
Features suggesting PMC

Diffuse colonic involvement
Marked low attenuation mural thickening
Accordion sign
Ascites
Onset following antibiotic therapy

Clostridium difficile toxin
Clinical features
Symptoms within days or weeks following antibiotic therapy
Copious watery diarrhea
Abdominal pain
Fever
Leukocytosis
Epithelial necrosis
Inflammatory infiltrate
Crypt eruption
Pseudomembranes
Figure 2-14-2
Pseudomembranous Colitis: CT Features
Mural thickening
Low attenuation
Accordion sign
Target sign
Intraluminal plaques or nodules
Pericolonic inflammation
Ascites
Complications
Luminal dilatation, toxic megacolon
Perforation
Pseudomembranous Colitis:
Thumbprinting = Mural Thickening
Pseudomembranous Colitis:
Low Attenuation Wall with Accordion Sign
Pseudomembranous Colitis: Target Sign [Figure 2-14-2]
PMC typically has the greatest amount of mural thickening of the

compared to other colitis
Presence of ascites favors acute colitis over IBD
PMC, infectious, ischemia
65-year-old woman abdominal pain during chemotherapy for

leukemia [Figure 2-14-3]
Neutropenic Colitis (Typhlitis)
Suggestive features
Clinical history
Right-sided involvement
Clinical features
Children and adults
Neutrophil counts <500 to 1000 cells/mm3
Fever, diarrhea, pain, abdominal distension
392
Figure 2-14-3
Pathogenesis
Cecal stasis
Cytotoxic mucosal injury
Bacterial invasion
CT features
Predominant right-sided disease
Mural thickening, low attenuation
Ascites
Pneumatosis
70-year-old man with acute abdominal pain and

bloody diarrhea [Figure 2-14-4]
Ischemic Colitis
Most common in elderly

Underlying atherosclerosis, diabetes, hypertension
Low flow states
Occlusive disease
Complicates infectious colitis, especially CMV
Ischemic Injury
Acute, fulminant
Transmural necrosis and perforation
Transient, reversible
Confined to mucosa and submucosa
Chronic
Submucosal fibrosis
Locations
Diffuse
SMA and IMA watershed vunerable
Normal communication through Arc of Riolan (marginal
artery of Drummond)
Absent in 5%
Splenic flexure and rectosigmoid
Neutropenic colitis
Figure 2-14-4
Ischemic Colitis
Denuded mucosa
Pseudomembranes, Hemorrhage
Ischemic Colitis- Imaging Features
Mucosal ulceration
Mural thickening
Low attenuation
Target sign
Luminal dilatation
Ascites
Chronic changes
Fibrosis, stricture
Ischemic Colitis- Acute
[Figure 2-14-4 and 2-14-5]
Figure 2-14-5
Ischemic colitis
Ischemic colitis
393
Ischemic Enteritis - Pathophysiology
Figure 2-14-6
Blood supply reduced by >50%

Arterial occlusive
Venous occlusive
Nonocclusive (low flow states)
Ischemic Enteritis
Segmental
Diffuse
Segmental, necrotic, ulcerated mucosa
Mural thickening
Stack of coins
Thick wall, engorged mesentery
Target sign [Figure 2-14-6]
Thin wall, infiltrated mesentery
Infiltrated mesentery [Figure 2-14-7]
Mucosal cast, intramural fistula
Mucosal ulceration, pneumatosis
Pneumatosis, mesenteric venous gas
Figure 2-14-7
Ischemic enteritis due to SMV

thrombosis
Figure 2-14-8
. Ischemic enteritis
Ischemic Colitis or Enteritis [Figure 2-14-8]
Chronic
Imaging Findings Suggestive of Ischemia
Ileus
Dilated bowel
Gasless abdomen
Unchanging bowel
Mural thickening
Stack of coins
Target sign
Mucosal ulceration
Ulceration
Intramural fistulas
Loss of folds
Mucosal breakdown
Intraluminal mucosal cast
Pneumatosis
Mesenteric or portal venous gas
Intraperitoneal air
394
Chronic ischemic colitis in the

watershed region of the colon
50-year-old woman with abdominal pain and fever [Figure 2-14-9]
Figure 2-14-9
Diverticulitis - Hepatic Flexure
Diverticular Disease
Most common disease of the

colon
Diverticulosis increases with age
33% to 50% of people over
50
50% of people over 80
Acute diverticulitis
Diverticular Disease
Etiology of Pulsion Diverticula
Pressure gradient
Between lumen and serosa
Compartmentalized by haustra
Highest in sigmoid
Weakness in bowel wall
Intramural vasa recta penetrate wall
Between taenia mesocolica and taenia libera
Between taenia mesocolica and taenia omentalis
Diverticular Disease - Pathologic Features
False diverticula
Mucosa and submucosa only
0.5 to 1.0 cm
Myochosis
Thickening of circular muscle
Shortening of taenia
Narrowing of the lumen
Results in corrugated appearance
Diverticular Disease: CT Features
Mural thickening
Diverticular outpouchings
Diverticular Disease: Complications
Diverticulitis
Hemorrhage
Giant sigmoid diverticulum
Diverticulitis
Most common complication

10% to 20% of patients with diverticulosis
Pathogenesis
Stagnation of fecal material
Inflammatory erosion of the mucosa
Perforation
Intramural abscess
Extramural abscess
Diverticular Disease: Complications
Diverticulitis
Luminal obstruction
Infection
395
Diverticulitis: CT Features
Figure 2-14-10
Diverticula
May have hyperdense fecal material
Inflammatory changes
Pericolonic stranding
Pericolonic phlegmon
Intramural abscess
Pericolonic abscess
Circumferential mural thickening
Usually < 1 cm
Rarely exceeds 2-3 cm
Tethered lumen
Saw-tooth configuration
Due to muscular spasm
Pneumoperitoneum, abscess
Diverticulitis: Hyperdense Fecal Material

Diverticulitis: Intramural Fistula - Pneumatosis
[Figure 2-14-10]
Diverticulitis: Complications
Hemorrhage 25%
Muscular hypertrophy and obstruction 25%
Pericolic abscess 20%
Free perforation 18%
Debilitated patients
Corticosteroid therapy
Vesicocolic fistula 8%
Pyelophlebitis and liver abscess
Acute diverticulitis with intramural

fistula and pneumatosis
Diverticulitis: Perforation
Diverticulitis: Pericolic Abscess
Diverticulitis: Colovesical Fistula
Colon carcinoma
Colitis
Infectious
Ischemic
Crohn disease
Foreign body perforation
Epiploic appendagitis
Diverticulitis vs. Carcinoma
Wall thickening
Mild circumferential thickening in diverticulitis (4 to 5 mm)
Carcinoma usually > 2 cm
Zone of transition
Abrupt change in lumen caliber favors carcinoma
Lobulated soft-tissue favors carcinoma
Tethered (saw-tooth) lumen favors diverticulitis
Favors diverticulitis
Regional adenopathy
Favors carcinoma
396
Diverticulitis vs. Carcinoma
Figure 2-14-11
Diverticulitis: CT Pitfalls
Differential diagnosis of colon cancer

Problematic in 10% of cases
If immediate surgery not performed: mucosal evaluation
(endoscopy or BE) to exclude cancer
Diverticulitis: CT and Surgical Management
Antibiotic therapy
Mild Diverticulitis
CT guided drainage
Focal abscess
Immediate Surgery
Free perforation with peritonitis
Severe hemorrhage
Elective surgical resection
Following successful abscess drainage
Recurrent diverticulitis
Persistent pain
Bleeding
Diverticular Hemorrhage
Most common cause of adult rectal bleeding

Site of hemorrhage
Single diverticulum
Right side of colon in 2/3 of cases
Rupture of the vasa recta
Giant sigmoid diverticulum
Diverticular Hemorrhage
Clinical features
Elderly patients
Sudden onset
Stops spontaneously in 80%
Rebleeding in 25%
Cecal Diverticulitis
Congenital or acquired diverticula

Congenital are true diverticula
Acquired are most common
CT features
Mural thickening
Demonstration of diverticula
Intramural or pericolonic abscess
Normal appendix
Giant Sigmoid Diverticulum [Figure 2-14-11]
Rare
Etiology
Subserosal perforation and inflammation
Air trapping
Ball-valve mechanism
Clinical Features
Chronic pain
Palpable mass
397
Giant Sigmoid Diverticulum
Figure 2-14-12
CT features
Mesenteric side of sigmoid colon
Usually 7 cm or greater
Thin wall
Unilocular
30-year-old man with diarrhea and pain following

vacation in Mexico [Figure 2-14-12]
Campylobacter Colitis
Infectious colitis
Distribution
Clinical
Summary
General approach
Location of disease
Degree of mural thickening
Ascites
Mesenteric disease
Clinical history
Summary - Pseudomembranous Colitis
History of antibiotics
Clostridium difficile
Mural thickening
Accordion sign
Ascites
Campylobacter colitis
Summary - Neutropenic Colitis/Typhlitis
Chemotherapy
Low neutrophil counts
Bacterial invasion
Predominant right-sided disease
Mural thickening
Pneumatosis
Ascites
Summary - Ischemic Colitis
Elderly patients
Diffuse
Watershed regions
Acute
Mucosal ulceration
Mural thickening
Chronic
Mural thickening
Stenosis
Summary - Diverticulitis
Focal disease
Diverticula
Air filled
Hyperdense
Adjacent inflammation
398
Summary - Diverticulitis vs. Carcinoma
Favor Diverticulitis
Lesser mural thickening
Gradual zone of transition
Tethered lumen
Favor carcinoma
Greater mural thickening
Lobular mural thickening
Sharp zone of transition
Pericolonic adenopathy
Summary
Infectious colitis
Clinical history
Right-sided colitis
Campylobacter, yersinia, salmonella
TB
Amebiasis
Diffuse colitis
PMC
E. Coli
Shigella, campylobacter, salmonella
Amebiasis
CMV
399
Gastrointestinal Seminar 1: Abdominal Gas

Case 1: 45 year old man with chronic pancreatitis and acute onset
of lower abdominal pain, distension, and constipation
Cecal volvulus
Marked Cecal Dilatation
Cecal volvulus
Cecal bascule
Pseudoobstruction (Ogilvie
syndrome)
Cecal Volvulus
Volvulus is an axial twist of at

least 90 degrees
Abnormal fixation to posterior
parietal peritoneum
Freely mobile cecum
Mechanical obstruction
Radiographic features
Cecal dilatation
Beak on contrast enema
Whirl on CT
Cecal volvulus
Seminar 1: Abdominal Gas
400
Cecal Bascule
Anterior-cephalad fold
May cause obstruction
Volvulus:Bascule = 10:1
Ogilvie Syndrome
Colonic pseudo-obstruction
Marked cecal dilatation
Sigmoid Volvulus
Transverse Colon Volvulus
Cecal bascule
Sigmoid volvulus
401
Case 2: 85 year old woman with abdominal pain, fever, and

shock
Intestinal ischemia with infarction and hepatic portal venous

gas
Intestinal Ischemia with Infarction and Hepatic Portal

Venous Gas
Hepatic Portal Venous Gas
Branching radiolucencies extending to within two cm of the

hepatic capsule
Must differentiate from pneumobilia
Bowel Necrosis (75%)
IBD (10%)
Abscess
Obstruction
Ulcer
Pneumobilia
Pneumobilia
Portal venous gas
Intestinal Infarction with Portal Venous Gas

Portal venous gas
402
Case 3: 60 year old man with progressive dyspepsia and acute,

severe upper abdominal pain
Pneumoperitoneum Gastric Ulcer
Perforation
Pneumoperitoneum Signs on the
Supine Abdominal Film
Diaphragmatic Slips (Leaping Dolphins)

Central Diaphragm (Cupola)
Morisons Pouch (Doges Cap)
Lesser Sac
Falciform Ligament
Fissure of Ligamentum Teres
Leaping Dolphin
Cupola Sign
Doges Cap
Pneumoperitoneum from gastric ulcer perforation
Case 4: 67 year old man with severe chest pain after vomiting
Boerhaaves Syndrome
Dr. Hermann Boerhaave

Emetogenic rupture
Distal esophagus or
Gastric cardia
Left posterolateral region
1.5 - 4 cm tear
Reduced muscle fibers
Entrance of nerves, vessels
Mediastinal gas
LLL infiltrate, atelectasis
Left effusion
Causes of Esophageal Rupture
Spontaneous
Boerhaave
Mallory-Weiss
Iatrogenic
Endoscopic
Dilation
Tube placement
Other
Caustic Ingestion
Trauma
Inflammatory
Neoplastic
Boerhaave syndrome
403
Case 5: 50 year old man with upper abdominal pain, epigastric

fullness, and constipation
Cecal Herniation through the
Foramen of Winslow
Foramen of Winslow
Foramen Of Winslow Hernia
8% of internal hernias
Involved bowel
Small intestine 70%
Cecum
25%
Cecal herniation
Persistence of ascending
mesocolon
Mobility
Alterations in intraabdominal
pressure
Cecal herniation through the foramen of Winslow
Circumscribed gas collection LUQ
Medial and posterior to stomach
Stomach displaced left and anterior
Gas in lesser sac from abscess or perforation
Cecal herniation through the foramen of Winslow
404
Gastrointestinal Seminar 2:
Nonneoplastic Disease of the Stomach
Case 1: 65-year-old woman presents with retching and the
production of little vomitus. The ER physician cannot pass a NG
tube into her stomach
Gastric Volvulus
Abnormal rotation of the

stomach
Rare
Children and adults
Most are secondary to fixation defect
Associated anomalies
Diaphragmatic defects
Malrotation
Wandering spleen
Asplenia
Mesenteroaxial volvulus
Gastric Volvulus
Classic Clinical presentation

Severe epigastric pain
Violent retching with production of little vomitus
Inability to pass NG tube into stomach
Outcomes
Recurrent
Resolve spontaneously
Resolve with NG placement
Complete obstruction
405
Seminar 2: Nonneoplastic Disease of the Stomach
Organoaxial
Rotation about a line extending from cardia to pylorus
Mesenteroaxial
Rotation about a line connecting middle of lesser curvature to middle of
greater curvature
Mixed types occur
30% associated with hiatal hernia
Gastric Volvulus - Radiologic Features
Double air-fluid level

Inversion of stomach
Greater curve above lesser curve
Positioning of cardia and pylorus at the same level
Downward pointing pylorus and duodenum
Organoaxial Volvulus
Mechanism for organoaxial volvulus
Mesenteroaxial Volvulus
Mechanism for mesenteroaxial volvulus
406
Case 2: 22-year-old woman developed epigastric pain when she

was dieting in preparation for her wedding
Gastric Bezoar
Accumulated ingested material

Trichobezoar
Hair
Phytobezoar
Vegetable matter
Pathophysiology
Altered gastric motility
Altered gastric anatomy
Trichotillomania
Gastric Bezoar
407
Case 3: 8-year-old girl with recurrent emesis and diarrhea

Imaging findings
Thick gastric folds

Thick duodenal wall
Liver metastasis
Large, enhancing pancreatic mass
Positive pentetreotide scan
Zollinger-Ellison Syndrome:
Pancreatic Gastrinoma
Zollinger-Ellison Syndrome
Affects all ages, peak 3rd to 5th decade

Gastrin-secreting neuroendocrine tumor
(gastrinoma)
Pancreas (75%)
Duodenum (15%)
Liver, ovary, lymph nodes
60% malignant
Clinical Features
One or more benign peptic ulcers
Diarrhea from hypergastrinemia (30%)
Elevated gastrin levels
May occur in MEN I syndrome
Pancreatic
gastrinoma
producing
Zollinger Ellison
syndrome
Zollinger-Ellison Syndrome
Radiologic features
Multiple ulcers
Increased gastric secretions
Thick gastric folds
Preoperative localization of gastrinoma
CT
MR
Somatostatin receptor scintigraphy
47-year-old man with recurrent PUD

Ectopic Gastrinoma in Retroperitoneal Lymph Node
Thick Gastric Wall? Gastric Air-Fluid Sign*
*Hammerman AM, Mirowitz SA, Susman N. Gastrointestinal Radiology 14:109112. 1989
Differential Diagnosis: Thick Gastric Folds
Hypertrophic Gastropathy
Menetrier disease
Zollinger-Ellison syndrome
Gastritis
Neoplasm
Adenocarcinoma
Lymphoma
Metastasis
Miscellaneous
Amyloid
Eosinophilic gastritis
Adjacent inflammation
408
Case 4: 70-year-old man presents epigastric pain and pedal

edema
Menetrier Disease
Menetrier Disease : Adult Form
Most common in men, 50 to 70 years

Symptoms
Epigastric pain
Vomiting
Weight loss
Peripheral edema
Hypoalbuminemia and hypochlorhydria
Diffuse enlargement of gastric folds
Proximal stomach
Mucus hypersecretion
Irreversible
Menetrier Disease : Pediatric Form
Associated with CMV infection

Allergic or autoimmune reaction
Symptoms
Periorbital and facial edema
Vomiting
Pain
Self-limited
Spontaneous resolution and reversal of protein loss
Antrum more commonly involved
Menetrier Disease : Pathology
Pathology
Foveolar hyperplasia, glandular atrophy, cysts
Enlarged folds (1-3 cm) resembling cerebral convolutions
H. pylori?
Menetrier Disease : Radiology
Thick folds
Nonuniform
Tortuous
Spiculation of greater curvature
Antral sparing
Flocculation of contrast
409
Case 5: UGI images from two different patients that complained

of epigastric pain. Both patients had a history of diarrhea
Case 5A: Radiologic Findings
Narrowed antrum
Multiple filling defects
Nodularity
Ulceration?
Effaced/nodular duodenal bulb
Case 5B
Case 5A
Case 5B: Radiologic Findings
Multiple ulcers
Aphthous ulcers
Nodularity
Crohn disease
Case 5 : Differential Diagnosis
Gastritis
H. pylori
Radiation
Caustic ingestion
Neoplasm
Adenocarcinoma
Lymphoma
Mets
Crohn
Sarcoid
Amyloid
TB
Gastric Crohn Disease
Histologically present in up to 33% of patients with Crohn disease

20% of patients with ileo-colic disease have abnormal UGI1
Antrum and duodenum most often affected
1Levine MS. Crohns disease of the upper gastrointestinal tract. RCNA 1987
Gastric Crohn Disease
Ulcers
Aphthous lesions
One or more large ulcers
Nodules
Focal nodules
Cobblestone mucosa
Abnormal gastric motility
Fibrosis
Tubular antrum (rams horn sign or shofar sign)
Obliteration of the pylorus (pseudo-Billroth I sign)
410
Pancreatic Duct
Normal Pancreatic Embryology
18 days
Normal Anatomy
22 days
Minor Papilla
Accessory PD
Duct of Santorini
Major Papilla
Main PD
Duct of Wirsung
5 weeks
Normal pancreatic and biliary duct

anatomy
5 weeks
Anatomic variants of the pancreatic duct
Normal pancreatic duct by ERCP and MRCP

411
Seminar 3: Pancreatic duct
Case 1: 25-year-old woman with a long history of nausea,

vomiting, and abdominal distension
Annular Pancreas
Bilobed ventral pancreatic bud

Buds migrate in opposite directions
Duodenal obstruction
Proposed mechanism for

annular pancreas
Annular pancreas
412
Case 2: 17-year-old female with abdominal pain and elevated

LFTs
Pancreatic divisum with focal chronic

pancreatitis
Pancreatic Divisum and Chronic Pancreatitis

Pancreatic Divisum
Incomplete fusion of dorsal and ventral pancreas

Body and tail drain through the duct of Santorini, minor
papilla
Incidence
4 to 11% (autopsy)
3 to 4% (ERCP)
Most asymptomatic
12-24% develop idiopathic recurrent pancreatitis
Choledocholithiasis in a patient with pancreatic
divisum by MRCP
413
Case 3: Two Different Patients with the same Disease
Chronic Pancreatitis
Chronic Pancreatitis: Ductal Features
Ectasia
Loss of normal tapering
Contour irregularity
Side branches
Clubbing
Stenosis
Opacification of cavities
Stenoses or occlusion
Chain of lakes
Intraductal calculi
Chronic pancreatitis by MRCP
414
Case 4: 50-year-old man with abdominal pain
Intraductal Papillary Mucinous Neoplasm

IPMN: Imaging
Duct dilatation
Focal or diffuse
Intraductal masses
Bulging duodenal papilla
Glandular atrophy
Main Duct IMPN

Side Branch IPMN
Bulging Papilla
415
Case 5: 45-year-old man with chest pain and elevation of serum

amylase
Mediastinal pseudocyst from acute

pancreatitis
Mediastinal Pseudocyst
Fluid Collections and Pancreatitis
50% of patients
Rupture of pancreatic duct
Exudation of fluid from gland surface
416
Hepatic Imaging
Case 1: 50-year-old woman with
vague abdominal discomfort
Complex Hepatic Cyst
Nonneoplastic
Echinococcal cyst
Simple cyst with
hemorrhage/infection
Post-traumatic cyst
Abscess
Ciliated hepatic foregut cyst
Neoplastic
Biliary cystadenoma
Biliary cystadenocarcinoma
Cystic metastasis
Peliosis
Teratoma
Biliary cystadenoma
Biliary Cystadenoma
Simple Cyst
417
Seminar 4: Hepatic Imaging
Case 2: 10-year-old girl with right upper quadrant pain
Differential Diagnosis: Hepatic Mass with a Scar

Hemangioma
Hepatic Mass with a Scar

Hepatocellular Carcinoma (HCC)
Hepatocellular Adenoma (HCA) with Fibrosis
Hemangioma
Hemangioma: Tagged RBC Scan
418
Case 3: 54-year-old man with right upper quadrant pain and

jaundice
Rim-like Enhancement
Hemangioma
Metastatic disease
Angiosarcoma
Hemangioma
419
Case 4: 50-year-old male with vague abdominal pain
Differential Diagnosis: Liver Mass with Fat
Angiomyolipoma
Myelolipoma
Metastasis
Liposarcoma
VERY RARE, Teratoma
Hepatocellular Adenoma: Focal Fat and Capsule
Hepatocellular Adenoma: Diffuse Hypodensity
Angiomyolipoma
Myelolipoma
Hepatic Teratoma
420
Case 5: 26-year-old woman with RUQ pain and mild elevation of

serum AST
Differential Diagnosis: Calcified Liver Mass
Nonneoplastic
Hematoma
Simple cyst
Parasitic infection
Healed infection
Benign neoplasm
Hemangioma
Teratoma
Malignant neoplasm
Hepatoblastoma (kids)
Colon Adenocarcinoma Metastases
Echinococcus multilocularis
421
Complications of Meckel Diverticulum
Cases 1-5
All patients have the same disease

The underlying disease is a congenital anomaly
Each presents with a different manifestation
Omphalomesenteric
(Vitelline) Duct Anomalies
Meckel Diverticulum
Most common anomaly of the GI tract

2% - 3% of the population
M=F
Symptoms more common in males
60% of patients present before age 10
Omphalomesenteric duct anomaly
Improper closure and absorption
Omphalomesenteric (Vitelline) Duct
Embryonic connection between yolk sac and midgut

10th week of embryogenesis
Midgut returns to abdomen
Duct is a thin fibrous band connecting midgut to umbilicus
Disintegrate
Absorption
Umbilico-ileal fistula
Umbilical sinus
Umbilical cyst
Persistent fibrous cord
Meckel diverticula
Meckel diverticula
with a fibrous attachment to the
umbilicus
Meckel diverticula
supported by a mesentery
Seminar 5: Meckel Diverticulum
422
Omphalomesenteric (Vitelline) Duct Anomalies
Umbilico-ileal fistula
Umbilical sinus
Umbilical cyst
Persistent fibrous cord
Meckel diverticulum
With a fibrous cord
With a portion of mesentery
Meckel Diverticulum: Pathology
Antimesenteric side of distal ileum

Within 100 cm of ileocecal valve
True diverticulum
Composed of all layers of the small bowel wall
Heterotopic tissue
50% of resected diverticula
Gastric most common (23% - 50%)
Pancreas (5% to 16%)
Rare, Brunner glands, colonic, biliary
Meckel Diverticulum: Heterotopic Gastric Mucosa

Meckel Diverticulum: Heterotopic Pancreatic Mucosa
423
Case 1: 22-year-old man with fever and guaiac positive stools
Meckel diverticulitis located in the midline because of persistent

attachment to the umbilicus

Urachal remnant
Colonic Diverticulitis
Meckel diverticulitis
Idiopathic ileal diverticula
Meckel Diverticulitis
Case 2: 22-year-old man with chronic abdominal pain and
anemia
Hemorrhagic Meckel diverticulum
Neoplasm
Ulcer
Vascular ectasia
Meckel diverticulum
Hemorrhagic Meckel Diverticulum

Angiographic Features of Meckel Diverticulum
Vitellointestinal artery
Arises from a distal ileal branch of the SMA
Tubular shaped angiographic blush
Intraluminal contrast if brisk bleeding
Hemorrhage in Meckel Diverticulum
Most frequent complication

Tc99-pertechnetate
Localizes in ectopic gastric mucosa
Modality of choice in pediatric population
Sensitivity 85%, specificity 95% in kids
Sensitivity 63%, specificity 2% in adults
424
Case 3: 61-year-old woman with intermittent abdominal pain
Inverted Meckel diverticulum
Lipoma
Inverted Meckel diverticula
Inverted Meckel Diverticulum

Inverted Meckel's Diverticulum
with Intussusception
Illustration of an inverted Meckel diverticulum
Case 4: 57-year-old man with abdominal pain and fever
Meckel diverticulitis with perforation and a stone
Meckel Diverticulitis with Perforation and a Stone

Meckel Diverticulitis with a Stone
Meckel Diverticulitis: Etiology
Luminal obstruction
Enterolith
Foreign body
Edema of orifice
Peptic ulceration
Torsion
425
Meckel Diverticulitis
Appendicitis
Idiopathic ileal diverticula
Helpful CT features
Blind-ending pouch
Mural contrast enhancement
Connection to ileum
Midline location
Associated SBO
Case 5: 40-year-old man with pain and vomiting
Small bowel obstruction from Meckel diverticulitis
Inflamed Meckel
with Small Bowel Obstruction
Small Bowel Obstruction due to Meckel Diverticula
Second most common complication of Meckel

Etiology
Inversion with intussusception
Diverticulitis
Volvulus from attachment to umbilicus
Congenital mesodiverticular bands
Foreign body impaction
Inclusion of Meckel in a hernia (hernia of Littre)
Neoplasm
Inclusion of Meckel in a true knot
Summary: Complications of Meckel Diverticula
Hemorrhage
Obstruction
Diverticulitis
Inversion
Intussusception
Stones
Torsion
Neoplasm
Summary: Complications of Meckel Diverticula
426
Beyond Appendicitis
Angela D. Levy, LTC (P), MC, USA
Case 1: 23-year-old man with a 1-day history of left lower
quadrant pain and bilious emesis
Imaging Features
Reversal of SMA and SMV

Swirling vessels about SMA
Absent colon right side of
abdomen
Inflammatory process in LLQ
Inflamed tubular structure
Mesenteric inflammation
Malrotation
Inflammation
Diverticulitis
Meckel diverticulum
Appendicitis
Malrotation with Left-sided

Appendicitis
Malrotation with left-sided appendicitis
Various locations of the cecum and appendix

within the abdomen
427
Seminar 6: Beyond Appendicitis
Case 2: 47-year-old man complains of fever and right lower

quadrant pain
Appendiceal mucinous cystadenoma causing appendicitis
Appendiceal Mucinous Cystadenoma Causing Appendicitis

Appendiceal Neoplasms
Uncommon
<0.4% of intestinal tumors
Histologic subtypes
Carcinoid
Mucinous cystadenoma/cystadenocarcinoma
Adencarcinoma
Non-mucin producing adenocarcinoma
Appendiceal Carcinoid
Most common location for GI tract carcinoid

45% of gastrointestinal carcinoids
Most common appendiceal tumor
50% to 85% of appendiceal tumors
Majority benign clinical course
70% to 90% discovered incidentally
>95% of appendiceal carcinoids have benign biologic behavior
Mucinous Cystadenoma/Cystadenocarcinoma
Mucin producing epithelial neoplasm

M=F
27 to 77 years of age
Presentation
Right lower quadrant pain, nausea, vomiting, abdominal swelling
Complications
Bowel obstruction, torsion, perforation, intussusception,
appendicitis
20% with a synchronous colonic adenocarcinoma
Mucinous Cystadenoma/Cystadenocarcinoma
Radiologic Findings
RLQ mass on plain film
Mass effect medial cecal wall
Nonfilled appendix on BE
Cross-sectional imaging
Fluid-filled, complex mass on CT or US
Mass bulges into cecal lumen
Short T1 and long T2 signal on MR
May be the lead point for intussusception
Mucinous Cystadenoma
Mucinous cystadenoma of the
appendix
428
Appendiceal Adenocarcinoma
Non mucin producing

Less common than mucinous
tumors
Radiologically resembles colonic
adenocarcinoma
Focal soft tissue mass
Soft tissue mural infiltration
Neoplastic vs. Nonneoplastic

Appendicitis
Appendiceal Adenocarcinoma
CT findings suggestive of neoplasm

Focal soft tissue mass
Cystic dilatation of the appendix
Nonspecific inflammatory changes may be seen in neoplasms of the
appendix
95% sensitivity for neoplasm if you combine morphologic changes with a
diameter > 15 mm1
1Pickhardt PJ, Levy AD, Rohrmann CA, Kende AI. Primary Neoplasms of the
Appendix Manifesting as Acute Appendicitis: CT Findings with Pathologic
Correlation. Radiology 2002. 224 (3): 775-781
429
Case 3: 65-year-old man with acute RLQ pain
Epiploic appendagitis
Epiploic Appendagitis
Appendix epiploica
Torsion
Infarction
Ischemia
Clinical course
Self limited
Spontaneous resolution
Epiploic Appendagitis: Imaging Features
Pericolonic fatty mass

Peripheral inflammatory change
Central high attenuation from vascular thrombosis
May have mass effect on adjacent bowel
Epiploic Appendagitis
Case 4: 17-year-old man with RLQ pain and poor appetite
Mesenteric adenitis
Mesenteric Adenitis
Inflammation of ileocecal nodes

Coexistent inflammation of the TI and cecum may be present
Children, young adults
Self limited
430
Case 5: 64-year-old man with RLQ pain that progressed to

involve the entire abdomen, fever, and vomiting
Cecal adenocarcinoma resulting in perforation, appendicitis, and

small bowel obstruction
Cecal Adenocarcinoma
Acute Appendicitis: Pathogenesis
Luminal obstruction followed by infection

Stones
Food, mucus, adhesions
Mucosal edema, lymphoid hyperplasia
Parasites
Tumors
Endometriosis
Cecal Adenocarcinoma
Mural thickening
Eccentric or asymmetric
Intraluminal mass
Often near appendiceal orifice if patient presents with appendicitis
Pericolic lymph nodes
Peritoneal implants, distant mets
Non Hodgkin Lymphoma

Case 6: 42-year-old woman with RLQ pain and peritoneal signs
on physical exam
Omental Infarction
Omental Infarction
Omental torsion
Most commonly site free edge of the right lateral omentum
CT features
Focal inflammation of omental fat
Normal appendix, colon, terminal ileum
Fatty mass with concentric or swirling lines
431
Tumors and Tumor-like Lesions of the
Gallbladder
Angela D. Levy, LTC (P), MC, USA
Case 1: 45-year-old woman with RUQ pain
Adenomyomatous hyperplasia
Sonographic Findings

Reverberation artifact
Differential Diagnosis: Gallbladder Wall Thickening
Infection/inflammation
Acute cholecystitis
Chronic cholecystitis
Xanthogranulomatous cholecystitis
Edema
Cardiac, liver, renal failure
Hepatitis
Neoplasm
Tumor-like lesions
Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder
432
Reverberation (comet-tail) Artifact
Gas in gallbladder wall

Coronal T2 MR Findings

String of pearls
Adenomyomatous Hyperplasia

String of pearls
Adenomyomatous Hyperplasia
Common
9% cholecystectomy specimens
More common in women than men
Gallstones frequently present
Three variants
Diffuse
Segmental
Localized (fundic adenomyoma)
Fundal Adenomyomatous Hyperplasia: "Adenomyoma"
433
Case 2: 47-year-old man complains of fever and right lower

quadrant pain
Xanthogranulomatous cholecystitis
CT Findings
Gallstone
Hypodense nodules in gallbladder wall
Ill-defined hepatic margin
Inflammatory change
Cholecystitis
Acute
Chronic
Xanthogranulomatous
Neoplasm
Xanthogranulomatous Cholecystitis
Aggressive inflammatory process

Pathophysiology
Intermittent cystic duct obstruction
Bile enters gallbladder wall
RUQ pain, fever, tenderness
Surgical treatment
Reported association with gallbladder carcinoma
Difficult to preoperative distinguish from carcinoma
Involvement of adjacent organs
Xanthogranulomatous Cholecystitis: Pathology

Xanthogranulomatous Cholecystitis: Imaging
Wall thickening
Mural nodules or bands
Hypoechoic on sonography
Low-attenuation on CT
Stones
Pericholecystic fluid
Adjacent invasion
Lymphadenopathy
434
Case 3: 75-year-old woman with RUQ pain and weight loss

MR Findings
Intraluminal gallbladder mass

Hepatoduodenal ligament mass
Gallbladder carcinoma
Bile duct carcinoma
Metastatic disease
Gallbladder Carcinoma
Sixth most common GI tract malignancy

Worldwide: stomach, colorectal,
liver, esophagus, pancreas,
gallbladder
US: colorectal, pancreas, stomach,
liver, esophagus, gallbladder
More common in women (3:1)
Mean age 72 years
Gallbladder Carcinoma:
Pathology
Epithelial malignancies (98%)

Adenocarcinoma (90%), squamous
cell, adenosquamous, small cell
carcinoma
Other (2%)
Sarcomas, lymphomas, carcinoid,
metastases
Gallbladder Carcinoma
Imaging patterns
Intraluminal polypoid mass (15% to 25%)
Focal or diffuse wall thickening (20% to 30%)
Mass replacing the gallbladder (40% to 65%)
Adenocarcinoma: Diffuse Wall Thickening

Papillary Adenocarcinoma : Polypoid Mass
Adenocarcinoma: Mass Replacing the Gallbladder Fossa
Gallbladder Adenocarcinoma: Direct Extension to Liver
Gallbladder Adenocarcinoma: Direct Extension to
Hepatoduodenal Ligament
435
Case 4: 30-year-old man with RUQ pain
Metastatic melanoma
Differential Diagnosis: Polypoid Gallbladder Mass
Cholesterol polyp
Gallbladder adenoma
Carcinoma
Metastatic disease
Metastatic Melanoma
Management of Gallbladder Polyps
Size < 5 mm
Do nothing
Size 5 to 10 mm
Follow
Size >10 mm
Remove
Features suggesting malignancy
Adjacent gallbladder wall thickening
Abnormal gallbladder/liver interface
Abnormal liver parenchyma
Hepatoduodenal ligament adenopathy
436
Case 5: 35-year-old woman with RUQ pain
Cholesterol polyp
Sonographic Findings
Small, nonshadowing echogenic mass

Adherent to gallbladder wall
Cholesterol Polyp
Common
50% of polypoid lesions in the gallbladder
More common in women
3:1
Cholesterol Polyp
Lipid laden macrophages

Normal gallbladder epithelium
Cholesterol Polyp: Sonography
Most <10 mm
Small lesions
Echogenic nodules
Larger lesions
Hypoechoic
Internal echogenic foci
Summary
Look for features to suggest a specific process tumor-like process

Ring-down artifact, pearl necklace sign for adenomyomatous hyperplasia
Focal mural nodules for XGC in the right clinical setting
Look for features to suggest a malignancy
Gallbladder wall thickening in association with a polypoid mass
Abnormal gallbladder/liver interface
Abnormal liver parenchyma
Hepatoduodenal ligament mass or adenopathy
437
Cholelithiasis and Cholecystitis

Robert K. Zeman, MD
Outline/Objectives
Detection of cholelithiasis
Gravel versus sludge
Acute cholecystitis
Complications of acute cholecystitis
Gangrenous cholecystitis
Emphysematous cholecystitis
Empyema of the gallbladder
Gallbladder perforation
Choledocholithiasis
Premise
The radiologist plays a central role in identifying the cause of the patients
symptoms and
Detecting complications of cholecystitis (inflammatory and neoplastic) that will
dictate the therapeutic approach
Cholelithiasis
30 million American adults harbor stones

Should silent stones be treated?
22% of patients with stones are symptomatic (Sirmione study)
In symptomatic patients, 50% chance of colic in 1 year; 12% cumulative risk
of acute cholecystitis.
Cholelithiasis
For symptomatic stones, recommend elective laparoscopic cholecystectomy

For acute cholecystitis:
Delayed surgery allows for better vizualization of surgical field
Early surgery means less adhesions
Figure 2-22-1
US of Cholelithiasis [Figure 2-22-1]
3 common appearances
Solitary stone
Gravel
Double-arc (WES)
Solitary Gallstone
Gravel
Double-Arc Sign (WES)
How Sensitive is US?
Remember the neck of the gallbladder
Sludge vs Gravel?
Solitary stone
Gravel
Double-arc (WES)
Gravel represents small, discrete calculi

Sludge is viscous, lithogenic bile
438
Tumefactive Sludge [Figure 2-22-2]
Figure 2-22-2
Baseline
After walking
Is there any role for the OCG?

[Figure 2-22-3]
No stones on OCG
See 5mm stone on US
Acute Cholecystitis
Uncomplicated vs complicated
Treatment options if complications
(do imaging findings influence
operative approach?)
Cholescintigraphy vs US
Cholescintigraphy vs.
Ultrasound
Tumefactive sludge mimics GB mass-but changes shape with

change in patient position
Both equally sensitive and specific

Emergency availability is key
Ultrasound screens for more non-biliary diseases
If biliary obstruction present, scintigraphy does not identify cause
despite high sensitivity; US may see cause
Scintigraphy great problem solver; can add EF when confusing
symptoms
Figure 2-22-3
Cholescintigraphy In AC
The only reliable indicator of acute cholecystitis is non-visualization

of the gallbladder remember the lateral
High sensitivity, moderate specificity
Cholescintigraphy: Positive study for acute

cholecystitis
Potential Causes of False-Positive Scintigraphy For
Acute Cholecystitis
Lack of adequate fasting

Failure to obtain delayed views
Pancreatitis
Hyperalimentation
Biliary obstruction
Prolonged fasting
Intercurrent illness
Alcoholism
Overly conservative pathologic criteria of Acute Cholecystitis
Trauma
Gallbladder Neoplasm
Missed stones often hide in the

gallbladder neck
Is There Anything That Can Reduce False-Positives?

Pharmacologic Enhancement of Cholescintigraphy
Can dramatically reduce false-positives*

Two approaches CCK versus morphine (in setting of suspected AC)
CCK to pre-empty GB
Morphine (.04 mg/kg) given if GB fails to visualize by 3050 minutes
*Kim et al, AJR 147:1177,1986
439
Use of Morphine Reduces False-Positives [Figure 2-22-4]
Figure 2-22-4
35 minutes (pre-MS)
55 minutes (post-MS)
Sonographic Findings Associated

With Acute Cholecystitis
Most useful signs

Cholelithiasis
Intramural sonolucency
Sonographic Murphy Sign*
GB wall hyperemia**
Secondary signs
Pericholecystic fluid
GB distention
Sludge
Gas in the gallbladder wall or lumen
Pericholecystic abscess
35 minutes (pre-MS)
Normal scan after morphine

55 minutes (post-MS)
*Laing et al, Radiology 140:449, 1981

**Uggowitzer AJR 168:707, 1997
Intramural Sonolucency
Described in 11 patients as first specific sign for acute cholecystitis*

Consists of a hypo-reflective or sonolucent band, continuous or interrupted,
within the hyper-reflective gallbladder wall
Focal lucency or concentric rings (striate)
most suggestive of inflammation**
Figure 2-22-5
*Marchal et al, Radiology 133:429, 1979

**Cohen et al, Radiology 164:31, 1987
Acute Cholecystitis-Striate GB
Walls [Figure 2-22-5]
Acute Cholecystitis-Focal Lucency
Lucency in GB Wall is not always
Edema
Gallbladder varices in portal hypertension
Which one has Varices?

Gallbladder Wall Thickening
Failure to fast
Acute cholecystitis
Hypoalbuminemia
Hepatitis
Ascites
Varices
AIDS
Carcinoma
Cholesterolosis
Mononucleosis
Beware of sliver of edema in some of

these entities
Multiple patients
Figure 2-22-6
Gallbladder Wall Thickening

[Figure 2-22-6]
Hepatitis
Ascites
Gallbladder wall thickening has many causes and is not as

specific as a striate wall
440
Hyperemia of Acute Cholecystitis

Complications/Severity of Acute Cholecystitis
Options for Treatment of Acute Cholecystitis
PCCL
OC
LC
Temporize
Role of the Radiologist in Acute Cholecystitis
Identify findings that make temporizing ill-advised or that would potentially

result in open cholecystectomy:
Extreme striations
Gangrenous cholecystitis
Emphysematous cholecystitis
Perforation
Biliary obstruction / Mirizzi syndrome
Incidental findings that preclude LC
Figure 2-22-7
Incidental Finding
Hemangioma next to GB neck
Gangrenous Cholecystitis
Not always Clostridal infection

Implies severe inflammation
Sonography-may see desquamated
mucosa/membranes
Scintigraphy-increased pericholecystic
activity* due to:
delayed excretion from perihepatitis
hyperemia with increased tracer
delivery
Two different patients show a band of tracer where the liver

abuts the inflamed gallbladder (arrow)
*Smith et al, Radiology 156:197, 1985
Gangrenous Cholecystitis-Rim Sign [Figure 2-22-7]
Two different patients
Gangrenous Cholecystitis
Sloughed membranes
Emphysematous Cholecystitis
Elderly patients, 2030% are diabetic

Male predominance
1/3 infected with Clostridia welchii
Perforation 5 times as common as for non-emphysematous cholecystitis
Dirty shadowing and echogenic GB wall on sonography is suggestive
Dont forget plain film differential diagnosis of RUQ air
Figure 2-22-8
US-echogenic foci=gas
KUB
[Figure 2-22-8]
US-ring-down
KUB
US-ring-down (reverb)
441
KUB
Not all GB Walls with Ring-Down Contain Gas
Diagnosis?
Not all GB Walls with Ring-Down Contain Gas
Adenomyomatosis
Emphysematous Cholecystitis-CT
Gallbladder Empyema
Figure 2-22-9
Gas confined to GB wall

Gas in lumen, hepatic ducts
[Figure 2-22-9]
Infection in obstructed, inflamed

gallbladder
25% incidence-rises to 80% if untreated
after 7 days*
Results in marked GB distention in 38%
of patients**
US, CT-Nonspecific. See distention,
bile/debris level, snow storm
*Goldman et al, Gastro 11:318, 1948

**Fry et al, Am J Surg 141:366, 1981
Perforation of the Gallbladder
Seen both in the context of chronic cholecystitis (eg., gallstone ileus) and AC
In older literature, occurs in 315% of patients with acute cholecystitis
Patient feels transiently better and then develops peritoneal signs
Cholescintigraphy extravasated activity maybe
Sonography, CT-pericholecystic collection, non-specific
GB Perforation in AC [Figure 2-22-10]

GB Perforation
Figure 2-22-10
Choledocholithiasis
May occur as primary duct stone (usually pigment),

secondary to gallstones, or following
cholecystectomy
Most small stones will pass spontaneously. The duct
caliber and dynamics may rapidly change
CT and US are approx. 70-80% sensitive for
detection of choledocholithiasis
If you suspect CBD stonesoptions are MRCP,
ERCP, intraop cholangiogram
When to Perform MRCP
Jaundice
Dilated ducts on US
Delayed egress on IDA
Anatomic finding that suggests process that may result in altered duct
anatomy or make laparoscopic cholecystectomy risky
Post-cholecystectomy complications
Contracted GB
MRCP
T2 wt TSE or FSE, thin sections or slab

Extra sections as needed
442
RUQ Pain (789.01) and Fever: Approach
R/O Acute calculous cholecystitis...IDA, US

R/O Stones...US
R/O Acute acalculous cholecystitis...?
R/O a reason to operate... if suspect biliary do US, if suspect acute abdomen/GI disease do CT
Remember MRCP
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
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18.
19.
20.
21.
22.
23.
24.
25.
26.
27.
28.
29.
Zeman RK, Garra BS. Gallbladder Imaging: The State-of-the-art. Gastroent Clin N. Am 2:127, 1991.
Garra BS, Davros WJ, Lack EE, Horii SC, Zeman RK: Visibility of gallstone fragments at ultrasound and
fluoroscopy. Implications for monitoring of gallstone lithotripsy. Radiology 174:343, 1990.
Mathieson JR, So CB, Malone DE, Becker CD, Burhenne HJ: Accuracy of sonography for determining the number
and size of gallbladder stones before and after lithotripsy. AJR 153:977, 1989.
duPlessis DJ, Jersky J. Management of acute cholecystitis. Surg Clin North Am 53:1071, 1973.
Halasz NA. Counterfeit cholecystitis: A common diagnostic dilemma. Am J Surg 130:189, 1975.
Zeman RK, Burrell MI, Cahow CE, Caride V. Diagnostic utility of cholescintigraphy and ultrasonography in acute
cholecystitis. Am J Surg 141:446, 1981.
Weissmann HS, Badia J, Sugarman LA et al. Spectrum of 99m Tc-IDA cholescintigraphic patterns in acute
cholecystitis. Radiology 138:167, 1981.
Eikman EA, Cameron JL, Colman M et al. A test for patency of the cystic duct in acute cholecystitis. Ann Int Med
82:318, 1975.
Fonseca C, Greenberg D, Rosenthall L et al. Assessment of the utility of gallbladder imaging with 99m Tc-IDA.
Clin Nucl Med 3:437, 1978.
Freitas JE. Cholescintigraphy in acute and chronic cholecystitis. Semin Nucl Med 12:18, 1982.
Shuman WP, Gibbs P, Rudd TG et al. PIDIDA scintigraphy for cholecystitis: False positives in alcoholism and total
parenteral nutrition. AJR 138:1, 1982.
Kalff V, Froelich JW, Lloyd R et al. Predictive value of an abnormal hepatobiliary scan in patients with severe
intercurrent illness. Radiology 146:191, 1983.
Laing FE, Federle MP, Jeffrey RB et al. Ultrasonic evaluation of patients with acute right upper quadrant pain.
Radiology 140:449, 1981.
Ralls PW, Colletti PM, Lapin SA et al. Real-time sonography in suspected acute cholecystitis: Prospective
evaluation of primary and secondary signs. Radiology 155:767, 1985.
Cohan RH, Mahony BS, Bowie JD, Cooper C, Baker ME, Illescas FF: Striated intramural gallbladder lucencies on
US studies. Predictors of acute cholecystitis. Radiology 164:3135, 1987.
Teefey SA, Baron RL, Bigler SA: Sonography of the gallbladder. Significance of striated (layered) thickening of
the gallbladder wall. AJR 156:945, 1991.
Shaler WJ, Leopold GR, Scheible FW: Sonography of the thickened gallbladder wall. A nonspecific finding. AJR
136:337, 1981.
West MS, Garra BS, Horii SC, Zeman RK et al. Gallbladder varices: Imaging findings in patients with portal
hypertension. Radiology 179:179, 1991.
Weissmann HS, Berkowitz D, Fox MS et al. The role of technetium-99m iminodiacetic acid (IDA)
cholescintigraphy in acute acalculous cholecystitis. Radiology 146:177, 1983.
Shuman WP, Rogers JV, Rudd TG et al. Low sensitivity of sonography and cholescintigraphy in acalculous
cholecystitis. Radiology 142:531, 1984.
Swayne LC. Acute calculous cholecystitis: Sensitivity in detection using technetium-99m iminodiacetic acid
cholescintigraphy. Radiology 160:33, 1986.
Mirvis SE, Vainright JR, Nelson AW, et al. The diagnosis of acute acalculous cholecystitis: A comparison of
sonography, scintigraphy, and CT. AJR 147:171, 1986.
Jeffrey RB, Laing FC, Wong W, Callen PW. Gangrenous cholecystitis: Diagnosis by ultrasound. Radiology
156:797, 1985.
Wales LR. Desquamated gallbladder mucosa: Unusual sign of cholecystitis. AJR 139:810, 1982.
Smith R, Rosen J, Gallo LN, Alderson PO. Pericholecystic hepatic activity in cholescintigraphy. Radiology
156:797, 1985.
Siskind B, Hawkins M, Cinti D, Zeman RK, Burrell MI. Perforation of the gallbladder: Radiologic-pathologic
correlation. J Clin Gastroenterol 9:67078, 1987.
Clemett AR, Lowman RM. The roentgen features of the Mirizzi syndrome. AJR 94:480, 1965.
Weltman D, Zeman RK. Imaging of acute diseases of the gallbladder and bile ducts. Radiological Clinics of North
America 32:933-950, 1994.
Fulcher AS, Turner MA, Capps GW. Technical Advances and Clinical Applications. RAdiographics 19:25-41,
1999.
443
Inflammatory Diseases of the Esophagus

Marc S. Levine, MD
Figure 2-23-1
Technique
Double-contrast:
Upright
Right lateral cardia
Single-contrast:
Separate swallows
Prone esophagus
Reflux Esophagitis
Most common inflam condition

Purpose of Ba study not simply to show HH/GER but to R/O morphologic
sequelae of GERD
Pathogenesis
Frequency of GER
Decreased LES tone
Mult trans LES relaxations
Duration of GER
Abnormal motility
(scleroderma)
Pathogenesis
Acidity of refluxate
ZES (increased acid)
Billroth II (bile or panc)
Resistance of mucosa
Age
Debilitation
Clinical Findings
Heartburn and regurg

Epigastric or RUQ pain
Upper GI bleeding
Dysphagia (peptic stx)
Reflux esophagitis with

granular mucosa
Figure 2-23-2
Hiatal Hernia and GER
Occur independently
Spont GER at fluoro:
3060% in esophagitis
4050% in volunteers
Reflux Esophagitis: Radiographic Findings

[Figures 2-23-1 and 2-23-2]
Abnormal motility
Granularity
Thickened folds
Inflammatory EG polyp
Ulceration
Peptic Scarring: Radiographic Findings
Radiating folds
Deformity of wall
Peptic stricture
Sacculations
Transverse folds
Inflammatory Disease of the Esophagus
Reflux esophagitis with

ulceration
444
Radiologic Dx of Esophagitis
Koehler
Ott
Creteur
Gr 1
13%
22%
53%
Gr 2
90%
83%
93%
Gr 3
100%
95%
100%
Schatzki Ring
Variant of peptic stx

Episodic dysphagia (meat)
(Steakhouse syndrome)
Symm ringlike constriction
Vertical height 24 mm
Usually sx if < 13 mm diam
Best seen on prone views
Barretts Esophagus
Prog columnar metaplasia from GER and esophagitis

Prevalence:
10% with esophagitis
40% with peptic stx
Clinical Findings
Men > Women, W > B

Reflux sx, dysphagia
40% asymptomatic
Tx of GER may not cause Barretts to regress
Histologic Findings
Projections or islands of columnar epith separated by squam epith

Foveolar epith > 3 cm above LES or intestinal metaplasia with goblet cells
Premalignant Condition
Figure 2-23-3
Risk of adenocarcinoma
Dysplasia-Ca sequence
Endoscopic surveillance
Radiographic Findings [Figure 2-23-3]
Classic: High stx or ulcer or reticular pattern

Common: GER, hiatal hernia, reflux esophagitis, or peptic stricture
Dx of Barretts by D/C Tech
200 pts with reflux sx

Classified at high, mod, or low risk for Barretts
AJR 150:97102, 1988
Classification of Risk
High: High stx or ulcer or reticular pattern

Mod: Distal stx or reflux esophagitis
Low: None of above
Radiologic vs Endoscopic Dx
Risk
High
Moderate
Low
10
73
117
Endo
9 (90%)
12 (16%)
1 (1%)
Barrett esophagus
with mid esophageal stricture
and reticular pattern
Radiologic Diagnosis
Less sensitive than endoscopy

Most false negative exams in mild disease
Vast majority do not have Barretts esophagus
445
Reflux Symptoms
Figure 2-23-4
Candida Esophagitis
Most common type

Immunocompromised (75%)
Local esophageal stasis (25%)
(achalasia, scleroderma)
Clinical Findings
Dysphagia/odynophagia
OP Candidiasis (50%)
Marked clinical response to antifungal agents (ketoconazole)
Candida esophagitis
with plaques
Radiographic Findings [Figures 2-23-4 and 2-23-5]
Mucosal Plaques (90%)

Linear
Etched in white
Shaggy esophagus (AIDS)
Plaques and membranes
Superimposed ulcers
Figure 2-23-5
Herpes Esophagitis
2nd most common type

Herpes simplex virus type 1
Immunocompromised
Viral Cx or Bx (intranuclear inclusions in cells adjacent to ulcers)
Clinical Findings
Dysphagia/odynophagia
Oropharyngeal herpes
Marked clinical response to antiviral agents (acyclovir)
Discrete ulcers in upper and midesophagus

Ulcers and plaques (mimics Candida)
Herpes Esophagitis in Healthy Pts
Young men (1530 y/o)

Sexual partners with OP herpes
Flu-like prodrome (310 days)
Severe odynophagia
Multiple tiny ulcers
Sx resolve in 314 days
Candida esophagitis
with shaggy esophagus
446
CMV Esophagitis
Pts with AIDS

Odynophagia
Viral Cx or Bx (intranuclear inclusions in cells at ulcer base)
Tx with ganciclovir (endo for confirmation)
Figure 2-23-6
Radiographic Findings [Figures 2-23-7]
Nodular mucosa
Small ulcers (mimics herpes)
Giant ulcers
Figure 2-23-8
Figure 2-23-7
Herpes esophagitis
with tiny ulcers
HIV esophagitis
with giant ulcer
CMV esophagitis
with giant ulcer
HIV Esophagitis
Odynophagia and giant ulcers

Palatal ulcers
Maculopapular rash
Recent seroconversion
Dx of exclusion (No CMV)
Treatment with steroids
Giant ulcers
(mimics CMV)
Satellite ulcers
Giant Ulcers in 21 HIV + Pts
Cause
HIV
CMV
Both
No Pts
16
3
2
%
76
14
10
Radiology 194:447451, 1995
Giant Ulcers in 21 HIV + Pts
All had AIDS (CD4 ct < 200)

Avg time from serodetect 2 yrs
Only 1 pt had palatal ulcers or rash
447
Conclusions
Most giant ulcers in HIV+ Pts caused by HIV not CMV

Impossible to diff by clin or rad criteria
Endoscopy for definitive Dx
Figure 2-23-9
Drug-Induced Esophagitis [Figure 2-23-9]
Contact esophagitis (doxycycline, tetracycline, KCl, quinidine, NSAIDs,

alendronate)
Aortic arch or lt main bronchus
Superficial ulcers
Severe odynophagia but rapid clinical improvement after withdrawal of
offending agent
Radiation Esophagitis
25000 rad: self-limited esophagitis (1 2 weeks)

5000 or more rad: stx, progressive dysphagia (48 months)
Adriamycin potentiates XRT (only 500 rad)
Radiographic Findings
Acute
Ulceration
Granular mucosa
Decreased distensibility
Chronic
Abnormal motility
Strictures
Tetracycline-induced
esophagitis with three ulcers
Caustic Esophagitis
Strong acids or alkali (liquid lye)

Three phases of injury:
Acute necrosis
Ulceration and granulation
Cicatrization
Chest pain, odynophagia, hematemesis,
shock
Figure 2-23-10
Figure 2-23-11
Acute
Abnormal motility
Ulceration
Perforation
Chronic
Strictures
(13 months)
Esophageal Intramural
Pseudodiverticulosis
Dilated excretory ducts

Ductal obstruction
Candida, diabetes, alcohol
High strictures classic
Peptic stx more common
[Figures 2-23-10 and 2-23-11]
Flask-shaped outpouchings
Floating outside wall
Associated strictures
(especially peptic stx)
Diffuse esophageal
intramural
pseudodiverticulosis with
high stricture
448
Localized esophageal
intramural
pseudodiverticulosis with
peptic stricture
Esophageal Varices
Uphill
Downhill
Location
Distal
Mid
Diffuse Nodules/Plaques
Cause
Reflux
Candida
Glycogenic acanthosis
Cause
Portal HTN
SVC obst
Finding
Granularity
Plaques
Nodules/plaques
Localized Nodules/Plaques
Cause
Candida
Sup spr Ca
Barretts
Ulcers
Cause
Reflux
Herpes
Drugs
CMV
HIV
Finding
Plaques
Coalesce nodules
Reticular pattern
Finding
Distal
Small, mid
Small, mid
Giant
Giant
Thickened Folds
Esophagitis
Varices
Varicoid Ca
High Strictures
Barretts esophagus
Mediastinal irradiation
Caustic ingestion
Primary or metastatic tumor
Distal Strictures
Peptic stricture
Lower esoph ring
Barretts Ca
449
Tumors of the Esophagus

Marc S. Levine, MD
Mucosal Lesions
Squamous papilloma
Adenoma
Glycogenic acanthosis
Squamous Papilloma: Pathologic Findings
Coral-like excrescence
Fibrovascular core
Hyperplastic squamous epithelium
Clinical Findings
Malignant degeneration rare
Multiple papillomas (papillomatosis)
Figure 2-24-1
Small, sessile polyp

Lobulated mass
Bubbly appearance
Diff Dx early Ca
Glycogenic Acanthosis
Accum of cytoplasmic glycogen

White nodules/plaques
Rarely causes esophageal sx
No risk of malignant degeneration
Round nodules/plaques
15 mm in diameter
Predominantly midesophagus
DDx Candidiasis
Intramural Lesions
Fibrovascular polyp
Leiomyoma
Granular cell tumor
Duplication cyst
Idiopathic varix
Leiomyoma: Pathologic Findings
Most common benign tumor

Bands of smooth muscle
60% DT, 30% MT, 10% PT
Up to 20 cm in diameter
Patterns submucosal, exophytic, intraluminal, circumferential
Glycogen acanthosis with nodules
Clinical Findings
Most pts asx

Dysphagia
GI bleed rare
Enucleation
CXR soft tissue mass, Ca++ rare
450
Ba submucosal mass
CT soft tissue mass
DDx fibroma, hemangioma, granular cell tumor, duplication cyst
Figure 2-24-2
Unusual Findings
Annular lesion
Giant intraluminal mass
Gastric involvement
Multiple lesions
Leiomyomatosis
Leiomyomatosis
Proliferation of smooth m.
Children/adolescents
Long-standing dysphagia
Familial autosomal dominant
Alports syndrome (nephritis, deafness, ocular lesions)
Esophageal leiomyoma in
profile
Ba tapered narrowing of distal esophagus (1 achalasia?)

Length > achalasia
Symmetric fundal defects
CT thickened wall (2 achalasia?)
Figure 2-24-3
Fibrovascular Polyp: Pathologic Findings
Benign intraluminal tumor

Fibrous/adipose/vascular tissue with nl squam epith
Hamartoma/fibroma/lipoma/fibrolipoma/angiolipoma
All classified as FVPs
Malig degen rare
Pathologic Findings
Arises in cervical esophagus

Loose submucosal conn tiss
Dragged inf by peristalsis
Occas prolapses into fundus
Pedicle in cervical esophagus
CXR with right superior mediastinal mass

caused by fibrovascular polyp
Clinical Findings
Figure 2-24-4
Dysphagia
Resp sx inspiratory stridor, choking, wheezing
Regurgitation of fleshy mass
Asphyxia/sudden death
Radiographic Findings [Figures 2-24-3 to 2-24-5]
CXR
Rt sup med mass
Retrotracheal bowing
Ba
Smooth, expansile
intraluminal mass
Var size & location
Lobulation common
Prox pedicle rare
DDx
Air bubble, achalasia,
malignant tumor
Figure 2-24-5
[Figures 2-24-5 and 2-24-6]
Path
CT
Fibrovascular polyp with fat

attenuation on CT
451
Fibrovascular polyp on barium

study
(same patient as Figure 2-22-3)
Adipose
Mixed
Fibrous
Figure 2-24-6
Lipid density [1]

Heterogeneous
Soft tissue density
[1] High-signal intensity on T1MR / High echo on endoscopic U/S
Duplication Cyst
Abnl embryo development

Sequest from prim foregut
Ciliated columnar epith
Most pts asymptomatic
Occas bleeding/infection
Fibrovascular polyp with geographic

areas of fat and soft tissue
attenuation on CT
CXR: Mediastinal mass

Ba: Submucosal mass
CT: Homogen low atten
MR: High-signal on T2
Malignant Tumors

Adenocarcinoma
Spindle cell carcinoma
Leiomyosarcoma
Kaposis sarcoma
Malignant melanoma
Lymphoma
Metastases
Epidemiological Factors
Tobacco and alcohol
Geographic variations (China, Iran, S Africa)
Low molybdenum in soil (accum of nitrosamines)
Predisposing Factors
Achalasia
Lye strictures
Head and neck tumors
Celiac disease
Plummer-Vinson
Tylosis
Definitions
Early: mucosa or submucosa without lymph node mets

Superficial: mucosa or submucosa with or without lymph node mets
Small: < 3.5 cm regardless of depth of invasion or lymph node mets
Routes of Spread
Direct extension trachea, bronchi, lungs, pericard, aorta, diaphragms

Lymphatic spread nodes in med, neck, upper abdomen (paracardiac, lesser
curv, celiac)
Hematogenous lungs, adrenals, liver
Clinical Findings
Dysphagia and wt loss

Odynophagia (if ulcerated)
Chest pain (poor sign)
Paroxysmal coughing (if TEF)
5-year survival < 10%
452
Early Squamous Cell Carcinoma: Radiographic Findings

[Figure 2-24-7]
Figure 2-24-7
Small, sessile polyp

Plaquelike (central ulcer)
Focal irregularity in wall
Superficial spreading
Adv Squamous Cell Carcinoma [Figures 2-24-8 and 2-24-9]
Plain Film
Widened med
Ant tracheal bowing
Thick RT stripe
A/F level in esoph
Barium
Infiltrating
Polypoid
Ulcerative
Varicoid
Squamous Cell Carcinoma: Staging
CT: Sens limited by adenopathy (mets in nl-sized nodes)

MRI: Comparable to CT
US: Depth of invasion & lymph node mets
Superficial spreading
carcinoma with focal nodularity
Adenocarcinoma
Arises in Barretts mucosa

Dysplasia-Ca sequence (low-grade, high-grade, ca-in-situ, invasive)
Comprises 2050% of esoph Cas
Predominantly in distal esophagus
Often invades proximal stomach
Prevalence 10% in Barretts esoph
3040X greater risk than gen pop
Dysphagia and weight loss
Same prognosis as squamous Ca
Endoscopic surveillance
Figure 2-28-9
Figure 2-28-8
Primary ulcerative carcinoma with

giant meniscoid ulcer surrounded
by rind of tumor
Varicoid carcinoma with

large submucosal defects
in lower esophagus
453
Radiographic Findings [Figures 2-24-10 and 2-24-11]
Early: sessile polyp, plaque, sup spreading, stricture

Adv: infiltrating, polypoid, ulcerative, varicoid (often invades cardia)
Figure 2-24-10
Figure 2-24-11
Adenocarcinoma in Barrett esophagus

invading gastric cardia
Early adenocarcinoma in Barretts
esophagus with plaque-like lesions
Figure 2-24-12
Spindle Cell Carcinoma
Carcinomatous and sarcomatous elements

Spindle cell metaplasia
Dysphagia and weight loss
Same prognosis as squamous Ca
Ba-polypoid intraluminal mass expanding lumen without obstruction

CT-expansile esophageal mass
DDx-malignant melanoma
Spindle cell carcinoma seen as

polypoid mass expanding
esophagus without causing
obstruction
454
Radiology of Peptic Ulcer Disease

Marc S. Levine, MD
Hypertrophic Gastritis [Figures 2-25-1 and 2-25-2]
Glandular hyperplasia
Increased acid secretion
Thickened folds
Diff Dx:
Menetriers
Lymphoma
Figure 2-25-1
Figure 2-25-2
Erosive gastritis with varioloform erosions in

antrum
Antral gastritis with

hypertrophied antralpyloric fold on lesser
curvature of distal antrum
Figure 2-25-3
Antral Gastritis [Figure 2-25-1]
Thickened antral folds

Longitudinal or Transverse
Crenulation of lessercurvature
Hypertrophic antral-pyloric fold
Causes of Erosive Gastritis [Figure 2-25-3]
PUD
NSAIDs
Alcohol
Stress
Trauma
Crohns
Acute Aspirin Ingestion (28 tabs/day in nl pts)
Finding
Erosions
Max damage
Healing
Time Span
824 hrs
13 days
37 days
Duodenitis
Spastic bulb
Thickened folds
Nodules
Erosions
NSAID-induced erosive gastritis with

linear erosions clustered in gastric
body near greater curvature
455
Peptic Ulcer Disease
Gastric Ulcers
Shape
Size
Location
Morphology
Figure 2-25-4
Figure 2-25-5
Benign posterior wall gastric ulcer
Anterior wall gastric ulcer seen as ring shadow on

double contrast view
Figure 2-25-6
Figure 2-25-7
Ulcer fills with barium on prone compression
Giant NSAID greater curvature ulcer
Multiple Gastric Ulcers
230% of pts with GUs

Association with aspirin in 80%
Each ulcer evaluated separately
Figure 2-25-8
Upper GI vs Endoscopy
More than 95% GUs Dx in North America are benign

616% of benign-app GUs on S/C studies are
malignant
Is endo always necessary?
Benign Gastric Ulcers [Figures 2-25-4 to 2-25-8-]
En Face
Round or ovoid crater
Smooth mound of edema
Symmetric radiating folds
In Profile
Projection outside lumen
Hamptons line or ulcer mound or collar
NSAID-induced greater curvature ulcer with

gastrocolic fistula
456
Malignant Gastric Ulcers [Figure 2-25-9]
Figure 2-25-9
En Face
Irregular crater in mass
Loss of areae gastricae
Nodularity, clubbing, or fusion of radiating folds
In Profile
Projection of crater inside lumen within mass
Acute angles of mass
Equivocal Ulcers
Irregularity of ulcer shape

Asymmetry of mass effect
Nodularity, irregularity, or clubbing of radiating folds
Enlarged areae gastricae
Location on greater curve
Radiologic Dx of Gastric Ulcers
Rad Dx
Benign
Equiv
Malig
No Pts
191
69
72
Endo
164
63
68
Malignant lesser curvature ulcer with clubbed folds

abutting ulcer on prone compression
Final Dx
All ben
56 ben / 7 malig
2 ben / 66 malig
AJR 141:331333, 1983
Radiologic Dx of Gastric Ulcers
Rad Dx
Benign
Equiv
Malig
No Pts
68
37
3
Endo
24
33
3
Final Dx
All ben
All ben
All malig
AJR 164:913, 1987
Gastric Ulcer Investigation
Advantages of Upper GI over Endoscopy
Shorter procedure time

Negligible risk
Lower cost
Upper GI vs Endoscopy
D/C upper GI
Endoscopy procedure
Pathology
Hospital
Total
Cost
$218
$540
$180
$102
$822
Cost of Evaluating 1 Million GUs in United States
UGI + endo
UGI + sel endo
Diff in Cost
$1 billion
$490 million
$510 million
457
Ulcer Healing
Figure 2-25-10
Change in size and shape

Avg pd for healing 8 wks
Ulcer scar in 90%
Ulcer Scar
Central pit or depression

Radiating folds
Retraction of adjacent wall
Duodenal Ulcers [Figure 2-25-10]
90% < 1 cm in size

50% on anterior wall
85% with deformed bulb
5% linear
15% multiple
Duodenal bulbar ulcer
Giant Duodenal Ulcers
Greater than 2 cm in size

Higher frequency of complix (bleeding, obst, perforation)
Fixed configuration at fluoro
Figure 2-25-11
Postbulbar Duodenal Ulcers [Figure 2-25-11]
5% of all duodenal ulcers

Medial wall of prox descending duodenum above papilla
Indentation of lateral wall
Notoriously difficult to Dx
Can result in development of ring stricture
Investigation of Duodenal Ulcers
Post-bulbar duodenal ulcer with ulcer niche

in proximal descending duodenum
H. Pylori
Gram-negative bacillus
Increases with age (50% of pop > age 60)
Eradicated by antibiotics and antisecretory agents
Figure 2-25-12
Thickened gastric folds (predom antrum and body)

Polypoid gastritis with thickened, lobulated folds
Association with Gastric Carcinoma
Increased risk of gastric Ca

Less than 1% develop Ca
Not enough evidence to treat all pts with H.pylori
Polypoid H. pylori gastritis with markedly
thickened, lobulated folds in gastric body
458
H. Pylori & Gastric Lymphoma
Stomach devoid of lymphoid tissue

Development of lymph follicles with H. pylori (MALT)
Low-grade MALT lymphoma (MALTOMA)
Characteristic pathologic features
Gastric MALT Lymphoma
Regress with antibiotics in 70-80%

Precursor of high-grade lymphoma
50-72% of all gastric lymphomas
More common than prev recognized
Figure 2-25-13
Nodularity of mucosa (rounded 2-7 mm nodules)

Diff Dx:
Focal gastritis
Intest metaplasia
Risk of Ulcers
Prevalence
Gastric ulcer
60-80%
Duodenal ulcer 95-100%
Gastric MALT lymphoma with confluent nodules in

gastric body
Detection of H.Pylori
Endoscopic bx
Urea breath test
Serum Ab test
459
Pancreatitis: Imaging Has Made a

Difference
Bruce P. Brown, MD
Is this heaven? No. Its the anterior pararenal space.
Normal Pancreas
Acute Pancreatitis
(Marseilles 1985)
Sudden onset abdominal pain
Increased pancreatic enzymes, blood, urine
Pancreatic edema, fat and gland necrosis, hemorrhage
Variable involvement of regional or remote tissues (Atlanta 1992)
(Marseilles 1985)
Recurrent or persistent abdominal pain
+/- increased enzymes
Irreversible morphologic change in pancreas
Fibrosis
Acinar destruction
Calcification
Diffuse, Focal
Loss of function
Acute Pancreatitis: Who Gets It?
Biliary stones (45%)

Alcohol (35%)
Idiopathic (1015%)
Hypercalcemia
Hypertriglyceridemia
Drugs
Post ercp
Hereditary
Trauma
Infection
Vasculitis
Pancreatic cancer
Pancreas divisum
Sludge?
Acute Pancreatitis: Pathophysiology
Alcohol
Alters duct permeability -> protein precipitation in ductules
Gallstones
Common channel of bile and
pancreatic ducts -> bile reflux into
pancreatic duct
Acute Pancreatitis: A Cascade of

Events
Pancreatitis
460
Acute Pancreatitis: Good and Bad
Interstitial
Edema
Architecture preserved
No hemorrhage
Hemorrhagic
Tissue necrosis, pancreas, fat
Hemorrhage
Vascular thrombosis & inflammation
Acute Pancreatitis: Clinical Dx
Abdominal pain->back
Nausea, vomiting
DDx
Perforated ulcer, bowel ischemia, cholecystitis
Labs
Hyperamylasemia
Elevated lipase, more specific for pancreatitis
Degree of enzyme elevation: no correlation w. severity
Acute Pancreatitis: Complications
Early ( 2-3 days ) multi-organ failure

Cardiovascular, pulmonary, renal
Phospholipase A2, elastase, tumor necrosis factor, cytokines, IL-1,2,6,
trypsinogen activated peptide (TAP)
Intermediate ( 2-5 weeks )
Infection, pseudocyst, GI, biliary
Late ( months years )
Vascular, pseudoaneurysm
Balthzar 2002 Radiol Clin
Acute Pancreatitis: Plain Films
Chest film
Pleural effusion 43% w. severe pancreatitis
ARDS
Pulmonary infarction
Duodenum or colon distention
Sentinal loop = focal dilation
Colon cutoff = gas-filled colon -> abrupt cutoff at splenic flexure
Acute Pancreatitis: GI Contrast
No primary role; may screw up CT

Perigastritis, duodenitis, colitis -> Thick folds
Mucosa intact
Mass effect from pancreatic fluid
Fundal varices from splenic vein thrombosis
Acute Pancreatitis: Ultrasound
Of limited use in Dx
Is pancreatitis associated w gallstones?
Fluid collections?
Vascular complications?
Intervention
461
Pancreatitis
Acute Pancreatitis: MRI
Advantages
Gadolinium easy on kidneys
Able to view biliary tract
Sick Patients
Motion artifacts
Difficult to monitor
Specialized equipment
Intervention difficult
Figure 2-26-1
Acute Pancreatitis: CT
Best overall modality

Global view
Prognosis & followup
Understand widespread nature of pancreatitis
Routes for intervention
Large, well-encapsulated pseudocyst

adjacent to pancreatic tail
Acute Pancreatitis: Terminology

Atlanta Symposium 1992
Confusion of terms
Acute pancreatitis
Mild = minimal organ involvement, uncomplicated recovery w. supportive
Rx
Severe = organ failure or complications eg. pseudocyst, necrosis, infected
necrosis, abscess
Acute Pancreatitis: Acute Fluid Collections
Figure 2-26-2
Extravasated pancreatic fluid

Anterior pararenal space, lesser sac
Not loculated
No capsule
40% patients w. acute pancreatitis
50% resolve spontaneously
May develop into pseudocyst
Acute Pancreatitis: Pseudocyst [Figure 2-26-1]
Loculated collect. of panc. enzymes

Non-epithelialized wall of fibrous or granulation
tissue
4-6 wks to develop
Arise from acute fluid collect. (30-50%)
> 5 cm less likely to resolve
Pseudocyst: Complications [Figure 2-26-2]
Infection
Bile duct or GI obstruction
Perforation -> adjacent organs
Vascular
Venous stenosis,occlusion
Gastric varices
Pseudoaneurysm
Hemorrhage
(top)Large gastric varices produced by splenic

vein thrombosis from pancreatic pseudocyst
adjacent to splenic hilum.
(bottom) Pseudocyst projecting from the
pancreatic tail to the splenic hilum with no
visualization of hilar splenic vein
Pancreatitis
462
Acute Pancreatitis : Necrosis
Non-enhancing pancreas or peripancreatic tiss. old phlegmon

Non-viable tissue
Poor prognosis
Type determines Rx
Sterile-trial of med Rx
Infected-debridement
If infected, mortality 15-50%
Needle aspiration to Dx
Acute Pancreatitis: Abscess
Circumscribed collection of pus

Develops after several weeks
Needle aspiration
Acute Pancreatitis: Location
Central location affords several routes for spreading disease

Anterior pararenal space
Pancreas
Duodenum
Colon
Bare area = reflection of post parietal peritoneum to form the transverse
mesocolon
Root of the small bowel mesentery contiguous w. transverse mesocolon
Tail = intraperitoneal -> splenorenal ligament
Posterior to the lesser sac
Barium Left Anterior Pararenal Space-o-Gram [Figure 2-26-3]

Figure 2-26-3
(top left) Extensive necrosis in the anterior

pararenal space on both sides with air anterior to
the left kidney mistaken for air in the colon.
(bottom left). Delayed views showing contrast
anterior to the left kidney.
Thought to be in the colon.
(right) Barium upper gi contrast study showing
erosion of the pancreatic inflammation into the
small bowel with barium contrast leaking
throughout the entire left anterior pararenal space
Acute Pancreatitis: Good and Bad
Interstitial (Good) 80-90%

< 10% organ failure
1-3% mortality
Hemorrhagic (Bad) 10-20%
Necrosis
50-60% organ failure
15-20% mortality
Banks. Gastro Endoscopy 2002
463
Pancreatitis
Acute Pancreatitis: Can we predict trouble?
75% acute pancreatitis resolve w/o complications

520% mortality
Cant biopsy
Who is really sick?
Clinical Predictors
Ranson Criteria Sens = 5785%; Spec = 6885%
APACHE II = 77% pos. pred. value on admit; 88% after 48 hrs
Clinical Assessment of Pancreatitis Severity
RANSON
Non-Gallstone pancreatitis
Admission (any 3)
> 55yrs old; WBC > 16K,
Blood sugar >200
LDH >350 ; AST > 250
At 48 hrs (any 3),
Hct decr >10
Rise in BUN > 5
Calcium < 8
PO2 < 60
Fluid deficit > 6L
Base deficit > 4
APACHE II
Vitals signs
PO2
pH
Electrolytes
Creatinine
HCT; WBC
Glasgow coma score
Can Imaging Alone Predict Trouble? Yes
CT grading (Balthazar 1985;1990)

A = Normal
B = Focal or diffuse enlargement
C = Peripancreatic inflammation
D = Single fluid collection outside gland
E = 2 or more fluid collections or gas in or near panc.
83 PTS
All As discharged w/o complications within 2 wks
A or B -> no abscess
D or E -> 5 of 6 deaths; 89% of abscesses
Imaging Predicts Trouble. Can we refine this further?
Problem: After classifying patients as high-risk, fluid collections resolved in

54%
Pancreatic necrosis
Poor gland enhancement correlates w. degree of necrosis at surgery
(Kivisaari GI Radiol 1984)
Gland necrosis correlates with development of complications (Balthazar
Radiol 1990)
No necrosis = no mortality; 6% morbidity
Necrosis = 23% mortality; 82% morbidity
Pancreatitis
464
Acute Pancreatitis: Can we predict trouble? [Figure 2-26-4]
CT Severity Index (Balthazar Radiol: 1990)

CT anatomic changes
A = 0, B = 1, C =2, D = 3, E = 4
Gland necrosis
< 30% = 2, 30-50% = 4, > 50 = 6
0-1 = no mortality or complications
2 = no mortality; 4% complications
7-10 = 17% mortality; 92% complications
Figure 2-26-4
Acute Pancreatitis: The Power of CT
Suspected pancreatitis Dx in doubt

Severe pancreatitis suspected of complications
Pancreatitis w/o improvement in 72 hrs of med. Rx
Improving pancreatitis that deteriorates
Severe pancreatitis w. initial scan D-E; CTSI 310
follow-up may detect asymptomatic complications
Definition (Marseilles 1985)

Recurrent or persistent abdominal pain
May or may not see increase enzymes
Irreversible morphological change in pancreas
Fibrosis
Acinar destruction
Calcification, duct /parenchyma
Focal, segmental, diffuse
Progressive loss of exocrine/endocrine function
Who Gets It?

Chronic alcohol abuse (6070%)
Idiopathic (30%)
Biliary tract disease
Hereditary
Hyperlipidemia
Hyperparathyroid
Pancreas divisum
Clinical
Recurrent abdominal pain (95%),
Pancreatic insufficiency ,
Malabsorption,
Diabetes,
Amylase/Lipase levels +/ abnormal
Balthazar Classification of severity of acute

pancreatitis
(top). Mild Pancreatitis: CTSI = 0-1. Small amount
of peripancreatic stranding. No fluid collections.
Entire gland enhances.
(bottom). Severe Pancreatitis: CTSI = 8-9.
Pancreas outlines are obliterated with necrosis.
No enhancement with contrast
Chronic Pancreatitis: Pathophysiology
Poorly understood
Etoh increases ductal secretion ->
Precipitation of protein plugs ->
Calcification
Chain of lakes / dilated duct
Inflammatory infiltrate + fibrosis
Chronic Pancreatitis: Plain Films
Pancreas Ca++ (75-90%)

Most common in Etoh pancreatitis,
Ductal or parenchymal
May be focal
Increase w. progression pancreatic dysfunction
Also w. hereditary pancreatitis, cystic fibrosis
465
Pancreatitis
Chronic Pancreatitis: Barium [Figure 2-26-5]
Inflammation/scar -> perigastritis

Not primary disease of bowel
Figure 2-26-5
(left) Severe distortion of the gastric

contours on double-contrast barium study
from chronic pancreatitis with inflammatory
changes and scar in the perigastric tissues,
so-called "perigastritis." There is no primary
gastric disease.
(right) CT appearance of the same patient
showing changes of chronic pancreatitis
with parenchymal calcifications and gland
atrophy
Chronic Pancreatitis: Ultrasound
Heterogenous echotexture
Hyperechoic foci = Ca++/ fibrosis,
Bile &/or pancreatic duct dilation
40% focal mass DDx = cancer
Complications
Pseudocyst portal / splenic vein thrombosis
Endoscopic ultrasound?
98% sensitivity / 90% specificity?
Chronic Pancreatitis: Endoscopic Ultrasound
Difficult to establish a gold standard esp. for mild to moderate disease

Few studies with histology
Sens = ?87%; Spec = ?64%
Chronic Pancreatitis: CT
Not as useful as in acute pancreatitis

Gland enlargement (30%)
Mass (30%) ? Cancer
Atrophy (15%)
Sens. 50-90%: Spec. 55-85%
Acute + chronic w. exacerbation of disease
Chronic Pancreatitis: MRI
Parenchymal enhancement
T1 fat-supressed, pre & post Gd dynamic
Decreased signal/Delay in peak vs. controls
Sens = 79%; Spec = 75%
Better than morphologic changes alone
MRCP- ductal anatomy
Highly T2 weighted, single breath-hold sequences
85-90% agreement w. ERP for duct caliber
Limited ability to dx early chronic pancreatitis
Functional exam w secretin not conclusive
Remer EM Radiol. Clin. of N. Am.2002
Chronic Pancreatitis: ERCP
Cambridge Classification of chronic pancreatitis

Mild = 3 side branches dilated; main duct 24 mm
Moderate = small cysts, irregular duct
Severe = any of above +
Cyst >10mm, intraductal filling defect, calculi, main duct obstruction,
severe irregularity
Pancreatitis
466
Chronic Pancreatitis: Pancreas Divisum
Dorsal & ventral ducts fail to fuse (5%)

Minor papilla (Santorini) atretic
Most of pancreas drained through atretic minor papilla
Pancreatitis?
Dx = ERCP
Pancreatitis Requests We Have Known and Loved

Pancreatitis: Febrile. Please aspirate fluid. What then?
You are in charge of thinking ahead.

Modality?
Route?
Transgastric? Not for diagnosis only
What is the plan?
Pus -> tube
Indeterminate -> Gram stain +/- tube
Clear fluid -> Gram stain, culture
Solid stuff -> no flow -> saline -> culture. BX?
Pancreatic Pseudocyst
Pancreatic Fluid Collection: I am happy to help, but what is the
indication for drainage? My staff wants it
Indication for access to evolving fluid collection or necrosis decided on full

evaluation of clinical, lab, and imaging
Percutaneous drain useless if wont flow through tube
No tube for necrosis or hematoma
Aspiration to dx infected necrosis
Uninfected collections and small pseudocysts may resolve on their own
References
1.
Topazian M, Gorelick GS. Acute Pancreatitis. In: Yamada T, Textbook of

Gastroenterology, Third Edition, Volume 2. Lippincott Williams and Wilkins, 1999,
2121-2150.
2. Owyang C. Chronic Pancreatitis. In: Yamada T, Textbook of Gastroenterology, Third
Edition, Volume 2, Lippincott Williams and Wilkins, 1999, 2151-2177.
3. Banks PA. Epidemiology, natural history, and predictors of disease outcome in acute
and chronic pancreatitis. Gastrointestinal Endoscopy 2002; 56 (6) S226-S230
4. Meyers MA. Dynamic Radiology of the Abdomen Normal and Pathologic Anatomy,
Fifth Edition.Springer, New York 2000.
5. Balthazar EJ. Staging of Acute Pancreatitis. Radiol. Clin. of N. Am. 2002;40:6, 11991209.
6. Balthazar EJ. Complications of Acute Pancreatitis. Radiol. Clin. of N. Am 2002; 40:6,
1211-1227.
7. Remer EM, Baker ME. Imaging of Chronic Pancreatitis. Radiol. Clin of N. Am 2002;
40:6, 1229-1242.
8. Fulcher AS, Turner MA. MR Cholangiopancreatography. Radiol. Clin of N. Am 2002:
40:6, 1363-1376.
9. Strate T, Knoefel WT, Yekebas E, Isbicki JR. Chronic Pancreatitis: etiology,
pathogenesis, diagnosis, and treatment. Int. J Colorectal Dis. 2003; 18: 97-106,.
10. Chatizicostas C, Roussomoustakaki M, et al. Balthazar Computed Tomography
Severity Index Is Superior to Ranson Criteria and APACHE II and III Scoring Systems
in Predicting Acute Pancreatitis Outcome. J. Clin. Gastroenterol. 2003; 36: 3, 253-260.
11. Wiersema MJ, Hawes RH, et al. Prospective evaluation of endoscopic ultrasonography
and endoscopic retrograde cholangiopancreatography in patients with chronic
abdominal pain of suspected pancreatic origin. Endoscopy 1993;25:555-564.
467
Pancreatitis
Gastrointestinal Bleeding In The Age of

the Endoscope. What Does a Radiologist
Have To Contribute?
Bruce P. Brown, MD
GI Bleeding: Demographics
Older
Male
Use alcohol, tobacco
Aspirin, non-steroidal anti-inflammatory
Anticoagulants
Peura et al, Am.J.Gastro. 1997
Gastrointestinal Bleeding: Presentation
Hematemesis Bloody vomitus, red, coffee grounds; indicates upper GI

bleeding
Melena Black, tarry stools; usually indicates upper GI bleeding
Hematochezia Red blood per rectum; lower GI bleed, large-volume upper GI
bleed (> 1000 cc)
Acute GI Bleeding: Demographics
Upper GI 76%
Duodenal & gastric ulcers >50%,
Lower GI 24%
Diverticular 30-50%
79% Anemia
31% Hypovolemia
59% Transfused
45% Endoscopic Rx
7% Surgery
2% Death
Peura et al, Am.J.Gastro. 1997
Gastrointestinal Bleeding: How Bad Is It?
Hypovolemia - 30% of GI bleeders

5 L (10 Units) = normal volume
Hct poor measure of acute bleeding
20% blood loss -> 10 mmHg drop BP w. standing
40% blood loss = Shock = resting supine tachycardia, hypotension, pallor,
agitation
Massive GI bleed = > 6 units transfusion needed in 24 hours
Acute Gastrointestinal Bleeding: Diagnosis is NOT the first

priority
Resuscitation
Two BIG lines 18 gauge
Fluids immediately
Blood when available; 6 u typed & crossed
ICU
Gastrointestinal Bleeding
468
Gastrointestinal Bleeding: Where Is It?
Upper GI
Proximal to ligament of Treitz,
Usually melena
NG tube 16% negative even w. UGI bleed
Lower GI
Distal to the ligament of Treitz
Usually hematochezia
Upper GI Bleeding: Causes
Diagnosis
Duodenal Ulcer
Gastric Erosions
Gastric Ulcer
Varices
Mallory-Weiss tear
Esophagitis
Neoplasm
Other
% of total
24
23
21
10
7
6
3
11
Silverstein et al,Gastro.Endosc. 1981
Lower GI Bleeding: Causes
Diagnosis
Diverticulosis
Vascular Ectasia
Idiopathic
Neoplasia
Colitis
Radiation
Ischemia
Ulcerative colitis
Other
% of total
43
20
12
9
6
2
1
7
Reinus et al GI Clin NA 1990
GI Bleeding: Endoscopy
First line procedure in UGI bleed

9095% accurate Dx
Useful for prognosis, treatment
Performed immediately
Alcoholics,
Large volume loss
Aorto-enteric fistula
Performed more electively
Young, no evidence of hypovolemia
Nuclear Scintigraphy
Most sensitive non-invasive test

Detects bleeding rates 0.1ml/min
Two techniques
Tc 99m sulfur colloid
Tc 99m labeled red blood cells
Used to
Delineate obscure sources small bowel, intermittent bleeding
Enhance the efficacy of angiography
469
Angiography
Usually preceded by RBC study

Detects 0.5 ml/min
Upper GI bleeding
When endoscopy inconclusive
Anticipation of transcatheter intervention
Lower GI bleeding
Procedure of choice?
Upper GI Bleeding: Peptic Ulcer Disease
Gastric, duodenal, stomal ulcers = 50% UGI bleeding

Etiology: Non-steroidals, H. Pylori
Anatomic risk factors
High lesser curve
Posterior-inferior duodenal bulb
Giant gastric (>3 cm) & duodenal (>2 cm)
Endoscopic risk factors
Risk of Rebleeding: Endoscopy
Peptic ulcer disease rebleeding

Clean fibrin base
Flat spot
Adherent clot
Visible vessel
Spurting vessel
5%
10%
22%
43%
90%
Laine NEJM; 717; 1994. UCLA-CURE studies.
Gastritis
Hemorrhage, erythema, erosions

Erosion = superficial break in mucosa w. punctate bleeding, fibrin base
Causes
Non-steroidals -> antral erosions, ulcer
bleeding usually not severe, resolve w. D/C
Alcohol ingestion
Direct toxin? ->erythema
Gastritis
Portal hypertension
Diffuse or patchy erythema, punctate bleeding, vascular ectasia
Requires reduction of portal hypertension
Stress Erosions
ICU patients
One or more bleeding erosions
Bleeding may be severe
Acute Hemorrhagic Gastritis

Esophageal Varices
50% cirrhotics develop esoph. varices.

1/3 of these bleed
Portal v. pressure >12 mmHg. above Hep.v
At risk to bleed
Large size
Located near GE Junct.
Vascular ectasia on the varices
Rapid bleeding
Emergent endoscopy
50% of cirrhotics w. bleed = non-variceal
Poor prognosis
3050% mortality for first bleed
2/3 die within one year
470
Esophageal Varices: Rx
Vasopressin ( somatostatin/octreotide )
50% effective
Sclerotherapy 8595% effective
Probably improves survival; complications
Band ligation
As effective as sclero Rx; few complications
Balloon tamponade
7090% effective
3050% rebleed after balloon down,
1030% severe complications
TIPS (Transjugular Intrahepatic Portosystemic Shunt)
Expandable stent hepatic to portal v.
95% technically successful
As effective as sclero Rx
1015% complications
1025% encephalopathy
3050% stenosis at 1 year
Surgical porta-caval shunts
5080% mortality for emergency shunt
Elective shunts for endoscopic Rx failures
Gastric Varices Without Esophageal Varices

Mallory-Weiss Tear
510% GI bleeds
Hx of retching; 40% no retching
Non-penetrating linear tear(s) near GEJ
25% multiple lesions; 75% have o. pathol.
Rx ->endo.oversewing
Gut Hemangioma
Rare
Described in young and old
Esophagus, stomach, sm. bowel, colon
Classification
Capillary collection of thin-walled vess.
Cavernous large, dilated channels w. thrombosis -> Ca++
Tendency to bleed
Angiomatosis large area of hemangioma
Gut hemangioma
Cavernous hemangioma
Phleboliths on plain film
UGI = Submucosal mass
CT
Thick wall
Early enhancement network of vessels & sinuses thickening the wall
Late enhancement confluent sinus fill-in
Endoscopy
Soft, submucosal mass or thickened folds, blue-red discoloration
Small Bowel Bleeding: Tough to Dx
35% GI bleeds occur in small bowel (2nd portion duod. to ileocec. valve)
Bleeding is intermittent
Most common causes are vascular
Inaccessible
Anatomy variable
471
Small Bowel Bleeding: Causes
Vascular lesions
Angiodysplasia, hemangioma, AVM, vasculitis
Small bowel tumors
Leiomyoma/sarcoma, adenoma/carcinoma, lymphoma, mets
Ulcers
Crohns, Meckels diverticulum, ZE syndrome
Diverticula
Aortoenteric fistula
Small Bowel Bleeding: How Well Does Imaging Do?
Small bowel series vs enteroclysis

71% lesions missed on small bowel series [1]
Small bowel series for occult bleeding
5% yield for bleeding site [2]
Enteroclysis
10 % yield for bleeding site [3]
Enteroscopy
Cumbersome, not generally available
[1] Maglinte, Radiol 144:737; 1982

[2] Rabe, Radiol. 140:47; 1981
[3] Rex, Gastro 58;89; 1997
Small Bowel Bleeding

Nuclear Scintigraphy
Most sensitive non-invasive test

Detects bleeding rates of 0.1 ml/min
Two techniques
Tc 99m sulfur colloid
Tc 99m labeled RBCs
Used to
Delineate obscure sources small bowel, intermittent bleeding
Enhance the efficacy of angiography
Technetium 99m Labeled RBCs
New in vitro process (Ultratag) >95% eff.

Continuous dynamic imaging
Large FOV camera over abdomen
60 images q 15 min
Stored for dynamic playback to detect labeled RBCs outside normal blood
pool
Technetium 99m Labeled Red Blood Cells
Disadvantages
Origin of bleed unclear on delayed scans
Vascular organs may interfere w. detection
Loss of tag can produce false +/Advantages
Detects intermittent bleeding
Labeled RBCs
Sensitivity = 8595%; Specificity = 7085%,
Method of choice
472
Meckels Diverticulum
Most common congenital GI tract anom.

Vitelline duct fails to resorb
True diverticulum
2% of population
2 x more common in males
2 cm long ( 110 cm),
2 feet from ileocecal valve
50% ectopic gastric or pancreatic mucosa
2540% symptomatic
Complications
Bleeding usually in kids <5 yr,
Intussusception kids & adults
Volvulus, diverticulitis, perforation
Bleeding ulceration of gastric mucosa
Aortoenteric Fistula
Erosion of aorta into 3rd portion of duod,

Dacron graft, atheroma, mycotic aneurysm
Herald bleed stops spontaneously followed by exsanguinating bleed
Preemptive surgery
Pill Endoscopy
Ingestible capsule
7 hour recording
2 images per second
Localizing surface antennae
View in real-time
Contraindicated w. obstruction
22% Capsule vs. 3% barium-positive in 52 pts w. occult gi bleed
Hara AK, Radiol 2004, 230: 260-265)
Lower GI Bleeding: Causes
Diagnosis
Diverticulosis
Vascular Ectasia
Idiopathic
Neoplasia
Colitis
Radiation
Ischemia
Ulcerative colitis
Other
% of total
43
20
12
9
6
2
1
7
Reinus et al GI Clin NA 1990
Colonic Diverticulosis
Colon Diverticula = herniations of mucosa and submucosa through muscular

layers at site of penetration of vasa recta through bowel wall.
Colonic Diverticular Bleeding
3550% prevalence of diverticula

15% pts. tics bleed
5% massively
The major cause of lower GI bleed
75% of tics in left colon
70% of bleeding tics in right colon [1]
Not assts w. diverticulitis
[1] Cassarella, NEJM 286:450;1972

473
Colonic Diverticular Bleeding: RX
Colonoscopic vasoconstrictor injection, heater probe, laser select patients

Angiography
Selective catheterization
Vasopressin 50-90% success
Embolo Rx Gelfoam, coils
Surgery
Angiodysplasia
2040% acute LGI bleeding

Vascular ectasia
2/3 in pts >70 yrs old
Aortic valve disease
Von Willebrand factor depletion?
< 5mm vascular tufts
Cecum & right colon
Bleeding
Not massive, intermittent
Stop spontaneously, 85% bleed again
Pathogenesis
Increased tension in cecal wall
Repeated, intermittent obstruction of submucosal veins -> dilation &
tortuosity
Develop small A-V malformation
Colonoscopy 8090% sensitive
Angiography
early tangle of vessels
early filling & slow emptying dilated veins
Treatment
Abnormal vessels poor response to vasoconstrictors; may temporize
Endoscopic electrocoagulation
Embolo Rx
Diffuse disease estrogen-progesterone
Surgery
References
1.
2.
3.
4.
5.
6.
7.
Peura DA, Lanza FL, et al. The American College of Gastroenterology Bleeding Registry: Preliminary Findings.
Am J Gastroenterol. 1997, Jun: 92(6): 924-8.
Reinus JF, Brandt LJ. Upper and lower gastrointestinal bleeding in the elderly. Gastroenterology Clinics of North
America. 1990 Jun; 19(2): 293-318.
Mitros FA, Atlas of Gastrointestinal Pathology. Gower Medical Publishing.
Elta GH, Approach to the patient with gross gastrointestinal bleeding. In Textbook of Gastroenterology, Lippincott,
Williams and Wilkins. Philadelphia, 1999, Yamada T, et al eds.
Fritscher-Ravens A, Swain CP. The wireless capsule: new light in the darkness. Dig. Dis. 2002;20(2): 127-33.
Hara, AK et al. Small bowel: preliminary comparison of capsule endoscopy with barium study and CT
Radiology 2004, 230: 260-265.
Hara AK. Capsule endoscopy: the end of the barium small bowel examination?Abdom Imaging, 2005, Jan.
474
Small Bowel Obstruction

Figure 2-28-1
Small Bowel Obstruction
Impaired passage of contents thru SB.

Partial vs Complete (High Grade)
Intermittent vs Continuous
Mechanical vs Paralytic (Ileus)
SBO
Review
Mechanical
Classic Acute Complete SBO
Simple SBO
Closed Loop Obstruction (CLO)
- Urgent Emergency !
Classic Appearances
Intermittent Chronic SBO
Partial SBO
Motility
Common Ileus
Unusual dysmotilities
SBO
Motility
Paralytic Ileus
Scleroderma Collagen Vasc Disease
Sprue, MAB diseases
Radiation enteritis, earliest stage
Hypothyroidism, metabolic
Chronic Intestinal Pseudo-obstruction
DYSMOTILITY is a FUNCTIONAL Obstruction !
Slow passage acts / looks obstructive
Acute mechanical SB obstruction

showing uniformly distended bowel
Figure 2-28-2
Chronic vs Acute SBO:

Concept to help analyze SB in CT, KUB, SB Series
Distention vs Dilatation: 2 variables

Dilatation: SB diameter larger than expected
A few loops or entire SB
May or may not be Distended
Distention: SB uniform appearance of maximum possible
diameter
Like a sausage shaped balloon inflated to its capacity
Appears tensely filled, to capacity
Simple SBO
A tapered distension meandering back toward Treitz.

A single transition point
Scleroderma SB shows dilated loops
with segments whose diameter are
greater than last case of acute SBO.
Loops are not uniformly distended
with segments that are partly
collapsed
Chronic Intermittent SBO
Dilated but not distended
Chronic vs Acute SBO [Figures 2-28-1 to 2-28-3]
Distention vs Dilatation
Distended, not (XS) Dilated:
Acute, initial SBO
Dilated, not Distended:
Chronic, intermittent SBO or
DYSMOTILITIES !
475
Small Bowell Obstruction
Figure 2-28-3
Dilated and Distended:

Acute, recurrent SBO
Chronic Idiopathic Intestinal Pseudo-obstruction

CIIPO
No cause (readily) apparent.

Myopathic forms:
More common
Dilated,
Atonic
Conceptually like: Scleroderma
Neuropathic forms:
Spastic,
Non propulsive peristalsis
Pulsion divertics of SB
Conceptually like: Diffuse Esophageal Spasm
Bloating, Obstruction
Prior Colectomy for constipation with

Ileo-rectal anastomosis
Idiopathic Intestinal Pseudoobstruction
Myopathic type
[Figure 2-28-4]
MNGIE = POLIP
Mitochondrial Neuro Gastro Intestinal Encephalopathy MNGIE

Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy,
Intestinal Pseudo-obstruction. P-O-L-IP
Rarenth power (73 cases up to 2005)
Familial, Autosomal Recessive
Blondon H, et al Digestive smooth muscle mitochondrial myopathy in

pts with mitochondrial-neuro-gastro-intestinal encephalomyopathy
(MNGIE), 3 cases & review of literature; Gastroenterologie 2005 Aug. SB appears both dramatically dilated
and uniformly distended.(double
VOL 29 - N 8-9,p. 773 - 778
arrow). This suggests acute
Simon et al, Polyneuropathy, Ophthalmoplegia,
obstruction in a patient with chronic
Leukoencephalopathy, Pseudoobstruction: POLIP Syndrome; Ann
recurring obstruction from Crohns
Neurol 1990;28:349-360
Disease (arrow)
Figure 2-28-4
48 Hr films shows barium in proximal SB.

Dysmotility diseases may produce massive
dilatation. Segments that do not propel act like
mechanical obstruction.
Diseases affecting nerves and muscles of the
bowel are multiple, infrequent, and require
extensive work-up. A history of prior partial
colon resection, SMA syndrome requiring
duodeno-jejunostomy, volvulus of colon in a
young pt, or recurrent obstructions without
apparent cause should raise suspicion
476
Radiology of POLIP MNGIE [Figure 2-28-5]
Slow GI Transit
Non-propulsive SB hypermotility
LIKE DES Corkscrew esophagus
SB Tics from segmental spasm
Malabsorption pattern: wet, moulage,
delay
MRI: White matter changes, high signal
on T2
Transition Point: Single Discordance
Tapering: Normal @ Treitz to max
obstruction
SB Meanders to max allowed by
mesentery
Figure 2-28-5
CT: Acute SBO [Figure 2-28-6]
Early (ER) CT:

Reduce mortality & morbidity
Cost effective
Diagnose SBO
Grade Severity
Simple
Closed Loop
Strangulating
Dead Bowel
Etiology
Segmental non coordinated SB

contractions of the small bowel may
produce PULSION DIVERTICULA
(T) and marked delay in transit,
findings both evident on a 24 hr follow
up film with contrast still in stomach
and proximal SB. This neuropathic
intestinal pseudo-obstruction pattern
may be seen in other rare diseases of
disordered mitochondrial activity
altering peristaltic neuromuscular
bowel coordination
CT: Acute SBO
Holy Grail = Transition Point

Define Lesion:
Tumor, hernia
No Lesion = Adhesion
Study:
Colon -?
Collapsed SBO
Fluid filled: Ileus, MAB
Ileocecal Valve
Duodenal Crossing
Mesenteric Vessels
Figure 2-28-6
Adhesion
MAJOR CAUSE SBO:

Benign Adhesions
Surgical
Inflammatory -itis
Radiation
Endometriosis
Ischemia
Neoplastic Adhesions
(Carcinoid)
Transition point without discernable

mass or hernia indicates adhesion
477
Tethered Adhesion [Figure 2-28-7]
Figure 2-28-7
Bezoar [Figure 2-28-8]
Rarely sole cause SBO:

THINK: motility dis.
Radiation, scleroderma
Nl person: Fiber binge
More often part of SBO:
Fibrous Food impaction just above
minor lesion. (adhesion) SB
Feces sign
POINTS TO OBSTRUCTION
Mayo-Smith WW, Wittenberg, et al. CT SB

faeces sign: description & clinical
significance. Clin Radiol. 1995
Nov;50(11):765-7
Tenting or sharp
angulation of a loop is
suspicious for adhesion
or entrapment of its
mesentery
Abdominal Hernias
By Location
External vs Internal
Inguinal, Femoral, Sciatic, Hiatal, Spigelian, etc
By Type
Complete vs Partial (Richter)
By Content
Littre (pre-existing tic), Amyand (appendix)
By Severity
Reducible
Non-Reducible or Incarcerated
Ischemic or Strangulated
Figure 2-28-9
Infarcted
Figure 2-28-8
Obturator Hernia
[Figure 2-28-9]
Elderly F 10 to 1 M
R >> L
Assoc w recent wt loss
Not palpable
SBO in Obturator
Pectineal Space
Howship Romberg Sign
Pain medial thigh +/50%
Hannington Kiff
Absent thigh adductor
reflex
Ijiri R, et al Oburator H:
usefulness of CT in DX.:
Surg.1996 Feb;119(2):137-40.
SB Bezoars are useful to point to a
site of obstruction. They are
composed of residual fibrous material
that begins fermentation. Carrots,
mushrooms, and other fibrous foods
may cause transient symptomatic
obstruction in normal patients when
the bolus reaches the ileocecal valve.
Poor peristaltic tone may play a role
in pts with dysmotility disorders
Obturator Hernias are unusual

hernias difficult to detect by
simple physical examination.
Special maneuvers are
needed. Tiny barely visible
hernias may produce
significant obstruction
478
Spigelian Hernia SBO [Figure 2-28-10]
Note hernia under extern obl muscle
Figure 2-28-10
Spigelian Hernias may

not be evident on
physical examination.
Classic location is either
lower quadrant lateral to
rectus muscle. The
hernia sac protrudes
through a normal weak
point where the
transversalis muscle
fascia changes from
posterior to anterior attachment to the rectus
sheath. Often the hernia stays under the external
oblique muscle
Figure 2-28-11
Incarcerated & Strangulated Parastomal

Hernia
[Figure 2-28-11]
SBO upstream
Efferent Limb collapsed
Neck squeezed
CLO
Distended Hernia Loop
Strangulation
Hernia Sac Fluid
Incarceration
Compressed Abd Wall
Chronic incarceration, or nonreducibility, may cause inward bowing

of the anterior abdominal wall.
Incarceration often is associated with
intermittent obstruction and
predisposed to vascular compromise
479
Gallstone Ileus [Figure 2-28-12]
Figure 2-28-12
Zalcman M, et al Helical CT signs in Dx of

intestinal ischemia in SBO. AJR 2000
Dec;175(6):1601-7
Value of Combining 2 signs for Dx

of Ischemia in SBO
Sign Combination
Sens% Spec %
Mural Thickening +
Mesen Fluid
Mural thickening +
Mesen Vasc Congest
Mural Thickening +
Ascites
Mesenteric Fluid +
Mesen Vasc Congest
Mesenteric Fluid +
Ascites
Mesenteric Vasc
Congestion + Ascites
29.2
99.2
29.2
93.3
25
94.2
50
94.2
66.7
94.2
41.7
94.2
Zalcman et al Helical CT Signs in Dx of

Intestinal Ischemia in SBO; AJR
2000;175:1601-1607
SB Series: Value of Tangent

Value of Tangentwith Valvalsa
? ISCHEMIA
Gallstone Ileus with compromised bowel. Note fistula to
duodenum, air in hepatic bile duct, and stone in distal SB. Top
right image shows a target sign loop (left of diamond) with white
internal mucosa and (right of diamond) adjacent loop which has
less mucosal enhancement . The lower right arrow points to
the longitudinal equivalent of the target sign. Acute high grade
unrelieved obstruction causes edema from compression of
mucosal venous drainage. Although above findings are of
obstructive edema, simple SBO may lead to bowel infarction
Parastomal Hernias: Tangent with Valsalva

Mesenteric Volvulus
Assoc w
Malrotation
Left Colon
Right SB
Weak Treitz
Internal Hernia
External Hernia
Post operative
Short / bunched mesentery
Closed Loop Obstruction
Lumen occluded at 2 adj. sites

Adhesion,
Hernia - Internal, External
Tumor,
Volvulus
Obstructed loop fills w fluid,
Distends, elongates
Base narrows, loop twists
Venous & Art Occlusion - Infarct may result or may be chronic intermittent
Maglinte, Herlinger, Nolan Rad of CLO: 25 confirmed cases. Radiology. 1991

May;179(2):383-7 (Chronic)
480
CT Closed Loop Obstruction (CLO) [Figure 2-28-13]
Closed Loop knot

Clustering of SB loops
Blocked at two ends
Very distended
Mesentery:
Bunching of engorged vessels
SBO above CLO
Less distended
Ascites
Figure 2-28-13
Single Discordance of Simple SBO

Double Discordance of CLO - SBO
SB CLO Ischemic & Hemorrhagic [Figure 2-28-14]
Bowel wall changes

Thickening,
High attenuation I-,
Target sign,
Abnormalities in attached mesentery,
Absence of findings of ischemia or infarction in CLO
does not rule out strangulation
Balthazar et al Closed-loop & strangulating intestinal

obstruction: CT signs. Radiology. 1992 Dec
CLO SBO Problems in Dx
Knot may be elongated

Mesenteric engorgement
Knot angle on slice may obscure
Obst loops prox to CLO, and decomp loops distal may
mingle with CLO
Ascites may mask mes changes
BOW TIE CLO SBO
High Suspicion on all SBOs:
Diameter CLO big & uniform
Criss Crossing: Vessels / SB
Thick walls
No oral contrast gets in
It is critical to
diagnose Closed
Loop SB Obstruction.
With early CLO SBO,
fluid will fill the lumen
of the closed loop.
Then the fluid heavy
loop will spin slightly
around the vessels.
This compromises venous outflow engorging
the wall. Then the elevated hydrostatic
pressure forces more fluid into the lumen
distending it to the maximum. Additional serum,
plasma,and eventually blood may ooze into the
wall. This increases the weight of the loop and
it tends to twist further. This progressive
twisting begins compromising arterial inflow.
Rapid bowel infarction may then occur. This
may all happen before the attending radiologist
comes in the next morning
Figure 2-28-14
A non-contrast CT shows high density in the wall of distended

loops of SB indicating intramural hemorrhage is occuring
481
CLO SBO in Camouflage [Figure 2-28-15]
Figure 2-28-15
CLO SBO Dead Bowel

Criss-Crossing Vessels / Bowel
[Figure 2-28-16]
Bow Tie CLO SBO [Figure 2-28-17]

Gastric Bypass [Figure 2-28-18]
Gastric Bypass Simple
Obstruction
Gastric Bypass Closed Loop
Obstruction
CLO: Stomach to Roux [Figures 2-28-19
and 2-28-20]
Sandrasegaran K, Maglinte DD, et al CT of

acute bilio-pancreatic limb obst. AJR 2006
Jan;186(1):104-9
Scheirey C, Scholz F, et al. Radiology Lap
Roux-Y Gastric Bypass: Conceptualization
and Precise Interpretation Radiographics in
press Sept 2006
CLO SBO may be obscured by location of the knot, by small

or large size of knot, by the angle of the knot relative to the
slice, by ascites obscuring the mesenteric bunching, and by
intertwining of other loops of decompressed distal SB and
simply-obstructed proximal SB among the loops of the CLO
SBO complex
Figure 2-28-16
Note that on sequential sections there is an abrupt

crisscrossing of bowel and vessels. This finding is suggestive
of entrapment of mesentery by adhesions or internal hernia
482
Obst post Roux
Figure 2-28-17
Paraduodenal Hernia
50% IH = Paraduodenal
Mortality pre-CT era (20%)
Clinically:
Asymptomatic,
Pain,
SBO,
Left 3X > R; M > F
SB entrapment =
Congenital anomaly
J Comp. Assist. Tomo. 10:542, 1986
Figure 2-28-18
Bilio
Pancreatic
Limb
Alimentary
Limb
The Bow Tie CLO SBO

occurs when two or
more loops are involved
in the closed loop.
There will be the simple
SBO above the first
closed loop. There will
be two or more CLOs
with each having uniform
but differing degrees of distension. CLO 1 in the
upper abdomen is shorter in length, more
distended, and shows thin perfusing walls. CLO2
involves a more distal longer limb with thicker
walls
Common Limb to Cecum

Gastric bypass for morbid obesity offers many
opportunities for SBO and one unique situation for
CLO SBO
Figure 2-28-19
Closed Loop SBO obstruction

may occur in Gastric Bypass
patients when the bypassed
Bilio-Pancreatic Limb obstructs
at the Roux Y anastomosis.
The Stomach has been stabled
shut so a closed loop
obstruction is created. While
there is not the risk of a
twisting of vessels, this
obstruction puts high pressure
on the closed gastric stump
staple line
483
Ascending Meso-colic H [Figure 2-28-21]
Figure 2-28-20
Right PDH
Ascending Mesocolic H
Absent lig. Treitz
Normal Cecum
Transverse colon not displaced caudally
CT: Ascending Colon vessels anterior to
SB loops
Desc Meso-colon H
Left PDH
Descending mesocolic H
Ligament of Treitz OK
Cecum OK
Stomach displaced to right
Neck contains IMVein & Left Colic Art.
displaced anteriorly by hernia
CT: IMV ant to SB loops
Desc Meso-colon Hernia
Treatment with percutaneous draininage will allow elective

surgery if the obstruction does not resolve
[Figure 2-28-22]
1. Ligament of Treitz OK
2. IMV in front of SB
3. SMA, Vs into hernia
4. Bunched SB possible
If you want to diagnose a Left PDH, look for the IMV
Figure 2-28-21
Figure 2-28-22
Right and left meso-colic hernias, or PDHs, can

be defined by the relationship of the right and left
colic vessels to the proximal small bowel.
Normally they pass behind the upper SB. Note the
anterior course of the right colic vessels anterior to
the SB (arrows) in a right PDH). Note the IMV (
(large pointers) in its normal position behind the
SB on the left
A Left PDH or Ascending Meso-colic hernia with

the Inferior Mesenteric Vein coursing anterior to
the entrapped proximal small bowel
484
Truth about PDHs [Figure 2-28-23]
PDH: Bad name.

Retro-mesocolic congenital hernia
Behind ascending, transverse, or descending mesocolon
(and colon).
Space usually obliterated as embryo
Mesocolon fuses to parietal peritoneum.
Rotation and fixation anomaly
SB does not get out of the way
SB prevents fusion
Figure 2-28-23
Foramen of Winslow
Epiploic Foramen
SB above antrum
ascending
Cecal Volvulus into lesser sac
SBO
Potential in Cecal Volvulus
SB follows IC Valve
Pre op DX: Infarcting Internal Hernia
Assoc w prior bowel surg ( Clips )

See
Mesenteric:
Bunching
Engorgement
Twisting
Criss-crossed vessels
transverse
SB Obstruction
Common disease.
Mechanical: Acute, Chronic, Internal, External
Dysmotilities
Critical
Diagnose
Stage
Establish etiology
SBO
The Paraduodenal Hernia will

remain in our literature and our
Board Examinations even
though they are not hernias but
errors of rotation in which the
SB is trapped behind the
returning meso-colon.
Because there is a wide arc of
returning colon, from the
ascending, the transverse,and
the descending colon, there will
be variation in radiographic
appearance depending on
where the SB is trapped.
Above one can see ascending,
transverse, and descending
retro-meso-colic entrapment
Reviewed
Mechanical
Classic Acute Complete SBO
Simple SBO
Closed Loop Obstruction (CLO)
Urgent Emergency !!!!!!!!
Classic Appearances
Intermittent Chronic SBO
Partial SBO
Paralytic
Common
Unusual
485
descending
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Balthazar et al Closed-loop & strangulating intestinal obstruction: CT signs. Radiology. 1992 Dec
Blondon H, et al Digestive smooth muscle mitochondrial myopathy in pts with mitochondrial-neuro-gastrointestinal encephalomyopathy (MNGIE), 3 cases & review of literature. Gastroenterologie 2005 Aug. 29N, 89:773-778
Frager D, Medwid SW, et al . CT of SBO: Value in Establishing Diagnosis and Determining Degree and
Cause.AJR 1994;162: 37-41.
Ljiri R et al. Oburator H: usefulness of CT in DX.:Surg. 1996 Feb; 119(2): 137-40
Luedke, Scholz. Larsen CT of Spigelian H. Comp Med Imag Graph. 198812(2):123-9.
Maglinte, Herlinger, Nolan. Rad of CLO: 25 confirmed cases. Radiology. 1991 May;179(2):383-7 (Chronic)
Mayo-Smith WW, Wittenberg, et al. CT SB faeces sign: description & clinical significance. Clin Radiol. 1995
Nov;50(11):765-7.
Megibow A, Balthazar E, Cho K, et al. Bowel Obstruction: evaluation with CT. Rad 1991;180:313-318.
Passas V, Karavias D, Grilias D, Birbas A. Computed tomography of left paraduodenal hernia. J Comput Assist
Tomogr. 1986 May-Jun;10(3):542-3.
Sandrasegaran K, Maglinte DD, et al CT of acute bilio-pancreatic limb obst. AJR 2006 Jan;186(1):104-9
Scheirey C, Scholz F, et al. Radiology Lap Roux-Y Gastric Bypass: Conceptualization and Precise Interpretation
Radiographics in press Sept 2006.
Simon et al, Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy, Pseudoobstruction: POLIP Syndrome; Ann
Neurol 1990;28:349-360
Zalcman et al Helical CT Signs in Dx of Intestinal Ischemia in SBO; AJR 2000;175:1601-1607
Zalcman M, et al Helical CT signs in Dx of intestinal ischemia in SBO. AJR 2000 Dec;175(6):1601-7.
486
Acute Mesenteric Ischemia

Bowel Ischemia
Small Bowel or Mesenteric ischemia

SMA distribution: SB and Right Colon
Colonic Ischemia - a different disease
Watershed: Sigmoid, Splenic
ESD: never: unless
Surgery
Radiation
Vasculitis
The Radiology of Mesenteric Ischemia
1. Review classifications & pathophysiology

2. Rad Findings: Ischemia & Infarction
3. Clues to Etiology, emphasis on CT

3 categories
Arterial Occlusive
Without Reperfusion
With Reperfusion
Venous Occlusive
Non-Occlusive Arterial - Low Flow
Pathophysiology of Ischemia
Initial Damage to Endothelial cells of pre-capillary arteriole and capillaries.

Blood vessels leak fluid, then cells
Mucosa & submucosa
Most sensitive, high metabolism
Edema, hemorrhage, & slough
Muscularis propia
Initial spasm
Then atonia
Then perforation / death
(healing with stricture)
Serosa
Petechiae, Ascites
With healing may see adhesions
Ischemia
Rad Findings, Symptoms & Prognosis depend on:

Duration
Momentary to Permanent
Degree
1%-100%
Extent
% of SB
Fast Ischemia
Cell, Tissue, Organ & Organism death -24-48 H

Eg. Embolus to SMA
Eg. Hypotension: Profound & prolonged
487
Mesenteric Ischemia
Slow or Minimal Ischemia
Cellular & localized tissue death

Organ dysfunction
Eg Radiation Enteritis
Eg Scleroderma
Eg Arteriosclerotic Abdominal Angina
Ischemia
Chronic - recurrent - slow

Acute - sudden - fast
Often both
Chronic for months then Acute
Wet vs Dry Ischemia
Wet: Ischemia w arterial inflow p insult (reperfusion)

SEE: Thickest wall, bleeding into wall, ascites.
Eg: Venous occlusion, Transient hypotension, fleeting, partial embolism
Dry: Ischemia w no arterial inflow. (non-reperfused)
SEE: Thinner or normal wall, no / min ascites.
Eg. Complete proximal SMA embolus, sudden thrombosis.
Chou C, CT Manifestations of Bowel Ischemia. AJR2002;178-87

Chou C, CT of SB ischemia. Abd Imaging 2004; 29:18-22
Figure 2-29-1
Wet vs Dry Ischemia : Personal experience
Two extremes: Prune vs Plum

Wet: Classic
Radiologists overcall on CT
Surgeons undercall at Surgery
Dry: Puzzling SBO
Radiologists undercall - miss completely
Surgeons baffled by our stupidity
(SECRET: Study Mesenteric Vessels I+)
Most Specific Single Finding of Acute Mesenteric

Ischemia
[Figure 2-29-1]
No CT perfusion of bowel wall

100% specific, 30%-50% sensitive
Diagnosis depends on
History
Summation of findings
Wall thickening
Mesenteric Edema
Ascites
Absence of wall opacification is

the most specific sign of wall
ischemia. It is not sensitive in all
cases. Because of the length of
the SB, small segments that are
not perfusing may not be
evident. In this image one
segment is perfusing while
another loop is not
WBC
Elevated Lactic Acid
History
Suggestive History:
Pain in excess of Physical Exam
Risk Factors
High Risk Patients (Boley, Clark)
Pt > 50 yrs with:

Valvular or Atherosclerotic Ht Dis
Longstanding CHF
Arrythymia
Hypovolemia or hypotension
Dig or diuretic Rx
Recent MI
Mesenteric Ischemia
488
Figure 2-29-2
Also: AAA w or wo repair

Also: Any Abd, Cardiac, Thoracic Surg
Dry Infarct [Figure 2-29-2]
Tendency to:
Thinner Wall
Absent Target
No intramural blood
Ascites min /absent
No perfusion
Beware: Ileus or SBO in Sick Pt at high risk
Dry Infarct, Dead Bowel - CLO SBO

Spectrum: Ischemia to Infarction [Figure 2-29-3]
Gasless abdomen
Ileus
Thick Folds
Target - CT, US
Stack of Coins - Films
Loss of Folds in Unchanging Thick-walled Loop
Focal ulcer
Shaggy gas pattern
Collar button ulcers
Intramural fistulas
Intralum mucosal cast
Mesenteric or portal vein gas
Intraperitoneal air
Stricture
Pseudodiverticulum
Many findings possible in same pt
Four sequential images (A-D) of a patient with

infarction WITHOUT REPERFUSION. Thin walls
without target sign or intramural blood. No ascitic
fluid. Good example of a dry infarct with minimal
or absent inflow of arterial blood. Dry infarcts may
be due to a large central SMA embolus or smaller
embolic or thrombi with lack of adequate
collaterals to allow inflow. This form of ischemia
and infarction is less common and is hard to
diagnose unless mesenteric vessel contrast is
studied carefully
Usually there is REPERFUSION or extensive
inflow via collaterals producing edema or
hemorrhage into the bowel wall and ascitic fluid
Remember the Law of Burps & Farts

[Figure 2-29-4]
Air rises & thins normal walls

(Also note engorged mesentery)
Figure 2-29-4
Figure 2-29-3
With reperfusion ischemia the wall thickens with fluid

and blood from leaking capillaries. Stack of coin
appearance is due to fluid and blood in the valvulae
conniventes
489
Image shows normal thin wall effaced by air

(arrows) while arrowheads show thick wall not
effaced by air. A loop with a target sign is also
noted (curved arrow).
The slightest amount of SB (or colon) air will rise
and thin wall of normal bowel. Walls thickened by
blood or tumor will not thin out. Understanding this
concept allows for easy detection of abnormal
bowel
Mesenteric Ischemia
The Law of Bowel Gas ll [Figure 2-29-5]
Figure 2-29-5
Air in lumen coalesces into one bubble.(unless

trapped in pneumatosis, blood, stool, or bezoar).
Note dense blood layering
Regular Stack [Figure 2-29-6]
Blood / Edema in Wall

Suggests:
Acute
Recent
Severe
Not specific for ischemia
Irregular Stack [Figure 2-29-7]
Blood / Edema in wall

Suggests:
Chronicity
Recurrence
Fibrosis
Images show non-coalescent air bubble (small

arrow) suggesting either pneumatosis or air
trapped in viscous blood. There is a low density /
high density fluid-fluid level (large arrow)
indicating bleeding into lumen
Loss of Folds [Figure 2-29-8]
Figure 2-29-7
Figure 2-29-6
An irregular stack of coins

suggests re-bleeding and
chronicity with fibrosis
Figure 2-29-8
A regular stack of coins with relatively

uniform appearance of folds is
suggestive of recent bleeding into
wall. A stack of coins appearance
can be due to blood or fluid. It is a
longitudinal image of cross sectional
target sign. Pts with coagulopathies
or with leaking capillaries due to
vasculitis will have a similar
appearance
Loss of folds in an unchanging loop is

an ominous sign for infarction. It
indicates extreme wall thickening and
loss of peristalsis
Mesenteric Ischemia
490
Thick Wall & Ulcers [Figure 2-29-9]
Figure 2-29-9
Ischemic Pneumatosis
[Figure 2-29-10]
Intramural and
Intravenous Air [Figure 2-29-
11]
V Air & Lack of Wall

Perfusion [Figure 2-29-12]
CT Equivalent to stack of coins with arrows

pointing to collections of contrast in the wall
suggesting loss of mucosal integrity
Figure 2-29-11
Figure 2-29-10
Image A: Intramural and intravenous air (arrows),

evidence of a degree of bowel infarction. The
degree of infarction is difficult to determine and
may not correlate with amount of air. A small
focus of wall infarction may allow a large amount
of air to enter veins. Once air enters a vein it may
travel long distances. When SB obstruction is
present air will be under pressure and large
amounts may enter veins through even tiny foci of
loss of mucosal integrity. With even the severest
mesenteric ischemia and infarction, the unaffected
stomach muscles will continue to peristalse fluid
and air into the SB creating SB distension
Two spot films(images A & B) from a water soluble

contrast ileostomy enema shows multiple
ulcerations filling with contrast or with air. The CT
scan slice (image C) shows pneumatosis
Figure 2-29-12
Look carefully for AIR in VESSELS

USE lung windows in Ischemic and in SBO cases
to look for venous air. Use narrow windows to
find subtle wall density changes indicating either
blood or lack of perfusion
491
Mesenteric Ischemia
Extreme Perfusion Variations [Figure 2-29-13]

Intrahepatic Portal Venous Air [Figure 2-29-14]
Splanchnic air will go everywhere splanchnic. It doesnt stay near its origin
Figure 2-29-13
Figure 2-29-14
Loops show EXTREME variations in wall perfusion

from absent to HYPERperfusion. This pt with
infarction and perforation from vasculitis. Pt is
being treated for hypotension and has dense
enhancement from shock bowel in unaffected
loops not involved with vasculitis
Air is seen in portal branches and within veins in

the otherwise normal stomach. (Arrow) Air in
portal system may distribute anywhere within
portal circulation by gravity. In prone position, air
in intra-splenic veins would likely be seen
Figure 2-29-15
Sloughed Mucosa / Serosa [Figure 2-29-15]

Mucosal Cast [Figure 2-29-16]
Inside Serosa
SBO Pearl [Figure 2-29-17]
SBO makes isch / Infarxn look worse

We overestimate Infarction
Edema greater
Air dissects great distances under pressure:
Neck crepitus reported
Pneumatosis not = Infarxn
Figure 2-29-16
The mucosa (long arrows) is seen sloughed in
lumen outlined by contrast reaching to smooth
as yet intact serosa (short arrows)
With infarction and diffuse loss of mucosal
integrity, there will be slough of mucosa. The
serosa is more resistant to ischemia (tough as
hot dog or sausage skins) and will be last to
perforate
Two CT images, A & B, show shaggy irregular
intramural air, a sign of a mucosal cast, a
sloughing of mucosa
Mesenteric Ischemia
492
Pneumatosis Intestinalis (PI)
Figure 2-29-17
Venous gas not allow prediction of transmural infarction,

observed with only partial wall damage
Outcome ..pneumatosis depends on underlying disease
Wiesner W, et al . PI and portomesenteric venous gas in
intestinal ischemia: correlation of CT with severity of ischemia
and clinical outcome. Am J Roentgenol. 2001
Dec;177(6):1319-23.
CT Dx of PI: Lactic Acid > 2.0 mmol/L at Dx assoc with > 80 %
mortality
Hawn MT, et al Serum lactic acid determines outcomes of CT
Dx of pneumatosis of GI tract. Am Surg. 2004 Jan;70(1):1923;
AIR GOES EVERYWHERELOOKS WORSE THAN IT IS
Ischemia Mimic: J Tube Jejunitis
Rad Findings:
Jejunal Edema
Ascites
Portal & SMV Gas
Pneumatosis
SB Necrosis
Clinical:
RARE, 4 / 1460 pts - .021%
J Tube Abd or ENT surg
Post op feeding day 1
Day 5 bloating, N & V
Day 7 hypotension - death.
They suddenly crump
CT Findings
Portal Venous Gas
SMV Gas
Pneumatosis
Penrose, J Tubes
OP:
Normal Bowel, pink, no resection
Two slices from a pt show a shaggy

irregular mucosal cast (image A)
indicating mucosal slough (long
arrow). Other loops of bowel have
extensive pneumatosis (image B).
The length of bowel involved with
pneumatosis may greatly exceed
amount of bowel that is infarcted
because air travels long distance in
veins and in areolar submucosal
tissue
Rare but recognize early
Carucci LR, Levine MS, Rubesin SE, Laufer I, Assad S, Herlinger H.

Evaluation of pts with jejunostomy tubes: imaging findings. Radiology. 2002
Apr;223(1):241-7
J Tube Jejunitis
Schunn CD, Daly JM. SB necrosis associated w post-op jejunal tube feeding.
J Am Coll Surg. 1995 Apr;180(4):410-6.
Lawlor DK, et al SB necrosis assoc w jejunal tube feeding. Can J Surg. 1998
Dec;41(6):459-62.
Rai J, et al SB necrosis in assoc w jejunostomy tube feedings. Am Surg. 1996
Dec;62(12):1050-4.
Munshi IA, et al.SB necrosis assoc with early post-op jejunal tube feeding in a
trauma pt.J Trauma. 2000 Jul;49(1):163-5.
Schloerb PR, et al. Bowel necrosis caused by water in jejunal feeding. J
Parenter Enteral Nutr. 2004 Jan-Feb;28(1):27-9.
Brenner DW, Schellhammer PF. Mortality assoc w feeding catheter
jejunostomy after radical cystectomy. Urology. 1987 Oct;30(4):337-40.
Gaddy MC, et al . SB ischemia: consequence of feeding jejunostomy? South
Med J. 1986 Feb;79(2):180-2.
Jorba R, et al. SB necrosis in assoc w early post-op enteral feeding after
pancreatic resection. Surgery. 2000 Jul;128(1):111-2.
493
Mesenteric Ischemia
Luxury Reperfusion Hyperemia [Figure 2-29-18]
Figure 2-29-18
Vessels lose auto-regulation

Tiniest vessels in mucosa
Dead, clotted,
Mucosa non-perfused
Bigger vessels
Lose muscular tone
Hyperemic bowel musculature
Shunt to veins
Reperfusion Bleeding [Figure 2-29-19]

Ischemia may cause strictures [Figure 2-29-20]
Figure 2-29-19
Luxury
Reperfusion with
gray mucosa and
hyperemic
muscularis and
mesentery
Intra-luminal bleeding may be obscured or

overlooked if pt is given opaque contrast material.
This pt had intraluminal bleeding from diffuse
vasculitis of Degos Syndrome initially assumed to
be ingested contrast. The pt did not drink opaque
material! Always look carefully at the density of
ascites. High density ascites indicates bleeding
into peritoneal cavity
Figure 2-29-20
A SB spot film (A) and a CT slice (B) show a

tapered stricture (arrows) and a long tubular
stricture (arrowheads). With recovery from a
severe ischemic insult, healing with permanent
stricturing can occur
Mesenteric Ischemia
494
Etiologies of Ischemia
Figure 2-29-21
Arterial Occlusive
Emboli 40%-50%
Atherosclerosis - Thrombosis 10%-20%
Mechanical
Closed Loop Obstruction
Volvulus,
Incarceration
Avulsion
Large and Small Vessel Vasculitides
Venous Occlusive
Arterial Non-Occlusive
Embolus [Figure 2-29-21]
Atrial Fibrillation
Valvular heart disease
Sharp cut off
Filling defect
Lateral aortogram shows a filling defect (arrow) in

SMA, a sign of embolus. AP injection of SMA in
another pt. shows long filling defect of embolic clot
(two arrows)
Embolism [Figure 2-29-22]
Figure 2-29-22
White SMA (short

arrows) Then Gray SMA
(long arrows)
ANGIO shows cutoff and
clot.
Dead bowel at surgery
within 8 hrs of
symptoms.
Pt died 16 hrs after
symptoms
495
Mesenteric Ischemia
Figure 2-29-23
Figure 2-29-24
Straddle embolus vs. mural

thrombus extending into
SMA Reperfusion by
collaterals distally
Slow filling of an irregular faint SMA

(arrow) usually indicative of
atherosclerotic thrombotic disease
Figure 2-29-25
Figure 2-29-26
Non-opacification of the SMA proximally with contrast in SMA

distally indicating distal perfusion by collaterals, confirmed with
a coronal reconstruction
A small segment of distal SB shows stack of coins

appearance (arrows) on UGI series ( Image A).
An angiogram (Image B) shows tapered narrowing
of small peripheral branch of SMA (arrow). A
careful SB series or enteroclysis may be needed
to diagnose short segment disease caused by
segmental dis. in peripheral SMA branches
Mesenteric Ischemia
496
Straddler: Embolus Vs Clot [Figure 2-29-23]
Figure 2-29-27
Thrombosis [Figures 2-29-24 and 2-29-25]
Absent segment
Slow filling distal vessel
Large collaterals
Reconstitution
Vascular calcification
Irregular lumen
SMA flow thru GD collaterals
Thrombosis vs. Embolus appearance can be similar,

Hx AF or Ht Valve important
A SB series (Image A) shows a stack of coins
appearance (within circle). An angiogram (Image
B) shows a bulging segment of SMA (arrow), an
ulcerating plaque showering cholesterol emboli.
(This could also be a mycotic aneurysm if patient
were septic.)
Peripheral focal lesion [Figure 2-29-26]

Ulcerating Plaque [Figure 2-29-27]
Dysplasia [Figure 2-29-28]
Becker Duodenal necrosis as presenting manifestation of

polyarteritis nodosa.
Clin Rheumatol. 2002 Aug;21(4):314-6 Chronic Radiation Enteritis
Degos Disease
Malignant atrophic papulosis

Porcelain-white, atrophic papules
Peripheral erythema
Telangiectases.
Small vessel thromboses
M3 X F, all ages
Skin presentation
May rarely remain dermal
When systemic, 2-4 yr prognosis
Coskun B Benign Cutaneous Degos' Disease:

case report and review of literature. J Dermatol. 2004 Aug;31(8):666-70
Degos GI Path
GI - Any portion, but SB predominant

60% Degos: GI perfs lead to death
Sub-serosal white or yellow plaque, transmural bowel
inflam., ulcers, hemorrhage, infarction
Figure 2-29-28
Arterial Occlusive
Atherosclerosis
Embolus
Venous Occlusive 5%-10%
Proximal Obstruction
Distal Disease
Etiology SMV Thrombosis
Idiopathic 20%
Recent Surgery, esp Colon
Hypercoagulable States
Protein S, C defic, polycythemia, hematological
Cirrhosis
Portal Vein Thrombosis
Pancreatic Inflamm / Neoplasm
Pelvic Infectious Processes
497
An SMA angiogram (Image A) shows an irregular

abrupt tapered narrowing of SMA branch vessel
(arrowhead), consistent with dysplasia. There is
also subtle corrugation of main trunk and small
branches within circle. There is a clip (arrow)
where right renal artery, resected previously for
renal artery dysplasia, originated
Mesenteric Ischemia
Symptoms SMV Thrombosis [Figure 2-29-29]
Figure 2-29-29
Duration of symptoms
9.1 days, range 1-42 d
Pain 84%, N & V 56%, Fever & Chills 56%
Diarrhea 23%, Blood in Stools 23%
Ischemia 21%
Bowel Wall Thickening
Mesenteric Congestion
Mortality in 7%, (rapidly falling in MDCT era)
(MDCT: Incr detection & Rx)
Warshauer DM, Lee JKT, Mauro MA, White GC; Superior

Mesenteric Vein Thrombosis w Radiologically Occult
Cause: Retrospective Study of 43 Cases;
AJR2001;177:837-841
Images A-C: CT images show thrombus (arrows)
in Portal Vein. Images D-F Images of mid
SB Infarction [Figure 2-29-30]
abdomen show engorged mesenteric SB leaves
Lymphoma of SB Mesentery
and thickening of SB loops (arrows) consistent
Nodes Compress Veins
with ischemia. While wall and mesenteric
Engorged Mesentery
thickening may mimic Closed Loop SBO, absence
Infarction / Slough
of bunching, lack of bowel distension, and
preservation of transit distinguish the two.In
absence of ongoing thrombotic coagulopathy,
Arterial Occlusive
venous thromboses often eventually resolve with
Atherosclerosis
or without therapy
Embolus
Venous Occlusive
Figure 2-29-30
Low Flow States
Shock
Steals
Arterial Vasospasm
SBO
Figure 2-29-31
Aortogram shows lush

perfusion and opacification of
even small peripheral
branches of upper abdomen
vessels with minimal
mesenteric vasculature
apparent. There is no IMA
visible. The main SMA trunk
stops abruptly (arrowhead).
An embolus is possible but
absence of visible filling defect
in SMA and extensive vascular
disease makes thrombosis
more likely
Mesenteric Ischemia
Images A-C show bulky nodes in the

SB mesentery (straight arrow). The
mesentery is engorged (arrowhead)
indicating compression of mesenteric
vessels. Shaggy irregular intramural
air (curved arrows) indicates
infarction with mucosal slough.
Compression of SB veins may lead to
venous engorgement and bowel
infarction
498
Abdominal Angina, a Clinical Syndrome
Figure 2-29-32
1. Pain following eating

2. Weight loss
3. Diarrhea, rapid transit
Classic
Occlusion of 2 of 3: Celiac, SMA, IMA
May be 1 vessel occlusion, part others
May be absent with full 3 vessel occlusion
Vasculitis, Radiation, Median Arcuate Ligament
Syndrome, Steal Syndromes, CA Pancreas
Vasculopath Blood Thievery[Figure 2-29-31]
Lush vasculature in upper abdomen

Abrupt SMA end.
Paucity lower abdomen
Vasculopath Collateral Steal from SMA

[Figure 2-29-32]
See steal from SMA to SMA, IMA and beyond

Reflex Arterial Vasospasm in SMA
Three images from SMA injection (A-C) show

abrupt
termination (arrow) and collateral flow filling
Abdominal Angina: Median Arcuate
an
enlarged
marginal arcade vessel (arrowheads)
Ligament
and
filling
of IMA branches (curved arrow).
[Figure 2-29-33]
Chronic
steal
syndromes in vasculopaths have
Median Arcuate Ligament of diaphragm
variations
in
amount
stolen depending on varying
Compression / fibrosis of Celiac Artery (occ SMA too)
demand.
Walking
may
deplete visceral flow and
Collateral Steal from SMA
produce Reflex Arterial Vasopasm and abdominal
angina
Figure 2-29-33
Figure 2-29-34
Image A in a pt with chronic intermittent abdominal pain shows

a stack of coins appearance to the jejunum. Image B and a
detail from it, image C, shows a short segment narrowing of
Celiac Axis with a normal SMA just caudal to it. The
appearance allows diagnosis of median arcuate ligament
syndrome. The median arcuate ligament of diaphragm may
compress Celiac Axis. This forces a physiologic steal from
SMA which may be asymptomatic when bowel is at rest.
Following eating, classic abdominal angina may occur because
steal creates a functional mesenteric ischemia
Expiration / Inspiration
Median Arcuate Ligament
Collateral Steal from SMA [Figure 2-29-34]
Large collaterals
SMA -> Celiac
Reflex Mesenteric Vasoconstriction
499
The AP projection during an SMA

injection shows collateral filling of
Celiac vessels and reflex mesenteric
vasoconstriction of mid and distal
branches of SMA, placing them at risk
of thrombosis. Celiac artery occlusion
may be cause of mesenteric angina,
ischemia, or infarction due to collateral
steal
Mesenteric Ischemia
Reflex Mesenteric Vasoconstriction
Figure 2-29-35
Shock Bowel
Dense persistent enhancement:

Bowel Wall, solid organs
Delayed pyelogram
Small aorta, IVC, Spleen
Ascites
Variable Distension - Wall Thickness
Periportal extravasation of fluid
Major trauma with
Resuscitation
Volume repletion
2 to Reflex vasoconstriction
Mirvis SE, et al Diffuse SB ischemia in hypotensive

adults after blunt trauma (shock bowel): CT findings &
clinical significance.
AJR Am J Roentgenol. 1994
Dec;163(6):1375-9
Shock Bowel
May develop:
Ischemia
Infarction
SB = Low Flow State usually caught in time
CT images (A-F) show wall thickening with fluid

density in pt with angioedema, a process where
capillaries leak serum. It may be due to allergies
to food, drugs, or other exogenous allergens. A
hereditary form occurs without specific causation.
Angiotensin Converting Enzyme inhibitor drugs
may produce this finding alone or in association
with glottic or generalized edema. It may be dose
related or seen with certain ACE inhibitors. Those
with bowel angioedema from ACE inhibitors may
present with a rad and clin picture suggesting
mesenteric ischemia. Pts on hypertensive or
cardiac medications should be questioned about
antihypertensive medication to exclude this as an
etiology. Cessation of offending ACE inhibitor may
provide relief and rad return to normal within 24 to
48 hrs
Ischemia Mimic: Angioedema

[Figure 2-29-35]
Enhancement of mucosa
Submucosa edema
Fluid in lumen
Ascites
Etio
Allergic reaction,
Hereditary,
ACE inhibitors.
DeBacker AI, et al; CT of Angioedema of the Small

Bowel, AJR 2001; 176: 649-52
3 cases: 3 different etiologies
1 case report of 1 pt
1 NEJM Images in Clinical Medicine
Ischemia Mimic: Angioedema

Serum Leak from Capillaries
ACE Inhibitors
Accupril
Aceon
Altace
Capoten
Captopril
Lisinopril
Lotensin
Mavik
Monopril
Prinivil
Univasc
Vasotec
Zestril
Mesenteric Ischemia
500
Ischemia Mimic: ACE I A E
7 Female 1 Male
Ascites > Bowel Change
Preserved Transit
ACE I stopped:
< 24 Hr resolution
Bowel changes mild enough to wait / watch, avoid surgery!!
Stiff Arcs in 6 of 8 pts
Figure 2-29-36
Lahey experience
Ischemia Mimic: Stiff Arc Sign
Serum leaks from capillaries

Intact
Arteries
Capillaries
Veins
Blood Flow
Oxygenation
Wall stiffest per degree of thickness
Long Arcs of erect SB possible
Ischemia Mimic: Edema Post Obstructive

9 Days Earlier: SBO & LBO Diverticulitis
Ischemia Mimic: Obstructive & Post Obst Edema
[Figure 2-29-36]
Drop in high IL pressure

Local Arteriolar Hypertension &
Increase Vasc Permeability =
Tissue Edema
Ischemia Mimic: Hemorrhage vs Ischemia [Figure 2-29-37]
Note thumbprinting of colon and

stack of coins appearance of SB.
Chronic high intra-luminal pressure
will affect the hemodynamics of
perfusion. Submucosal edema will
occur with severe obstruction. When
obstruction is relieved, the edema
and altered perfusion dynamics may
persist, and the edema may become
more prominent immediately after
relief of obstruction
Figure 2-29-37
Some CT features overlap: target sign, hemoperitoneum

Intramural Hemorrhage:
Short segment < 15 cm
Wall thicker = or > 1 cm
Ischemia:
Long > 30 cm
Wall less thick < 1 cm
15 -30 cm overlap
Macari M, et al Intestinal ischemia versus intramural hemorrhage: CT

evaluation. AJR. 2003 Jan;180(1):177-84
Ischemia Mimic: ITP
Immune Thrombocytopenic Purpura

Bowel Hemorrhage
Note: post splenectomy
Ascites
2 groups: Age 2-4, Adult
Causes
Idiopathic
Drug Induced
SLE
Infection
Pregnancy
Rx
Immune Suppression
Splenectomy
A regular stack of coins with relatively

uniform appearance of folds is
suggestive of recent bleeding into
wall. A stack of coins appearance
can be due to blood or fluid. Pts with
coagulopathies or with leaking
capillaries due to vasculitis will have a
similar appearance
501
Mesenteric Ischemia
Figure 2-29-38
Purpuras are a group of diseases that weep small

amounts of blood from many tiny vessels.
Henoch Schonlein is transient, often recurring
immune mediated vasculitis, usually affecting
children, can be seen in adults. Usually palpably
raised itchy red lesions (Arrows Image A) are
present and allow a diagnosis. Petechiae (arrows
in image B of ileal endoscopy) and purpuric
lesions also occur in bowel. Abd involvement is
seen in 50-75% of pts who present with dramatic
colicky abd pain and bleeding, which may be
massive in 1-2% of patients. Bleeding into wall of
bowel thickens it (Image C arrows) and gives a
stack of coins appearance (Image D arrows). This
intramural bleeding may cause obstruction, GI
bleeding, infarction, perforation, or intussusception
in distal SB. While no effective therapy, pts must
be monitored for complications until attack
subsides
Figure 2-29-39
The descriptive term

target sign has been
applied to a loop of bowel
with distinct demarcation
of circular layers of the
wall. It is seen whenever
fluid of some type, or fat,
or air enter submucosal
space between mucosa - muscularis mucosa and
muscularis propria-serosa.
Image A innermost gray is fluid in lumen. Then
there is a white line representing contrast opacified
mucosa and muscularis mucosa. Then there is a
gray circle which represents fluid in submucosa.
The outermost white ring represents contrast
opacified muscularis propria and serosa
Image B, the innermost white dot is ingested
opaque contrast material. Then a less opaque ring
represents mucosa and mucosal muscle. There is
a low density ring which is negative in Hounsfield
units indicating fat. The outermost layer is
muscularis propria-serosa. Fat prominence is seen
in pts with Crohns disease and for unknown
reasons may be seen in occasional normal pts in
the distal Ileum, possibly due to prior infectious
gastroenteritis
Mesenteric Ischemia
502
Ischemia Mimic: Henoch Schonlein Purpura

[Figure 2-29-38]
Target sign [Figure 2-29-39]
Blood, Serum, Plasma, Interstitial Fluid, Fat, Air

Ischemia
Vasculitis
Intramural Hemorrhage
Crohns: edema (or fat)
Angioedema
Portal Hypertension
NSAIDs Enteritis
ANY ENTERITIS
Chemo, Rad, Infect. etc
Bowel Damage Pathways
Loss of Barrier Integrity

Vascular Barrier
Leak of serum, plasma, cells
Edema
Ischemia
Loss of mucosal barrier
Mucosal Barrier
Inflow of excluded molecules
Edema
Loss of vascular barrier
Vascular compromise
Ischemia
Ischemia CT Mimics
Vascular or Mucosal Barrier Interruption

Ischemia
Vasculitides HSP
Coagulopathies
Bleeders - Purpuras, anticoags
Clotters - P Vera, S,C defic
Angioedema - ACE inhibitors
Regional Inflammation - tic appy itis
Crohns
Infectious Enteritis
Neutropenic Enterocolitis
Mesenteric Ischemia
Diagnosis
Imperative in Acute & Chronic Ischemias
Now earlier Dx by CT - study vessels
Think of it in every abd pain CT.
Physiological understanding is critical
Remember Steals
Surgeons undercall some, be brave, stay bold
We undercall some, explain plums & prunes
References
General References
1. Becker Duodenal necrosis as presenting manifestation of polyarteritis nodosa. Clin Rheumatol. 2002 Aug;
21(4):314-6.
2. Carucci LR, Levine MS, Rubesin SE, Laufer I, Assad S, Herlinger H. Evaluation of pts with jejunostomy tubes:
imaging findings. Radiology. 2002 Apr; 223(1): 241-7.
3. Coskun B. Benign Cutaneous Degos' Disease: case report and review of literature. J Dermatol. 2004
Aug;31(8):666-70
503
Mesenteric Ischemia
4.
5.
6.
DeBacker AI, et al; CT of Angioedema of the Small Bowel, AJR 2001; 176: 649-52
Mirvis SE, et al Diffuse SB ischemia in hypotensive adults after blunt trauma (shock bowel): CT findings &
clinical significance. AJR Am J Roentgenol. 1994 Dec; 163(6):1375-9.
Warshauer DM, Lee JKT, Mauro MA, White GC; Superior Mesenteric Vein Thrombosis w Radiologically Occult
Cause: Retrospective Study of 43 Cases; AJR 2001;177:837-841
Wet vs Dry Ischemia

1. Chou C, CT Manifestations of Bowel Ischemia. AJR2002;178-87
2. Chou C, CT of SB ischemia. Abd Imaging 2004; 29:18-22
Pneumatosis Intestinalis (PI)
1. Hawn MT, et al Serum lactic acid determines outcomes of CT Dx of pneumatosis of GI tract. Am Surg. 2004
Jan;70(1):19-23;
2. Wiesner W, et al . PI and portomesenteric venous gas in intestinal ischemia: correlation of CT with severity of
ischemia and clinical outcome. Am J Roentgenol. 2001 Dec;177(6):1319-23.
J Tube Jejunitis
1. Brenner DW, Schellhammer PF. Mortality assoc w feeding catheter jejunostomy after radical cystectomy. Urology.
1987 Oct;30(4):337-40.
2. Gaddy MC et al. SB ischemia: consequence of feeding jejunostomy? South Med J. 1986 Feb; 79(2):180-2.
3. Jorba R, et al. SB necrosis in assoc w early post-op enteral feeding after pancreatic resection. Surgery. 2000
Jul;128(1):111-2.
4. Lawlor DK, et al SB necrosis assoc w jejunal tube feeding. Can J Surg. 1998 Dec; 41(6):459-62.
5. Munshi IA, et al.SB necrosis assoc with early post-op jejunal tube feeding in a trauma pt.J Trauma. 2000 Jul;
49(1):163-5.
6. Rai J, et al SB necrosis in assoc w jejunostomy tube feedings. Am Surg. 1996 Dec; 62(12):1050-4.
7. Schloerb PR, et al. Bowel necrosis caused by water in jejunal feeding. J Parenter Enteral Nutr. 2004 JanFeb;28(1):27-9.
8. Schunn CD, Daly JM. SB necrosis associated w post-op jejunal tube feeding. J Am Coll Surg. 1995 Apr;
180(4):410-6.
Mesenteric Ischemia
504
Malabsorption
The Radiology of Malabsorption (MAB)
Review
Celiac Disease Sprue in detail
MAB Pattern Barium & CT
Other Diseases of MAB
CT Detection of MAB
Images from a Virtual Colonoscopy

75 F Asymptomatic
Celiac Disease
Vessel cloaking nodes, fluid in pelvic SB loops
1991: Sprue Presentations
Diarrhea
85%
Weight loss
57%
Abd distress
29%
Edema
29%
Bone pain
19%
Tetany
10%
Failure to grow, hematuria, foot drop, hypovolemic shock, each 2%
Trier J, Celiac Sprue NEJM 1991
2005: Sprue Presentations
50% of adult pts present w Fe Defic Anemia

Farrell RJ, Kelly CP. Celiac Sprue.N Engl J Med. 2002 Jan 17;346(3):1808
Occult GI Bleeding (FOBT+)detected in half of pts with Sprue
Fine KD, Prevalence of occult GI bleeding in Celiac Sprue, NEJM 1996
334:1163-7
Back & Leg Pain
-> Primary Care MD ->

Neurologist - back pelvis films ->
Radiologist - Osteomalacia ->
GI series ->
Gastroenterologist: Biopsy: Sprue
The Physiologists MAB
Maldigestion (no enzymes, no mixing)

Biliary - panc insuff, ZE, bacterial overgrowth, SB diverticulosis Luminal
Cellular MAB (Columnar Cell uptake failure)
Sprue, ischemia, villous tip infiltration
Malassimilation (Columnar Cell exit failure)
lymphangiectasia, abetalipoproteinemia, mesenteric diseases Mesenteric
The Radiologists MAB: Malabsorption Pattern
Dilution from XS intraluminal fluid

Acute & Chronic Diseases
Dilatation
Delay
MP to enteric fluid overload, greater chronicity: > Dilatation, Delay
505
Malabsorption
Malabsorption Pattern (MP)
Figure 2-30-1
MP = Dilution + Dilatation + Delay.

Due to chronic enteric fluid overload
Historically, radiologic MAB Pattern = Sprue
Sprue is king of MAB pattern BUT
Other diseases can cause MAB pattern
Not all Sprue pts have MAB pattern
Sprue: Gold Standard Dx
SB Biopsy
Antiendomysial antibody (EMA)
IgA Ab to extracellular reticular fibers
90% sensitive, 98% specific
Tissue Transglutaminase antibody (tTGab)
86% sensitive; 84% specific
AntiGliadin IGA antibody
76% sensitive, 79% specific
Image shows two jejunal biopsies, of similar

magnification, contrasting Celiac Sprue at top with
a normal biopsy at bottom. With Celiac Sprue
there is loss of normal fingerlike villi (arrows) seen
below and Crypt Hyperplasia (compare thickness
of double arrowheads)
Johnston SD, et al A comparison of antibodies to tissue transglutaminase with

conventional serological tests in the diagnosis of coeliac disease. Eur J
Gastroenterol Hepatol. 2003 Sep;15(9):1001-4.
Figure 2-30-2
Villous Atrophy & Crypt Hyperplasia

Normal Villi & Crypts [Figure 2-30-1]
Entero-Enteric Circulation
Crypts secrete fluid into lumen

Villi absorb fluid + nutrients from lumen
Nutrients into portal veins, lymphatics
Crypts recycle fluid back into lumen
Sprue Pathophysiologic Sequence
Mucosal Villous Atrophy + Crypt hypertrophy

Chronic Fluid Overload
Dilatation (SB Muscle exhausted- CGF)
Delay in Transit >
Increases Malabsorption
Bacterial overgrowth
Other Degradations
Sprue 1 Rad findings l [Figure 2-30-2]
Dilution
WET !!
Dilatation
WIDE !!
Delay in transit WAY LATE !!
Segmentation
Folds: normal > nodular > flat
MALABSORPTION PATTERN
Figure 2-30-3
The Malabsorption Pattern is

characterized by Dilution evident with
watery low density of barium (arrow)
caused by fluid mixing with it,
Dilatation evident by wide diameter
(double arrow head) and Delay
evident by a 7 hr marker without any
barium reaching colon
Jejunal Peristalsis
Feathery Fishtails
Ileal Peristalsis
Esophageal
[Figure 2-30-3]
The MAB pattern results in part from loss of normal peristalsis. Long
arrow in A is normal feathery or fish-tail appearance of jejunal
peristalsis. Short arrows in B show normal contractile pattern of
Ileum with parallel folds in tapered segments mimicking esophageal
contraction
Malabsorption
506
Look at the difference in tone: diameter and peristalsis

[Figure 2-30-4]
Sprue 1 Rad Findings ll
Proximal SB mucosal villous atrophy

reversal of jejuno-ileal fold pattern
toothpaste jejunum (moulage, < 4 folds/inch)
jejunization of ileum (increased ileal folds)
flattened bald duodenal mucosa
foamy mucosal pattern mosaic
Intussusceptions, momentary + non-obstructing (loss of wall thickness AND
tone allow for loops to slide in and out.)
Toothpaste Reversal [Figure 2-30-5

Foamy, Thick, Bald [Figure 2-30-6]
The Jejunum looks like Ileum, the Ileum looks like

Jejunum, the Duodenum looks like hell
Figure 2-30-4
The MAB pattern is evident in A with dilution giving

gray watery barium ( short arrow) and dilated
loops(double arrowheads) with minimal peristaltic
events. The bowel looks baggy, flabby, like
chronically stretched tube socks. Contrast A with
a normal SB film in B. Numerous peristaltic events
(arrows) are apparent and there is a state of tonic
contraction allowing for visualization of the
mucosal detail
Figure 2-30-5
Figure 2-30-6
Image A shows a smooth fold-free segment of the

jejunum, called toothpaste or moulage caused
by atrophy of mucosal villi and thickening of the
wall by crypt hyperplasia.
Image B shows a bald jejunum in the LUQ and a
feathery abundant fold pattern in the ileum RLQ.
Chronic increase in the nutrient mix presented to
the ileum because of lack of jejunal absorption
cause compensatory hypertrophy of ileal mucosa,
hence Reversal of Fold Pattern
Image A shows a nodular lacy mucosal pattern

(arrow) in the duodenal bulb. This is due to
atrophy of the mucosa allowing the normal
submucosal glands to become apparent
507
Malabsorption
Mosaic Pattern [Figure 2-30-7]
Figure 2-30-7
Mucosal Atrophy [Figure 2-30-8]
Fissures
Pits
Acid burns thinned mucosa
Acid Burns, Ulcerates, Strictures [Figure 2-30-9]

Ulcer [Figure 2-30-10]
Occult GI Bleeding (FOBT+)detected in half of pts

with Sprue
Fine KD, Prevalence of occult GI bleeding in celiac

sprue, NEJM 1996 334:1163-7
Jejunal Webs [Figure 2-30-11]
Mucosal and fold atrophy may create a lacy

granular or mosaic pattern in jejunum seen only
with double contrast or mucosal detail
compression images
Figure 2-30-8
Figure 2-30-9
Areas of narrowing ( arrows) may be

seen in the proximal jejunum due to
inflammation or scarring
Mucosal atrophy leaves the wall
susceptible to inflammation with
cracks, fissures (arrows A) , ulcers,
and pitting from chronic inflammation
(Arrows B)
Figure 2-30-10
Figure 2-30-11
Ulcerations may heal with stricture formation
Recurrent ulceration and healing may lead to

multiple short segment web-like strictures (arrows)
Malabsorption
508
Classic MAB Pattern [Figure 2-30-12]

Intussusception [Figure 2-30-13]
Figure 2-30-12
CT in Sprue
SB
Fluid filled pelvic SB
Dilated, Non distended SB - BAGGY
Dilution if O = Iodine
Flocculation if O = BA
Intussusceptions
Fragmentation
Colon
Big, Gassy, Wet
Foamy Feces (Stool Whip)
Nodes, incr number
Loss of body fat
Small Spleen
Fatty Liver
Classic Appearance of SB
Intussusception [Figure 2-30-14]
Classic MAB pattern. Dilution is evident with watery

appearing barium (curved arrows). Dilatation is seen
(double arrowheads). Puddles of isolated barium
evident (red dots) which, if large, are called
segmentation, if small, flocculation. Fluid and poor
peristaltic activity in MAB causes segmentation and
flocculation. Image A (Arrowhead): A worm or
threadlike collection of barium is caused by a small
amount of barium settling out in a fluid filled length of
bowel that has not had enough peristalsis to keep the
barium and water mixed
Figure 2-30-13
Figure 2-30-14
Intussusceptions are usually transient with dilated

flaccid thin walled loops of bowel sliding easily into,
and out of, each other. Because the muscle is
weak and stretched out in Celiac Sprue, it cannot
pull the intussusception deeper. With normal
bowel grabbing a lead point, peristaltic muscle
contraction pulls it further and tighter until it is
wedged
CT of Lower abdomen shows classic appearance

of SB intussusception. The classic dotted crescent
of fat(arrow) represents mesenteric fat and
mesenteric vessels pulled in with the
intussuscepting loop
509
Malabsorption
Dilated non-distented SB, Fluid filled distal SB loops, colon

Slow transit of contrast, Fluid filling Colon [Figures 2-30-15 and 2-30-16]
Figure 2-30-15
Figure 2-30-16
Image A shows fluid and gas in the right and left

colon (arrows). Image B show fluid filled mildly
dilated loops of SB in pelvis (arrow). Both show
minimal body fat. The fluid absorbing ability of
colon is preserved in Celiac Sprue. With severe
chronic malabsorption, the volume of fluid
delivered to colon may overwhelm its ability to dry
out wet foamy stool. The colon in severe cases
will be fluid filled and pt will have prominent
diarrhea
Large amount of stool (arrow), lack of body fat in

subcutaneous tissues (curved arrow) and in
peritoneal cavity, and dilated flaccid appearing SB
loops are consistent with MAB. Sprue should be
suggested if the history is appropriate
Figure 2-30-17
Reactive Lymphadenopathy
[Figure 2-30-17]
Nutritional Collapse
Hypoproteinemia
Hypoalbulminemia
Ascites
Vitamin deficiencies
K (Coagulation defects)
Iron Deficiency Anemia
Jejunum absorbs Fe
Slowwww bleeding
Electolyte Disturbances
Tetany
Seizures
Multiple small and moderate sized lymph nodes

are seen in the SB mesentery (arrows)
surrounding mesenteric vessels. They are
reactive lymph nodes chronically stimulated by the
low grade SB autoimmune inflammatory process.
They are notable for number but rarely for size
Sprue = Immune Disease
Lymphatic activity
Reactive mesenteric nodes
Para-aortic LNs
Large cavitating nodes = poor prognosis
Peripheral lymphadenopathy
Splenic atrophy & clinical hyposplenism
Antibody Tests define Autoimmunity
Genetic Disease
Unique histo-compatability complex in 80% of sprue HLA B8 , DR3

(vs 20% of normal population)
Increased prevalence of Sprue in families
10% latent Sprue in 1st order relatives
Malabsorption
510
Allergic Disease
Wheat, rye, barley

Alpha-gliadin component of Gluten
Grass Allergy
Sprue: Immune Sequence
Genetic susceptibility
?Viral exposure immune memory
Gluten + endothelium antigen
Lymphocytes flood villous tips
Antibodies destroy villi
Determinants of Severity
Genetic Dose +
Gluten Dose +
Time +
Other Factors
Adapted from Marsh, Gastroenterology 1992: 102:330-54
Marsh Biopsy Categories
0. Normal (Latent)
1. Intraepithelial lymphocytes increased
2. Infiltration with lymphocytes
3. Early villous atropy
4. Severe villous atrophy and crypt hyperplasia
Marsh M N, Gluten, major histocompatibility complex, and SB. A molecular and

immunobiologic approach to spectrum of gluten sensitivity ('celiac sprue')
Gastroenterology 1992 Jan;102(1):330-54)
Sprue Associated Auto-immunities
Skin
Dermatitis Herpetiformis
Pancreas
Autoimmune Pancreatitis, Macroamylasemia
Kidney
IGA mesangial glomuleronephritis
Insulin dependant diabetes
3 X increase incidence of Sprue
Hair
Alopecia areata
MULTIPLE emerging associated auto-immunities
Dermatitis Herpetiformis
Pruritic papulovesicular lesions

IG A deposits - dermal-epidermal junction
Goes away with gluten restriction
CD in High Risk Patients
General Population
Superfamily DR 3 / 3
1 Relative of Celiac
Trisomy 21, Turners,
Williams Synd
Type 1 Diabetic
Autoimmune Thyroid
1 Relative of Diabetic
IG A Deficiency
0.4%-2% 1%
33 %
4%-10%
5%-10%
4%
3%
2%
2%
511
Malabsorption
Nodules !! In Celiac Sprue ?? [Figure 2-30-18]
Figure 2-30-18
Healing Sprue
Folds slowly return, first

Diameter slowly shrinks
May take 3-4 years
Sprue
Occult Marsh 1
Nl SB exam, Asympt,
bx +, labs +
Intraepithelial lymphocytes
1 relatives of sympt pts: 5%-15 %
Nodular Marsh 2
Sandy Nodules, irritable
Classic MAB pattern Marsh 3, 4
Wet, wide, way late
Non-responsive
Diet errors, misdiagnosis
Recalcitrant 5%-20% - Bact Overgr; Panc Insuffic
Lymphoma
Recalcitrant Sprue
Responsive to initial Rx 8+yrs

Loss of responsiveness
Smoldering symptoms
Thick folds
Ulcerative Jejunitis
High incidence Lymphoma
Recalcitrant Relapsing Sprue
One phase of Sprue is an early

transient phase in which lymphoid
infiltration occurs. This will produce
prominent or even nodular folds in
SB, not typical appearance of Sprue.
The stage is not often radiographed
and it may be a fleeting phase of this
disease
Misty Mesentery
Perivascular Cloaking
Lymphoma in Sprue
Loss of response to gluten restriction.

Rising Ig A, Sepsis.
Increasing lymphadenopathy.
Thickened bowel loops.
Mesenteric Perivascular Cloaking ???
1 yr lymphoma survival - 31 %, 5 yr - 11%
Survival improves every yr, better
CT Dx, Rx
3.4% malignancy in CD (Lymphoma,
CA Esophagus, other)
Figure 2-30-19
Think Physiologically
[Figures 2-30-19 and 2-30-20]
Maldigestion (no enzymes, no mixing)

Biliary - panc insuff, ZE, bacterial
overgrowth, SB diverticulosis
Luminal
Cellular MAB (columnar C uptake
failure)
Sprue, ischemia, villous tip
infiltration
Malassimilation (columnar cell exit
failure)
Lymphangiectasia,
abetalipoproteinemia, mesenteric
diseases Mesenteric
Malabsorption
A patient with bloating, cramps, gas, indigestion, occasional

diarrhea for yrs. CT Scan shows a big gassy colon, fluid filled
loops of pelvic small bowel, and baggy proximal SB. The
diagnosis was made because of these findings and because of
obvious findings in upper slices evident in the first two images
512
Luminal (Lumenal) [Figure 2-30-21]
Figure 2-30-20
Pancreatic Insufficiency
College Freshman, Wt Loss,

Diarrhea
[Figure 2-30-22]
Fluid
Colon Gas
Minimal Fat
Luminal MAB: Shwachman Diamond Syndrome [Figure 2-30-23]
Exocrine Pancreatic Insufficiency

50% outgrow in adolescence
Neutropenia
Chronic Infections
Myeloid Leukemia
Metaphyseal Chondrodysplasia
Dwarfism
Pancreatic calcifications. Pancreatic insufficiency can cause

malabsorption. The patient may have no other symptoms other
than abdominal bloating, discomfort, and diarrrhea
Figure 2-30-21
Figure 2-30-23
The pancreas is absent in this pt with MAB due to

pancreatic insufficiency. Only fat is seen (arrows)
where pancreas should be
Figure 2-30-22
CT of upper abdomen shows no

glandular tissue with fat in the
pancreatic bed (arrows). Pt had been
diagnosed with Shwachman Diamond
previously and had stopped
replacement therapy at the start of
college
A patient with diarrhea shows fluid filled loops of

SB (arrow) in the pelvis indicating MAB and slow
transit
513
Malabsorption
Luminal MAB: ZE Syndrome [Figures 2-30-24 and 2-30-25]
MAB X-Ray pattern 2 to

Increased gastric fluid
Decreased pH
> Enzyme non-activation
> Poor digestion
> Hypermotility
> Edema/hyperemic folds
Clinical MAB variable
Figure 2-30-24
Figure 2-30-25
Patients with ZE will have

radiographic features of MAB. SB
series shows dilated loops (double
headed arrow) with dilution and with
some fragmentation (curved arrows).
The proximal SB and stomach shows
fold thickening due to edema of folds(
arrows) from large volumes of acid
reaching the proximal SB
See dilution, dilatation from ZE syndrome
ZE
Figure 2-30-26
Excess fluid
Thick folds
Hyperemic mucosa
Luminal MAB: Gastric Surgery [Figure 2-30-26]
MAB pattern 2 to
Vagotomy
Loss of pylorus > bolus into SB
> Poorly mixed food
> Lack of acid digestion
> Poor enzyme synchronization
Clinical MAB not common 1yr p Surg
Absorption occurs distally
Pt changes eating patterns
Cellular Villous Dysfunction
Sprue
Cong / Acq Enzyme Deficiencies
Sugar splitting enzymes (Lactase)
Bacterial / Viral Toxins
Crypts Hypersecrete
Capillaries Leak
Enterocytes Malfunction
Lymphatic Congestion
Cellular Poisons - Drugs - Chemo - Rad Rx
Malabsorption
With a Bilroth L, II,

gastrojejunostomy, or other surgery
to increase gastric emptying, the
proximal SB will become dilated due
to surges of fluid and poor
synchronization of the digestive
process. Absorption equilibrates
and occurs in distal SB and pts
usually become asymptomatic as
they modify their dietary habits
514
Malabsorption from Bacterial Overgrowth [Figure 2-30-27]
Motility Diseases, eg Scleroderma, Chronic Narcotics

SB Diverticulosis
Figure 2-30-27
Weight loss and diarrhea [Figure 2-30-28]

Wt Loss, episodic Diarrhea 30 F, MD, Wife of
MD,
2 m in US from India for training [Figure 2-30-29]
Bacterial infections affect absorption in a number
of ways. Invasive bacteria may stun or destroy
absorptive endothelial cells, may impair small
capillary or lymphatic vessel drainage, compete
for nutrients, or degrade critical enzymes
MAB pattern is apparent with dilated loops (double
arrow in A) and diluted barium (arrows A & B).
Transit delay evident image B at 285 minutes.
Numerous diverticula (red dots) shelter bacteria
from peristaltic cleansing, allowing them to
multiply to such a degree that they degrade or
utilize nutrients and enzymes. Note how more
diverticula are apparent in image B, diverticula
may be difficult to assess, hiding amid dilated
loops. Pts with numerous large SB diverticula
may develop Megaloblastic anemia from B12 and
folate deficiency
Figure 2-30-28
Weight loss and diarrhea. There are dozens of large SB

diverticula not apparent. Easily overlooked unless you
very very very carefully scroll. The MAB pattern with
baggy SB loops, fluid levels, foamy feces should make
you think hard and look for subtle causes of MAB
Figure 2-30-29
Marcha MAB Tropical
515
Malabsorption
SB Fluid - Dilution with FLOCCULATION !! [Figure 2-30-30]
Figure 2-30-30
Tropical Sprue
Villous and Crypt Atrophy

Malabsorption
Glossitis, wt loss, diarrhea, skin changes
Folate & B12 deficiency prominent
Rx folate, B12 improves partly
Antibiotic Rx cures
Relapses common in tropics
Westergaard H.Tropical Sprue Curr Treat Options

Gastroenterol. 2004 Feb;7(1):7-11.
Haghighi P, Wolf PL. Tropical Sprue and subclinical
enteropathy.. Crit Rev Clin Lab Sci. 1997 Aug; 34(4):
313-41.
Scleroderma
[Figure 2-30-31]
Scleroderma always Dilated Delayed Dry

Scleroderma WET =
BACTERIAL OVERGROWTH
Marcha MAB Tropical Fluid
Giardiasis - Campylobacter [Figure 2-30-32]
Figure 2-30-31
Chemotherapy Enteritis
MAB: 2 Villus Blockage by
Lymph cells (immune disease, lymphoma, Crohn

Disease)
PMNs (infections)
Eosinophils (eosinophilic gastroenteritis)
Mast cells (mastocytosis)
Macrophages (Whipples)
Amyloid (amyloidosis)
Giardia on CT Req [Figure 2-30-33]
In image A, typical changes of Scleroderma

involving SB are apparent with dilated loops,
hidebound appearance and pseudo-sacculations.
Scleroderma creates a motility disturbance without
affecting absorption, creating a dry pattern without
dilution. However, with severe Scleroderma and
dysmotility, patients may have episodes of
bacterial overgrowth which then produce MAB as
seen in the pt in image B with dilution apparent
Villus Dysfunction
Engorged Veins & Lymphatics

Blocked Arteries (ischemia)
Paraplegic with diarrhea
MAB pattern
Villous atrophy on Bx
No response to gluten restricted diet
Physical Exam -> NO LEG PULSES
Figure 2-30-32
Arterial Insufficiency [Figure 2-30-34]

Lymphangiectasia [Figure 2-30-35]
Malabsorption
Radiologists may still be first on scene in pts with

Sprue & MAB diseases
MAB major radiographic pattern
Fluid is the hallmark of MAB pattern
Increased production +/or
Decreased absorption
Image A shows MAB associated with Giardiasis.

Image B is a patient with Mab from Campylobacter
Malabsorption
516
Figure 2-30-33
Figure 2-30-34
The oral contrast type determines the appearance of the fluid

filled loops of pelvic SB. If barium is given, flocculation may be
seen. If water soluble oral contrast is given, dilution may be
seen. Acute and subacute infectious diseases may cause SB
fluid to increase but may not be chronic enough to cause
distention and delay. This patient has dilute water soluble oral
contrast in the colon. Clips from a lymph node biopsy are
present
Figure 2-30-35
Arterial insufficiency may

cause dysfunction of all cells
of the small bowel creating
dysmotility and malabsorption.
Note dilatation (double arrow)
and dilution (curved arrow).
Usually these bowel
symptoms are accompanied
by abdominal pain leading to a
correct diagnosis. This
paraplegic patient had an
element of sensory
denervation which caused the
diagnosis of aortic thrombosis
(image B arrow) to be missed
initially
Lymphangiectasia may cause degrees of clinical

and radiographic MAB due to engorged
lymphatics and mucosal edema which will impede
absorption. Usually folds remain prominent
517
Malabsorption
Sprue is King of Rad MAB
Nodular Phase
Rarely seen, ? short phase
MAB Phase
Commonest for Radiology, Classic
Recalcitrant
Dietary indiscretions, edema, nodes, MALIG Bacterial Overgrowth,
Pancreatic Insuffic
Lymphoma
Nodes, weight loss, loss of gluten response
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Farrell RJ, Kelly CP. Celiac Sprue. N Engl J Med. 2002 Jan 17;346(3):180-8
Fine KD, Prevalence of occult GI bleeding in Celiac Sprue. NEJM. 1996 334: 1163-7
Haghighi P, Wolf PL. Tropical Sprue and subclinical enteropathy. Crit Rev Clin Lab Sci. 1997 Aug; 34(4): 313-41.
Johnston SD et al. A comparison of antibodies to tissue transglutaminase with conventional serological tests in the
diagnosis of coeliac disease. Eur J Gastroenterol Hepatol. 2003 Sep; 15 (9): 1001-4.
Jones B, Bayless TM, Hamilton SR, Yardley JH. "Bubbly" duodenal bulb in celiac disease: radiologic-pathologic
correlation. Am J Roentgenol. 1984 Jan; 142(1): 119-22
Lomoschitz F et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol.
2003 Apr;13(4):890-6.
Lomoschitz F et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol.
2003 Apr;13(4):890-6.*
Marsh MN. Gluten, major histocompatibility complex, and small intestine. A molecular and immunobiologic approach
to spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology 1992 Jan;102(1):330-54)*
Marsh M N, Gluten, major histocompatibility complex, and the small intestine. A molecular and immunobiologic
approach to the spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology 1992 Jan;102(1):330-54)
Rubesin SE, Herlinger H, Furth EE." Bubbly" duodenal bulb in clinically unsuspected or refractory adult celiac
disease. Abdom Imaging. 1998 Jul-Aug; 23 (4): 449-52.
Schweiger GD, Murray JA. Postbulbar duodenal ulceration and stenosis associated with celiac disease. Abdom
Imaging. 1998 Jul-Aug; 23(4):347-9.
Tomei E et al. CT of SB in adult celiac disease: jejunoileal fold pattern reversal. Eur Radiol. 2000; 10(1):119-22.*
Trier J. Celiac Sprue. NEJM. 1991
Westergaard H. Tropical Sprue. Curr Treat Options Gastroenterol. 2004 Feb; 7(1): 7-11.
Malabsorption
518
Things that go bump in the bowel:

Familial Polyposis and Other Such
54 y/o M, Vomiting
2003: Vomiting 2-3 hrs p meals

Liquid diet wks
30 lb wt loss - 4 mos
Mother deceased, colon CA.
1996 Proctocolectomy, end-ileostomy
2001 Laparotomy, gastrotomy: 3 big gastric polyps removed
Figure 2-31-1
2 yrs earlier
Endoscopy
Many gastric polyps, Large obstructing duodenal

polyp(s)
Gastrectomy:
Stomach: inflamm, mucosal papillary
hyperplasia with atypia.
Duodenum : Hyperplastic mucosa, foci suggestive of
adenomatous change
FAP
Usually:
Dx known clinically ~75%
Colonoscopic Dx, not BE, not CT
Not Dx challenge except:
~25% de novo mutation
Professors quiz
Differential important:
More things look like FAP than are FAP
Only a small percentage of Colorectal Cancers are

related to FAP
Figure 2-31-2
Colorectal Cancer CRC Perspective

[Figure 2-31-1]
150,000 new CR CA / yr,
only ~ 1% = FAP
FAP = Colonic Polyposis [Figure 2-31-2]
Onset of polyps
Age 10 -- 15%
Age 20 -- 75%
Age 30 -- 90%
Risk of CA nearly 100% by age 40 yrs
FAP Genotypes & Phenotypes
VARIABILITY IN:
Age onset
Polyp #: 100 - 1000s
Bone, Eye, Skin, Brain, Thyroid, Desmoid
APC is BIG gene, 300+ variable mutations
Gardners Syndrome
Turcotte, etc
Early non-colonic changes may help early Dx
There are many components possible in the

patient with Familial Adenomatous Polyposis
519
Familial Polyposis
Uncountable
Figure 2-31-3
Barely Countable
Countable, Pedunculated
Variable size [Figure 2-31-3]
1,000s of Small Early Polyps
FAP Colon Polyps = Adenomas
No matter what size
100s of Bigger Older Polyps
1,000s of Small Polyps
FAP UGI Polyps [Figure 2-31-4]
Fundal gland hyperplastic polyps

100s of Bigger Older Polyps
Adenomatous change in hyperplastic
polyps in 100% of FAP pts. eventually.
5%-8% develop duodenal or ampullary CA, stomach, SB
LIFETIME ENDO SURVEILL

Fundal refers to normal location of nl type of gland
Polyp number, size, and age of

appearance vary in FAP
patients
Figure 2-31-4
FAP UGI
Start Flat
Fundal first
If you look you will find them.
Follow colon polyps, occ precede.

Duodenal: Hyperplastic vs Adenoma vs CA ?
Lifetime Surveillance Needed
Figure 2-31-5
[Figure 2-31-5]
While they
occur first
and most
prominently
in the
gastric fundus, the term Fundal
Gland refers to the histologic
characteristics of the polyp.
Fundal Gland hyperplastic
polyps in FAP can occur
throughout the stomach and
duodenum
While they start as hyperplastic polyps, they may

undergo adenomatous change and then may
undergo malignant degeneration. We cannot
differentiate the three types of polyps:
Hyperplastic, Adenoma, Adenocarcinoma.
Lifetime endoscopy is required
Familial Polyposis
520
FAP Duodenal AdenoCa [Figure 2-31-6]
Figure 2-31-6
FAP Ampullary Tumor (Clue: Stoma)

[Figure 2-31-7]
Figure 2-31-7
Obvious Duodenal Adenocarcinoma
Figure 2-31-8
Ampullary tumor and stoma: a link worthy of being

an Aunt Minnie
FAP Duo - Ampulla [Figure 2-31-8]
1. Cystic Glandular Hyperplasia

2. Adenomas
3. Carcinoma
Jaundice, abn LFTs
Pancreatitis
FAP - 33 yr p Colectomy [Figure 2-31-9]

Relative Risk: Upper GI CA in FAP
Site
No of Carcinoma
Duodenum
7
Ampulla
4
Gastric
2
Non-duodenal
1
F/u 1391 pts
Rel Risk
331
124
2.4
12.7
18,679 pt - yrs
Offerhaus GJA, et al. Gastroenterology 1992; 102:1980
Osteomas [Figure 2-31-10]
Ampullary Polyp and absent colon.

Another linkup creating an almost
Aunt Minnie
Figure 2-31-9
Sinuses
Mandible
Anywhere
Osteomas Benign Bone Islands
Note absent colon gas
Mandibular Osteomas
Exostotic Osteomas [Figure 2-31-11]
Diffuse Cortical Thickening
Cortical Endo & Exos [Figure 2-31-12]
Ileostomy and SB tumor. With history of remote
total colectomy, another almost Aunt Minnie.
Without history, a great differential
521
Familial Polyposis
Figure 2-31-10
Figure 2-31-11
Osteomas growing out from the angle of the

mandible, a favorite place for these rare
manifestations to occur in FAP
Figure 2-31-12
Osteomas may be innie or

outie osteomas
Figure 2-31-13
Osteomas of paranasal sinuses
FAP Dental ~80% [Figure 2-31-13]
Fused roots 1st & 2nd molars

Missing
Impacted
Supernumerary
Long tapered roots post teeth
Mal-erupted
Carl, W. Herrera, L. Dental and bone abnormalities in patients with

familial polyposis coli; Semin Surg Oncol 73-83, 1987
Dental anomalies, mild, moderate, or

severe, occur in FAP
CHRPE: Congenital Hypertrophy of Retinal Pigment

Epithelium
Evident in subset of FAP

Screening marker for subset of certain families
~Earliest clinical marker
FAP Skin
Sebaceous Cysts
Pigment Changes
Desmoids
Rare benign tumors,

Never metastasize, invade locally,
FAP associated, occ spontaneous
In FAP pts:
Familial Polyposis
522
Figure 2-31-14
50% abd wall (surg excision)

50% intra-abdom,
85%-100% are mesenteric:
-SBO or ischemia,
-Hydronephrosis.
Rx: NSAID in comb. w tamoxifen
Knudsen AL, Bulow S. Desmoid tumour in familial adenomatous polyposis. A
review of literature. Fam Cancer. 2001;1(2):113-21
Desmoid Radiology [Figure 2-31-14]
1. Desmoid Tumor
Geometric: muscle or intraperitoneum
2. Mesenteric Fibromatosis
Infiltration: mesentery, retroperitoneum
Both can occur in same pt
Desmoid
NOTE: Colectomy, Hazy Dense Mesentery
Desmoid tumors may be

geometric or infiltrative
NON FAP DESMOID: TUMORAL & INFILTRATIVE with

Ischemia
Infiltrative / Tumoral
FAP: Causes of death
Cause
Desmoid tumor
Periampullary cancer
Trauma/accident
Perioperative death
Rectal cancer
Other
Number
11 (31%)
8 (22%)
3 ( 8%)
3 ( 8%)
3 ( 8%)
8 (22%)
Yrs after Colectomy

7
23
5
11
13
11
Mean age
35
49
31
37
42
50
Figure 2-31-15
DCR 1990, 33:639
Peritoneal Inclusion Cysts: PIC [Figure 2-31-15]
F, usually premenopausal
Prior proctocolectomy
(Endometriosis, PID)
Large pelvic cyst(s), multilocular
Ovary trapped in pelvic loculation.
Cysts lined by mesothelial cells.
May see ovary on wall
Peritoneal Inclusion Cyst [Figure 2-31-16]
Entrapped Ovary Syndrome

Ovary secretes more fluid than can be absorbed by peritoneal
locule.
Mimic Ovarian Cystadenoma or CA
Hx important.
Rx Cycle suppression
PIC [Figure 2-31-17]

FAP Mimics
Numerically, more things resemble FAP than the cases of FAP that
we see.
More common entities mimic classic polyposis syndromes
Peritoneal Inclusion Cysts are a
complication produced by pelvic
peritoneal adhesions in
premenopausal women.
523
Familial Polyposis
Classification of Hereditary GI
Polyposis Syndromes (Gene)
Figure 2-31-16
Familial adenomatous polyposes

Adenomatous polyposis coli (APC)
incl: Gardner, Turcot, Attenuated (APC)
Hamartomatous polyposes
Peutz Jeghers syndrome 1/8 Less common
Familial juvenile polyposis rarer still
Cowdens disease
Intestinal ganglioneuromatosis
Ruvalcaba-Myrhe-Smith syndrome
Tuberous sclerosis
Classification of Hereditary GI
Polyposis Syndromes (Gene)
Familial adenomatous polyposes

Born Adenomas -> Malignant transformation
Hamartomatous polyposes HPs
Born Hamartomas of varying types
Some undergo epithelial atypia from overwork
Some adenomas may develop, at risk CA
Bowel CA can occur in all HPs
More than general population
Far less than FAP
Non-Hereditary GI Polyposes
Inflammatory and post inflammatory

CUC, Crohns, Infectious colitides
Lymphoid
Reactive nodular lymphoid hyperplasia
Lymphoma
Pneumatosis cystoides intestinalis
Lipomatosis
Angiomatosis
Leiomyomatosis
Cronkhite-Canada syndrome
Peritoneal Inclusions Cyst (s) progressively

enlarge unless cycle suppression therapy is
instituted. Reoccurrence may occur after lysis of
adhesions
Figure 2-31-17
Peutz Jeghers [Figure 2-31-18]
3 SB Hamartomas
Figure 2-31-18
Ovary is evident in the wall of the PIC. A cystic

lesion with septations and a mural nodule may
simulate ovarian carcinoma
Peutz Jeghers with three large polyps in SB.

Polyps may be large, medium, small,
endoscopically visible, or so teeny weeny to be
visible only by microscopic biopsy. All sizes
may be encountered in the same patient
Familial Polyposis
524
Peutz Jeghers [Figure 2-31-19]
Figure 2-31-19
Episodic Pain due to intussusceptions
Peutz Jeghers
One 5 X 8 mm, many 1-2 mm polyps
Peutz-Jeghers [Figure 2-31-20]

Cowdens Syndrome [Figure 2-31-21]
Autosomal Dominant - rare

Multiple Hamartoma Synd.
Age onset: 1st to 3rd decade
Hamartomatous GI polyps,
Non GI manifestations.
CA, Breast~ 50% of F
Thyroid
Colon
Peutz-Jeghers pts may

have painful episodes
due to intussusception
requiring surgery
Figure 2-31-20
Cowdens Syndrome
Papillomas / fibromas mucosa, tongue, (cobblestone

appearance)
Acral keratosis
Tricholemoma of face
Also: angiomas, lipomas, skin vitiligo
Juvenile Polyposis Syndrome [Figure 2-31-22]
The polyps are juvenile hamartomas

Syndrome can be adult presentation.
Anywhere
Peutz-Jeghers hamartomatous polyps in the

stomach and duodenum
Figure 2-31-21
Figure 2-31-22
Cowdens Syndrome
Gastric Juvenile Polyposis refers to the type of
polyp defined by microscopy, not the age of
presentation
525
Familial Polyposis
SB Juvenile Polyps [Figure 2-31-23]
Figure 2-31-23
Ruvalcaba-Myhre-Smith
Bannayan-Riley-Ruvalcaba syndrome (BRRS)

Riley - Smith syndrome
Bannayan - Zonana syndrome
R M S is best
Macrocephaly hamartoma papilledema syn
RMS [Figure 2-31-24]

RMS Syndrome
Rare2, autosomal dominant ? (80% male)

Men more common
Ileal & Colonic polyps in 45%
Other:
Colonic tumors
GI tumor/polyp/hemangioma
Lobulated tongue (including hamartomas)
Skin
Abnormal genital pigmentation
Acanthosis nigricans
Cafe au lait spots
Nevi or lentigines
Lipomata
Capillary hemangioma
Other tumors of skin
Supernumerary nipples
Bones
Delayed bone age
Asymmetric limbs
Joint laxity
Muscle weakness/myopathy
Brain
Tumors/cysts
Intra-cranial calcification
Vascular malformations of brain
Macrocephaly
Papilledema
Eye
Anterior chamber abnormalities,
Visible nerve fibers on cornea
Palpebral fissures slant down
High birth wt (> 90th %centile)
Large Juvenile Polyps in the SB
Figure 2-31-24
Ruvalcabre Myhre Smith in SB and Colon
Figure 2-31-25
Cronkite-Canada syndrome [Figure 2-31-25]
Hyperpigmentation
Alopecia
Glossitis
Dystrophic nails
GI manifestations
Harmatomatous polyps
Exocrine pancreatic insufficiency)
Diarrhoea
Pancreatic insufficiency
Protein losing enteropathy, low protein
Familial Polyposis
Cronkhite-Canada Syndrome
526
CCS [Figure 2-31-26]
Figure 2-31-26
Lymphoma
[Figure s 2-31-27 and 2-31-28]
Nodularity one form of

Lymphoma.
Remember L is a PoLyposis+
Nodular Lymphoid
Hyperplasia Colon [Figure 2-31-29]
Can be:
Related to GI infection
Assoc w
hypogammaglobulinem
Assoc w Giardia
Diff to histo diff from
Lymphoma
TI most frequent
Anywhere in GI tract
Cronkhite Canada Syndrome
Figure 2-31-27
Figure 2-31-28
Lymphoma probably produces diffuse

polyposis of the colon, and other
organs, more frequently than does
any one of the more famous Polyp
Syndromes. Remember Lymphoma
!!!!! In your differential for polyposis
cases
Diffuse Colonic Polyposis. One of the most frequent

causes of diffuse polyposis is not due to a syndrome
Nodular Lymphoid
Hyperplasia
Small Bowel [Figure 2-31-30]
Figure 2-31-29
Figure 2-31-30
Radiology
Uniform, 1-4 mm
May be umbilicated
Diffuse
Regional
Clustered -patches
Nodular Lymphoid
Hyperplasia
Colon
Radiology
Uniform, 1-4 mm
May be umbilicated
Diffuse
Regional
Clustered
NLH of SB
527

may mimic a polyposis syndrome
or Lymphoma
Familial Polyposis
Mastocytosis [Figure 2-31-31]
Figure 2-31-31
Secret:
Small nodules differential:
Think:
White Blood Cell Differential:
PMN
Lymph
Eo
Mast
Macroph
Hyperplastic Polyps CUC

[Figure 2-31-32]
Filiform Polyposis [Figure 2-31-33]
Crohns
CUC
CUC
Figure 2-31-32
NLH of SB Nodules in the SB are usually caused by cellular

infiltrates. To ease your differential brain pain, think of a WBC
smear and all the types of WBCs seen. So nodularity in the SB
from ordinary PMNs caused by infections, acute or chronic eg Whipples Lymph cells: Lymphoma, Nodular Lymphoid
Hyperplasia, Lymph Cell Granulomas of Crohns or TBC,
Immunoproliferative Small Intestinal Disease (IPSID)
Eosinophils: Eosinophilic Gastroenteritis
Mast Cells: Mastocystosis
Macrophages: Chronic infections: IPSID, Tropical Sprue,
Amyloid, Whipples
Figure 2-31-33
Polyps in CUC may be due to islands

of residual mucosa persisting after
slough of diseased mucosa or may
due to inflammatory polyps
developing as the mucosa attempts
regeneration. Note ahaustral colon
Giant Hyperplastic Polyposis
Hyperplastic polyps often have random shapes including

wormlike or filiform polyps
[Figure 2-31-34]
Seen in
CUC
Crohns
Infect. Colitis
Figure 2-31-34
Hyperplastic polyps may be massive. Imagine polyps so long, so numerous that

they fill the colon like hamburger stuffing the colon like a sausage. Any colitis that
sloughs the mucosa may create localized of diffuse inflammatory polyposis. Each
polyp weeps serum creating hypoproteinemia. Pts may have inactive colitis and
present with CHF, peripheral edema, or anasarca
Familial Polyposis
528
All Granulomatous Disease may give bumps !!
Figure 2-31-35
Granulomatous change in .
Crohns Stomach & Colon

Crohns Stomach [Figure 2-31-35]
Hyperplastic Polyps [Figure 2-31-36]
Benign
Assoc w Atrophic Gastritis
Atrophic Gastritis assoc w Gastric CA
HP - AG - CA !!
? Assoc w Acid Suppression therapy:
Purple Pill Polyps
Crohns of the Stomach!!
Declich P, et.al. Fundic gland polyps under omeprazole treatment. Am J Clin

Pathol. 1999
Carcinoid Hyperplasia [Figure 2-31-37]
IN:
Atrophic Gastritis
Zollinger-Ellison
2 to:
Absent Acid
Acid Suppression
Elevated Gastrin
Cause:
Carcinoid gland stimulation
Figure 2-31-36
Solcia E et al Morphology & pathogenesis of endocrine hyperplasias,

precarcinoid lesions, & carcinoids arising in chronic atrophic gastritis.
Scand J Gastroenterol Suppl. 1991;180:146-59
Figure 2-31-37
Hyperplastic polyps are the most

common polyp of stomach. 1 to 2 cm
size, PLUS multiple, PLUS mucosal,
PLUS fundal and body location,
PLUS approximately equal in size
add up to allow statistical call of
benign hyperplastic polypsl They are
in themselves truly benign but are
associated with degrees of atrophic
gastritis which has risk for developing
cancer. I call these the purple pill
polyps because of suggested
association with Acid Suppressive
therapies. In your lifetime the
argument will be settled
Carcinoid Glandular Hyperplasia
529
Familial Polyposis
Pneumatosis Cystoides Coli [Figure 2-31-38]
Figure 2-31-38
Familial Polyposis
Protean manifestations
Multiple mimics
Worthy of further study:
For itself
As starting point for Bumps of the
Bowel
Pneumatosis Cystoides Coli may fool the unwary
References
1.
2.
3.
Carl, W. Herrera, L. Dental and bone abnormalities in patients with familial polyposis coli; Semin Surg Oncol
73-83, 1987
Knudsen AL, Bulow S. Desmoid tumour in familial adenomatous polyposis. A review of literature. Fam Cancer.
2001;1(2):113-21.
Offerhaus GJA et al. Gastroenterology 1992;102:1980
Familial Polyposis
530
The Spleen
Embryology
Formed from the mesenchymal cells between the layers of the dorsal
mesentery, which lies between the stomach and pancreas
Rotates to the left pulling the mesentery with it and forming the lesser sac
between the stomach and pancreas. Left side of the dorsal mesentery fuses
with the parietal peritoneum covering the left kidney and adrenal to form
Gerotas fascia. This fusion brings the splenic vessels and the pancreas into
the retroperitoneum.
Hilum of the spleen is retroperitoneal, while most of the spleen is
intraperitoneal, with a bare area similar to that of the liver, along posterior
surface adjacent to the left kidney
Anatomy
Crescent shaped, convex toward the diaphragm and concave medially, located
in the LUQ
Bounded by ribs, stomach, left kidney and splenic flexure of the colon.
Splenic hilum contains splenic vessels and tail of pancreas (retroperitoneal)
Splenic artery-tortuous, often containing aneursyms
Splenic vein-straight. Confluence with SMV forms the portal vein. Splenic
vein often enlarges with splenomegaly. Upper normal is 1.5cm
Important Connections
Splenorenal ligament (retroperitoneal)

Connects the splenic hilum to the left kidney
Contains the pancreatic tail, and splenic artery and vein
Gastrosplenic ligament (peritoneal)
Peritoneal fusion of the lesser and greater sacs, connecting the splenic
hilum to the stomach
Phrenicocolic ligament (peritoneal)
Connects the lower pole of the spleen to the splenic flexure of the colon
and to the diaphragm
Histopathology
Stroma supports functional red and white pulp

White pulp: the functional cells of the spleen; lymphocytes, plasma cells and
macrophages.
Red pulp: surrounds white pulp and is comprised of arteries and sinuses filled
with blood. Also contains chords which slowly filter the blood, removing aging
cells
Splenic Function
Adult: filtration of aged rbcs, sequesters platelets, removes foreign particles

with macrophages.
Childhood: adult functions plus production of lymphocytes and monocytes.
Fetal hematopoesis
Maintains immunity against bacterial pathogens (streptococcus D)
Normal Size
Long axis from diaphragm to inferior pole

Usual size (US and CT and MRI) length 12cm, width 7cm and thickness 34cm. (you can allow greater length if the spleen is thinner)
Spleen may be horizontal or longitudinal in orientation
531
The Spleen
Normal Appearance (CT) [Figure 2-32-1]
Figure 2-32-1
Normally less dense than liver by 15 HU precontrast, if greater than liver, then liver is fatty, or
spleen contains iron. Normal is 40-60 HU.
Arterial phase imaging (30 sec p/injection)
Heterogenous enhancement with a serpentine,
zebra-like pattern.
Portal phase imaging (70 sec p/injection)
Homogenous enhancement of 100-150 HU,
generally 25 HU greater than the liver. If more
than that, then liver is fatty
Normal Spleen. Normal arterial phase (left) and

portal venous phase (right) CT images of the
Measures 12 cm cranial-caudal
spleen. Note the striped pattern on the arterial
Normally does not extend beyond the left kidney
phase at 30 seconds post injection has become
Homogeneous mid-level echoes, slightly greater than
uniform by the 70 second delay portal scan
liver and equal to or greater than left kidney.
Image in longitudinal and transverse planes, often easier to see in expiration
Normal Spleen (US) [Figure 2-32-2]
Vos PM, Mathieson JR, Cooperberg PL, The Spleen: in Diagnostic Ultrasound;
Rumack CM, Wilson SR, and Charbonneau JW eds. .Elsevier Mosby 2005, St
Louis, pp 147-170
Figure 2-32-3
Figure 2-32-2
Normal US. Longitudinal image between the ribs

(left) and transverse image (right) shows the
uniform medium level echoes of the spleen with
the concave hilum containing the anechoic splenic
vessels in the pancreatic tail
Normal signal intensity of the spleen on T1 (top

left) T2 fat suppressed (lower left), Early arterial
T1 Gadolinium enhanced (top right) and portal
phase Gadolinium T1 enhanced (lower right) MRI
Normal MR Appearance [Figure 2-32-3]
scans. The early arterial phase in MRI shows
T1-low signal intensity, similar to muscle, lower than
heterogeneous enhancement which becomes
liver.
uniform in the portal phase
T2 - high signal intensity, greater than liver.
Gradient echo: in and out of phase
Figure 2-32-4
Post contrast appearance: Arciform with serpiginous bands of low signal
intensity separating larger regions of intense
enhancement, similar to that seen on CT
Normal Variants
The spleen is formed from multiple cell aggregates

which coalesce.
This gives rise to:
Accessory spleens
Clefts
Splenosis
Splenic rests
Accessory Spleens [Figure 2-32-4]
30%-40% incidence in normal population and 10% of

patients have >1 focus
Frequently (75%) in splenic hilum
The Spleen
532
Normal accessory spleen

Left: normal spleen in left upper quadrant
Right: Isoattenuating 2cm enhancing nodule
lateral to the left kidney and adjacent to the splenic
flexure is a normal accessory spleen
Figure 2-32-5
Remainder usually adjacent to splenic poles or along

splenic artery or in pancreatic tail
Size ranges from microscopic to 2-3cm. May
hypertrophy following splenectomy up to 5cm.
Importance is not to mistake for other soft tissue
pathology, usually lymphadenopathy
Accessory Spleen: Appearance [Figure 2-32-5]
Isoattenating to spleen on CT, isointense on MRI,

and isoechoic on US.
Ultimate diagnostic test is Tc99mSC nuclear
medicine scan (macrophages take up
radiopharmaceutical, differentiating accessory
spleen from lymphatic tissue)
Spleen Cleft [Figure 2-32-6]
These are the residual spaces between partially

fused lobules.
These are sharp and well defined. They may be as
deep as 2-3cm
Most commonly they occur along the lateral margin
of the spleen and on the superior diaphragmatic
portion.
They may mimic splenic lacerations
Enlarged spleen with accessory spleen (arrows)

on US (top left), CT (lower left) and MRI T1
weighted ( right top), T2 weighted ( right middle)
and Gadolinium enhanced (right lower) images.
The image characteristics of the accessory spleen
match those of the adjacent prinicipal splenic
tissue on all imaging modalities
Splenosis [Figures 2-32-7 and 2-32-8]
Residual splenic tissue following splenectomy. Fairly large (up to 5cm

Figure 2-32-6
diameter) multiple nodules may occur, but typically small, multiple and
enhancing.
Location: most frequently in the LUQ,
but may be anywhere in the abdomen
along the peritoneal surfaces and/or
mesentery.
May involve the diapharagm and pleura
DDX: endometriosis, mesothelioma
Dx with Tc99mSC or RBC study
Splenic clefts. Left: fine shallow clefts are noted at the superior
and posterior margins of the spleen adjacent to the diaphragm.
Note the lack of any fluid, and the sharp margins. The superior
cleft extends from the medial to the lateral surface.
Center: a deeper cleft in the posterior spleen, adjacent to the
diaphragm.
Right: An unmistakable cleft at the junction of 2 lobules, not a
splenic tumor. Note the normal enhancement similar to the rest
of the spleen
Figure 2-32-7
Splenosis. Left: multiple splenules in the left upper quadrant

after splenectomy. Middle CT show splenules adjacent to the
gallbladder and in the hepatorenal fossa (right) as well as in
the left splenic and renal fossae
533
The Spleen
Splenic Gonadal Fusion
Splenic tissue contained within epididymis, spermatic

cord, or testis
Male: female ratio is 17:1
Mimics tumor
Believed to arise from adhesion between the
gonadal primordial tissue and the spleen prior to
gonadal descent.
A fibrous band between gonad and spleen contains
additional splenules in 50% and is associated with
other congenital anomalies (cardiac or limb defects,
hernias, undescended testes, micrognathia)
Unconnected gonadal rests not associated with other
anomalies
Figure 2-32-8
Warshaer DM. Spleen; in Computed Body Tomography

with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and
Heiken JP, eds. 4th Edition, Lippincott Williams and
Wilkins, Philadelphia, 2006: 973-1006
Splenosis.Top left: 2 cm enhancing round mass

anterior to the aortic arch. Lower left: Round soft
Polysplenia
tissue mass posterior to the left atrium. Top right:
Bilateral left-sidedness with multiple other organ
Bowel fills the left upper quadrant in the splenic
system manifestations
fossa. Lower right: Technesium 99m sulfur colloid
ie 2 left lungs or left sided azygous or interrupted
study shows the normal liver, and enhancing
IVC, biliary atresia, absent gallbladder, GI
nodules in the chest at the level of the aortic arch
malrotation
and the heart
Associated with cardiac abnormalities including
VSD, ASD, right sided arch, partial anomalous pulmonary venous return
(PAPVR), transposition of the great vessels
Asplenia
Bilateral right-sidedness
2 right lungs in 2/3
Midline liver
More complex cardiac anomalies including single AV valve, pulmonary
stenosis or atresia, TAPVR, transposition of the great vessels, ASD, single
ventricle
Mortality is as high as 80% in first year.
Impaired immune response due to asplenia
May present with serious bacterial infections
Wandering Spleen
Long mesentery if dorsal mesentery fails to fuse with the posterior peritoneum.
Diagnosis made by US, CT or MR showing classic splenic tissue in abnormal
location
May torse and lead to acute or chronic abdominal pain. Lack of enhancement
is present in complete infarction.
Chronic torsion may lead to hypersplenism, splenomegaly, or gastric varices
Enlarged Spleen
Moderately large
Portal hypertension most common (check for cirrhosis and collaterals)
Anemia
Infection
AIDS
Very large (17 cm or more)
Leukemia or lymphoma
Infectious mononucleosis
Myelofibrosis
Portal hypertension
The Spleen
534
Splenomegaly [Figure 2-32-9]
Figure 2-32-9
Splenomegaly with Spontaneous

Rupture
81 yo female with polycythemia

presented acutely with abdominal pain.
Note massively enlarged spleen with
anterior disruptions and
hemoperitoneum.
Splenomegaly. Right: transverse US shows an enlarged spleen
anterior to the kidney. Center and right: massive splenomegaly
Benign Focal Lesions
in a patient with portal hypertension
Cysts
Hemangiomas
Figure 2-32-10
Granulomas
Abscesses
Infarcts
Trauma
Simple Splenic Cysts [Figure 2-32-10]
Most commonly post traumatic in origin and lack an

epithelial lining, thus are really pseudocysts.
Appearance:
US: anechoic with imperceptible wall and
posterior acoustic enhancement.
MR: low on T1, bright on T2
CT: water attenuation without enhancement
Splenic cysts may rarely contain debris (cholesterol
crystals, post trauma)
No enhancement on CT or MR after contrast
Epidermoid Cysts
Splenic Cyst by CT (left) is often calcified post

trauma. Post-traumatic splenic cyst in a different
patient. Sagittal ultrasound (right) shows a largely
anechoic mass with some near field echoes but a
sharp back wall, and posterior acoustic
enhancement. This one also has some rim
calcifications
True congenital lesions discovered incidentally

Often with trabeculations or septations and occasional peripheral calcification
Other Cysts
Figure 2-32-11
Echinococcal cysts
Extensive wall calcification
Pancreatic pseudocysts may arise within the spleen.
Check for accompanying features of pancreatitis.
Pseudocysts may contain debris or hemorrhage
Abscesses may mimic cysts by US, but on CT or MR
should have an enhancing rim. On US they may
contain gas or debris, differentiating them from
Echinococcal cyst. Left: CT shows a heterogenous
simple cysts
cystic and solid lesion replacing the splenic tissue.
The lesion contains central soft tissue and a
Echinococcal Cyst [Figure 2-32-11]
spoke-wheel appearance of multiple cysts.
38-year-old Russian woman with persistent left flank
Right:
Gross pathology shows multiple cysts in an
pain
encapsulated mass within the spleen
Pancreatic Pseudocyst [Figure 2-32-12]
Figure 2-32-12
36 yo female with 3 days of LUQ and flank
pain. Cysts occupy the spleen (left and
center) and the gastric wall and supcapsular
regions of the spleen (right). Surgery
revealed multiple pseudocysts. The
pseudocyst in the gastric wall is a clue to
the origin of the cysts
535
The Spleen
Hemangioma [Figures 2-32-13 and 2-32-14]
Figure 2-32-13
Most common benign splenic neoplasm

Echogenic on ultrasound
Low signal on T1 and high on T2 (MRI)
Low attenuation with early phase enhancement on MR or CT,
often lacking the nodules and centripetal fill-in seen with liver
hemangiomas, especially if <2cm
Other Benign Lesions
Lymphangiomas-cystic with septations, may be septated and/or

calcified
Hamartomas-normal splenic tissue, predominantly red pulp,
single or multiple, variable size (1-15cm)-slow and prolonged
enhancement noted.
Littoral Cell angioma-vascular tumor unique to spleen, multiple
lesions of low attenuation on CT .2-9cm in size
Sagittal US shows an echogenic well

circumscribed mass on this
assymptomatic patient presenting for
renal ultrasound
Malignant Lesions
Figure 2-32-14
Lymphoma
Metastases
Angiosarcoma
Lymphoma
Most common splenic malignancy

Rarely as isolated lesion, usually as part
of diffuse disease
Low grade lymphomas usually diffuse
enlargement
Splenic hemangiomas. Left: early phase dynamic CT shows
Hodgkins and higher grade NHL cause
markedly
enhancing smooth round nodules. Right: Delayed CT
discrete low attenuation/echogenicity
image
at
the
same level shows delayed washout, characteristic
nodules.
of
a
benign
lesion. On CT splenic hemangiomas often show
Accuracy of CT prediction of splenic
diffuse
bright
enhancement, more than the puddling peripheral
involvement ranges from 30%-70%.
enhancement
seen in hepatic hemangiomas
Marked splenomegaly the best predictor
of involvement. FDG PET reported
98%-100% accuracy in predicting splenic lymphoma
Warshauer, D. Spleen: Computed Body Tomography with MRI Correlation. Lee

JKT, Sagel SS, Stanley RJ, Heiken JP eds. Lippincott, Williams and Wilkins.
Philadelphia, PA. 2006 pp 973-1006
Lymphoma
Often presenting with splenic enlargement, LUQ pain or fever, weight loss,
malaise.
US: nodules are hypoechoic
CT or MR, nodules usually not seen without contrast, but can be low signal on
T2w MRI.
Post contrast (CT or MR) are less intense than normal spleen but fill in quickly
(2 min).
Look for adjacent adenopathy
Figure 2-32-15
Lymphoma [Figure 2-32-15]
A 53 yr old female with hepatitis

C,splenomegaly and thrombocytopenia
is evaluated for portal hypertension
The Spleen
Focal Non-Hodgkins lymphoma. A 53 yo female with hepatitis

C, splenomegaly and thrombocytopenia is evaluated for portal
hypertension. Left US shows a well circumscribed hypoechoic
lesion. On Doppler imaging (center) it is vascularized. On CT
(right ) there are multiple ill defined low attenuation lesions
which are non-specific, and could be tumor, infarct or infection
536
Lymphoma [Figures 2-32-16 and 2-32-17]
Figure 2-32-16
Hodgkins Lymphoma
A 35 yr old man with past left seminoma presents

with new lymphadenopathy and focal splenic lesions.
Lymph node biopsy yielded Hodgkins lymphoma
Metastases
Common at autopsy difficult to image except on early

arterial phase contrast imaging (CT or MR)
Diffuse Non-Hodgkins lymphoma. 22 yo male with
Sources include islet cell tumors, melanoma, breast
fever and LUQ mass. Left: Longitudinal US shows
carcinoma and lung carcinoma
massive splenomegaly with multiple diffuse
hypoechoic lesions. Right: US guided biopsy of a
Angiosarcoma [Figure 2-32-18]
focal lesion returned non-Hodgkins lymphoma.
Most common primary nonlymphomatous splenic
The patient died within 2 weeks of splenic rupture
malignancy
and hemorrhage
Single or multifocal
Aggressive growth with hemorrhage and necrosis.
Very vascular and enhance intensely with contrast in arterial phase
Figure 2-32-17
Figure 2-32-18
Initial CT (left) shows isolated hypoechoic lesion of

Non Hodgkins Lymphoma. One year later (right)
the lesion has nearly resolved
44-year-old woman with abdominal pain, nausea,

vomiting, chills. Patient underwent US, CT, US
biopsy, laparoscopic biopsy with hemorrhage;
exploratory laparotomy to rebiopsy, and evacuate
hemoperitoneum, continued bleeding and dies 2
days later. CT (left) shows a low density lesion
with peripheral nodular enhancement and multiple
mixed attenuation partially enhancing lesions in
the liver as well as ascites. Ultrasound (right)
shows heterogenous echogenic lesions in the
liver, consistent with highly vascular lesions with
multiple interfaces
Figure 2-32-19
Infection [Figure 2-32-19]
Bacterial
Aerobic from GI tract, sepsis
TB
Viral
CMV, mononucleosis
Fungal
Candida
40yo male with AIDS presented with marked

splenomegaly. CT with contrast showed
inumerable tiny low attenuation lesions. US (upper
right) shows a 20 cm spleen with multiple tiny
hypoechoic lesions. Core biopsy (lower right) with
18 g needle revealed granulomas, eventually
proven to be TB
537
The Spleen
5 Years Later [Figure 2-32-20]
Figure 2-32-20
Crohns Disease [Figure 2-32-21]
27 yo male with Crohns and ileal

perforation presents 3 weeks
postoperatively (ileocecectomy) with
LUQ pain and fever. What is the cause
of the splenic lesion?
Abscess Evolution
2 weeks later
6 weeks later
Abscess
TB in the spleen. Left: original US

Right: repeat US 5 years later shows persistant splenomegaly,
now with inumerable calcifications. Patient was treated
successfully and was assymptomatic
What caused the abscess?

How did it get to the spleen?
Dx: Diverticulitis extending along the phrenicocolic ligament
What else do you see?
Splenic artery aneurysm
Figure 2-32-21
Sarcoidosis
Splenic involvement common on biopsy

(24%-59%)
Most patients asymptomatic
May show splenomegaly, or diffuse
hypoattenuating nodules on CT and MR
Splenic abscess. 27 yo male with Crohns disease presents 3
which lack peripheral enhancement
weeks
status post ileal and cecal resection with LUQ pain and
Associated abdominal
fever.
Left
CT shows portal vein thrombus. Center CT shows
lymphadenopathy common
splenic vein thrombosis. Right CT shows typical rosette shaped
abscess, in this case from septic thrombophlebitis from the GI
Trauma
tract
Spleen most frequently affected organ
in blunt abdominal trauma.
Highly associated with left lower rib fractures.
Four appearances:
Lacerations (check for splenic hilar involvement) are decreased
attenuation on contrast enhanced CT-perisplenic blood or clot often more
apparent than the laceration.
Intrasplenic hematoma (contusion) may be low attenuation or contain
higher attenuation clot
Subcapsular hematoma: non-enhancing fluid with crescentic compression
of underlying splenic tissue.
Infarcts-non-enhancing wedge shaped areas extending to the capsule.
Severe trauma shatters the spleen into fragments.
Active bleeding is identified as focal extravascular enhancement similar in
intensity to the aorta
Trauma
Clinically important injury is accompanied by hemoperitoneum

Active bleeding identified as area of contrast enhancement with arterial intensity.
Surgical intervention is based on clinical stability/hypotension, lacerations involving
the hilum, and presence of pseudoaneurysms or arteriovenous fistulae
Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of
Thoracoabdominal Trauma; Computed Body Tomography with MRI Correlation,
4th ed, Lippincott, Williams and Wilkins, Philadelphia 2006, pp1440-1429
Trauma
Massive splenic trauma with fragmentation of the spleen, active arterial

extravasation of contrast and hemoperitoneum
The Spleen
538
Splenic Trauma?
23 yo snowboarder with injury. Initial arterial phase

images (left) show multiple possible contusions,
without perisplenic hematoma. Portal venous phase
images on repeat exam (right) show a normal spleen
Trauma [Figures
Figure 2-32-22
2-32-22 and 2-32-23]
Lesion usually < 1cm

Signal void on all pulse sequences
Susceptibility artifact on GE images seen as
blooming artifact
Splenic Angiosarcoma
Exceedingly rare
Most common nonlymphoid primary malignant tumor
of the spleen
More common in patients with thorotrast exposure
Splenomegaly with well defined nodules or diffuse
involvement
Delayed Splenic Rupture
Not predicted by degree of splenic injury

Actual incidence unknown, but not uncommon.
May occur days to weeks after initial injury
Molina PL, Quinn MT, Bouchard EW, Lee JKT.

Computed Tomography of Thoracoabdominal Trauma.
Computed Body Tomography with MRI Correlation, Lee
JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th
Edition, Lippincott Williams and Wilkins, Philadelphia,
2006: 1417-1480
Trauma. Initial CT shows tiny low attenuation

lesion (top left ) without fluid, and subtle low
attenuation lesions (lower right) in an otherwise
normal spleen (lower left and upper right
Figure 2-32-23
Sequelae of Trauma
Injuries (lacerations, infarcts and hematomas) may

take months to a year to heal and may leave scars,
deformed splenic margins, or splenic pseudocysts.
Uncommon injuries requiring intervention include
pseudoaneurysms or arteriovenous fistulae
Blunt Splenic Trauma
Initial CT for blunt trauma shows a bright area of

extravasation superiorly (left) and multiple
lacerations more caudally (right). The patient was
discharged
Splenic Trauma
Trauma. Eleven days later patient presents with

diffuse abdominal pain. Top left CT shows medial
contusion and perisplenic blood and ascites
around the liver. Lower left and upper right show
additional lesions. Lower right CT in the pelvis
shows marked hemoperitoneum (high attenuation
ascites)
Vascular Abnormalities
Portal hypertension
Enlarged splenic vein and/or collaterals
Splenic vein thrombosis
Infection (Crohns, diverticulitis)
Pancreatitis
Splenic artery thrombosis (embolic)
Splenic artery aneurysms
Pseudoaneurysms ((trauma, pancreatitis)
539
The Spleen
Portal Hypertension
Splenomegaly-often up to 20cm c-c

Collaterals
Located at the spenic hilum and
most commonly directed into the
renal vein (a spontaneous splenorenal shunt)
Venous flow in the spleen is never
reversed even if it is reversed in the
splenic vein.
Gamma gandy bodies
Figure 2-32-24
Gamma Gandy Bodies. Spin echo T1 (top left) and T2

weighted series (center), show signal voids at the iron foci.
Gradient echo T1 images (Right) show the blooming effect of
iron due to magnetic susceptibility (pathognomonic)
Portal Hypertension
53 yo woman with cirrhosis and portal hypertension has massively enlarged

liver on ultrasound and LUQ collaterals (US and CT)
Figure 2-32-25
Gamma Gandy Bodies
Focal iron deposition

Common in patients with cirrhosis and
portal hypertension due to
microhemorrhages
Generally mm in size (less than 1cm)
Signal void on all MR images with
pathognomonic blooming on gradient
echo images due to susceptibility
artifact
Gamma Gandy Bodies [Figure 2-32-24]
Spin echo T1, T2, show signal voids at

the iron foci. Gradient echo T1 images
show blooming effect of iron due to
magnetic susceptibility (pathognomonic)
10 year old with sickle cell disease. Nn-contrast CT (left) shows

high attenuation spleen, greater than the liver.(normally should
be less dense than liver pre contrast). MR T1 weighted (center)
and T2 weighted (right) show very low signal intensity in the
spleen, which is normally equal to muscle on T1 and much
brighter than liver on T2. Note also the marked splenomegaly
and the gallstones (T2)
Hemosiderosis [Figure 2-32-25]
Hematoma
Sucapsular
Intrasplenic
Perisplenic
Look for sharp margin b/w spleen and peritoneal fluid
Fluid will accumulate in dependent areas
Infarcts
Common due to arterial emboli

Common in lymphomatous spleens which outgrow their blood supply
Autoinfarction with sickle cell disease
Infarct Appearance
Focal wedge-shaped peripheral lesions

Invisible on US
Decreased attenuation on contrast enhanced CT and low signal on T1
weighted post-Gd enhanced MRI
May rupture with peripsplenic hematoma
Splenic Infarcts [Figure 2-32-26]
53 yo man with MDS presents with increasing LUQ pain. Initial US (left) shows
large heterogeneous avascular cystic lesion in an enlarged spleen.
2 weeks later the lesion has retracted slightly. The patient underwent
splenectomy which showed hemorrhagic infarcts
The Spleen
540
Infarcts [Figure 2-32-27]
Figure 2-32-26
Patient with sarcoidosis and multiple infarcts.
Healing Infarcts
Several months later the splenic contour is

diminished and the areas of infarction still lack
enhancement
What Is Your DX?
Dx: Aortic valve vegetation with splenic infarcts

80 yo with abdominal pain, R/O aortic dissection or
aneurrysm
12 Hours Later
Conclusion
The spleen is easily evaluated on cross-sectional

imaging modalities.MRI is most sensitive for iron and
for small lesions such as diffuse Candidiasis.
Anatomic variants (splenules, clefts) are common
and should not be mistaken for pathologic
processes.
Splenomegaly is non-specific, but usually related to
hematologic abnormalities or portal hypertension.
Infection is acquired by hematogenous or direct
spread (splenic vein, colon, pancreas)
Splenic infarction is often the sequelae to other
disease processes and should encourage you to
search harder to make the diagnosis
Round, hemoorhagic splenic infarcts. Top left US

shows a large heterogenous mass with through
transmission. The mass is avascular (upper right).
Lower left Ultrasound 2 weeks later shows less
fluid in the lesion with smaller diameter, indicating
some clot retraction. Pathology specimen (lower
right) shows 2 infarcts, the larger one
corresponding to the US images
Figure 2-32-27
Classical infarcts going from superior spleen (left) to hilum (center) to inferior spleen (right)
show varying shapes of infarcts which all extend to the periphery of the spleen and lack
enhancement
References
1.
2.
3.
4.
5.
Vos PM, Mathieson JR, Cooperberg PL. The Spleen In: Diagnostic Ultrasound; Rumack CM, Wilson SR, and
Charbonneau JW eds.Elsevier Mosby 2005, St Louis, pp 147-170
Kelekis NL, Burdeny DA, and Semelka RC. Spleen In: MRI of the Abdomen and Pelvis. Semlka RC, Ascher SM
and ReinholdC, eds. 1997, Wiley-Liss New York, NY. Pp 239-256
Warshaer DM. Spleen; In: Computed Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and
Heiken JP, eds. 4th Edition, Lippincott Williams and Wilkins, Philadelphia, 2006: 973-1006
Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma; Computed
Body Tomography with MRI Correlation, 4th ed, Lippincott, Williams and Wilkins, Philadelphia 2006, pp1440-1429.
Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma. Computed
Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th Edition, Lippincott
Williams and Wilkins, Philadelphia, 2006: 1417-1480
541
The Spleen
Portal Venous Doppler

WHY DO WE USE DOPPLER?
To identify vascular from non-vascular structures

To find vessels invisible on gray scale
To make diagnoses based on arterial or venous spectral or color flow patterns
SCANNING PARAMETERS
3MHz transducer-small footprint often preferred for intercostal approaches

Doppler gain as high as possible without image or spectral noise
Wall filter as low as possible to avoid false diagnosis of thrombosis
Scale (PRF) as low as possible to localize vessels quickly with color, then
sample and angle correct for spectral Doppler
WHICH DOPPLER TO USE?
Spectral Doppler most sensitive to flow

Inefficient for quick overview of flow direction, requires precise gate
placement, suspended respiration.
Use wide gate and search for small hepatic arteries not seen with color or
power, and turn off color.
Color Doppler good for flow direction (portal and hepatic veins)
Quickly localizes hepatic arteries for spectral sampling.
Aliasing identifies areas of stenosis (HAs, TIPS)
Power Doppler has best flow sensitivity
Limited by motion (flash) artifact
Best used in TIPS (which often have poor Doppler angles) and in portal
vessels with slow flow
COLOR DOPPLER ALIASING IN TIPS
Color Doppler shows direction of flow and mean velocity. Spectral Doppler
shows angle-corrected true velocity
POWER vs. COLOR DOPPLER
Partial thrombosis suggested. Spectral Doppler implies PV flow.

Note HA visualized on power Doppler as well as intrahepatic vessels
THE NORMAL PORTAL VEIN
Monotonous continuous waveform normally directed into the liver

(hepatopedal)
Color doppler should fill out the entire vessel to exclude portal vein thrombosis
(may be anechoic)
Spectral doppler velocities are typically low, 15-40cm/sec and may even be bidirectional due to swirling flow in a large slow-flowing vein.
Portal vein flow reverses (out of the liver, hepatofugal) in portal hypertension,
and in patent, well functioning porto-systemic shunts
PORTAL HYPERTENSION
Most commonly caused by cirrhosis, but may also be caused by diffuse

metastatic disease and by venous outflow obstruction.
Color flow Doppler and spectral doppler are used in the evaluation of portal
hypertension to detect portal vein thrombosis, the presence of collaterals and
to assess bypass shunts (portocaval, splenorenal, mesocaval, and TIPS) for
patency.
A positive diagnosis can be made by reversed (hepatofugal) flow on Doppler
or by presence of porto-systemic collaterals
eMedicine - Portal Hypertension : Article by Ali Nawaz Khan, MBBS ...

Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and
Charboneau eds.2005, Mosby, Inc. pp 77-146
542
Seminar: Portal Venous Doppler
CASE 1: 55 YO WOMAN WITH ABNORMAL LFTS
A transverse image of the right lobe of the liver shows 2 equal-sized vessels
adjacent to the gallbladder
CASE 1 What is your diagnosis?
DX: Portal hypertension with hepatofugal flow
CASE 1: 55 year old woman with abnormal liver

function tests. (below) A transverse image of the
right lobe of the liver shows 2 equal sized vessels
to the right of the gallbladder
CASE 1: (below) Spectral display of the hepatic
artery (left) and portal vein (right). What is your
diagnosis?
PORTAL HYPERTENSION WITH HEPATOFUGAL FLOW
US offers information re: flow direction; into or out of the liver, which is
unavailable on CT.
Flow reversal out of the liver is a late, but diagnostic sign of advanced portal
hypertension.
Quick clues are the opposing colors and opposing spectral flow directions of
the hepatic artery and portal vein, which should normally both flow in the same
direction
CASE 2
Patient with AML and rising liver function tests. Baseline examination
CASE 2: 3 days later. What has happened and what is your

diagnosis?
Dx: Veno-occlusive disease with reversed portal flow
CASE 2: Three days later the patient returns with

abnormal liver function tests
(below) What has happened and what is your
diagnosis?
CASE 2: Patient with Acute myelogenous

leukemia and rising liver function tests.
Baseline examination shows normal portal Doppler
waveform (right)
543
VENO-OCCLUSIVE DISEASE WITH REVERSED PORTAL FLOW
Hepatic veno-occlusive disease involves hepatic venules in bone marrow

transplant patients.
Major veins appear normal the diagnosis is confirmed by liver biopsy.
Rapid development of portal vein hepatofugal flow has been reported with
veno-occlusive disease* and when present is diagnostic
*Brown BP, Abu-Yousef M, Farner R, La Brecque D, Gingrick R. AJR

1990;154:721-4
CASE 3 :Explain the spectral tracings in the right and left lobes
Dx: Liver metastases with localized left-sided portal hypertension
ATYPICAL PORTAL
HYPERTENSION
While portal hypertension is most

commonly the sequelae of cirrhosis, it
can be caused by any process which
obstructs the sinusoids including Venoocclusive disease, Budd Chiari
syndrome, and metastases.
Focal flow reversal should prompt a
search for localized disease, including
analysis of the gray scale images
PORTOSYSTEMIC COLLATERALS
CASE 3: 61 year old female with rising liver function tests and
jaundice. Prior Doppler ultrasound was normal.
Left: Doppler tracing of the right HA and PV.
Center: Gray scale transverse image of the left lobe
Right: Spectral Doppler tracing of the left HA and PV. Explain
the difference between the left and right lobe.
Diagnosis: Metastatic breast carcinoma with localized left portal
hypertension
These are diagnostic of portal

hypertension
Frequent locations include the
gastroesophageal junction,
paraumbilical vein in the falciform ligament, splenorenal and gastrorenal in the
left upper quadrant, intestinal veins in the retroperitoneum, and hemorrhoidal
veins in the pelvis. Visualization of the paraumbilical vein is specific for the
diagnosis of portal hypertension.
Visualization of collaterals requires slow flow settings, and a good sonographic
window, unobscured by bowel gas
CASE 4: 31 year old male with ascites

CASE 4: Can you account for the alternate flow directions in the
left and right portal veins? What is your diagnosis?
Portal hypertension with a patent umbilical collateral vein
CASE 4: Can you account for the alternate flow

directions in the left and right portal veins? What is
your diagnosis?
CASE 4: 31 year old male with ascites
544
PORTAL HYPERTENSION WITH COLLATERALS
Multiple collateral pathways carry portal blood around the liver into the
The most common is the coronary vein, however this is not specific for portal
hypertension
Another common pathway is the patent umbilical vein, seen in up to 20% of
patients and 100% specific for portal hypertension.
A patent umbilical vein collateral will preserve forward (hepatopetal) flow in the
left portal vein while the flow in the right portal vein is reversed (hepatofugal).
CASE 5: What is happening in the splenic vein?

CASE 5: What is your diagnosis now?
Portal hypertension with spontaneous
splenorenal shunt formation
CASE 5: Portal hypertension with spontaneous

splenorenal shunt formation seen on sequential
CT images and corresponding transverse US
image (bottom right)
CASE 6: A 30 yr old woman presents with acute

epigastric pain.
What is the diagnosis and possible etiology?
Case 5: A 68 year old woman

presents in liver failure.
Top: Midline transverse flow in the
splenic vein is toward the transducer
(toward the spleen). Bottom: Flow in
the splenic vein is away from the
spleen at the hilum
Dx: Splenic and portal vein thrombosis, patient taking birth control pills
Portal Vein Thrombosis
Bland thrombus is a frequent sequelae to slow flow and portal hypertension.

Other etiologies include hypercoagulability, pancreatitis, pyelophlebitis from
diverticulitis or Crohns disease or
cholangitis, and portocaval shunts.
Thrombus may be partial or complete,
involving main or branch vessels.
Early thrombus is hypoechoic, and may
require color Doppler to detect.
Older thrombus is hyperechoic and
recanalizes and/or forms collaterals,
which have typical portal spectral
waveforms.
CASE 6: A 30 yr old woman presents with acute epigastric
Tumor thrombus (HCCA) contains
pain. What is the diagnosis and possible etiology?
hepatic arterial waveforms and may be Left: US shows enlarged anechoic portal vein with normal color
biopsied for staging.*
flow in the IVC posteriorly. Right: Transverse image of
noncontrast CT corresponds to US on the left.
*Dodd GD, Memel DS, Baron RL, Eichner L,
Dx: Splenic and portal vein thrombosis in a patient taking birth
Santaguida LA. AJR 1995;165:573-577
control pills
545
CASE 7: A 64 year old man is being considered for liver

transplant.
Dx: Cavernous transformation of the portal vein
CAVERNOUS TRANSFORMATION OF THE

PORTAL VEIN
This refers to numerous collateral vessels at the

porta due to acute or longstanding thrombosis.
(Cavernous transformation may occur as soon as a
week after occlusion)
The absence of an adequate portal vein for
anastamosis precludes successful liver
transplantation
CASE 7: A 64 year old man is being considered

for liver transplant
De Gaetano AM, Lafortune M, Patriquin H, De Franco A, Aubin B, Paradis K.

Cavernous transformation of the portal vein: patterns of intrahepatic and
splanchnic collateral circulation detected with Doppler sonography. AJR 165,
1151-1155,
CASE 8: Is this normal or abnormal flow?
Right and left portal vein flow reversal in normally

functioning TIPS
PORTO-SYSTEMIC SHUNTS
These are used to decompress portal hypertension

to control bleeding
Color Doppler is useful to assess shunt patency,
providing an adequate acoustic window is present
(may be limited in mesocaval and splenorenal
shunts)
Intrahepatic portal venous flow should be hepatofugal
if the shunt is working properly
PORTOSYSTEMIC SHUNTS: TIPS
CASE 8: TIPS shunt. Is the portal vein flow normal

or abnormal?
These are the most common portosystemic shunt now used.

The entrance is percutaneous from the jugular vein into the right hepatic vein
through the liver to the main portal vein.
Spectral Doppler TIPS peak velocity is obtained in the proximal (portal venous
end), mid and distal (hepatic end) shunt. Absent flow indicates shunt occlusion
Color flow in the intrahepatic portal veins should be toward the TIPS, and the
involved hepatic vein flow should be toward the IVC
TIPS DOPPLER SURVEILLANCE
Monitoring is recommended post procedure, then q3 months and as clinically

indicated
Normal velocity is between 90 and 190cm/s in the mid and distal shunt.*
(although the original lower limit of normal was reported to be 50-60 cm/sec,**
this has been unreliable in the proximal shunt due to portal mixing)
A change in peak stent velocity by >50cm/sec over time, reversed flow in the
draining hepatic vein, and focal shunt stenosis are also useful ***
*Kanterman RY, Darcy MD, Middleton WD, Sterling KM, Teefey SA, Pilgram TK.
AJR 1997;168:467-472.
**Foshager MC, Ferral H, Nazarian GK, Castaneda-Zuniga WR, Letournea JG.
AJR 1995;165:1-7.
***Dodd GD, Zajko AB, Orons PD, Martin MS, Eichner LS, Santaguida LA. AJR
1995;164:1119-1124.
546
PROXIMAL TIPS SHUNT VELOCITY
Doppler signal from the proximal end of the TIPS shunt. Flow
velocity increases as the cursor is moved from the main portal
vein (less than 40 cm/sec) into the shunt (69 cm/sec)
NORMAL TIPS
ABNORMAL TIPS
28mm gradient between TIPS and right atrium due to tight

stenosis at the hepatic venous end which was balloon
angioplastied. Note monophasic portal wave form
Proximal TIPS Shunt Velocity
Doppler signal from the proximal end
Increased shunt velocity at the distal end of the shunt indicated by of the TIPS shunt. Note the increase
color Doppler aliasing
in flow velocity as the cursor is
Spectral Doppler signal demonstrates the focal distal shunt
moved from the main portal vein into
stenosis, velocity is 256.7 cm/sec
the shunt
TIPS Shunt Stenosis
Abnormal US, Normal Angiogram
US abnormal by strict velocity criteria, but note

normal pulsatility
TIPS Monitoring: Low Velocities
Subsequent venogram showed severe stenosis

throughout the stent, which was replaced with an
intrastent following balloon angioplasty
CONCLUSION
Portal venous Doppler US is an effective way to

assess the patency and function of the hepatic portal
TIPS Shunt Stenosis. (Right) Increased shunt
system.
velocity at the distal end of the shunt indicated by
It permits rapid identification of portal flow direction
color Doppler aliasing. (Left)Spectral Doppler
and can identify collaterals, specific to portal
signal demonstrates the focal distal shunt
hypertension.
stenosis, velocity is 256.7 cm/sec
It is particularly useful to serially monitor TIPS shunt
function

Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and Charboneau eds.2005,
Mosby, Inc. pp 77-146.
References
1.
2.
3.
4.
5.
6.
7.
8.
Brown BP, Abu-Yousef M, Farner R, La Brecque D, Gingrick R. AJR 1990; 154:721-4.

De Gaetano AM, Lafortune M, Patriquin H, De Franco A, Aubin B, Paradis K. Cavernous transformation of the
portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR
165, 1151-1155,
Dodd GD, Memel DS, Baron RL, Eichner L, Santaguida LA. AJR 1995;165:573-577.
Dodd GD, Zajko AB, Orons PD, Martin MS, Eichner LS, Santaguida LA. AJR 1995; 164:1119-1124.
eMedicine - Portal Hypertensions: Article by Ali Nawaz Khan, MBBS.
http://www.emedicine.com/radio/topic571.htm
Foshager MC, Ferral H, Nazarian GK, Castaneda-Zuniga WR, Letournea JG. AJR 1995;165:1-7.
Kanterman RY, Darcy MD, Middleton WD, Sterling KM, Teefey SA, Pilgram TK. AJR 1997;168:467-472.
Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and Charboneau eds.2005, Mosby,
Inc. pp 77-146.
547
548
549
550
Imaging of Uterine Disorders

Figure 3-1-1
Overview
Normal Anatomy
Imaging Techniques
Benign Lesions
Malignancies
Uterus
Fundus
Corpus
Cervix
Uterine Corpus
Serosa
peritoneal reflection
Myometrium
involuntary smooth muscle
Endometrium
stratum basalis
stratum functionalis
Normal cervix
Cervix [Figure 3-1-1]
Internal os
Endocervical canal
columnar epithelium
plicae palmatae
surrounded by fibrous stroma and muscular layer
External os
squamocolumnar junction
Figure 3-1-2
Uterine Ligaments
Broad ligaments
double sheet of peritoneum
Cardinal ligaments
Uterosacral ligaments
Uterovesical ligaments
Round ligaments
Blood Supply [Figure 3-1-2]
Uterine artery
branch of internal iliac
passes superficial to the ureter
enters myometrium at internal os
Ovarian artery
branch of the aorta
anastomosis with uterine artery
Dual blood supply to uterus
Imaging Techniques
Ultrasound
Hysterosalpingography
Sonohysterography
MRI
551
Ultrasound
Figure 3-1-3
Transabdominal
full baldder
2.55.0 MHz transducer
Transvaginal
empty bladder
5.07.5 MHz transducer
Myometrium [Figure 3-1-3]
Homogeneous intermediate echogenicity

Can sometimes see hypoechoic inner and outer layers
Blood supply
uterine
arcuate
radial
spiral (endometrium)
Endometrium [Figures 3-1-4 and 3-1-5]
Early proliferative phase

thin echogenic line
Late proliferative phase
hypoechoic thickening, 48mm
Secretory phase
hyperechoic thickening, 714mm
Menstrual phase
thin broken echogenic line
The uterine arteries give rise to the

arcuate arteries located in outer
third of myometrium. Radial arteries
course through the myometrium and
terminate as spiral arteries in the
endometrium
Figure 3-1-4
Figure 3-1-5a
Before ovulation (days 1 14), the ovary is in the

follicular phase and the endometrium is in the
proliferative phase. After ovulation (days 14-28)
the ovary is in the luteal phase and the
endometrium is in the secretory phase
Endometrium, proliferative phase
Figure 3-1-5b
Endometrium, secretory phase
Hysterosalpingography: HSG
First ten days of menstrual cycle

Active PID contraindication
Radiation dose 75750 mrad
Only visualizes internal contour
Primary use tubal patency
552
Pelvic MRI
Figure 3-1-6
Phased array coils

Fast T2WI images
Pelvic Protocol
Pelvic Coil
Coronal localizer FMSPGR
Always include kidneys
FSE T2 sagittal, axial,
coronal, oblique
TR 4,0005,000
TE 90130
ETL 16
FOV 2024 cm
Thickness 45 mm, 1
mm gap
Matrix 256x256
24 NEX
T1 SE
Axial
TR 300500
TE min
T1 Fat Sat with Gd
Normal Uterus: Sagittal T2WI and HSG view
Figure 3-1-7
Uterus
T1 uniform intermediate
signal
T2 zonal anatomy
Endometrium high
signal
Junctional zone low
signal
Myometrium intermediate signal
Normal Cervix: sagittal and donut view
Figure 3-1-8
Normal Uterus
[Figure 3-1-6]
Normal Cervix
[Figure 3-1-7]
Embryology
Figure 3-1-9
Embryologic paramesonephric ducts.
Metanephrosis (kidney) with concurrent
development
Uterus forms from fused paramesonephric

ducts. Distal vagina forms from urogenital
sinus
553
Mullerian Duct Anomalies

[Figure 3-1-10]
Class
II
III
IV
VI
Figure 3-1-10
Description
Agenesis or hypoplasia
Unicornuate
Didelphys
Bicornuate
Septate
VIDES-related
Unicornuate / Didelphys
[Figure 3-1-11]
Low rate of pregnancy loss

Limited surgical options
Unicornuate highest rate of renal
agenesis
Didelphys 75% have vaginal
septum
Bicornuate
Classification of Mullerian duct anomalies
[Figures 3-1-12 and 3-1-13]
Partial fusion of ducts

Concave external contour
Bicollis or unicollis
Figure 3-1-12
Figure 3-1-11
Bicornuate bicollis (2 cervices)

and
bicornuate unicollis (1 cervix)
Unicornuate and didelphys
Figure 3-1-13
Bicornuate, unicollis
554
Septate
Figure 3-1-14
[Figures 3-1-14 and 3-1-15]
Most common uterine malformation

Highest spontaneous abortion rate
Septum may be complete or partial
Septum may be fibrous or composed of myometrium
Figure 3-1-15
Complete and partial septum
Septate uterus with a complete fibrous

septum through the cervix
Bicornuate vs. Septate

[Figure 3-1-16]
Angle between horns

External morphology
Complications
Treatment
Bicornuate
Septate
>90
<90
concave
normal
abnormall lie
increased spontaneous
premature labor
abortion rate
metroplasty
hysteroscopic resection
Arcuate Uterus
[Figure 3-1-17]
Figure 3-1-16
Figure 3-1-17
At least 1 cm of remaining
myometrium should be present for
hysteroscopic resection
Arcuate uterus is a normal variant. 1 1.5 cm indentation
555
Diethylstilbestrol: DES Related
Figure 3-1-18
[Figure 3-1-18]
11.5 million female progeny exposed

50% have a uterine anomaly
Associated with clear cell carcinoma of the vagina
No associated urinary tract abnormality
Mullerian Duct Anomalies
Form a continuum
Renal anomalies in 25%
Obstructions are common risk for endometriosis and adenomyosis
Septate has highest spontaneous abortion rate
DES exposure risk factor for clear cell carcinoma of the vagina
Benign Uterine Masses
Leiomyomas
Adenomyosis
Leiomyoma
Benign smooth muscle tumor

Dysmenorrhea, hypermenorrhea, fertility problems
25% of premenopausal women
DES exposure
Leiomyoma
[Figure 3-1-19]
Submucosal, intramural, subserosal

Well circumscribed
US generally hypoechoic
MRI low signal on T1 and T2 unless they have
undergone degeneration
Figure 3-1-19
US, MRI, and hysteroscopic image of a submucosal fibroid
Leiomyoma Degeneration [Figure 3-1-20]
Figure 3-1-20
Hyaline
Myxomatous
Cystic
Hemorrhagic (carneous)
Sarcomatous
Cystic and hemorrhagic degenerated

fibroid
556
Indications for MRI
Figure 3-1-21
Pre-myomectomy
Rapidly growing fibroid
When US is confusing
Adenomyosis
[Figures 3-1-21 and 3-1-22]
Heterotopic implants of endometrium within the

myometrium
Dysmenorrhea, hypermenorrhea
25% of hysterectomy specimens
Adenomyosis [Figure 3-1-23]
May be diffuse or focal (adenomyoma)

MRI
junctional zone > 1 cm
low signal on T1 and T2
punctate areas of high signal
irregular borders
Diffuse adenomyosis
Adenomyosis
Figure 3-1-22
Ultrasound
Enlarged heterogeneous uterus
Focal form often confused for fibroids
Endometrial Thickness
< 15 mm in premenopausal patient (secretory phase)

8mm in asymptomatic post-menopausal patient (if on hormones
scan after withdrawl bleeding)
4mm postmenopausal and bleeding
Abnormal Uterine Bleeding
Polyps
Submucosal fibroids
Hyperplasia
Carcinoma
Atrophy
most common cause of post-menopausal bleeding
Sonohysterography
Adenomyosis
Figure 3-1-23
[Figure 3-1-24]
Figure 3-1-24
Focal adenomyosis (adenomyoma)
Sonohysterography. Saline is infused while

scanning
557
Endometrial Polyps
[Figure 3-1-25]
Focal overgrowth of endometrial tissue

Pedunculated or sessile
20% are multiple
May be cystic
Figure 3-1-25
Endometrial polyp
Submucosal fibroid
Figure 3-1-26
Endometrial Hyperplasia [Figure 3-1-26]
Increased estrogen stimulation

hormone replacement (unopposed estrogen)
tamoxifen
anovulatory cycles, polycystic ovarian
disease
obesity
estrogen producing tumors (granulosa cell,
thecoma)
Risk factor for carcinoma
Tamoxifen [Figure 3-1-27]
Has an antiestrogen effect on the breast

but weak estrogen effect on the uterus
Increased risk of endometrial carcinoma,
hyperplasia, and polyps
Cystic changes often present
Endometrial hyperplasia
Postmenopausal Bleeding
Figure 3-1-27
4mm atrophy
> 4mm sonohysterogram
diffuse thickening random bx or D&C
focal thickening hysteroscopy
Endometrial Carcinoma
Most common GYN malignancy- 33,000

cases/year
Risk factors:
unopposed estrogens, tamoxifen
nulliparous
diabetes
obesity
Cystic endometrium from tamoxifen

558
Endometrial Carcinoma
Histology
adenocarcinoma (80%90%)
adenosquamous
papillary serous **
clear cell carcinoma **
Grade
I well differentiated
II moderately well differentiated
III poorly differentiated
Endometrial Carcinoma: FIGO Staging

Stage
0
Ia
Ib
Ic
II
III
IVa
IVb
Description
Carcinoma in situ
Limited to endometrium
Less than 1/2 myometrium
Greater than 1/2 myometrium
Invades cervix but not beyond uterus
Beyond uterus but not outside pelvis
Outside true pelvis / bladder / bowel
Distant metastases
MRI Findings
Figure 3-1-28
Intermediate signal mass

Expands endometrial cavity
Enhances less than myometrium
Not for screening
MRI Staging [Figure 3-1-28]
Disruption of junctional zone

Depth of myometrial invasion
Extension into cervix
Extension beyond uterus
Adenopathy
Prognostic Factors
Histology
Tumor grade
Depth of myometrial invasion
Lymph node involvement
Cervical Carcinoma
Endometrial carcinoma extending to serosa
14,000 cases/year, 4,900 deaths/year

Begins at squamocolumnar junction
90% are squamous cell
Association with papilloma virus, herpes, and HIV
Cervical Carcinoma
Stage
0
I
II
IIa
IIb
III
IVa
IVb
Description
Carcinoma in situ
Confined to cervix
Invades beyond cervix but not to pelvic sidewall or lower third of vagina
No parametrial invasion
Parametrial invasion
Extension to pelvic sidewall / lower third of vagina / causes
hydronephrosis
Invasion into bladder / rectum
Distant metastases
559
Staging
Clinical
errors in 32% for stage IB (greater than 5mm deep and 7mm wide)
62% for II-IV
MRI
93% accuracy for tumor size within 5mm
Staging accuracy 87%- 92%
Figure 3-1-29
Prognosis
Tumor size
Depth of invasion
Parametrial extension
Lymph node involvement
MRI
Intermediate signal T2WI

Check list
tumor size
depth of stromal invasion
parametrial invasion
hydronephrosis
lymphadenopathy
Cervical Carcinoma Stage I

[Figure 3-1-29]
Cervical Carcinoma Stage II
Stage I cervical carcinoma. The tumor remains confined to the

cervix
Figure 3-1-30
[Figure 3-1-30]
Stage IIB cervical carcinoma with obvious parametrial invasion

References
1.
2.
3.
4.
5.
6.
7.
8.
Bazot M, Cortez A, Darai E, et al. Ultrasonography compared with magnetic resonance imaging for the diagnosis
of adenomyosis: correlation with histopathology. Hum Reprod 2001; 16:2427-2433.
Davis PC, O'Neill MJ, Yoder IC, Lee SI, Mueller PR. Sonohysterographic findings of endometrial and
subendometrial conditions. Radiographics 2002; 22:803-816.
Nicolet V, Carignan L, Bourdon F, Prosmanne O. MR imaging of cervical carcinoma: a practical staging approach.
Radiographics 2000; 20:1539-1549.
Reinhold C, Khalili I. Postmenopausal bleeding: value of imaging. Radiol Clin North Am 2002; 40:527-562.
Reinhold C, Tafazoli F, Mehio A, et al. Uterine adenomyosis: endovaginal US and MR imaging features with
histopathologic correlation. Radiographics 1999; 19 Spec No:S147-160.
Scheidler J, Heuck AF. Imaging of cancer of the cervix. Radiol Clin North Am 2002; 40:577-590, vii.
Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. Radiology 2004; 233:19-34.
Ueda H, Togashi K, Konishi I, et al. Unusual appearances of uterine leiomyomas: MR imaging findings and their
histopathologic backgrounds. Radiographics 1999; 19 Spec No:S131-145.
560
Approach to Renal Masses

Approach to Renal Masses: 4 Questions
Cyst vs Solid
Infiltrative vs Expansile
Fatty vs Soft Tissue
Solitary vs Multiple
Tumor Growth: Ball vs. Bean
Expansile Ball
Infiltrative Bean
Expansile Renal Mass
Spherical, exophytic, frequently encapsulated

DDx
Malignant adenocarcinoma, metastases, lymphoma
Benign cyst, angiomyolipoma, oncocytoma, etc.
Infiltrative Renal Masses
Invades parenchyma, preserves renal contour, poorly marginated

DDx
Malignant transitional cell, squamous cell, lymphoma, atypical
adenocarcinoma
Benign pyelonephritis, XGP, TB
Intravenous Urography
Only good for expansile masses

Misses 1/3 of masses <3cm
All lesions require further work up
US, CT, MRI
Ultrasound
Cyst
anechoic
acoustic enhancement
sharp posterior wall
RCCA
can be hypo, iso, or hyperechoic
Computed Tomography
94% sensitive for lesions 3 cm or less

90%-95% accuracy in staging
Pre and post contrast of lesion
Scan in both corticomedullary and nephrographic phase
561
Renal Neoplasms
Volume Averaging All Tissues in the Slice

Volume are Averaged
Figure 3-2-1
[Figures 3-2-1 to 3-2-3]
Phases of Excretion
Corticomedullary phase
25 80 sec
Nephrographic phase
90120 sec
Excretory phase
3 5 min
Varies with injection rate, cardiac output, and renal
function
Corticomedullary Phase (CMP)
Cortex and medulla > 100 HU difference

Best for metastases and vascular invasion
Pitfalls: Can miss hyperdense cortical masses and hypodense
medullary masses, pseudotumor in the IVC
Volume averaging
Figure 3-2-2
Nephrographic Phase
Renal lesions are best seen in the nephrographic phase

1.1 > 2.4 more masses detected
Venous extension
Contiguous vs. overlapping

reconstruction
Figure 3-2-3
[Figure 3-2-4]
Excretory Phase
Decreasing density of nephrograms

Worsening streak artifact especially with non-ionic contrast
Excretory Phase
Decreasing density of nephrograms

Worsening streak artifact especially with non-ionic contrast
New role in CT urography
Affect of volume averaging
on an AML
Figure 3-2-4
Surgical approach is based on extent of venous invasion
Enhancement
< 10 HU no enhancement
> 15 HU enhancement
1015 HU gray zone
Renal Neoplasms
562
De-enhancement
Decrease 15 HU at 15 minutes
CT Technique for Renal Mass Characterization
Scan kidneys both pre and post contrast

Slice thickness of 57mm
Scan during corticomedullary and nephrograhic phase
Perform overlapping reconstruction if the lesion is small
Delayed scans
Magnetic Resonance Imaging
Equivalent to CT in accuracy with Gd

Calcification difficult to detect
Excellent for vascular invasion
Magnetic Resonance Imaging
Cysts
low-signal T1WI
high-signal T2WI
no enhancement
Solid
15% enhancement with Gd
Calculating % Enhancement
(Post SI Pre SI) / Pre SI x 100 = % enhancement
Malignant Neoplasms
Adenocarcinoma
Uroepithelial tumors
Transitional cell
Squamous cell
Lymphoma
Metastases
Renal Cell Carcinoma
25,000 30,000 new cases

12,000 deaths per year
Peak incidence sixth and seventh decade
M:F (23:1)
Bilateral 2%, multicentric 15%
Expansile cortical mass

90% originate from proximal convoluted tubule
Slow growing low grade malignancies may be encapsulated

Can have areas of necrosis, cyst formation, hemorrhage, or calcification
Histology
Clear cell 70%80%

Deletion on chromosome 3p
Lipid rich
Papillary 10%15%
Slower growing, less vascular, calcification more common, often
encapsulated, better prognosis
Other chromophobe, sarcomatoid, medullary, etc.
563
Renal Neoplasms
Renal Cell Carcinoma - Risk factors
Figure 3-2-5
Dialysis [Figure 3-2-5]

von Hipple Lindau [Figure 3-2-6]
often multiple RCCAs
multiple renal cysts
affects other abdominal organs
Tuberous sclerosis (much less common)
Renal Cell Carcinoma: Presentation
Classic triad hematuria, flank pain, mass < 50%

Paraneoplastic hypertension, erythrocytosis,
hypercalcemia
Other fever, weight loss, anemia, varicocele
30% present with metastases
Cystic disease of dialysis with RCCA
Calcification
20%30% of RCCA
1%2% of benign cysts
Rim calcification 80% benign
Central calcification 87% malignant
Figure 3-2-6
Cystic Changes
1525% of RCCA
Necrosis 75%
Cystic 25% often papillary histology
Mural nodule or septations [Figure 3-2-7]
Malignant cell lining (VHL)
Benign Lesions
Simple Cyst
Water density
Thin (1-2 mm) wall
No enhancement
Minimally Complicated Cyst
High density cysts (protein or blood)
Thin septations
Thin curvilinear calcifications
VHL with multiple RCCA
Figure 3-2-7
Surgical Lesions
Enhancing lesions
Nodularity
Thick wall (>2mm)
Thick septations
Irregular or central calcifications
Less important
Spontaneous Renal Hemorrhage
RCAA (men)
AML (women)
Infarction
Infection
AV malformation
Vasculitis
Glomerulonephritis
Cystic RCCA
Infiltrating Renal Cell
7% of adenocarcinomas
Arise from the renal medulla difficult to differentiate from
invasive uroepithelial tumor
Medullary carcinoma, collecting duct carcinoma, sarcomatoid neoplasms
Poor prognosis
Renal Neoplasms
564
Medullary Carcinoma [Figure 3-2-8]
Figure 3-2-8
Young Black male with sickle cell

trait
From epithelium of papilla or distal
collecting duct
Survival < 4 mos.
Robson Staging [Figure 3-2-9]
I Confined to kidney
II Within Gerotas fascia
III A Renal vein or IVC invasion
III B Lymph nodes
III C Vascular invasion plus nodes
IV A Direct organ invasion
IV B Distant metastases
Medullary carcinoma with infiltrative pattern
TNM Staging
T1 < 7cm
T2 > 7 cm
T3a local invasion not beyond Gerotas fascia
T3b venous invasion below diaphragm
T3c venous invasion above diaphragm
T4 extension beyond Gerotas fascia
N0 no regional lymph nodes
N1 metastasis in a single regional lymph node
N2 metastasis in more than one regional lymph
node
M0 no distant metastasis
M1 distant metastasis
Stage I
T1,N0,M0
Stage II
T2,N0,M0
Stage III
T1,N1,M0
T2,N1,M0
T3a,N1,M0
T3b,N0,M0
T3b,N1,M0
T3c,N0,M0
T3c,N1,M0
Stage IV
T4,N0,M0
T4,N1,M0
Any T,N2,M0
Any T,any N,M1
Figure 3-2-9
Stage 1
Stage II with invasion

into Gerotas fascia
Figure 3-2-10
American Joint Commitee on Cancer
Nephron-sparing Surgery
Margins of at least 5 mm normal tissue

< 4cm
Away from renal hilum (polar, cortical)
Survival rates comparable to radical nephrectomy
RF ablation non-surgical alternative
Stage II vs. Stage I

[Figure 3-2-10]
Nodule in perinephric space most specific

but present < 50%
Perinephric stranding unreliable
Stage II with nodule in perirenal space
565
Renal Neoplasms
Stage III [Figure 3-2-11]

Figure 3-2-11
Stage III
Stage III Renal Carcinoma: Imaging

[Figure 3-2-12]
Figure 3-2-12
Vascular invasion extending to the right atrium

Renal Neoplasms
566
Stage IV [Figure 3-2-13]
Figure 3-2-13
Stage IV
Stage IV renal carcinoma: Imaging

Figure 3-2-14
[Figure 3-2-14]
Renal Cell Carcinoma: Metastases
Lung
Bone
Liver
Nodes
Brain
Adrenal
69%
43%
34%
22%
5%
4%
Abdominal CT Checklist
Renal vein, IVC

Regional lymph nodes
Adrenal glands
Contralateral kidney
Review lung and bone windows
Stage IV with invasion into the descending colon
Uroepithelial Neoplasms
5%10% of all urinary tract malignancies

Transitional cell 85% 95%
Squamous cell carcinoma 5%10%
Rare adenocarcinoma, sarcoma, metastases
Transitional Cell Carcinoma
5070 yo
Males > females (3:1)
Risk factors
Smoking
Aniline dyes
Benzene
Analgesic nephropathy (phenacetin)
Balkan nephropathy
Transitonal Cell Carcinoma
Hematuria 75%
Multicentric 30%50%
Bilateral 10%
Incidence by location:
Bladder 92%
Pelvis 6%
Ureter 2%
567
Renal Neoplasms
Transitional Cell Carcinoma

[Figure 3-2-15]
Figure 3-2-15
Small, hypovascular masses

Majority papillary with endophytic growth
Renal invasion in 25%
Imaging
Retrogrades, IVP with compression
CT urography
US and conventional CT poor

[Figure 3-2-16]
Squamous metaplasia from chronic irritation

Associated with stones (>50%)
Aggressive behavior, commonly infiltrative
Survival < 1 yr
Renal Lymphoma
Invasive transitional cell carcinoma presenting as

a renal mass
[Figures 3-2-17 to 3-2-19]
Common in widespread disease

Primary lymphoma very rare
Hematogenous spread or direct invasion
50-70% bilateral
Homogeneous
Multiple masses
50%
Infiltrating hilar
mass
25%
Perirenal
10%
Renal enlargement 10%
Solitary mass
5%
Figure 3-2-16
Figure 3-2-17
Sqaumous cell carcinoma with a staghorn calculus and large soft tissue
mass. SCCA often has overlapping features with XGP
Figure 3-2-18
Renal lymphatics are

located around the
capsule and renal hilum
Figure 3-2-19
Renal lymphoma with homogeneous
expansile and infiltrative masses
Perirenal
lymphoma with
capsular
invasion
Renal Neoplasms
568
Metastases
Figure 3-2-20
7%20% of autopsy cases

Generally asymptomatic
Primaries
Lung
Breast
GI (esp colon)
Melanoma
Metastates [Figure 3-2-20]
Spread
Hematogenous usually cortical
Lymphatic perirenal
Direct invasion
Expansile or infiltrative pattern
Solitary or multiple masses
Metastases with adenopathy
Figure 3-2-21
Benign Renal Neoplasms
Cystic
Multilocular cystic nephroma
Solid
Parenchymal
Oncocytoma
Juxtaglomerular tumor
Leiomyoma (capsuloma)
Mesenchymal
Angiomyolipoma
Multilocular Cystic Nephroma

[Figure 3-2-21]
Bimodal age distribution

< 2 yo (M:F, 3:1)
> 40 yo (F:M, 9:1)
Multiple well-defined cysts with enhancing septa, no hemorrhage
Can herniate into renal pelvis
DDx: cystic renal cell carcinoma, MCDK complicated benign cyst,
abscess
Oncocytoma
[Figure 3-2-22]
Oncocyte Greek swollen cell

Large epithelial cells with granular eosinophilic cytoplasm (abundant
mitochondria)
Found in kidney, salivary glands, thyroid, parathyroid, and pancreas
Oncocytoma
Large 7 cm avg. at detection
Older males
Solid exophytic enhancing mass
Can not distinguish from RCCA

with herniation into the renal
pelvis
Figure 3-2-22
Oncocytoma
Helpful features all non-specific

Central scar
Spoke wheel angio appearance
Necrosis, hemorrhage, calcification rare
No adenopathy or metastases
Gross tan/brown tumor with pale central scar
Oncocytoma with a central scar
569
Renal Neoplasms
Juxtaglomerular Cell Tumor
Figure 3-2-23
Renin producing tumor

Rare cause of hypertension, may also have headache
and muscle weakness
Young adults (F:M, 2:1)
Hypovascular mass imaging non-specific
Leiomyomas [Figure 3-2-23]
Small solid masses on renal surface (capsuloma)

Usually incidental finding
Imaging non-specific
Low signal on T2-weighted MRI is suggestive
Angiomyolipoma: AML
Renal hamartoma with blood vessels, smooth muscle, and fat

Prevalence 0.3%3%
80% sporadic
Females 3050 yo
Usually solitary
20% tuberous sclerosis
Angiomyolipoma: Imaging
Ultrasound non-specific [Figure 3-2-24]

AML
Shadowing
Markedly hyperechoic
RCCA
Hypoechoic rim
Cystic spaces
Must prove with CT or MRI
Low-signal, non-enhancing
leiomyoma (capsuloma)
Figure 3-2-24
Angiomyolipoma: Imaging
CT
HU < 10 will detect 85% of AMLs
No calcifications
Vascular phase imaging can detect aneurysms
MRI
fat bright on T1 and T2
fat saturation sequence
Angiography
Tortuous, abnormal vessels with small
aneurysms
Embolization
AML with shadowing
Tuberous Sclerosis [Figure 3-2-25]
Autosomal dominant
Clinical triad seizures, adenoma sebaceum, mental retardation
Multiple hamartomatous lesions including: retinal hamartoma,
cortical tubers, subependymal nodules, ungual fibroma,
angiofibroma, pulmonary lymphangiomyomatosis, cardiac
rhabdomyoma
Figure 3-2-25
Tuberous Sclerosis
[Figure 3-2-25]
Renal involvement
Approx 3/4 will have AML
75% multiple
50% bilateral
Cysts can also be seen especially in children
1%2% develop RCCA
Renal Neoplasms
Tuberous sclerosis with large bilateral

AMLs
570
Angiomyolipoma: Presentation
Figure 3-2-26
[Figure 3-2-26]
Incidental finding
Usually < 4 cm
Hemorrhage
Usually > 4cm
May be spontaneous or minor trauma
Bleeding may be life-threatening in up to 25% of cases
Vessels thick walled with decreased elastin
Predisposition for aneurysm formation
RCCA with Fat

[Figure 3-2-27]
Osseous metaplasia calcification

Lipid necrosis large necrotic masses
Engulfed perirenal or sinus fat large masses, irregular
invasive appearance
Angiomyolipoma with perinephric

hemorrhage
Figure 3-2-27
RCCA with osseous metaplasia
571
Renal Neoplasms
Helpful Tips
No enhancement (etc.)
Benign cyst
Fat
AML
Multiple AMLs
Tuberous sclerosis
Infiltrative + expansile
Lymphoma
Herniation into renal pelvis + female
Cysts + solid masses
VHL or dialysis
Central scar + no adenopathy or vein invasion
Oncocytoma
References
1.
Catalano C, Fraioli F, Laghi A, et al. High-resolution multidetector CT in the preoperative evaluation of patients
with renal cell carcinoma. AJR Am J Roentgenol 2003; 180:1271-1277.
2. Choyke PL, Glenn GM, Walther MM, Zbar B, Linehan WM. Hereditary renal cancers. Radiology 2003; 226:3346.
3. Khan A, Thomas N, Costello B, et al. Renal medullary carcinoma: sonographic, computed tomography, magnetic
resonance and angiographic findings. Eur J Radiol 2000; 35:1-7.
4. Rendon RA, Stanietzky N, Panzarella T, et al. The natural history of small renal masses. J Urol 2000; 164:11431147.
5. Sheth S, Scatarige JC, Horton KM, Corl FM, Fishman EK. Current concepts in the diagnosis and management of
renal cell carcinoma: role of multidetector ct and three-dimensional CT. Radiographics 2001; 21 Spec No:S237254.
6. Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologicpathologic correlation. Radiographics 1995; 15:653-669.
7. Israel GM, Bosniak MA, Slywotzky CM, Rosen RJ. CT differentiation of large exophytic renal angiomyolipomas
and perirenal liposarcomas. AJR Am J Roentgenol 2002; 179:769-773.
8. Urban BA, Fishman EK. Renal lymphoma: CT patterns with emphasis on helical CT. Radiographics 2000; 20:197212.
9. Wong-You-Cheong JJ, Wagner BJ, Davis CJ, Jr. Transitional cell carcinoma of the urinary tract: radiologicpathologic correlation. Radiographics 1998; 18:123-142; quiz 148.
10. Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal angiomyolipoma: relationships
between tumor size, aneurysm formation, and rupture. Radiology 2002; 225:78-82.
Renal Neoplasms
572
Urinary Tract Trauma

GU Trauma
Evaluate lower tract before upper tract if both may be injured

Males always perform retrograde urethrogram before foley is inserted if there
is blood at the meatus or pubic rami fx/diastasis
Figure 3-3-1
Male Urethra [Figure 3-3-1]
Posterior
Prostatic
Membranous
Anterior
Bulbous
Penile
Retrograde Urethrogram: RUG

[Figure 3-3-2]
Inflate pediatric foley (35cc) in fossa navicularis

50 cc of 30%60% contrast
Inject 2030 cc and take film while continuing to inject
Oblique if possible or gently move penis laterally
May perform pericatheter RUG if foley is in place
Figure 3-3-2
Normal urethral anatomy
Figure 3-3-3
Intact urethra
Urethral Trauma
[Figures 3-3-3 to 3-3-8]
Posterior pelvic fractures

I stretch
II rupture above UGD
retropubic extravasation
III rupture above and below
UGD
perineal/scrotal
extravasation
IV bladder neck and urethra
Type 1 - Stretch injury
Figure 3-3-4
Figure 3-3-5
Type II rupture above UGD

573
Figure 3-3-7
Figure 3-3-6
Type III rupture

above and below
UGD
Type V Anterior urethral trauma [Figures 3-3-9 and 3-3-10]
Figure 3-3-8
Bony injury uncommon

Partial/complete
Corpora/venous extravasation
Associated scrotal trauma
Figure 3-3-9
Figure 3-3-10
Type IV Bladder neck and

urethra
Type V Anterior urethral trauma (straddle injury)
Complications
Strictures
Fistulas
Bladder Trauma
Blunt or penetrating
5%10% of pubic rami fx
Pelvic fractures in 80% of ruptures
83% of extraperitoneal
62% of intraperitoneal
Bladder Trauma Evaluation
Standard cystogram
300500cc
incomplete filling may miss leak
15%30% I concentration
AP, obliques
post drainage important for small leaks
CT
clamp foley
delayed images, post drain
574
Extraperitoneal Bladder
Rupture
[Figures 3-3-11 and 3-3-12]
Figure 3-3-11
Figure 3-3-12
60%
focal extravasation,
flame-shaped
conservative therapy
Intraperitoneal Bladder
Rupture
[Figures 3-3-13 and 3-3-14]
40%
free flowing extravasation,
outlines intraperitoneal
organs
surgical therapy
Extaperitoneal bladder rupture
Bladder Trauma Evaluation

[Figure 3-3-15 and 3-3-16]
CT cystogram
perform routine CT
drain bladder
refill with 2%3% I
solution (300 cc)
scan full and post
drain
Figure 3-3-13
Figure 3-3-14
Intraperitoneal bladder rupture
Figure 3-3-15
Figure 3-3-16
Intraperitoneal bladder rupture

on CT cystogram
Ureteral Injury
Least common site of injury (<3%)

Penetrating trauma anywhere
UPJ disruption
Extraperitoneal bladder rupture on CT cystogram
575
Renal Injuries
[Figures 3-3-17 to 3-3-21]
Figure 3-3-17
Category I - Minor (85%)

contusion
intrarenal hematoma
small subcapsular/perirenal hematoma
segmental infarction
superficial laceration
Conservative management
Figure 3-3-18
Figure 3-3-19
Hematoma
Figure 3-3-20
Subcapsular hematoma with

delayed nephrogram
Subcapsular hematoma has

an abrupt start and stop
point and deforms renal
parenchyma
Figure 3-3-21
Renal Injuries [Figures 3-3-22 and 3-3-23]
Category II - Serious (10%)

deep lacerations
laceration through the collecting
system
large perinephric/subcapsular
hematoma
Conservative management vs. surgery
Figure 3-3-22
Laceration breaks through
renal capsule and causes a
perinephric hematoma
Figure 3-3-23
Segmental infarction
Laceration into the collecting

system
Laceration into the collecting

system with urine leak
576
Renal Injuries [Figures 3-3-24 to 3-3-30]
Category III - Catastrophic

fractured/shattered kidney
renal artery occlusion/avulsion
renal vein occlusion/avulsion
UPJ avulsion
Often surgical treatment
Figure 3-3-24
Figure 3-3-25
Figure 3-3-26
Shattered kidney
Vascular avulsion
Figure 3-3-29
Vascular avulsion with contrast
extravasation
Figure 3-3-27
Figure 3-3-28
Acute arterial thrombosis with
subsequent development
of a rim sign
Figure 3-3-30
Renal artery thrombosis.

Non-functioning normal sized
kidney
Rim sign: collateral
circulation forming after
thrombosis
Renal vein thrombosis. Kidney

will be enlarged
577
UPJ disruption [Figures 3-3-31 and 3-3-32]
Deceleration injury
3:1, children:adults
3:1, R:L
Stent, nephrostomy, surgery
Figure 3-3-31
Figure 3-3-32
UPJ disruption. Leak obvious on

delayed films
UPJ disruption
Conclusion
If there is blood at the meatus, perform urethrogram first

A normal bladder on CT does not rule out a leak
Post-drainage films are key for small leaks
Dont forget delayed images
References
1.
Ali M, Safriel Y, Sclafani SJ, Schulze R. CT signs of urethral injury. Radiographics 2003; 23:951-963; discussion
963-956.
2. Blankenship B, Earls JP, Talner LB. Renal vein thrombosis after vascular pedicle injury[clin conference]. AJR Am
J Roentgenol 1997; 168:1574.
3. Fishman EK, Horton KM. CT evaluation of bladder trauma: a critical look. Acad Radiol 2000; 7:309-310.
4. Goldman SM, Sandler CM, Corriere JN, Jr., McGuire EJ. Blunt urethral trauma: a unified, anatomical mechanical
classification. J Urol 1997; 157:85-89.
5. Herschorn S, Radomski SB, Shoskes DA, Mahoney J, Hirshberg E, Klotz L. Evaluation and treatment of blunt
renal trauma. J Urol 1991; 146:274-276; discussion 276-277.
6. Kamel IR, Berkowitz JF. Assessment of the cortical rim sign in posttraumatic renal infarction. J Comput Assist
Tomogr 1996; 20:803-806.
7. Kawashima A, Sandler CM, Corriere JN, Jr., Rodgers BM, Goldman SM. Ureteropelvic junction injuries
secondary to blunt abdominal trauma. Radiology 1997; 205:487-492.
8. Kawashima A, Sandler CM, Corl FM, et al. Imaging of renal trauma: a comprehensive review. Radiographics
2001; 21:557-574.
9. McAndrew JD, Corriere JN, Jr. Radiographic evaluation of renal trauma: evaluation of 1103 consecutive patients.
Br J Urol 1994; 73:352-354.
10. Nunez D, Jr., Becerra JL, Fuentes D, Pagson S. Traumatic occlusion of the renal artery: helical CT diagnosis. AJR
Am J Roentgenol 1996; 167:777-780.
11. Roberts JL. CT of abdominal and pelvic trauma. Semin Ultrasound CT MR 1996; 17:142-169.
578
Retroperitoneum
Figure 3-4-1
Retroperitoneum
Non-neoplastic
Fluid collections
Pancreatic, urinoma, hematoma, abscess
Retroperitoneal fibrosis
Lymphadenopathy
Inflammatory/infectious
Castleman disease
Lymphoma
Metastatic adenopathy
Organs
Pancreas, colon, duodenum
Kidneys, adrenal, ureters
Primary ( > 100 benign and malignant tumors)
Neurogenic
Nerve sheath, ganglioneuroma,
ganglioneuroblastoma, neuroblastoma
Paraganglioma
Mesenchymal
Lipoma/sarcoma, leiomyoma/sarcoma, malignant
fibrous histiocytoma (MFH), lymphangioma,
hemangioma, hemangiopericytoma, angiosarcoma
Germ cell
Teratoma (benign and malignant)
Retroperitoneal spaces
Figure 3-4-2
Anatomy: 3 spaces [Figure 3-4-1]
Anterior pararenal
Perirenal
Posterior pararenal
Updated view of the perirenal space with

complex fascial boundaries
Anterior Pararenal Space (the GI space)
Colon (ascending and descending)

Pancreas
Duodenum (2nd and 3rd portions)
Figure 3-4-3
Perirenal Space (the GU space) [Figure 3-4-2]
Kidneys
Adrenal glands
Upper portion of ureters
Posterior Pararenal Space (the nothing space)
No solid organs
Fat, connective tissue, nerves
Borders [Figure 3-4-3]
Parietal peritoneum separates peritoneal space from APRS

Anterior renal fascia separates APRS from perirenal space (Gerotas
fascia)
Posterior renal fascia separates PPRS from perirenal space
(Zuckerkandls fascia)
Lateral conal fascia demarcates the lateral
Sagittal view of the
extent of the APRS
retroperitoneum. All 3
Separates the APRS from the PPRS
compartments
All spaces communicate inferiorly
communicate inferiorly
579
Retroperitoneum
Retroperitoneal Fibrosis
Microscopically: collagen, fibroblasts, and inflammatory cells

Typical distribution: below kidneys to bifurcation
40% may have atypical distribution
8%10% malignant
Desmoplastic reaction to infiltrating metastases
Breast, lung, colon, prostate, cervix
Prognosis poor (3-6 months)
Figure 3-4-4
Retroperitoneal Fibrosis: Etiology
2/3 idiopathic (Ormands Disease)

Methysergide toxicity
Aortic aneurysm
Surgery
Hemorrhage
Radiation/surgery
Fibrosing conditions elsewhere
Retroperitoneal Fibrosis [Figure 3-4-4]
IVP, retrogrades
Medial deviation of ureters
Hydronephrosis
Retroperitoneal Fibrosis [Figure 3-4-5]
CT
Wispy plaque-like deposits to confluent masses
Enhancement variable
Aorta is encased but not deviated
MR
Fibrotic phase
Low on T1 and T2
No enhancement
Active phase
High on T2
Enhancement
Can not rule out malignancy
Must biopsy
RPF with medial deviation of the ureters and

hydronephrosis
Figure 3-4-5
Treatment
Stents
Steroids
Immunosuppression
Surgery
Neurogenic Tumors
Nerve sheath
Schwannoma (neurilemmoma), neurofibroma,
malignant nerve sheath tumor
Ganglionic
Ganglioneuroma, ganglioneuroblastoma,
neuroblastoma
Paraganglionic
Paraganglioma (pheochromocytoma)
Neurogenic Tumors
Paraspinal masses
Mass often elongated and well-defined
RPF with low signal on T2WI
Smooth or mildly lobular
Generally benign
Rapid growth, increased vascularity, poorly circumscribed suggest malignancy
Retroperitoneum
580
Neurogenic Tumors
Figure 3-4-6
Low density on CT
Low signal on T1WI
May be hyperintense on T2WI (myxoid matrix)
May have calcifications
Nerve sheath tumors [Figure 3-4-6]
Often appears as psoas mass

Look at neuroforamen
May have intraspinal (extradural) extension
Multiple consider neurofibromatosis
Ganglion Cell Tumors [Figure 3-4-7]
Form from primitive neural crest cells

Sympathoblast
Neuroblastoma
Ganglioneuroma
Pheochromoblast
Paraganglioma
Nerve sheath tumor presenting as a psoas mass
Figure 3-4-7
Ganglioneuroma
Benign
More common in mediastinum
Generally asymptomatic
Elongated low-density masses
Maybe be hyperintense on T2WI
Paraganglioma
(Extra-adrenal pheochromocytoma)
About 10% of pheochromocytomas

Most (60%80%) have known catecholamine excess
Hypertension, palpitations, sweating, tremor, diarrhea,
nausea
More commonly malignant than adrenal pheochromocytomas
Paraganglioma [Figure 3-4-8]
Organs of Zuckerkandl
CT non-specific
Enhance avidly
Contrast contraindicated
High signal on T2 is suggestive but is not universally seen
Uptake on MIBG scan
Figure 3-4-8
Sympathetic chain
Paraganglioma with high signal on T2WI

581
Retroperitoneum
Leiomyosarcoma [Figure 3-4-9]
More commonly necrotic than other tumors

May have intravascular invasion
May arise in the wall of the IVC
Figure 3-4-9
Leiomyosarcoma with IVC invasion
MFH / Malignant Fibrous Histiocytoma [Figure 3-4-10]
Generally large masses

Necrosis less common
T2WI helpful Bowl of fruit sign
Mosaic of low and high signal
Fibrous tissue low
Myxoid stroma hyperintense
Soft tissue - intermediate
Lymphangioma
Figure 3-4-10
[Figure 3-4-11]
Benign
Fluid-filled
Uni-multiloculated
Insinuates itself around organs
Can be huge
Figure 3-4-11
MFH with bowl of fruit on T2WI
Lymphangioma
Retroperitoneum
582
Liposarcoma [Figure 3-4-12]
Figure 3-4-12
The most common primary retroperitoneal tumor

85% have fat detected by CT or MR
Well-differentiated, pleomorphic, myxoid, de-differentiated
Poorly differentiated tumors have no detectable fat by
imaging studies
Often present late
Weight gain
Infiltrative margins
Complete surgical excision may be difficult
Local recurrence common
Hyperintense T2WI: Things with a myxoid matrix
Neurogenic tumors
Myxoid liposarcoma
Liposarcoma
Figure 3-4-13
Germ Cell Tumors
Teratoma
Mature
Immature
Malignant germ cell tumors
Teratoma
Most are mature (benign) and are cured by surgery

Children (less than 6 months) and young adults
(1525 years)
Female:male = 3:1
Teratoma [Figures 3-4-14 and 3-4-15]
Fat (sebum or adipose tissue)

Calcification in 90% (may be clump-like)
Cystic portion in 75%
Figure 3-4-14
Myxoid liposarcoma with high signal on T2WI
Figure 3-4-15
Embryo with germ cells in the

retroperitoneum
Retroperitoneal teratoma
583
Retroperitoneum
Malignant germ cell tumors are more common in males and are
much more likely to be secondary to a testicular tumor (rather
than primary retroperitoneal)
Fat containing retroperitoneal masses
Liposarcoma: large, heterogeneous

Teratoma: young patients, calcification, cystic area
Myelolipoma: usually arising from adrenal
Angiomyolipoma: arises from kidney, but attachment may be hard to find
References
1.
2.
3.
4.
5.
6.
Engelken JD, Ros PR. Retroperitoneal MR imaging. Magn Reson Imaging Clin N Am 1997; 5:165-178.
Granstrom P, Unger E. MR imaging of the retroperitoneum. Magn Reson Imaging Clin N Am 1995; 3:121-142.
Kim T, Murakami T, Oi H, et al. CT and MR imaging of abdominal liposarcoma. AJR Am J Roentgenol 1996; 166:829833.
Nishimura H, Zhang Y, Ohkuma K, Uchida M, Hayabuchi N, Sun S. MR imaging of soft-tissue masses of the
extraperitoneal spaces. Radiographics 2001; 21:1141-1154.
Nishino M, Hayakawa K, Minami M, Yamamoto A, Ueda H, Takasu K. Primary retroperitoneal neoplasms: CT and
MR imaging findings with anatomic and pathologic diagnostic clues. Radiographics 2003; 23:45-57.
Morton A. Meyers. Dynamic Radiology of the Abdomen. Springer-Verlag. A must read book in your residency
Retroperitoneum
584
Radiologic Evaluation of the Scrotum

Embryology
Sex is chromosomally determined at fertilization

No morphologic differentiation until week 7 (indifferent stage)
Testis determining factor on short arm of Y chromosome
induces formation of seminiferous tubules
Figure 3-5-1
3 Components of Gonad [Figure 3-5-1 to 3-5-4]
Germ cells
arise from yolk sac
migrate to genital ridges
Mesothelium
primitive sex cord
Sertoli cells
Mesenchyme
Interstium
Leydig cells
Migration of germ cells along the

hindgut to the genital ridges
Figure 3-5-3
Figure 3-5-2
Normal seminiferous tubules
Figure 3-5-4
Embryologic formation of the testes
Leydig cells secrete testosterone

stimulate mesonephric ducts
Sertoli cells secrete mullerian-inhibiting factor
Sex organs at indeterminate stage

585
Mesonephric (Wolffian) Ducts
Epididymis
Vas deferens
Ejaculatory ducts
Seminal vesicles
Figure 3-5-5
Embryologic Remnants
Mullerian
appendix testis
Wolffian
appendix epididymis
Testis [Figure 3-5-5]
200-300 lobules
Seminiferous tubules (300-980 meters)
Efferent ductules (15-20) converge at mediastinum
Epididymis
Form single convoluted tubule in head (600 cm)

Tail loosely attached inferiorly
Exits as single tube
Normal testis
Spermatic cord [Figure 3-5-6]
Figure 3-5-6
Vas deferns
Testicular, deferential, cremasteric arteries
Pampiniform plexus
Nerves, lymphatics
Ultrasound
Testes
homogeneous low level echoes
linear echogenic mediastinum testis
Epididymis
globus major (head), body, tail
iso- to slightly hyperechoic
MRI
T1WI - homogeneous intermediate signal

T2WI - high signal with low signal mediastinum testis and linear
septations
MRI [Figure 3-5-7]
Tumors are low signal masses
Spermatic cord
Goals of Ultrasound
Intra-testicular vs. extra-testicular

Cyst vs. solid
Figure 3-5-7
Testicular Neoplasms
Germ Cell Neoplasms (95%)

Sex cord, Stromal Tumors
Sertoli cell
Leydig cell
Lymphoma
Metastases
Epidermoid Cysts
Testicular tumors are low signal on T2WI

586
Germ Cell Neoplasms
Figure 3-5-8
Seminoma (most common pure tumor)

Embryonal Cell Carcinoma
Yolk Sac Tumor (Endodermal Sinus Tumor)
Teratoma
Choriocarcinoma
MGCT - Mixed Germ Cell Tumor (most common
overall)
Seminoma [Figure 3-5-8]
Homogeneous, well-defined
May be lobular and multifocal
Bilateral 2%
Peak age 30-40 years
Radiosensitive
Good prognosis
Seminoma
Non Seminomatous Germ Cell Tumor [Figure 3-5-9]
Embryonal
Rare in pure form
87% of MGCT
Yolk Sac (endodermal sinus tumor)
Most common childhood tumor
44% of MGCT
Teratoma
Mature and immature (always malignant in
adults)
Cysts/calcifications common features
Choriocarcinoma
Very rare
Dismal prognosis
Figure 3-5-9
Non Seminomatous Germ Cell Tumor
MGCT with large amount of teratoma

Mixed germ cell tumors much more common than
any pure tumor
Heterogeneous, ill-defined
Peak age 20s
Not radiosensitive
Often higher stage and less favorable prognosis than seminoma
Germ Cell Tumors: Modes of Spread
Lymphatic
ipsilateral renal hilum
Hematogeous
common with choriocarcinoma
lung, liver, brain
Tumor Markers
Alpha-fetoprotein (AFP)
from fetal liver, GI tract, and yolk sac
elevated in tumors with yolk sac elements
Human chorionic gonadotropin (HCG)
produced by syncytiotrophoblasts from developing placenta
elevated in tumors with choriocarcinoma (occasional seminoma)
LDH
Non-specific, correlates with bulk of disease
Elevated in 80% of non-seminomatous tumors
587
Burned-Out Germ Cell Tumor
Presents with metastases (often extensive)

The primary may not contain any active tumor and
may be difficult to identify
Orchiectomy performed if any suspicious area seen
Figure 3-5-10
Testicular Microlithiasis
0.6% in general population

Present in 40% of germ cell tumors
Usually bilateral
Consider annual screening
Risk Factors for Testicular Carcinoma
Prior testicular tumor

Cryptorchidism
Infertility
Family history
Intersex syndormes (hermaphrodite)
??? microcalcifications
Bilateral undescended testes
Figure 3-5-11
Cryptorchidism [Figure 3-5-10]
5% testicular agenesis
65% migratory testis
30% undescended
Increased incidence of malignancy
Risk is also increased in the contralateral testis
Sex Cord, Stromal Tumors [Figure 3-5-11]
Sertoli (sex cord) and Leydig (stromal)

90% benign
More common in pediatric age group
May be be hormonally active
precocious puberty, gynecomastia
more common with Leydig
Sertoli cell tumors may be bilateral and calcified
Sertoli cell tumor with calcification
Figure 3-5-12
Testicular Lymphoma Presentation
Most common testis tumor > 60 yo

5% of testicular neoplasms
< 1% of patients with lymphoma
Often presents as the site of recurrent disease because of
blood-testis barrier (Sertoli cells)
Testicular Lymphoma Imaging [Figure 3-5-12]
Most common bilateral tumor

Homogeneous
Epididymis and spermatic cord often involved
Lymphoma with bilateral testicular

masses
Epidermoid Cyst [Figure 3-5-13]
Benign
? germ cell tumor
Filled with keratin
Well-defined
Ringed-appearance
Can not differentiate from a malignant neoplasm
May do focal resection rather than orchiectomy
Figure 3-5-13
Concentric rings in a
epidermoid cyst
588
Tumors Summary
Children
Yolk sac tumor
Sertoli, Leydig cell
Younger men (20s)
Mixed germ cell tumor
Heterogeneous, poorly defined
Somewhat older (30s)
Seminoma
Homogeneous
Older males (> 60 yo)
Lymphoma
Bilateral
May involve paratesticular structures
Figure 3-5-14
Non-neoplastic Testicular Masses
Tubular ectasia
Cysts
Sarcoidosis
Adrenal rests
Acute scrotum
infection
infarction
trauma
Tubular ectasia with an intratesticular cyst
Figure 3-5-15
Tubular Ectasia
Dilatation of the rete testis

Often bilateral
Associated with a spermatocele
Tubular US appearance
Iso- to hyperintense on T2WI
Testicular Cysts [Figure 3-5-14]
Peripheral
Tunica albuginea cyst
Central
Must be careful to differentiate from cystic
neoplasm
Can not have any solid component
Often associated with dilated rete testis
Sarcoidosis
Sarcoidosis with multiple testicular masses
[Figure 3-5-15]
Multisystem chronic granulomatous disorder

5% will have genital involvement
Epididymis more commonly involved
More common in Blacks (testicular tumors are rare)
Figure 3-5-16
Adrenal rest hypertrophy secondary to

congenital adrenal hyperplasia [Figure 3-5-16]
Adrenal rests in 7.5-15% of newborns, 1.6% adults

Hypertrophy when exposed to elevated ACTH
Bilateral, multiple, eccentric
Tx glucocorticoids not orchiectomy
Bilateral Testicular Masses
Lymphoma
Seminoma (rarely)
Metastases
Sarcoidosis
Adrenal rests
Developing adrenals. Adrenal tissue may become

entrapped within the developing testis
589
Extratesticular Scrotal Masses [FigureS 3-5-17 and 3-5-18]

Figure 3-5-17
Figure 3-5-18
Adult scrotum. Processus vaginalis

closes to form tunica vaginalis
Developing scrotum. Testicular descent is aided by

the processus vaginalis
Hydrocele
Fluid between the parietal and visceral layers of the tunica vaginalis
Small amount is normal
Hydrocele
Figure 3-5-19
Congenital
Patent processus vaginalis, may have an
inguinal hernia
Acquired
Infection, infarction, trauma, tumor
Scrotal Calculi
Torsion of appendix or inflammatory deposits

Repeated microtrauma
bikers
Variable size and calcification
Mobile
Epididymal Masses
Cyst, Spermatocele [Figure 3-5-19]

Infection
Bacterial (acute)
TB (chronic)
Tumors
Adenomatoid tumor
Papillary cystadenoma (von Hippel-Lindau)
Lymphoma
Sarcoidosis
Epididymal cyst
Figure 3-5-20
Adenomatoid Tumor [Figure 3-5-20]
Benign
Most common epididymal tumor
Solid, small, well-circumscribed
Papillary Cystadenoma
Associated with VHL (70%)

40% bilateral
Benign
Adenomatoid tumor
590
Epididymal and Testicular Mass
Figure 3-5-21
Lymphoma
Testicular involvement greater than epididymis
Sarcoidosis [Figure 3-5-21]
Epididymal involvement greater than testis
More common in Blacks
Infection
Bacterial (acute)
TB (chronic)
Tuberculosis
Epididymis primary site with testis secondarily

involved
30% bilateral
50% will have abscess or fistulas
Sarcoidosis with marked epididymal enlargement
Acute Scrotum
Trauma
Epididymitis/orchitis
Torsion
Acute Epididymitis
Bacterial infection from lower urinary tract - chlamydia, gonococcus, E coli

US findings - enlarged, hypoechoic, hyperemia, hydrocele, skin thickening
20% have associated orchitis
Orchitis
Usually secondary to epididymitis

May rarely be focal
US findings - enlarged, heterogeneous echogenicity, hyperemia
May lead to focal ischemia/infarction
Fournier Gangrene
Diabetics or other immunosuppression

Scrotal abscess with necrotizing infection of the perineum
Surgical emergency
Torsion
Gray scale US may be normal early

Decreased or absent flow with Doppler
Compare with normal side
Venous compromise occurs first
Look for mass in inguinal canal
Testis becomes enlarged and hypoechoic with time
Torsion
< 6 hrs at diagnosis salvage rate 80%-100%

12 hr salvage rate 20%
Paratesticular masses
Varicocele
Fibrous pseudotumors
Polyorchidism
Neoplasms
Lipomas
Half of all spermatic cord tumors
Liposarcoma
Rhabdosarcoma, leiomyosarcoma, MFH
Mesothelioma
591
Varicocele [Figure 3-5-22]
Figure 3-5-22
> 3mm
Idiopathic
incompetent valves
more common on left (bilateral 10%)
longer course
more perpendicular insertion
nutcracker effect of left renal vein under SMA
Secondary to abdominal mass
Varicocele
15% of general population

40% of men with infertility
? increased temperature
Improved pregnancy rates (35%-50%) with repair even if
subclinical
Large varicocele
Fibrous Pseudotumor [Figure 3-5-23]
Figure 3-5-23
Hylanized collagen and granulation tissue

Attached to tunica albuginea
US non-specific
MRI low signal intensity
Polyorchidism
Abnormal division of genital ridge

Often abnormal spermatogenesis
Increased risk of torsion
Paratesticular Neoplasms
Lipomas [Figure 3-5-24]

Rhabdosarcoma
Leiomyosarcoma
MFH
Mesothelioma
Lipoma [Figure 3-5-24]
Most common extratesticular neoplasm

Half of all cord tumors
Variable by ultrasound
may be homogenously hypoechoic
MR with fat suppression helpful
Fibrous pseudotumor. Round lowsignal mass involving the tunica

albuginea
Figure 3-5-24
Figure 3-5-25
Liposarcoma
Hypoechoic lipoma
592
Mesothelioma [Figure 3-5-26]
Figure 3-5-26
Tunica vaginalis lined with mesothelial cells

Much less common then pleural or peritoneal
Benign and malignant
Often present with hydrocele
Mesothelioma with nodules and

hydrocele
References
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2.
3.
4.
5.
6.
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Doherty FJ. Ultrasound of the nonacute scrotum. Semin Ultrasound CT MR 1991; 12:131-156.
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1991; 179:55-59.
Kassis A. Testicular adenomatoid tumours: clinical and ultrasonographic characteristics. BJU Int 2000; 85:302304.
Kim ED, Lipshultz LI. Role of ultrasound in the assessment of male infertility. J Clin Ultrasound 1996; 24:437453.
Kutchera WA, Bluth EI, Guice SL. Sonographic findings of a spermatic cord lipoma. Case report and review of the
literature. J Ultrasound Med 1987; 6:457-460.
Mattrey RF. Magnetic resonance imaging of the scrotum. Semin Ultrasound CT MR 1991; 12:95-108.
Ragheb D, Higgins JL, Jr. Ultrasonography of the scrotum: technique, anatomy, and pathologic entities. J
Ultrasound Med 2002; 21:171-185.
Sudakoff GS, Quiroz F, Karcaaltincaba M, Foley WD. Scrotal ultrasonography with emphasis on the
extratesticular space: anatomy, embryology, and pathology. Ultrasound Q 2002; 18:255-273.
Tessler FN, Tublin ME, Rifkin MD. Ultrasound assessment of testicular and paratesticular masses. J Clin
Ultrasound 1996; 24:423-436.
Woodward PJ, Schwab CM, Sesterhenn IA. From the archives of the AFIP: extratesticular scrotal masses:
radiologic-pathologic correlation. Radiographics 2003; 23:215-240.
Woodward PJ, Sohaey R, O'Donoghue MJ, Green DE. From the Archives of the AFIP: Tumors and Tumorlike
Lesions of the Testis: Radiologic-Pathologic Correlation. Radiographics 2002; 22:189-216.
593
First Trimester Ultrasound

Ovarian Period: (Weeks 12)
Ovarian follicle matures

Ovulation
Corpus luteum formation
Figure 3-6-1
Conceptus Period: (Week 35)
Fertilization
Morula (16 cells)
Blastocyst
Trilaminar embryo
Embryonic Period: (Weeks 610)
C-shaped embryo
Major organs develop
Yolk sac detaches
Intradecidual sac sign
Fetal Period: (Weeks 1112)
Fetal growth
Amniotic and chorionic membranes approach each other
Gestational Sac
Visualized as early as 4 - 4.5 wks (TV)

Intradecidual sac sign [Figure 3-6-1]
Double decidual sac sign [Figure 3-6-2]
Basalis [DB]
Capsularis [DC]
Parietalis [DP]
Gestational Sac = Chorionic Sac

Chorionic laeve = Smooth chorion = Chorionic membrane

Chorionic frondosum + Decidua basalis = Placenta
The sac is eccentrically located with

respect to the endometrial cavity
Figure 3-6-2
Figure 3-6-3
Double decidual sac sign
Chorionic sac with chorionic

frondosum and laeve (smooth
chorion)
594
Figure 3-6-4
Series of 5 illustrations showing
normal 1st trimester development
with expansion of the amnion and
detachment of the yolk sac
595
Yolk sac [Figure 3-6-5 ]
Figure 3-6-5
Visualized at 5 5.5 weeks
Embryo [Figure 3-6-6 ]
Visualized 6 6.5 wks
Major First Trimester Landmarks [Figures 3-6-7 and 3-6-8]
Gestational sac
Yolk sac
Embryo
4.5 wks
5.5 wks
6.5 wks
Multiple Gestations
Figure 3-6-6
Normal yolk sac at 5.5 wks
Types of Twinning
Dizygotic (70%)
2 eggs
Monozygotic (70%)
single egg
Figure 3-6-7
Multiple Gestations
# of chorions equals
# of placentas
sharing is bad
Normal 6.5 week embryo with
risk for twin/twin
double bleb or diamond ring sign
transfusion
# of amnions equals
# of separate sacs
sharing is really bad
risk for cord accidents
Dizygotic Twins [Figure 3-6-9]
8 week embryo surrounded by

amnion
Figure 3-6-8
Dizygotic must be dichorionic (2 placentas)

and diamniotic (2 sacs)
Figure 3-6-9
Normal first trimester US including rhombencephalon

and bowel herniation
Dichorionic, diamniotic twins
596
Monozygotic Twins
1/3 are Dichorionic/Diamniotic [Figure 3-6-10]

cleavage by day 3
2/3 are Monochorionic/Diamniotic [Figures 3-6-11 and 3-6-12]
cleavage day 4-8
Rare (approx 1%) Monochorionic/Monoamniotic [Figure 3-6-13]]
cleavage > 8 days
Conjoined twins
cleavage > 14 days
Figure 3-6-10
Figures 3-6-11 and 3-6-12

Dichorionic, diamniotic twins
Figure 3-6-13
Monochorionic, monoamniotic twins
Monochorionic, diamniotic twins
AIUM Guidelines: First Trimester
Gestational sac
Location
Mean Sac Diameter (MSD)
MSD = (L+W+D)/3
Yolk sac
Embryo
Crown rump length
Cardiac activity
Fetal number
Chorionicity/Amnionicity
Uterus, adnexa, cul-de-sac
Threshold Level - the size at which a finding may be seen
Discriminatory Level - the size at which a finding must be seen
597
Threshold
Level MSD
Discriminatory
Level MSD
5 mm
TV10 mm
TA 20 mm
TV 18 mm
TA 25 mm
5 mm (CRL)
Gestational Sac
Yolk Sac
2 mm
TV 4 mm
Embryo
TV 8 mm
Heartbeat
2 mm (CRL)
Major: Discriminators
MSD > 10 mm must have a yolk sac

MSD > 18 mm must have an embryo
CRL > 5mm must have a heartbeat
Cardiac Activity
Must be present if embryo is > 5 mm

56 weeks
100110 bpm
89 weeks
150170 bpm
Abnormal Frist Trimester
25% threatened abortion

Embryonic demise
Bradycardia
Anembryonic pregnancy [Figure 3-6-14]
Perigestational hemorrhage [Figure 3-6-15]
Abnormal yolk sac
Poor growth
Figure 3-6-14
Anembryonic Pregnancy [Figure 3-6-14]
Major discriminators
MSD > 10 mm without a yolk sac
MSD > 18 mm without a fetal pole
Minor discriminators
weak decidual reaction
abnormal shape or location
empty amnion
Yolk Sac
First landmark in gestational sac

In the chorionic cavity
Abnormal findings:
>6mm
irregular shape
calcifications
multiple yolk sacs
Anembryonic pregnancy
with empty amnion
Figure 3-6-15
Growth
Normal growth rate 1 mm per day
Ectopic pregnancy
Tubal 95%
Unusual locations 5%
Interstitial
Cervix
Ovary
Abdominal
1:50-1:200 live births
Risks factors: IUD, prior ectopic, PID, tubal surgery,
infertility treatment
Perigestational hemorrhage
598
Ectopic pregnancy: Uterine Findings
Figure 3-6-16
[Figures 3-6-16 and 3-6-17]
No gestational sac
Thickened endometrium
Pseudogestional sac
Discriminatory hCG levels
hCG >1,000 IU/L (2nd IS)
hCG >2,000 IU/L (3rd IRP)
Figure 3-6-17
Double decidual sac sign vs. pseudosac
Figure 3-6-18
Ectopic with echogenic ring

and blood in the cul-de-sac
Pseudosac
Ectopic Pregnancy: Adnexal Findings

[Figure 3-6-18]
Living extrauterine embryo

Echogenic ring +/- yolk sac
ring of fire
Adnexal mass (clot)
Cul-de-sac blood
Normal adnexa
Figure 3-6-19
Heterotopic Pregnancy
1 in 30,000 spontaneous pregnancies

1 in 4,000 assisted pregnancies
Interstitial Pregnancy [Figure 3-6-19]
Isthmus
Rupture later catastrophic bleeding
Eccentric
Lack of encircling myometrium
Interstitial line sign
Cornual ectopic with interstitial line sign
599
Management of Ectopic Pregnancy
Surgical
Salpingectomy
Salpingostomy
Medical
Systemic methotrexate
Intragestational methotrexate
Intragestational KCI
Systemic Methotrexate
Preserves fallopian tube

Non-invasive
Outpatient
Criteria
Mass < 4cm
No bleeding
hCG <3,000 IU/L (2IS)
No formed fetal parts
Figure 3-6-20
Cutting Edge
Sonoembryology
Early diagnosis of major malformations
Screen for aneuploidy
nuchal translucency
hypoplastic nasal bone
abnormal flow in ductus venosus
Nuchal Translucency
Screen for Trisomy 21 [Figure 3-6-20]
Accredited lab
11-14 weeks
>3mm abnormal
Risk assessment based on age, NT, serum screen
High detection rates (75%-90%)
Increased nuchal translucency in

Down syndrome
References
1.
Ackerman TE, Levi CS, Dashefsky SM, Holt SC, Lindsay DJ. Interstitial line: sonographic finding in interstitial
(cornual) ectopic pregnancy. Radiology 1993; 189:83-87.
2. Brown DL, Emerson DS, Felker RE, Cartier MS, Smith WC. Diagnosis of early embryonic demise by endovaginal
sonography. J Ultrasound Med 1990; 9:631-636.
3. Brown DL, Doubilet PM. Transvaginal sonography for diagnosing ectopic pregnancy: positivity criteria and
performance characteristics. J Ultrasound Med 1994; 13:259-266.
4. Dickey RP, Olar TT, Curole DN, Taylor SN, Matulich EM. Relationship of first-trimester subchorionic bleeding
detected by color Doppler ultrasound to subchorionic fluid, clinical bleeding, and pregnancy outcome. Obstet Gynecol
1992; 80:415-420.
5. Frates MC, Brown DL, Doubilet PM, Hornstein MD. Tubal rupture in patients with ectopic pregnancy: diagnosis
with transvaginal US. Radiology 1994; 191:769-772.
6. Frates MC, Benson CB, Doubilet PM, et al. Cervical ectopic pregnancy: results of conservative treatment. Radiology
1994; 191:773-775.
7. Frates MC, Laing FC. Sonographic evaluation of ectopic pregnancy: an update. AJR Am J Roentgenol 1995; 165:251259.
8. Jarjour L, Kletzky OA. Reliability of transvaginal ultrasound in detecting first trimester pregnancy abnormalities.
Fertil Steril 1991; 56:202-207.
9. Jurkovic D, Gruboeck K, Campbell S. Ultrasound features of normal early pregnancy development. Curr Opin Obstet
Gynecol 1995; 7:493-504.
10. Nyberg DA, Mack LA, Laing FC, Patten RM. Distinguishing normal from abnormal gestational sac growth in early
pregnancy. J Ultrasound Med 1987; 6:23-27.
600
11. Nyberg DA, Filly RA, Laing FC, Mack LA, Zarutskie PW. Ectopic pregnancy. Diagnosis by sonography correlated
with quantitative HCG levels. J Ultrasound Med 1987; 6:145-150.
12. Oh JS, Wright G, Coulam CB. Gestational sac diameter in very early pregnancy as a predictor of fetal outcome.
Ultrasound Obstet Gynecol 2002; 20:267-269.
13. Rempen A. Diagnosis of viability in early pregnancy with vaginal sonography. J Ultrasound Med 1990; 9:711-716.
14. Sohaey R, Woodward P, Zwiebel WJ. First-trimester ultrasound: the essentials. Semin Ultrasound CT MR 1996; 17:214.
15. van Leeuwen I, Branch DW, Scott JR. First-trimester ultrasonography findings in women with a history of recurrent
pregnancy loss. Am J Obstet Gynecol 1993; 168:111-114.
601
Fetal CNS Malformations

AIUM: Fetal Brain [Figure 3-7-1]
Figure 3-7-1
Ventricular Plane
atrium and choroid
BPD Plane
thalami
third ventricle
cavum septi pellucidi
Posterior Fossa
cerebellum
cisterna magna
nuchal skin thickeness
Fetal MRI
Fast T2WI (SSFSE, HASTE)

Safety issues
No known deleterious effects
Do not perform in the first trimester
Do not give gadolinium
Obtain informed consent
Congenital CNS Malformations
Dorsal Induction
anencephaly
encephalocele
spina bifida
Ventral Induction
holoprosencephaly
Dandy-Walker malformation
Neuronal Proliferation
microcephaly
macrocephaly
tumors
Migration
agenesis of corpus callosum
3 required images of the fetal brain
There is too much fluid in there
Hydrocephalus
Holoprosencephaly
Hydranencephaly
Hydranencephaly
Absent cerebral hemispheres

Occlusion of ICA -? etiology
infection
vasculitis
emboli
Falx
Normal facial development
602
Holoprosencephaly [Figures 3-7-2 to 3-7-4]
Spectrum of arrested development

alobar
semilobar
lobar
Absent cavum, absent falx, fused thalami, dorsal sac
Midline facial defects
proboscis
cyclopia
midline cleft
Trisomy 13
Figure 3-7-2
Figure 3-7-3
Alobar, semilobar lobar
holoprosencephaly
compared to normal.
Alobar holoprosencephaly with single ventricle
Figure 3-7-4
Hydrocephalus
Dilated ventricles and enlarged head
Ventriculomegaly
Dilated ventricles
Signs
Lateral ventricle > 10mm

Medial ventricular wall to choroid > 3mm
Dangling choroid
Hydrocephalus: Differential
Semilobar holoprosencephaly with a dorsal sac.

Face shows a midline cleft.
Aqueductal Stenosis
Dandy-Walker Malformation
Chiari II
Communicating Hydrocephalus
Figure 3-7-5
Aqueductal Stenosis [Figures 3-7-5]
Block at aqueduct of Sylvius

Most common cause of hydrocephalus
Male predominance (X-linked form)
Aqueductal stenosis with dilatation

of the lateral and third ventricles.
603
Dandy-Walker Malformation [Figures
Communicating PF cyst
Hydrocephalus +/50 have accociated abnormality
3-7-6 and 3-7-7]
Figure 3-7-6
Figure 3-7-7
Dandy-Walker Malformation with

enlargement of the 4th ventricle and
posterior fossa cyst.
Dandy-Walker
Malformation
Chiari II [Figures 3-7-8 to 3-7-10]
Downward herniation of the 4th ventricle and vermis

Myelomeningocele
Hydrocephalus
Normal
Lemon and Banana sign
Chiari II
Figure
3-7-8
3-7-9
3-7-10
Normal vs. Chiari II

604
Communicating Hydrocephalus [Figure 3-7-11]
Dilatation of all ventricles and subarachnoid space

Etiology
hemorrhage
? abnormal arachnoid granulations
? abnormal superior sagittal sinus
Figure 3-7-11
Hydrocephalus: Differential
Aqueductal Stenosis
Dandy-Walker Malformation
Chiari II
Agenesis of the Corpus Callosum [Figure 3-7-12]
Tear-dropped shaped ventricules (Colpocephaly)

Absent cavum septi pellucidi
Associated with lipomas and arachnoid cysts
Often missed or confused with mild ventriculomegaly
Neural Tube Defects
Anencephaly
Spina Bifida
Encephalocele
Acrania
Figure 3-7-12
Anencephaly [Figure 3-7-13]
Absent cranium and cerebral hemispheres

Area cerebrovasculosa
Figure 3-7-13
Agenesis of the CC with colpocephaly

and arachnoid cyst
Figure 3-7-14
1st trimester anencephaly
Encephalocele [Figure 3-7-14]
75% occipital
Frontal Southeast Asia
Evaluate brain tissue
80% have associated malformations
Choroid Plexus Cysts
30% of trisomy 18
1%2% of normals
1/500 chance of trisomy 18
Occipital encephalocele
605
Trisomy 18 [Figure 3-7-15]
Overlapping Fingers
Cardiac Defects
Omphalocele/Diaphragmatic Hernia
Choroid plexus cysts
Figure 3-7-15
Trisomy 18 with overlapping fingers
References
1.
Chang MC, Russell SA, Callen PW, Filly RA, Goldstein RB. Sonographic detection of inferior vermian agenesis in
Dandy-Walker malformations: prognostic implications. Radiology 1994; 193:765-770.
2. Chatzipapas IK, Whitlow BJ, Economides DL. The 'Mickey Mouse' sign and the diagnosis of anencephaly in early
pregnancy. Ultrasound Obstet Gynecol 1999; 13:196-199.
3. Coleman BG, Adzick NS, Crombleholme TM, et al. Fetal therapy: state of the art. J Ultrasound Med 2002; 21:12571288.
4. d'Ercole C, Girard N, Cravello L, et al. Prenatal diagnosis of fetal corpus callosum agenesis by ultrasonography and
magnetic resonance imaging. Prenat Diagn 1998; 18:247-253.
5. Ghidini A, Strobelt N, Locatelli A, Mariani E, Piccoli MG, Vergani P. Isolated fetal choroid plexus cysts: role of
ultrasonography in establishment of the risk of trisomy 18. Am J Obstet Gynecol 2000; 182:972-977.
6. Goldstein RB, LaPidus AS, Filly RA. Fetal cephaloceles: diagnosis with US. Radiology 1991; 180:803-808.
7. Johnson SP, Sebire NJ, Snijders RJ, Tunkel S, Nicolaides KH. Ultrasound screening for anencephaly at 10-14 weeks
of gestation. Ultrasound Obstet Gynecol 1997; 9:14-16.
8. Levitsky DB, Mack LA, Nyberg DA, et al. Fetal aqueductal stenosis diagnosed sonographically: how grave is the
prognosis? AJR Am J Roentgenol 1995; 164:725-730.
9. McGahan JP, Nyberg DA, Mack LA. Sonography of facial features of alobar and semilobar holoprosencephaly. AJR
Am J Roentgenol 1990; 154:143-148.
10. Pilu G, Romero R, Rizzo N, Jeanty P, Bovicelli L, Hobbins JC. Criteria for the prenatal diagnosis of holoprosencephaly.
Am J Perinatol 1987; 4:41-49.
11. Ulm B, Ulm MR, Deutinger J, Bernaschek G. Dandy-Walker malformation diagnosed before 21 weeks of gestation:
associated malformations and chromosomal abnormalities. Ultrasound Obstet Gynecol 1997; 10:167-170.
12. Vergani P, Ghidini A, Strobelt N, et al. Prognostic indicators in the prenatal diagnosis of agenesis of corpus callosum.
Am J Obstet Gynecol 1994; 170:753-758.
606
Fetal Body Anomalies

Paula J. Woodward, M.D.
Neck Masses
Figure 3-8-1
Neural Tube Defects

Cystic Hygroma
Teratoma (Epignathus)
Thyroid
Cystic Hygroma [Figure 3-8-1]
Lymphangioma
Chromosomal Abnormalities
Turners Syndrome XO
Trisomy 21 (2nd trimester nuchal thickening)
Often associated with hydrops
Sagittal and transverse images through the fetal

neck show a cystic hygroma
Iniencephaly [Figure 3-8-2]
Fixed hyperextension of neck (stargazer

position)
Rachischisis
Cephalocele
Shortened or absent vertebral bodies
First trimester
head appears large
CRL less than expected
Figure 3-8-2
Figure 3-8-3
AIUM: Chest [Figures 3-8-4 and 3-8-5]
Four chamber heart

side (stomach and heart both on left)
axis ~35-45
equal chamber size
excludes 90% of cardiac defect
LVOT, RVOT if feasible
First trimester scan of

iniencephaly shows a
hyperextended head and
short body
Epignathus (teratoma)
Figure 3-8-5
Figure 3-8-4
Four-chamber view of normal heart
Left ventricular outflow tract and

right ventricular outflow tract
607
Fetal Anomalies
Hypoplastic Left Heart [Figure 3-8-6]
Figure 3-8-6
Lethal in neonate if untreated

Norwood or transplant
Small or invisible LV
Hypoplastic aortic arch
RA < LA
Consider Turner syndrome in female fetuses
Chest Masses
Congenital Diaphragmatic Hernia

Cystic Adenomatoid Malformation
Extralobar Sequestration
Teratoma
Congenital Diaphragmatic Hernia [Figure 3-8-7]
90% left-sided through foramen of Bochdalek

50% have other anomalies
Pulmonary hypoplasia
Liver-up poor prognosis
Hypoplastic left ventricle
Cystic Adenomatoid Malformation [Figure 3-8-8]
Figure 3-8-7
Lung Hamartoma
Types I III
Fetal CCAMs classified as micro- or macro cystic
Arterial supply from pulmonary artery
May spontaneously regress
In utero surgery for hydrops
Figure 3-8-8
Congenital diaphragmatic hernia

with deviation of the heart
Figure 3-8-9
Sagittal scan of the fetal chest and neonatal CXR

showing type II CCAM
Extralobar Sequestration [Figure 3-8-9]
Non-communicating (sequestered) lung segment

Arterial supply from aorta
90% left sided
10% below diaphragm
AIUM: Abdomen
Stomach
Kidneys
Bladder
UC insertion site
Umbilical cord vessel number
Amniotic Fluid Balance
Production
Fetal/ Embryo plasma
volume
Uterine Perfusion Metanephros (>10 wks)
Lungs
Fetal Anomalies
Extralobar sequestration with feeding vessel from

the aorta.
Removal
Intramembranous
Transmembranous
Swallowing
Lungs
608
Polyhydramnios
Figure 3-8-10
2/3 idiopathic
1/3 definable cause
macroscomia
GI obstruction
CNS malformation
hydrops
Oligohydramnios
Never normal
A DRIP of fluid
Demise
Renal, also bladder
often anhydramnios
IUGR
PROM, post dates
Double bubble, oblique view confirms

duodenal atresia
Figure 3-8-11
Fetal GI Tract
Atresias
Abdominal Wall Detect
Gastroschisis
Atresias
Esophageal
Duodenal
Small Bowel
Anorectal
Esophageal Atresia
Stomach may be present but small

Polyhydramnios after 20 wks
IUGR common
ingested fluid important for nutrition
Figure 3-8-12
Double Bubble [Figure 3-8-10]
Duodenal Atresia
30% have trisomy 21
50% have other structural abnormalities
Ladds bands, annular pancreas usually do not present in utero
Jejunal/Ileal Atresia
1/3 have cystic fibrosis

5%-10% may perforate
Meconium peritonitis
ascites
calcifications
pseudocyst formation
Omphalocele
Figure 3-8-13
Bowel
Normal bowel herniation at 8 weeks

Rotates counterclockwise 270
Returns to abdomen in 12 weeks
Abdominal Wall Defects
Gastroschisis [Figure 3-8-11]

Omphalocele [Figure 3-8-12 and 3-8-13]
Limb-body-wall defect
Gastroschisis
Location
Right
Membrane
No
Cord Insertion
NL
Associated Anomalies
No
Omphalocele
Central
Yes
On sac
50-75%
609
Omphalocele
Fetal Anomalies
Limb-Body-Wall Defect (Body Stalk Anomaly)
Fetus attached to placenta

Absent or short umbilical cord
Severe (lethal) malformation
Scoliosis common
Figure 3-8-14
Renal Anomalies [Figure 3-8-14]
Agenesis
Renal Cystic Disease
Hydronephrosis
Masses
Autosomal recessive polycystic kidney disease [Figure 3-8-15]

Multicystic dysplastic kidneys [Figures 3-8-16 and 3-8-17]
Cystic dysplasia due to obstruction
Autosomal dominant polycystic kidney disease
a) Normal vs. b) renal agenesis
Figure 3-8-15
Associations
VACTERL Syndrome
Vertebral, anal atresia, cardiac, TE fistula, renal, limb
Inherited Disorders
Meckel-Gruber (renal cystic dysplasia, encephalocele,
polydactyly)
Chromosomal Abnormalities
Trisomy 13
Autosomal recessive polycystic
kidney disease
Hydronephrosis
UPJ Obstruction
UVJ Obstruction
Duplications
PUV, Urethral Atresia
Reflux
Figure 3-8-16
Hydronephrosis [Figure 3-8-18]
Renal Pelvis
> 4 mm before 33 weeks
> 7 mm after 33 weeks
AP pelvis diam/AP kidney diam >50%
Calyceal Dilatation
Any degree of dilatation when accompanied by cystic renal
changes
Multicystic dysplastic kidney
Figure 3-8-17
Figure 3-8-18
Bilateral MCDK
Fetal Anomalies
UPJ obstruction
610
Retroperitoneal Masses

Renal Tumors
Mesoblastic Nephroma
Wilms Tumor
Adrenal
Neuroblastoma
Hemorrhage
Figure 3-8-19
Cystic Abdominal/Pelvic Collections
Bladder Obstruction
Dilated Bowel
Cysts
Ovarian
Duplication
Mesenteric
Choledochal
Meconium pseudocyst
Posterior Urethral Valves [Figures 3-8-19 and 3-8-20]
Bladder funnels into dilated posterior urethra

Oligohydramnios common
Renal dysplasia (echogenic cystic kidneys) bad prognostic sign
Posterior urethral valves
Figure 3-8-20
Figure 3-8-21
Severe posterior urethral valves
with oligohydramnios
Ovarian Cyst [Figure 3-8-21]
Most common cyst in 3rd trimester

Anywhere in abdomen
Complexity suggests torsion or hemorrhage
Resolve by 6 mos
Sacral Mass
Sacrococcygeal teratoma
Myelomeningocele
Ovarian cyst
611
Fetal Anomalies
Sacrococcygeal Teratoma [Figures 3-8-22 and 3-8-23]
Solid, cystic, or mixed

Location
Type 1: completely external
Type 2: external and internal into pelvis
Type 3: external and internal into abdomen
Type 4: completely internal
Figure 3-8-23
Figure 3-8-22
Solid sacrococcygeal teratoma

with marked growth
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
Leung JW, Coakley FV, Hricak H, et al. Prenatal MR imaging of congenital diaphragmatic hernia. AJR Am J
Roentgenol 2000; 174:1607-1612.
Coleman BG, Adzick NS, Crombleholme TM, et al. Fetal therapy: state of the art. J Ultrasound Med 2002;
21:1257-1288.
Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and
outcome. Am J Obstet Gynecol 1998; 179:884-889.
Lopoo JB, Goldstein RB, Lipshutz GS, Goldberg JD, Harrison MR, Albanese CT. Fetal pulmonary sequestration: a
favorable congenital lung lesion. Obstet Gynecol 1999; 94:567-571.
Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: a
25-year experience with 277 cases. Arch Surg 1998; 133:490-496; discussion 496-497.
Nyberg DA, Resta RG, Luthy DA, Hickok DE, Mahony BS, Hirsch JH. Prenatal sonographic findings of Down
syndrome: review of 94 cases. Obstet Gynecol 1990; 76:370-377.
Corteville JE, Gray DL, Langer JC. Bowel abnormalities in the fetus--correlation of prenatal ultrasonographic
findings with outcome. Am J Obstet Gynecol 1996; 175:724-729.
Stringer MD, McKenna KM, Goldstein RB, Filly RA, Adzick NS, Harrison MR. Prenatal diagnosis of esophageal
atresia. J Pediatr Surg 1995; 30:1258-1263.
Meizner I, Levy A, Katz M, Maresh AJ, Glezerman M. Fetal ovarian cysts: prenatal ultrasonographic detection and
postnatal evaluation and treatment. Am J Obstet Gynecol 1991; 164:874-878.
Muller-Leisse C, Bick U, Paulussen K, et al. Ovarian cysts in the fetus and neonate--changes in sonographic
pattern in the follow-up and their management. Pediatr Radiol 1992; 22:395-400.
Hutton KA, Thomas DF, Davies BW. Prenatally detected posterior urethral valves: qualitative assessment of
second trimester scans and prediction of outcome. J Urol 1997; 158:1022-1025.
James CA, Watson AR, Twining P, Rance CH. Antenatally detected urinary tract abnormalities: changing incidence
and management. Eur J Pediatr 1998; 157:508-511.
Abuhamad AZ, Horton CE, Jr., Horton SH, Evans AT. Renal duplication anomalies in the fetus: clues for prenatal
diagnosis. Ultrasound Obstet Gynecol 1996; 7:174-177.
Fetal Anomalies
612
14. Pryde PG, Bardicef M, Treadwell MC, Klein M, Isada NB, Evans MI. Gastroschisis: can antenatal ultrasound
predict infant outcomes? Obstet Gynecol 1994; 84:505-510.
15. Luton D, De Lagausie P, Guibourdenche J, et al. Prognostic factors of prenatally diagnosed gastroschisis. Fetal
Diagn Ther 1997; 12:7-14.
16. Getachew MM, Goldstein RB, Edge V, Goldberg JD, Filly RA. Correlation between omphalocele contents and
karyotypic abnormalities: sonographic study in 37 cases. AJR Am J Roentgenol 1992; 158:133-136.
17. Salihu HM, Boos R, Schmidt W. Omphalocele and gastrochisis. J Obstet Gynaecol 2002; 22:489-492.
613
Fetal Anomalies
Cystic Diseases of the Kidney

Peter L.Choyke, MD
Cystic Disease of the Kidney
Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Autosomal Recessive Polycystic Kidney (ARPKD)
Tuberous Sclerosis Complex (TS or TSC)
Von Hippel-Lindau Disease (VHL)
Acquired Cystic Kidney Disease (ACKD)
Figure 3-9-1
ADPKD
Occurs in 1:1000 Individuals

Genetics: Autosomal Dominant
ESRD in 50%
Risk of Cancer = Not increased
Types of ADPKD
Illustration of the microvilli

on the surface of lumenal
cells that are abnormal in
ADPKD
PKD 1 (85-95%)
16p13.3
Polycystin I
Mean age of ESRD=55y
PKD 2 (~5% )
4q2123
Polycystin II
Mean age of ESRD= 71.5y
PKD3? PKD4?
Mechanism [Figure 3-9-1]

Clinical Manifestations
Pain
Hypertension
Infection (Women > Men)
Stones
Loss of Renal Function
Renal Failure
Imaging [Figures 3-9-2 to 3-9-5]

Figure 3-9-2
Figure 3-9-3
Montage of ultrasounds at different stages of

life. From upper left clockwise: 14 years, mid
twenties, mid fifies, mid sixties
Retrograde pyelogram in
ADPKD
614
Figure 3-9-4
Figure 3-9-5
Montage of CT scans of different patients at different stages of

their disease
MRI of ADPKD
Complications [Figure 3-9-6]
Figure 3-9-6
Acute Infection
Manifestations of ADPKD
Intracranial Aneurysms
Cardiovascular Disease: Mitral, Aortic
valve, aortic aneurysm
Cysts: Hepatic, Pancreatic, Spleen
Diverticula: Colon
Intracranial Aneurysms [Figure 3-9-7]
Gas forming organism requiring percutaneous drainage

ICA
Figure 3-9-7
1826% of ADPKD
Rupture ~211%
4661% Mortality Rate
Mean age 3947 years of rupture
Screening (MRA) performed periodically in patients
with ADPKD
Extrarenal Cysts
Occur in 7075% of ADPKD

Complications (liver):
Pain
Biliary Obstruction
IVC compression
Dissecting aneurysm of the abdominal aorta in
ADPKD
Screening
US Screening begins in teenage years

~ 2/3 of affected children will show cysts
between 1120
~ 95% by age 30
Figure 3-9-8
Localized PKD [Figure 3-9-8]
Unilateral, segmental
Non Hereditary
Possible mosaic form of ADPKD
Two cases of unilateral PKD
615
Autosomal Recessive Polycystic Kidney Disease (ARPKD)

[Figures 3-9-9 and 3-9-10]
Figure 3-9-9
Prevalence: Variable 1:6000-50,000

Genetics: Autosomal Recessive
ESRD: >50%
Cancer: None
Unrelated to ADPKD
Congenital Hepatic Fibrosis
Carolis, Biliary Cystic Disease
Renal ductal ectasia, abnormal cilia
Enlarged echogenic kidneys
Infantile Form: Renal Failure leading to Oligohydramnios,
Pulmonary dysplasia
Childhood form: Portal Hypertension, Carolis, Renal
enlargement, Late renal failure
Mechanism: Disease of Microvilli

Tuberous Sclerosis
Prevalence: 1:10,000
Genetics: Autosomal Dominant**
Produces hamartomas throughout the body:
ESRD: 15% (cystic/AML bleeding)
Risk of Cancer: 1-2% (slight increase)
** mostly new mutations; not hereditary
Types of TSC
TSC 1
9q34
~1/3 TSC
Hamartin
Assoc with severe MR
TSC 2
16p 13.3 !!
2/3 of TSC
Tuberin
Assoc with worse renal disease
Typical appearance of increased

echogencity within the kidneys and
increased signal on T2W MRI in
ARPKD
Pathogenesis
Tuberous= nodular, Sclerosis= hard

Skin: Adenoma Sebaceum, Angiofibromas
CNS: Tubers, Subependymal nodules, Giant Cell Astrocytoma
Kidneys: Cysts, Angiomyolipomas
Heart: Rhabdomyomas
Lungs: Lymphangiomyomatosis (LAM)
Bone: Islands
Figure 3-9-10
Biliary cystic dilation in ARPKD

616
Cyst Predominant Forms of TSC
Figure 3-9-11
[Figure 3-9-11]
Renal Involvement
[Figure 3-9-12]
Angiomyolipoma predominant
Mild to severe
Risk of Hemorrhage
Treat conservatively
Partial nephrectomy
Angioembolization
Radiofrequency ablation
Non Fatty Angiomyolipoma [Figure 3-9-13]

Renal Manifestations
Carcinoma of the Kidney

12% of TS patients
Heterogenous solid lesions
Faster growing than AMLs
No screening recommendations
Cystic predominant form of TSC
Figure 3-9-12
Von Hippel Lindau Disease

[Figures 3-9-14 to 3-9-17]
Prevalence ~ 1:35,000 to 1:45,000

Genetics: Autosomal Dominant
ESRD: < 5% Usually due to nephrectomy
Risk of Cancer: 30-40%
Target Organs
CNS, Retina --Hemangioblastomas
Kidney--Cysts and Cancers
Pancreas-- Cysts and
Neuroendocrine tumors
Adrenal--Pheochromocytomas
Epididymis/ Broad Ligament
Cystadenomas
Renal Manifestations
Multiple Cysts
Virtually all will have neoplastic clear
cell lining
Cysts containing tumors
Solid (Clear Cell) tumors
Examples of angiogmyolipomas in TSC at differing degress of

severity
Management
Risk of Metastases - 3 cm rule - Risk of Renal Failure
Figure 3-9-13
Illustrates features of non fatty angiomyolipomas; hyperdense lesions that enhance uniformly
617
Figure 3-9-14
Figure 3-9-16
CNS hemangioblastomas in VHL
Figure 3-9-15
Solid islet cell or pancreatic

neuroendocrine tumors in VHL
Cystic lesions of the pancreas in VHL
Figure 3-9-17
Radiofrequency Ablation [Figure 3-9-18]

Acquired Cystic Kidney Disease
Rate: up to 100% of dialysis pts

Genetics: None
ESRD: 100%
Risk of Cancer: Increases with duration
Pathogenesis
Theory 1: Dialysis Toxin

Theory 2: Uremic Milieu
Mutations which lead to cysts, adenomas, tumors and
metastatic cancers
Bilateral pheochromocytomas
Figure 3-9-18
Successful treatment with RFA in patient with VHL
618
Renal Cancer in ACKD [Figure 3-9-19]
Figure 3-9-19
1050 Fold Risk of RCC

Mean dialysis duration 8 yrs
Multifocal & Bilateral
17% Risk of Metastases
Screening
Screening is not routinely justified

Relatively low risk of cancer
High risk of dying from other causes
Reserve screening for pts with good long term prognosis
Levine E, Abdom Imaging 1995 20:569-71
ACKD-RCC After Transplant
Transplantation
Cysts Regress
New Tumor Formation Decreases
Increased Risk of Metastases from Existing RCC
Immunosuppression
Take Home Points
When confronted with a polycystic kidney

Pure cysts ? Aneurysms ? ADPKD
Enlarged echogenic kidneys? ARPKD
Cysts and AMLs? Brain, skin? TSC
Cysts and RCCs? VHL
Renal failure on dialysis? ACKD
Genetic Testing and Screening:
PKD1, PKD2
TSC1, TSC2
VHL
Risk of Renal Cancer:
ADPKD None known
ARPKD None known
TSC ~ 1-2%
VHL ~ 35%
ACKD ~ 50% (after 8 years of dialysis)
Examples of tumors in patients on

dialysis due to ACKD
References
1.
2.
Witzgall R. New developments in the field of cystic kidney diseases. Curr Mol Med 2005; 5:455-465.
Tahvanainen E, Tahvanainen P, Kaariainen H, Hockerstedt K. Polycystic liver and kidney diseases. Ann Med 2005;
37:546-555.
3. Adeva M, El-Youssef M, Rossetti S, et al. Clinical and molecular characterization defines a broadened spectrum of
autosomal recessive polycystic kidney disease (ARPKD). Medicine (Baltimore) 2006; 85:1-21.
4. Okumura M, Bunduki V, Shiang C, Schultz R, Zugaib M. Unusual sonographic features of ARPKD. Prenat Diagn
2006.
5. Choyke PL, Glenn GM, Walther MM, Zbar B, Linehan WM. Hereditary renal cancers. Radiology 2003; 226:3346.
6. Sessa A, Righetti M, Battini G. Autosomal recessive and dominant polycystic kidney diseases. Minerva Urol
Nefrol 2004; 56:329-338.
7. Herring JC, Enquist EG, Chernoff A, Linehan WM, Choyke PL, Walther MM. Parenchymal sparing surgery in
patients with hereditary renal cell carcinoma: 10-year experience. J Urol 2001; 165:777-781.
8. Seizinger BR, Smith DI, Filling-Katz MR, et al. Genetic flanking markers refine diagnostic criteria and provide
insights into the genetics of Von Hippel Lindau disease. Proc Natl Acad Sci U S A 1991; 88:2864-2868.
9. Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau disease: genetic,
clinical, and imaging features. Radiology 1995; 194:629-642.
10. Ishikawa I, Saito Y, Asaka M, et al. Twenty-year follow-up of acquired renal cystic disease. Clin Nephrol 2003;
59:153-159.
619
Imaging of Prostate Cancer

Peter L.Choyke, MD
Prostate Cancer
Diagnosis
Staging
Image guided Therapy
Epidemiology
~220,000 new diagnoses per year

~29,000 cancer deaths (USA)
2nd most common cause of cancer deaths in males
21% decrease in cancer deaths in the PSA era
Only a small fraction of prostate cancers cause death
We are overdiagnosing and overtreating prostate cancer
Grading Prostate Cancer
Gleason Grading System

Two predominant cell types
Add together for score from 2-10
Screening and Detection
Recommendations:
For men > 50 years or > 40 in African Americans or with Family history :
Annual Digital Rectal*
Annual PSA
Figure 3-10-1
Prostate Specific Antigen
Ranges of PSA
04ng/ml Normal (PPV=5%)
410ng/ml Indeterminate (PPV=22%)
> 10 ng/ml Abnormal (PPV=67%)
Detecting Prostate Cancer
Elevated PSA or Abnormal Rectal Exam

Transrectal Ultrasound Guided Biopsies
Biopsy Mapping and Grading
Anatomy
Zonal Anatomy of Prostate

Peripheral Zone
Glandular
70% of Cancers
Transitional Zone
Stromal/Glandular
25% of Cancers, 90% of Hyperplastic nodules
Central Zone
Distribution of Prostate Cancers

Prostate Ultrasound [Figure 3-10-1]
Transrectal Ultrasound-History
The Chair (Watanabe 1968)
Zonal Anatomy (Stamey 1980)
Screening (Lee 1983)
TRUS Guided Biopsy (1985-)
Color Doppler (1995-)
Contrast Enhanced US (2000-)
Normal ultrasound of the prostate

showing echogenic peripheral zone
and ejaculatory duct on sagittal view
620
The TRUS Examination [Figure 3-10-2]
Figure 3-10-2
Examine the PZ for nodules

Examine the TZ for asymmetry
Examine Seminal Vesicles
Determine the Volume
TRUS Guided Biopsy
Prep:
Antibiotics before and after (Cipro)
Enema (Fleets)
Core Biopsies with Automatic Cutting Needle
Directed Biopsies at sites of abnormality
Label all specimens; send separately
Doppler power ultrasound can be

used to detect prostate cancer
vascularity
Tumor Mapping with Biopsy

Staging Prostate Cancer
Staging (TNM)
Non palpable
Detected by Bx
Palpable
Extracapsular
Fixed, invasive
Regional Nodes
Distant Mets
Treatment
A1, 2
B1, 2
C1, 2
D1
D2
A1, 2
**
B1, 2
C1
C2
D1
D2
T1 a-c
T2 a,b
T3 a-c
T4
Tx, N+, M+
Figure 3-10-3
T1a, b
T1c
T2a, b
T3a, b, c(sv)
T4
Tx, N+
Tx, Nx, M+
Surg/XRT/WW
Surg/XRT/WW
XRT/WW/Hormonal
XRT/WW/Hormonal
Hormonal/Chemotherapy
Staging with Imaging
Local Staging (Extracapsular) [Figure 3-10-3]

Ultrasound
MRI
Key Structures:
Neurovascular Bundles
Seminal Vesicles
Apex
Periprostatic Venous Plexus
Extracapsular extension on
ultrasound
TRUS Staging
Sensitivity
~4050%
Exceptions:
Seminal Vesicles
Neurovascular Bundle
Figure 3-10-4
Endorectal Coil MRI [Figure 3-10-4]
Sensitivity:
Early Studies ~8590%
Multi institutional Trial ~6070%
Motion
Microscopic Disease
Operator dependent
Observer dependent
Extracapsular extension on endorectal coil MRI
621
Endorectal Coil MRI
Figure 3-10-5
Improvements
Dynamic contrast enhancement
MR Spectroscopy
Dynamic Enhanced MRI of the Protaste

MRI and 1H-MRSI- Prostate: Metabolic
Interrogation
Nodal Staging [Figures 3-10-5 and 3-10-6]
CT/MRI only ~ 36% sensitive

Size threshold ~8mm + biopsy of nodes
Yield improves for high risk pts
(PSA >20 ng/ml)
Prostascint SPECT
USPIO (Combidex) MR Lymphography
Prostascint images demonstrate positive node despite

negative CT
Staging for Distant Metastases
Bone Metastases
Bone Scan
Yield increases
after PSA >10
**Superscan**
Quantitation/
Confirmation
Figure 3-10-6
Radioactive Ablation
Strontium-89 (Metastron)
~80% response rate
Up to 6 months relief of Examples of nodal metastasis due to prostate cancer. Percutaneous biopsy
can be performed to determine if a node is positive
bone pain
Samarium and
Rhenium
Figure 3-10-7
Image Guided Treatment
Brachytherapy
Cryotherapy
Brachytherapy [Figure 3-10-7]
Interstitial Radioactive Seeds

(Afterloaded)
Iodine, Iridium, Palladium, Gold
Introducers are placed within prostate at
regular spacing via:
CT
US
MRI
Examples of brachytherapy seeds immediately after seed

placement (left) and several years after placement (right)
Cryotherapy [Figure 3-10-8]
Liquid Nitrogen instilled via cannulas placed within Prostate under TRUS
Not enough Data
High rate of impotence
Steep learning curve
Figure 3-10-8
Cryotherapy of the prostate monitored by ultrasound

622
Take Home Points
Imaging currently plays minor role in prostate cancer detection:

MR spectroscopy, Dynamic MRI may change this
Staging depends on PSA/Grade
MRI for local staging
CT/MRI (USPIO) for nodal staging
Bone Scan/CT/MR for distant staging
Image Guided therapy is an important trend in treatment
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
Adusumilli S, Pretorius ES. Magnetic resonance imaging of prostate cancer. Semin Urol Oncol 2002; 20:192-210.
Campbell T, Blasko J, Crawford ED, et al. Clinical staging of prostate cancer: reproducibility and clarification of
issues. Int J Cancer 2001; 96:198-209.
el-Gabry EA, Halpern EJ, Strup SE, Gomella LG. Imaging prostate cancer: current and future applications. Oncology
(Huntingt) 2001; 15:325-336; discussion 339-342.
Engelbrecht MR, Barentsz JO, Jager GJ, et al. Prostate cancer staging using imaging. BJU Int 2000; 86 Suppl 1:123134.
Harisinghani MG, Barentsz J, Hahn PF, et al. Noninvasive detection of clinically occult lymph-node metastases in
prostate cancer. N Engl J Med 2003; 348:2491-2499.
Hocht S, Wiegel T, Bottke D, et al. Computed tomogram prior to prostatectomy. Advantage in defining planning target
volumes for postoperative adjuvant radiotherapy in patients with stage C prostate cancer? Strahlenther Onkol 2002;
178:134-138.
Hernandez J, Thompson IM. Prostate-specific antigen: a review of the validation of the most commonly used cancer
biomarker. Cancer 2004; 101:894-904.
Konety BR, Bird VY, Deorah S, Dahmoush L. Comparison of the incidence of latent prostate cancer detected at
autopsy before and after the prostate specific antigen era. J Urol 2005; 174:1785-1788; discussion 1788.
Karakiewicz PI, Eastham JA, Graefen M, et al. Prognostic impact of positive surgical margins in surgically treated
prostate cancer: multi-institutional assessment of 5831 patients. Urology 2005; 66:1245-1250.
Kumar R, Zhuang H, Alavi A. PET in the management of urologic malignancies. Radiol Clin North Am 2004; 42:11411153, ix.
Mathews D, Oz OK. Positron emission tomography in prostate and renal cell carcinoma. Curr Opin Urol 2002; 12:381385.
Ravery V, Boccon-Gibod L. T3 prostate cancer: how reliable is clinical staging? Semin Urol Oncol 1997; 15:202206.
Raja J, Ramachandran N, Munneke G, Patel U. Current status of transrectal ultrasound-guided prostate biopsy in the
diagnosis of prostate cancer. Clin Radiol 2006; 61:142-153.
Sanchez-Chapado M, Angulo JC, Ibarburen C, et al. Comparison of digital rectal examination, transrectal
ultrasonography, and multicoil magnetic resonance imaging for preoperative evaluation of prostate cancer. Eur Urol
1997; 32:140-149.
Sodee DB, Nelson AD, Faulhaber PF, Maclennan GT, Resnick MI, Bakale G. Update on fused capromab pendetide
imaging of prostate cancer. Clin Prostate Cancer 2005; 3:230-238.
623
Radiographic Evaluation of Urinary Stone

Disease
Learning Objectives
Pathogenesis of renal stone disease

Highlight CT as the modality of choice
Alternative modalities
Reinforce the critical role of Radiology
AFIP
Limited Rad-Pathology
Stone Dz
Major nuisance
Med/Urologic Advances
Previously Debilitating
Annual 2-3% incidence
White male LTR is 1 in 3-8
14% @ 1yr
35% @ 5yr
52% @ 10yr
Multi Billion $$ Cost
Genetics
Family Hx (3 X)
M : F : 3: 1
Recognized D/O
Familial RTA
Mutations in CLCN5 gene
Extrinsic
Climate
Water
Diet
Occupation
Stress
Predisposing Factors
Family Hx
Bone/GI Dz
Gout
Chronic UTI
Nephrocalcinosis
Stasis
Stone Makeup
Composition
Ca Oxalate/phosphate
Struvite/matrix
Uric Acid
Cystine
Other (incl indinavir)
Percent of all stones

75
10-15
6
1-2
<5
Heterogeneous Nucleation
Epithelial cells
Urinary Casts
RBCs
Homogeneous Nuc---
Radiographic Evaluation of Urinary Stone Disease
624
Inhibitors
Organic Molecule
Magnesium
Pyrophosphate
Citrate
Mucoproteins
RNA Fragmets
Promoters
Glycosaminoglycans
Tamm-Horsfall
Aggregation
Free crystals need to grow (2-5 min transit fm glomerulus to nephron)

Anatomic Abnl
UPJ
MSK
Lack of inhibitors
Light chain proteins
Figure 3-11-1
Formation Product
Real question?
Why dont we all form stones
Kf is 7-11 X Ksp
Calcium Stones [Figure 3-11-1]

Hypercalcuria
Idiopathic
Steroid Use
Immobilizaion
Hypocitrinuria
Hypomagnesuria
Left Ureteral Stone
Calcium Stones
Hyperoxaluria
Crohn disease
Celiac sprue
Pancreatic insufficiency
Small intestinal bypass surgery for obesity
CaPhos
PTH or RTA
Figure 3-11-2
Struvite Stone MgNH4 PO4 6H2 O

Magnesium Ammonium Phos [Figure 3-11-2]
(NH2 )2 --CO ? H2 O + 2NH3 + CO2

Urine pH increases because ammonia hydrolyzes
Urease
Proteus
Klebsiella
Struvite
Staghorn Calculus
Low Urine Volumes

Infection
F to M: 3 to 1
Sx
Malaise
Fever
Wt Loss
625
Uric Acid Stones
Hyperuricosuria
Gout 35% if Uric Acid > 700mg/24 hr urine
Newly Dxed Gout 1 per 114 with stones
Hereditary (Lesch-Nyhan)
Idiopathic
Dehydration
Markedly acid urine (< pH of 6.2)
UC is classic example
Ileostomy w/water and bicarb loss
Cystine Stone
Hereditary Cystinuria
Three Types
Auto Recessive
Abnormal renal tubule transport
Large amounts of cystine are excreted in the urine (10 X normal)
Younger Patients
Presentation
Autonomic System
Celiac ganglion
Confusion about source
Diaphragm to testicle
GI sx
N/V
Diarrhea
Ileus
Figure 3-11-3
Films
Visualize
Characterize
Sens 45%
Spec 50%
IVP
CT
Sensitivity: 64-97%
Specificity: 92-94%
Still 10-15% false negative rate
Distal left ureteral stone demonstrating a Rim Sign
1995
Sens of > 90%
Spec of nearly 100%
Quick
No contrast
Non-urologic Abnl
Figure 3-11-4
CT-Radiation
4 by 2.5, 120 KVP, 120mAs,1-1.5
500 mrem
150-350 mrem for full IVP
13 mrem for one image
Rim Sign [Figure 3-11-3]

Comet Tail Sign [Figure 3-11-4]
Calcifications in right pelvis demonstrating the

Comet Tail Sign
626
Secondary Signs
Hydronephrosis/ Hydroureter [Figure 3-11-5]
Figure 3-11-5
Secondary Signs
Unilateral renal enlargement [Figure 3-11-6]
Secondary Signs
Perinephric/ureteral edema [Figure 3-11-7]
Secondary Signs
Unilateral absence of the white pyramid
[Figures 3-11-8 to 3-11-10]
Figure 3-11-6
Asymmetric Right Hydronephrosis
Figure 3-11-7
Asymmetric left renal enlargement
Figure 3-11-8
Left Perinephric stranding
Figure 3-11-9
Normal hyperdense pyramids
Figure 3-11-10
Unilateral absence of right

hyperdense pyramids
Obstructed left system with lower parenchymal
attenuation
627
Conclusions
If H.U. Discrepancy > 5

Sens = 61%
Spec = 100%
Accur = 79%
Urologist
Size
Number
Location
Complications
Spontaneous Passage Rate of Ureteral Calculi as a Function of

Stone Size
Stone Size (mm)
1
2
3
4
5
6
7
8
9
10
No of Stones
15
43
23
18
15
18
17
9
3
11
Passage Rate (%)

87
72
83
72
60
72
47
56
33
27
Expectant Treatment
Figure 3-11-11
Non-infected
Two kidneys and normal renal function
Small Stones
<4mm 90% pass spontaneously
Distal Stone Protocol
Steroids
Calcium channel blockers
Fluids
Pain meds
Non-invasive treatment: ESWL
Extracorporeal Shock wave lithotripsy

< 2 cm
Ca Stones
Upper/Mid Pole Calyx
Invasive treatment: Ureteroscopy
Mechanical extraction
Homium Laser
Shadowing stone in central collecting system
Figure 3-11-12
Invasive Therapy: PCNL
Percutaneous Nephrolithotomy
Not amenable to ESWL or ureteroscopic approaches
1-2 day hospitalization
Alternative Modalities [Figure 3-11-11]

Quantifying Obstruction: Ultrasound [Figure 3-11-12]
Hydronephrosis
Examine ureteral jets
Potential use of resistive index (>.7)
Hydronephrotic Kidney
628
MR [Figure 3-11-13]
Figure 3-11-13
MR Urography shows distal ureteral stone
References
1. Walsh : Campbell's Urology, 8th ed. 2002 W. B. Saunders Company.
2. Tamm EP, Silverman PM, Shuman WP. Evaluation of the patient with flank pain and possible
ureteral calculus. Radiology. 2003 Aug;228(2):319-29. Epub 2003 Jun 20. Review.
3. Guest AR, Cohan RH, Korobkin M, Platt JF, Bundschu CC, Francis IR, Gebramarium A, Murray
UM. Assessment of the Clinical Utility of the Rim and Comet-Tail Signs in Differentiating Ureteral
Stones from Phleboliths AJR 2001;177:1285-1291.
629
Testicular Torsion - Case Based Review

Testicular Torsion
Defined as a twist of the spermatic cord or of the testis itself on its

attachments.
Degree of ischemia relative to the amount of twisting, beginning with venous
compromise, and progressing to arterial occlusion. A 360 degree twist may still
have arterial inflow.
Torsion most common in puberty (ages 12-18), but also occurs in neonates
and adults
Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum.
Radiology 2003;227:18-36
Types of Testicular Torsion
Extravaginal (Neonatal)
Intravaginal
Bell-Clapper deformity
Scrotal Anatomy and Torsion
Normal Tunica Vaginalis

Inner visceral layer covers the testis and epididymis and cord
Outer parietal layer lines the scrotum except posterolaterally where it fuses
with the visceral layer and the scrotal wall to form the bare area.
Hydroceles occur between these 2 layers
Scrotal Anatomy and Torsion
Bell-Clapper Deformity of the Tunica Vaginalis

Failure of fusion of the visceral and parietal layers to the scrotal wall, so
the space completely encircles the epididymis, distal spermatic cord and
the testis rather than attaching to the posterolateral aspect of the scrotum
to form the normal bare area.
It is bilateral in most cases
12% incidence found in one autopsy series
Dogra VS, Gottlieb RH, Oka M, Rubens DJ.

Sonography of the scrotum. Radiology 2003;227:18-36
Scrotal Anatomy
Figure 3-12-1
Tunica
Vaginalis
[Figure 3-12-1]
Bell-Clapper Deformity
Sagittal line drawings of bell-clapper (left) and

normal (right) testis. Tunica vaginalis is the
outermost layer (arrows).
Testicular Torsion
630
Testicular Torsion
Clinical presentation includes:

Acute onset of scrotal pain
Anorexia, nausea and/or vomiting
Lack of urinary symptoms or fever
As many as 35-50% of patients experience gradual onset of pain, similar to
epididymitis
Pain may be intermittent (detorsion)
Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum.
Radiology 2003;227:18-36
Testicular Torsion: Clinical Significance
Time from onset of symptoms to surgery associated with salvage rate:*

5-6 hours, 80-100%
6-12 hours 70%
After 12 hours 20%
If non salvageable, the necrotic testis is removed to decrease risk of
autoimmune reaction to the residual testis
Donohue RE, Utley WL. Urology 1978 11:33
Testicular Torsion: Grayscale Patterns
Acute torsion with viable testis: normal

Acute torsion with infarction: hypoechoic pattern which may be
total, or partial in the case of a partial infarct
Acute torsion with hemorrhagic infarction: hyperechoic and
heterogeneous pattern.
Chronic: hypoechoic with small testis
Figure 3-12-2
Middleton WD, Middleton MA, Dierks M, et al. Sonographic prediction

of viability in testicular torsion: preliminary observation. J Ultrasound
Med. 1997;16:2327.
Testicular Torsion: Doppler Patterns
Absent arterial and venous flow

Increased Resistive Index on affected side (diminished or reversed
diastolic flow)
Decreased flow velocity difficult to measure due to small
vessels/angle correction, but may be subjectively inferred by
relative difficulty in finding small low amplitude flow on symptomatic
side
Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the

scrotum. Radiology 2003;227:18-36
Dogra VS, Sessions A, Mevorach R, Rubens DJ Reversal of diastolic
plateau in partial testicular torsion. J Clin Ultrasound 2001; 29:105-108
CASE 1 [Figure 3-12-2]
Sudden right sided pain

Diagnosis: Acute right testicular torsion with viable testis.
Dx: Acute torsion with viable testis
Important imaging findings:

Note grayscale symmetry on transverse images
Color Doppler flow is absent in the affected testis, but not in the
epididymis
The epididymis has alternate blood supply and may be
perfused even if the testis is not
CASE 1: 21 year old man with

sudden right-sided testicular pain
631
Testicular Torsion
CASE 2 [Figures 3-12-3 and 3-12-4]
A seven year old presents with acute symptoms. Which side is abnormal?
Minimal right sided flow
Right testis is hypoechoic
Figure 3-12-3
Figure 3-12-4
CASE 2: A seven year old presents

with acute symptoms. Which side is
abnormal?
CASE 2: Minimal right sided flow. Right testis is hypoechoic
Left sided Doppler findings. What is your diagnosis?

Chronic left testicular torsion with hemorrhagic non-viable testis
From: Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular

Torsion Ultrasound Clin 2006; 1:55-66 with permission.
Figure 3-12-5
Chronic Torsion with

Hemorrhagic
Non-Viable Testis
In young children the testis may be

small and hypoechoic. Flow is often
minimal, but note the normal spectral
waveform pattern
The abnormal side has markedly
increased Doppler flow, but it is around
the testis, not within it. It is important
always to image in 2 planes and to
document flow within the actual testis.
CASE 2: Left sided Doppler findings in long axis (left) and in
Hemorrhage creates additional tissue
transverse (right) plane. What is your diagnosis?
planes and is hyperechoic and
heterogeneous. Hemorrhage is a result
of infarction and indicates a non-viable testis
Right sided pain, nausea and vomiting.

Is this torsion or epidydimitis?
Diagnosis:
Torsion with 360 degree twist of the
spermatic cord
Figure 3-12-6
RT
LT
CASE 3 Right sided pain, nausea and vomiting in a 15 year

old. Is this torsion or epidydimitis?
Testicular Torsion
632
Torsion with preservation of Doppler flow
Torsion is not an all or none phenomenon

Venous obstruction occurs first and is indicated by a high-resistance arterial
spectral Doppler waveform.
Flow may still be present even if the testis is twisted up to 720 degrees.
More flow will be detected with power Doppler and probably also with US
contrast.
THE PRESENCE OF FLOW DOES NOT EXCLUDE TORSION!
Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion

Ultrasound Clin 2006; 1:55-66
Figure 3-12-7
2 hours of left sided symptoms

Can this be torsion?
DX: Torsion/detorsion with increased flow post torsion
From: Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of

Testicular Torsion Ultrasound Clin 2006; 1:55-66 with permission.
TORSION/DETORSION
History is critical-classically that of intermittent acute and sharp pain

with long symptom-free intervals
Know which side hurts and if it still hurts during the examination.
If scanned immediately after detorsion, the affected testis may show
increased blood flow
Left sided symptoms progressing over several days. Prior US exam

showed an enlarged epididymis with increased flow to the
epididymis
Dx: Torsion/detorsion with focal infarction
Figure 3-12-8
CASE 4: 2 hours of left sided

symptoms. Can this be torsion?
From: Dogra VS, Bhatt S, Rubens,
DJ. Sonographic Evaluation of
Testicular Torsion
Ultrasound Clin 2006; 1:55-66 with
permission
CASE 5:Left sided

symptoms
progressing over
several days. Prior
US exam showed an
enlarged epididymis
with increased flow
to the epididymis
Torsion/detorsion with infarction
Focal hypoechoic areas with concave margins are typical of infarcts.

Focal infarcts when associated with normal or increased flow should alert you
to the possibility of intermittent torsion.
Frequently the epididymis is enlarged and hyperemic in torsion/detorsion and
can be mistaken for epididymitis.
Careful surveillance of the testis for focal infarction may lead to the correct
diagnosis
633
Testicular Torsion
A 2 month old had pain 1 month ago with a normal US exam,

Now he has new pain and right scrotal swelling for 18 hours
Figure 3-12-9
What is the finding adjacent to the testis.

Is this torsion?
Dx: Inguinal hernia with obstructed flow to
the spermatic cord
Figure 3-12-10
CASE 6: A 2 month old had pain 1 month ago with a

normal US exam, Now he has new pain and right scrotal
swelling for 18 hours
CASE 6: Note fluid filled

structure superior to the right
testis (top) with color Doppler
flow in the wall (bottom).
A hydrocele surrounds the
right testis. Is this torsion?
Inguinal Hernia With Obstructed Testicular Perfusion
Multiple etiologies of altered testicular perfusion may occur, including mass

lesions obstructing the spermatic cord.
Even in a newborn, flow should be obtainable in the testis
Figure 3-12-12
4 day old with large left hydrocele. Is there left-sided torsion?
Repeat scan 10 hours later
Figure 3-12-11
CASE 7: A 4 day old presents with a large left hydrocele. Is

there left-sided torsion?
Testicular Torsion
634
CASE 7: Repeat scans 10

hours later
Right testes (top)
Left testes (bottom)
Repeat spectral Doppler exam 10 hours later. What is your diagnosis?

Dx: Normal testes
Figure 3-12-13
Doppler Technical Considerations
Always use the highest frequency Doppler which will yield a signal
without attenuation
Initial examination was performed at Doppler frequency of 5MHz,
repeat examination at 10 MHz.
Always confirm a true arterial spectral waveform. The waveform on
the initial examination was only noise, and could have been
interpreted as no flow
CASE 8
[Figure 3-12-14]
Acute left sided pain

Patient is s/p Lt orchiectomy and s/p Rt Orchiopexy
Now with right sided pain. Repeat scan done 2 days later
[Figure 3-12-15]
Dx: Bilateral infarction due to polyarteritis nodosa
Figure 3-12-14
RT
CASE 7: Repeat spectral Doppler

exam 10 hours later. What is your
diagnosis?
Top: Bilateral normal testes with
inadequate Doppler on initial
examination
LT
Figure 3-12-15
Acute left sided pain

No flow in the left testis
Testicular Ischemia
Causes for ischemia other than torsion include:

Vasculitis
Polyarteritis Nodosa
Systemic Lupus Erythematosum
Severe edema from infection
Uncontrolled or unresponsive epididymo-orchitis
Venous thromboses (ie hypercoagulable patients)
CASE 8: 2 days later the patient presents with

right sided pain. He is post left orchiectomy and
right orchiopexy.
Gray scale image (left) is unremarkable.
Doppler image (right) shows a noise spectrum.
Dx: Bilateral infarction due to polyarteritis nodosa
Imaging Methods
Doppler examination is 86% sensitive, 100% specific and 97% accurate* when
using absent flow in the symptomatic side as the single diagnostic criteria. If
assymmetric abnormal spectral tracing were also used for diagnosis,
sensitivity would improve
In children, power Doppler is more sensitive than color Doppler to detect
normal flow, with rates of 97% vs 88% respectively **
*Burks DD, Markey BJ, Burkhard TK, Balsara ZN Haluszka MM, Canning DA.
Suspected testicular torsion and ischemia: evaluation with color Doppler
sonography. Radiology 1990;175:815-21
** Barth RA, Shortliffe LD. Normal pediatric testis: comparison of power Doppler
and color Doppler US in the detection of blood flow. Radiology 1997;204:389-93
635
Testicular Torsion
Torsion/Detorsion
Classic history of intermittent symptoms

If scanned when asymptomatic or after detorsed, will see increased flow in the
affected testis, which may suggest epididymitis
Testis may be enlarged, and focal infarcts may or may not be present
Torsion Mimics/Variants
Infarction may present with pain which mimics torsion

Partial infarction may occur from torsion/detorsion, from vasculitis, or from
variant arterial anatomy. In some patients, the epidydimal artery, a branch of
the testicular artery, supplies the superior and anterior pole of the testis. If this
artery is hypoplastic, a small twist may result in focal infarction involving the
superior pole
Total infarction is more unusual, but should be suspected in patients with
underlying vasculitides such as polyarteritis nodosa and systemic lupus
Artery to Epididymis
In some patients, the epidydimal artery, a branch of the testicular artery,

supplies the superior and anterior pole of the testis. If this artery is hypoplastic,
a small twist may result in focal infarction
Torsion Take Home Messages
Torsion may be present despite testicular flow.

Diminished or high resistance flow should suggest torsion in the proper
setting.
A history of intermittent symptoms should suggest detorsion, and
corresponding hyperperfusion should not be confused with epididymo-orchitis.
Other rare causes of decreased testicular perfusion include vasculitis, and if
torsion is not present, appropriate medical therapy should be pursued
References
1.
2.
3.
4.
5.
6.
7.
Barth RA, Shortliffe LD. Normal pediatric testis: comparison of power Doppler and color Doppler US in the
detection of blood flow. Radiology 1997;204:389-93.
Burks DD, Markey BJ, Burkhard TK, Balsara ZN Haluszka MM, Canning DA. Suspected testicular torsion and
ischemia: evaluation with color Doppler sonography. Radiology 1990;175:815-21
Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion. Ultrasound Clin 2006; 1:55-66 with
permission.
Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the Scrotum. Radiology 2003;227:18-36
Dogra VS, Sessions A, Mevorach R, Rubens DJ Reversal of diastolic plateau in partial testicular torsion. J Clin
Ultrasound 2001; 29:105-108
Donohue RE, Utley WL. Urology 1978 11:33
Middleton WD, Middleton MA, Dierks M, et al. Sonographic prediction of viability in testicular torsion:
preliminary observation. J Ultrasound Med. 1997;16:2327.
Testicular Torsion
636
Imaging of Ovarian Masses

Brent J. Wagner, MD
Ovarian Masses
Non-neoplastic
physiologic cyst, endometriosis, etc.
Neoplastic
epithelial tumors 65%
germ cell tumors 25%
sex-cord stromal tumors 5%
secondary malignancies 5%
gonadoblastoma <1%
Relative Incidence of Ovarian Neoplasms [Figure 3-13-1]

Major Ovarian Tumor Types
Epithelial
Serous
Mucinous
Endometrioid
Clear Cell
Brenner
(others)
Germ Cell
Mature teratoma
Dysgerminoma
Immature teratoma
(others)
Sex Cord Stromal
Fibrothecoma
Granulosa cell
Sertoli-Leydig
(others)
Figure 3-13-1
Relative incidence of
ovarian neoplasms
Common Ovarian Epithelial Tumors: Classification
Serous
Mucinous
Endometrioid
Clear Cell
Brenner
(others, including mixed, undifferentiated, etc.)
Ovarian Epithelial Tumors
65% of ovarian neoplasms

85% of ovarian malignancies
60% of epithelial tumors are benign
35% of epithelial tumors are malignant
5% of epithelial tumors are borderline, low malignant potential
major risk factor: incessant ovulation (Lancet, 1973)

infertility
celibacy
nulliparity
family history
high socioeconomic status
breast cancer
endometrial cancer
lack of oral contraceptive use
637
CA-125
abnormally elevated in 85% of ovarian cancer patients

false negative in 50% of Stage I disease
false negative in 30% of mucinous tumors
false positives occur (especially in pre-menopausal patients)
with benign neoplasms, endometriosis, etc.
most commonly used to follow known disease for remission and
recurrence
rarely of use in deciding on surgical vs. non-surgical
management at the time of initial presentation of a pelvis mass
benign
low malignant potential (LMP)
borderline tumors
based on histologic appearance of primary
may be a heterogeneous group (but histologic features
overlap)
95% five year survival overall
But the patients who have metastasis or recurrence are
clinically similar to patients with (true) ovarian cancer
malignant
Figure 3-13-2
Serous cystadenoma (bilateral)
Figure 3-13-3
Epithelial Tumors: Terminology
adenoma or adenocarcinoma
add prefix cyst- if cystic
Serous cystadenoma (LMP)
add suffix -fibroma if more than 50% fibrous
(e.g. cystadenofibroma or fibrous cystadenocarcinoma)
Figure 3-13-4
do not confuse with a true fibroma (one of the sex-cord stromal tumors)
Epithelial Ovarian Neoplasms: Serous

[Figures 3-13-2 to 3-13-6]
also known as papillary tumors

25% of benign neoplasms
50% of malignant neoplasms
63% benign, 30% malignant, 7% LMP
strongest association with CA-125
thin-walled cyst, usually unilocular
papillary soft tissue projections often seen
psammomatous calcification is more common than with other
ovarian neoplasms
solid or bilateral tumors suggest malignancy
Figure 3-13-5
Serous
cystadenocarcinoma
638
Serous cystadenoma (LMP)
Epithelial Ovarian Neoplasms: Mucinous
mucin-containing cells (cyst content varies)

up to 20% of benign ovarian neoplasms
10% of carcinomas
73% benign, 16% malignant, 11% LMP
serum marker (CA-125) is less reliable (falsely negative) with
mucinous tumors
Figure 3-13-6
Epithelial Ovarian Neoplasms: Mucinous [Figure 3-13-7]
thin-walled cyst, usually multilocular

often large, may be enormous
occasionally, linear calcifications (but calcifications are LESS
frequent than with mucinous tumors of colonic origin)
solid elements suggest malignancy
LMP tumors are associated with pseudomyxoma peritonei
cause / effect relationship is often unclear
Pseudomyxoma peritonei [Figure 3-13-8]
usually arises from appendix

often difficult to determine whether the process originated from
the appendix, ovary, or both (synchronous)
prolonged, uncomfortable survival (limited treatment options)
low density, scalloping seen on CT
Pseudomyxoma peritonei is a poorly understood condition and
it is unclear whether its continous production of gelatinous mucin
is due to peritoneal implantation of neoplastic mucinous cells or
to metaplasia of peritoneal cells into mucinous epithelium,
induced by mucin. [1]
Serous cystadenocarcinoma
Figure 3-13-7
[1] Trop CG et al. Surgery for borderline tumor of the ovary.

Seminars in Surgical Oncology 2000; 19:6975.
Epithelial Ovarian Neoplasms: Endometrioid
mimics endometrial ca, but is primary to ovary

may have malignant stroma (carcinosarcoma or malignant
mixed mesodermal tumor = MMMT)
almost all are malignant
1015% of ovarian cancers
25% of patients have an associated uterine abnormality
endometrial carcinoma (separate primary malignancy)
endometrial hyperplasia
15% of patients have coexistent endometriosis (cause/effect
unclear)
Mucinous cystadenoma (LMP?)
Figure 3-13-8
Epithelial Ovarian Tumors: Clear Cell [Figure 3-13-9]
mimics clear cell cancer of the vagina, but no association with in

utero DES exposure
5% of ovarian cancers (all clear cell tumors are malignant)
gross appearance is variable:
unilocular cyst with a mural nodule
multilocular
Figure 3-13-9
solid, etc.
Pseudomyxoma peritonei
Clear cell carcinoma

639
Ovarian Carcinoma Staging: Local disease (30%)
Stage I
limited to ovary [subtypes]
Stage II
extra-ovarian pelvic extension [subtypes]
Figure 3-13-10
Ovarian Carcinoma Staging: Advanced Disease

(70%)
Stage III
tumor within the peritoneum (outside the pelvis) [or]
retroperitoneal lymph nodes [or] surface of the liver [or] small
bowel/omentum (within the pelvis)
Stage IV
distant spread
hepatic parenchyma
lung
etc.
Typical Ovarian Cancer Therapy
Surgical: hysterectomy, oophorectomy, appendectomy,

omentectomy, removal of peritoneal masses
Medical: 68 (monthly) cycles of chemotherapy (a platinumbased agent, plus taxol)
Second look surgery? (only as part of a structured research
protocol)
Ovarian Masses
Non-neoplastic
Physiologic cyst, endometriosis, etc.
Neoplastic
Epithelial tumors
65%
Germ cell tumors
25%
Sex-cord stromal tumors
5%
Secondary malignancies
5%
Gonadoblastoma
<1%
Ovarian Germ Cell Neoplasms
most are mature teratomas:

the most common mature teratomas are mature cystic
teratomas (commonly referred to as dermoid cysts)
less common mature teratomas include carcinoid tumors,
struma ovarii, etc.
in the ovary . . .
all dermoid cysts are mature teratomas (and most, but not all,
mature teratomas are dermoid cysts)
Mature Cystic Teratoma [Figures 3-13-10 and 3-13-11]
Mature teratoma
Figure 3-13-11
unilocular cyst
cyst fluid is nearly sonolucent
posterior acoustic enhancement (fluid at body temperature)
images as fat (lipid) by CT, MRI but it is NOT adipose
tissue
Rokitansky nodule
contains various tissues (cartilage, gastrointestinal
epithelium, etc)
echogenic
Malignant transformation of
mature teratoma
640
Figure 3-13-12
child-bearing years
may undergo:
torsion
rupture
malignant transformation (very rare)
often discovered as an incidental finding
12% bilateral
Ovarian Malignant Germ Cell Tumors
dysgerminoma (similar to seminoma)

embryonal carcinoma
endodermal sinus tumor
immature teratoma
choriocarcinoma
mixed germ cell tumor (much less common than testicular mixed
GCT)
Dysgerminoma
Figure 3-13-13
Malignant Germ Cell Tumors [Figure 3-13-12]
in general:
younger age group (1530 years) than epithelial tumors
solid / heterogeneous
highly aggressive
differentiation among the various types is difficult (but
immature teratomas are the most likely to have fat,
calcification)
may have elevated markers (AFP, HCG)
many are very low grade malignancies

generally diagnosed at Stage I (and therefore surgery is often
curative)
hormonal manifestations include:
estrogenic effects: pseudoprecocious puberty, endometrial
stimulation
virilization (less common)
Fibroma / fibrothecoma
Figure 3-13-14
fibrothecoma
50% of all sex-cord stromal tumors
more common than either pure thecoma or pure fibroma
granulosa cell tumors
including juvenile variety
Sertoli-Leydig
more common than either pure Sertoli or Leydig cell tumors
rare, but the most common virilizing tumor of the ovary
Hemorrhagic infarction of fibroma
Figure 3-13-15
Fibrothecoma [Figures 3-13-13 and 3-13-14]
thecoma component produces estrogen

fibroma component accounts for low signal on T2-weighted MRI
sonographically, they tend to be homogeneously hypoechoic but
sound-attenuating
Granulosa cell tumors [Figure 3-13-15]
sponge-like appearance on imaging

multicystic lesion with hemorrhage in a patient under 30
suggests juvenile granulosa cell tumor (but these account for
only 5% of granulosa cell tumors overall)
Granulosa cell tumor

641
low signal on T2 suggests fibroma

hypoechoic sound-attenuating lesion suggests fibroma
diagnosis of this and other sex-cord stromal tumors may be possible if clinical
factors are taken into consideration (morphology is generally solid and nonspecific)
. . . difficult to suggest a simple algorithm for evaluation of women with
ovarian masses [1]
[Doppler U/S, CT, and MRI] yielded similar [results] for discrimination between
benign disease and cancer . . . Although differentiation of benign from
malignant disease is obviously clinically important and these detection rates
are higher than those previously reported, they are likely still not high enough
for surgery to be avoided in most cases. [1]
Whatever the modality used, it is hoped that correct staging of advanced
disease will lead to appropriate referral to a specialist in gynecologic
oncology. [1]
[1] Kurtz A et al Radiology 1999 Jul;212(1):1927
Scoring systems for ovarian tumors
wall thickness
nodularity
septations
echogenicity
ascites?
size?
Ovarian Masses: Sonographic scoring
wall irregularities
smooth --> papillary projections
wall thickness
thin --> thick (< or > 3 mm)
septa
none --> thin--> thick
echogenicity
low --> high
ascites? size?
Doppler sonography*
ideally, should allow more specificity and sensitivity for malignancy

based on low resistance flow (high diastolic flow) in malignant neovascularity
significant overlap with benign processes, especially in pre-menopausal
women
* = controversial
Doppler sonography of ovarian masses*
it should work:
in a large series of patients, the presence of high diastolic flow is predictive
of malignancy
however, it is of limited usefulness:
specificity is limited, especially in pre-menopausal patients
there is considerable overlap of benign vs. malignant
* = controversial
642
Doppler sonography of ovarian masses* [Figure 3-13-16]
Figure 3-13-16
proposed threshold values:

Resistive index (RI) = .45 (or .50)
Pulsatility index (PI) = 1.0
below these values: suggests malignancy
corpus luteum may give false positive
incomplete sampling may give false negative
* = controversial
Doppler sonography of ovarian masses*
do not use the RI or PI values at the exclusion of the

sonographic morphology
color / amplitude Doppler may be of use in characterizing areas
that may be confusing or indeterminate morphologically (for
example, clot vs. tissue)
Use of resistive index in assessment

of ovarian masses
* = controversial
Is pre-operative staging of ovarian cancer important? Maybe

not, because . . .
all patients go to surgery (cytoreduction)

in most centers, staging laparotomies are performed by a gynecologic
oncologist
Is prediction of malignancy in a neoplastic mass important?
May determine the surgical approach

May determine who the surgeon is:
If probably benign, general gynecologist
If probably malignant, gynecologic oncologist
Is the differentiation of a neoplasm from a non-neoplastic

ovarian mass important?
Yes (and its usually accomplished sonography)
Pre-menopausal (ovulating) patient
Acute symptoms?
check pregnancy test
check for fever, elevated white blood cell count
if severe acute pain, consider torsion
Pre-menopausal (ovulating) patient
If sub-acute or mild symptoms:

simple cyst < 30 mm, no follow-up
hemorrhagic cyst < 25 mm, no follow-up
simple cyst 3060 mm, follow-up in 610 weeks
hemorrhagic cyst 2560 mm, follow-up in 610 weeks
any appearance > 60 mm, consider surgery
any soft tissue component (septation, etc), consider surgery
Post-menopausal patient
simple cyst <16 mm: ignore

simple cyst 1650 mm: follow-up 4 months
presumed serous inclusion cyst vs. benign neoplasm
simple cyst > 50 mm OR any complex lesion: consider surgery
643
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Gajewski W, Legare RD. Ovarian cancer. Surg Onc Clin N Am 1998; 7:317.
Hricak H, Chen M, Coakley FV. Complex adnexal masses: detection and characterization with MR imaging -multivariate analysis. Radiology 2000; 214:39.
Jung SE, Lee JM, Rha SE, Byun JY, et al. CT and MR Imaging of Ovarian Tumors with Emphasis on Differential
Diagnosis. Radiographics 2002; 22:1305.
Kawamoto S, Urban BA, Fishman EK. CT of epithelial ovarian tumors. Radiographics 1999; 19:S85.
Kinkel K, Lu Y, Mehdizade A, et al. Indeterminate ovarian mass at US: incremental value of second imaging test
for characterization meta-analysis and Bayesian analysis. Radiology 2005; 236:85-94.
Koonings PP, Campbell K, Mishell DJ, Grimes DA. Relative frequency of primary ovarian neoplasms: a 10-year
review. Obstet Gynecol 1989; 74:921-926.
Kurtz AB, et al. Diagnosis and Staging of Ovarian Cancer: Comparative Values of Doppler and Conventional US,
CT, and MR Imaging Correlated with Surgery and Histopathologic AnalysisReport of the Radiology Diagnostic
Oncology Group. Radiology 1999; 212:19.
Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex-cord stromal and steroid cell tumors of the ovary.
RadioGraphics 1998; 18:1523.
Patel MD, Feldstein VA, Lipson SD, Chen DC, and Filly RA. Cystic teratomas of the ovary: diagnostic value of
sonography. Am J Roentgenol 1998; 171:1061-1065.
Siegelman ES, Outwater, EK. Tissue Characterization in the Female Pelvis by Means of MR Imaging. Radiology
1999; 212:5.
Sironi S, Messa C, Mangili G, Zangheri B, et al. Integrated FDG PET/CT in Patients with Persistent Ovarian Cancer:
Correlation with Histologic Findings. Radiology 2004; 233:433.
Tanaka YO, Tsunoda H, Kitagawa Y, Ueno T, et al. Functioning Ovarian Tumors: Direct and Indirect Findings at
MR Imaging. RadioGraphics 2004; 24:S147.
Wagner BJ, Buck JL, Seidman JD, McCabe KM. Epithelial Neoplasms of the Ovary: Radiologic-Pathologic
Correlation. RadioGraphics 1994; 14:1351.
Woodward PJ, Hosseinzadeh K, Saenger JS. Radiologic Staging of Ovarian Carcinoma with Pathologic Correlation.
RadioGraphics 2004; 24:225.
644
Adrenal Imaging in Adults

Brent J. Wagner, MD
Neoplastic
Adenoma
Metastasis
Lymphoma
Pheochromocytoma
Adrenocortical Carcinoma
Myelolipoma
Hemangioma (rare)
Non-neoplastic
Hemorrhage
Inflammation
Hyperplasia
Cyst
Pseudocyst*
*may be secondary to neoplasm (adenoma)
Clinical manifestations of adrenal tumors
Aldosteronism (hypertension, hypokalemia)

80% due to adenoma (Conns syndrome)
20% due to hyperplasia
< 1% due to adrenal cortical carcinoma
Virilization
most due to hyperplasia
15% due to adenoma
5% due to carcinoma
Cushings syndrome (hypertension, obesity, diabetes, etc) may be due to:
exogenous steroids (most common)
pituitary adenoma ACTH production bilateral adrenal hyperplasia
non-pituitary tumor ACTH production bilateral adrenal hyperplasia
20% due to adrenal adenoma
10% due to carcinoma
Catecholamine excess (hypertension, tachycardia, flushing, etc.)
pheochromocytoma
Adenoma [Figure 3-14-1]
(3% of the general population)

Non-hyperfunctional (vast majority)
Hyperfunctional (imaging features are the same as non-hyperfunctional)
Figure 3-14-1
Adenoma microscopic pathology
clear cells (high lipid content)

cords of fibrovascular tissue
Adenoma (typical) macroscopic pathology
well-circumscribed
homogeneous
small (usually less than 3 cm)
Adrenal adenoma
645
Adenoma with degeneration (atypical) [Figure 3-14-2]
heterogeneous
hemorrhagic
cystic / necrotic
calcifications
(gross and radiologic appearance mimics carcinoma)
Figure 3-14-2
Adenoma radiology
CT findings (NCCT):
small, homogeneous
hypodense due to lipid content (< 18HU?, <15HU?,
<10HU?)
CT findings (CECT):
decreased enhancement compared to metastasis, etc.
rapid wash-out of contrast?
Some adenomas are lipid poor
A mass that does not satisfy the density requirements for an
adenoma may still be an adenoma (biopsy or washout study
required?)
A mass that does not decrease in signal on an opposed phase
image may still be an adenoma
MR (opposed phase imaging) [Figure 3-14-3]
spleen used as internal reference
visual assessment is generally adequate, although signal
intensity ratios of lesion:spleen may be used
Opposed phase MRI operates on the same principle (lipid
content) as non-contrast CT, therefore will generally add little to
the patient work-up* (i.e. an indeterminate lesion by CT will likely
be indeterminate on opposed phase MRI).
* controversial
Degenerating adenoma
Figure 3-14-3
Adrenal Carcinoma [Figure 3-14-4]
rare
heterogeneous
large ( mean >10 cm)
necrotic
percutaneous biopsy unreliable
more than 1/3 are calcified
half are hyperfunctional (these are generally smaller)
Adrenal adenoma, including opposed

phase imaging
Figure 3-14-4
Adrenocortical carcinoma with extension to inferior vena cava
646
Pheochromocytoma [Figures 3-14-5 and 3-14-6]
Pheochromocytoma
Myelolipoma [Figures 3-14-7 and 3-14-8]
Figure 3-14-5
almost all are abdominal

depends on definition perhaps all are adrenal
90% are adrenal (the remainder are paragangliomas)
90% are unilateral
90% are benign benignity established by clinical follow-up
elevated catecholamines
imaging generally performed for localization, not diagnosis
CT:
3 - 6 cm mass
heterogeneous when large (often cystic)
calcification < 5%
MRI:
historically, characterized as very high signal on T2
not totally specific, but normally needed only for localization
MIBG (iodine-131-meta-iodobenzylguanidine):
high sensitivity and specificity
(but generally not needed and availability is limited)
Figure 3-14-6
marrow elements: blood precursors and fat

benign no malignant potential
small lesions very unlikely to bleed
usually incidental findings, but may present with hemorrhage
may be diagnosed by needle biopsy (often not needed)
rarely extra-adrenal (differential diagnosis liposarcoma)
most are predominantly fat attenuation (CT) or SI (MR)
one third have calcification
occasionally, associated with hormonal activity
Adrenal Mass Evaluation: Helpful features
Pheochromocytoma
Mass is very large: favor adrenocortical carcinoma
Mass is between 2- 6cm in patient with hypertension: hyperfunctioning
adenoma vs. pheochromocytoma
Mass contains a cystic portion and is less than 6 cm: pheochromocytoma
Mass is primarily very high signal on T2: suggests pheochromocytoma
Calcification: inflammatory, old hemorrhage, adrenocortical ca [unlikely: mets,
pheo]
Small, homogeneous, hypodense = adenoma
Figure 3-14-7
Figure 3-14-8
Myelolipoma
Myelolipoma
647
Absolute washout calculation
percentage of enhancement washout

(HUdyn HUdelayed) / (HUdynamic HUpre)
if greater than 60%, = adenoma
Relative washout calculation
(HUdyn HUdelayed) ?(HUdynamic)

if > 40%, = adenoma
Algorithm
NCCT:
If less than 10 HU, its an adenoma [STOP]
If more than 10 HU, proceed to:
CECT: (dynamic and 15* minute delay)
If less than 30* HU on delayed scan = adenoma ?
If more than 30 HU on delayed scan, what is washout value
* = controversial
Opposed phase MRI (OPMRI)

decrease in signal relative to spleen = adenoma no further evaluation
needed
no decrease biopsy
indeterminate consider CT evaluation [will probably need
washout/delay scans because the lipid content is probably too small to
make the lesion sufficiently hypodense]
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
Blake MA, Kalra MK, Sweeney AT, et al. Distinguishing beinign from malignant adrenal masses: multi-detector
row CT protocol with 10-minute delay. Radiology 2005; 238:578-85.
Blake MA, Slattery JMA, Kalra MK, et al. Adrenal lesions: characterization with fused PET/CT image in patients
with proved or suspected malignancy initial experience. Radiology 2006; 238:970-77.
Caoili EM, Korobkin M, Francis IR, et al. Adrenal masses: characterization with combined unenhanced and delayed
enhanced CT. Radiology 2002; 222:629-33.
Elsayes KM, Narra VR, Leyendecker JR, et al. MRI of adrenal and extraadrenal pheochromocytoma. Am J Roentgenol
2005; 184:860-67.
Haider MA, Ghai S, Jhaveri K, Lockwood G. Chemical shift MR imaging of hyperattenuating (>10 HU) adrenal
masses: does it still have a role? Radiology 2004; 231:711.
Kenney PJ, Wagner BJ, Rao P, Heffess CS. Myelolipoma: CT and pathologic features. Radiology 1998; 208: 8795.
Korobkin M. CT characterization of adrenal masses: the time has come. Radiology 2000; 217:629.
Mayo-Smith WW. CT characterization of adrenal masses (letter). Radiology 2003; 226:289.
Savci G, Yazici Z, Sahin N, et al. Value of chemical shift subtraction MRI in characterization of adrenal masses. Am
J Roentgenol 2006; 186:130-53.
648
Imaging of the Urinary Bladder and

Urethra
Brent J. Wagner, MD
Outline
Figure 3-15-1
Bladder
filling defects
wall thickening (+/ calcification)
abnormal contour
Urethra
anatomy
filling defects
obstructive processes (strictures, valves)
Bladder: Filling defects
neoplasm
calculus
clot
fungus ball
ureterocele
endometriosis
schistosomiasis
(prostate)
Bladder: Types of Neoplasms
transitional cell ca (TCC) (urothelial)

squamous cell ca
adenocarcinoma
leiomyoma/sarcoma
hemangioma
metastasis
invasion
other
embryonal rhabdomyosarcoma (child)
Bladder Neoplasms: (TCC), urothelial carcinoma

[Figure 3-15-1]
males > females

80% over age 50
typically, projects into lumen
papilloma = low grade TCC
irregular surface, papillary
occasionally (30% ?) multifocal
CT to assess extraluminal extent
enhances on early CT scan; filling defect on delayed scan
Urothelial carcinoma
Figure 3-15-2
Bladder neoplasms: Differential features

[Figures 3-15-2 and 3-15-3
TCC = common
squamous cell carcinoma = look for associated stones, history of
infection (Schistosomiasis?), or chronic indwelling catheter
adenocarcinoma = often of urachal origin; look for calcified
anterior midline mass with prominent extracystic growth
Urachal Anomalies
patent urachus
umbilical-urachal sinus
vesico-urachal diverticulum
urachal cyst
Urachal carcinoma
649
Imaging of the Urinary Bladder and Urethra
Filling Defects: (may be mobile)
clot
often smooth
stones
shadowing on U/S, midline on supine radiograph
occasionally radiolucent (or obscured) post-contrast
history of infection (and/or)
evidence for bladder outlet obstruction
trabeculation
hydroureter
prostate impression
fungus ball
laminated, gas-containing
Figure 3-15-3
Filling Defects: Miscellaneous: [Figures 3-15-4 and 3-15-5]
ureterocele
smooth
prostate
midline, generally smooth
endometriosis
can look like anything
gastrointestinal inflammation
Crohns
diverticulitis
Bladder leiomyoma
Figure 3-15-4
Wall thickening [Figure 3-15-6]
I. cystitis and variants

infection
TB*
Schistosomiasis*
malakoplakia
cystitis cystica (lobulated, diffuse)
radiation*
post-cytoxan*
II. Neoplasm (TCC)
III. Bladder outlet obstruction
IV. Inflammation/invasion
Prostate carcinoma
Figure 3-15-5
*may calcify
Malakoplakia
most common in females with recurrent infection

mimics infiltrating carcinoma
cysto/bx to diagnose
Michaelis-Gutman bodies
Cystitis cystica et glandularis
etiology/significance is controversial (? inflammatory)

regenerative / reparative
proliferative cystitis
may result in wall thickening, but typically there are no imaging
findings
prominent dilated glandular lumina
Endometriosis
Figure 3-15-6
Tuberculosis
650
Cystitis glandularis is it pre-malignant?
Cystitis glandularis is so common that it may be considered a normal feature

of the vesical mucosa.
There are 2 types of cystitis glandularis
typical
intestinal (less common)
Diffuse cystitis glandularis of the intestinal type is termed intestinal metaplasia
and usually occurs in chronically irritated bladders such as those of
paraplegics or in patients with stones or long term catheterization . . . it is
associated with an increased risk of bladder carcinoma.
It is only the intestinal type of cystitis glandularis that is associated with
adenocarcinoma.
Young RH, Eble JN. Non-neoplastic disorders of the urinary bladder. In: Urologic
Surgical Pathology. Mosby 1997. pp 1745.
Schistosomiasis
calcification in 50%
calcification is rare in transitional / urothelial carcinoma
Schistosomiasis is a risk factor for squamous cell ca of the bladder
Regional enteritis (Crohns) or other gastrointestinal disease
combination of regional wall thickening and invasion

diverticulitis more common than regional enteritis
associated findings with regional enteritis
calculi
anterior/right (posterior/left for diverticulitis)
progression:
impression/thickening
invasion
fistula
Figure 3-15-7
Emphysematous cystitis
urinary tract combined with uncontrolled diabetes mellitus

gas may be intraluminal* as well as intramural
linear, lucent streaks
non-surgical condition
treatment: antibiotics and insulin
* if gas is only intraluminal, consider fistula
Abnormal contour
smooth narrowing:
pelvic lipomatosis
pelvic hematoma
(irregular narrowing = lymphoma, other mass?)
focal outpouching (diverticula):
bladder outlet obstruction
stones/tumors/bleeding
reflux/ureteral obstruction
(especially in children)
Urethra: Anatomy
posterior:
prostatic
membranous
anterior:
bulbous
penile
Condyloma acuminata
651
Urethrography: Technique
Figure 3-15-8
Clamp vs. catheter

Fluoroscopic guidance
Hand injection
Usually, dilute (30%) contrast
Urethra: Masses/filling defects
urethral carcinoma
most are squamous (if proximal, consider
transitional/urothelial carcinoma)
70% of cases in males are associated with postinflammatory
stricture
filling defect or irregular stricturing
condyloma acuminata [Figure 3-15-7]
urethral disease in only 5% of pts with external lesions
viral
Acute and subacute

gonococcal urethritis
Figure 3-15-9
Urethra: Strictures [Figure 3-15-8]
post-inflammatory
especially gonococcal (40% of strictures in the U.S.)
post-traumatic
includes iatrogenic
may be associated with perineal fistula
Urethra: Obstructive processes [Figure 3-15-9]
Urethral diverticulum (male)
Figure 3-15-10
posterior urethral valve

anterior urethral valve
(vs. diverticulum)
acquired or congenital
may obstruct, or develop calculi
Urethra: Diverticulum of the female urethra [Figure 3-15-10
outpouching of contrast
may require double-balloon technique
fluid-filled mass on CT, MR, or sonography
associated with carcinoma (usually squamous)
Urethral diverticulum (female)
References
1. Beer A, Saar B, Rummeny EJ. Tumors of the urinary bladder: technique, current use, and
perspectives of MR and CT cystography. Abdom Imaging 2003; 28:868.
2. Hahn WY, Israel GM, Lee VS. MRI of female urethral and periurethral disorders. Am J
Roentgenol 2004; 182:677-82.
3. Pavlica P, Menchi I, Barozzi L. New imaging of the anterior male urethra. Abdom Imaging 2003;
28:180.
Yu J-S, Kim KW, Lee H-J, Lee Y-J, et al. Urachal remnant diseases: spectrum of CT and US findings.
RadioGraphics 2001; 21: 451.
652
Non-Neoplastic Disorders Of The Ovary

And Adnexae And GTD
Outline
Clinical and imaging characteristics of common non neoplastic ovarian and

adnexal pathology
Role of CT / MR
Gestational trophoblastic disease
Essential Clinical Information
Age of patient
Symptoms e.g fever, discharge
Menstrual status and time in cycle
Pregnancy status
Previous surgery and medical history
Drugs, e.g HRT, ovulation stimulation
Functional Ovarian Cysts
Very common incidental findings occurring during normal ovarian cycle

Failure of ovulation or development of fluid in corpus luteum
Most regress spontaneously
Functional Ovarian Cysts
Follicular
Corpus luteal
Theca lutein
Follicular Cyst [Figure 3-16-1]
Failure of mature follicle to rupture or regress

Usually 38 cm
Unilocular simple cyst
Well defined thin smooth wall
Regress spontaneously (if <5 cm) or may respond to hormonal suppression
Clinical or sonographic follow-up in 68 weeks
Figure 3-16-1
Follicular cyst of left ovary with resolution one month later. a. initial
transabdominal ultrasound. b. initial transvaginal ultrasound. Echoes
are artefactual. c. Follow up.
653
Non-Neoplastic Disorders Of The Ovary And Adnexae
Normal Ovaries-MR [Figure 3-16-2]
Figure 3-16-2
MR of normal ovaries. a. T1-w image: left ovary (arrow) is isointense to muscle. On T2-w (b
and c) images the right and left ovaries (arrows) contain multiple high signal follicles
Follicular Cyst [Figure 3-16-3]
Figure 3-16-3
Corpus Luteum Cyst

[Figure 3-16-4]
Persistence of corpus luteum or

bleeding into it
>3 cm
Unilocular
Thick vascular wall
Wall slightly echogenic
CL cyst of pregancy regresses by
16 wk
Corpora Lutea
Symptomatic functional
cysts
a. Sagittal and b. coronal T2-w MRI showing a right sided follicular

cyst (arrow) with surrounding ovarian parenchyma
Internal hemorrhage
Rupture
May rupture and bleed into peritoneal cavity with peritoneal
signs and hypotension
Or rupture with simple free fluid
Figure 3-16-4
Hemorrhagic Functional Cysts [Figure 3-16-5]
Hemorrage occurs into existing cyst

Acute pain or asymptomatic
More common in luteal cysts
Imaging spectrum depends on age
Rapid change
Thin linear fibrin strands reticular, fish net or lacy
Retracting hyperechoic clot
Fluid debris level
Mildly thickened wall
Diffuse low level echoes with acoustic enhancement ground
glass
Rare, usually a feature of endometriosis
Figure 3-16-5
Interval development of hemorrhage into a functional cyst.

Note fine lacy linear echoes (arrow)
654
Thick walled unilocular cyst with low

resistance arterial flow in wall
Hemorrhagic Cyst [Figure 3-16-6]
Figure 3-16-7
Figure 3-16-6
Acute left pelvic pain. US and CT of left ovarian hemorrhagic

cyst. Note fishnet appearance on US and hematocrit level on
CT (arrow)
MR
Blood products
High on T1
Usually high on T2
Acute pain from cyst rupture.Note free

fluid and crenated cyst (bottom)
Cyst Rupture [Figure 3-16-7]

Cyst Rupture with Hemorrhage [Figure 3-16-8]
Theca Lutein Cysts
Gestational trophoblastic disease

Associated with high levels of HCG
Ovarian hyperstimulation syndrome
Secondary to infertility drugs
Abdominal pain, distension, nausea,
vomiting
Figure 3-16-8
Hyperstimulation Cysts
Bilateral enlarged ovaries

Multiple large cysts
May bleed, rupture or torse
OHSS associated with ascites, pleural
effusion, hemorrhage, DIC
OHSS [Figure 3-16-9]

Endometriosis
Functioning ectopic endometrium

Pelvic peritoneum, ovary, tube
Endometriosis
Symptoms
Dysmenorrhea
Dyspareunia
Pelvic pain
Cyclic pain with menses
Associated with infertility
Prevalence 25%
Acute pain and pelvic guarding. Bleeding from left corpus

luteum cyst * into peritoneal cavity. U= uterus
655
Figure 3-16-9
Ascites and bilateral enlarged ovaries with multiple cysts
Endometrioma - US
Figure 3-16-10
[Figures 3-16-10 and 3-16-11]
Thick walled cystic lesion

Ground glass homogeneous low
level echoes (highly suggestive)
Unilocular or multilocular with
septations
Mural reflectors
Rarely fluid-fluid level
Endometriosis:
MR Technique
Ground glass appearance of endometriotic cyst. a. Transabdominal

Axial T1-w SE
*Axial fat-suppressed T1-w SE to and b. transvaginal ultrasound. Note homogeneous internal echoes
and posterior acoustic enhancement
distinguish fat from blood*
Axial/sagittal/coronal T2-w FSE
Dynamic enhanced T1-w (optional)
Endometrioma: MR [Figure 3-16-12]
Figure 3-16-11
Highly accurate, sensitive and specific (90-96%)

Thick walled cystic lesion
Hyperintense on T1-w
Hypointense on T2-w with shading
Endometrioma: MR
Less specific signs

Multiple homogeneous hyperintense lesions on
T1-w and T2-w
Low signal hemosiderin ring
Enhancement of cyst wall/peritoneum
Endometrioma
Homogeneous endometriomas (*) with mural

reflectors (arrows)
Endometrioma with hematosalpinx

Rectus Endometriosis
Diffuse Endometriosis
Figure 3-16-12
More common
Associated with fibrosis and adhesions
Laparoscopy is gold standard allows
staging and treatment
MR may be useful for inaccessible
sites or for evaluation of response to
medical treatment
High T1 signal ovarian endometrioma with no suppression on

fat sat. Low signal shading on FSE T2
656
Endometriosis
Figure 3-16-13
Endometrioma or Hemorrhagic Cyst?
Clinical history
Sequential imaging with US
MR
Less bright on T1-w
No shading on T2-w
Single
Rule Out Ovarian Torsion

Ovarian (adnexal) Torsion [Figure 3-16-13]
3% of gynecologic emergencies
Usually premenopausal
20% pregnant
80% associated mass
Acute pain, nausea, vomiting
Previous self limiting episodes
Ovarian Torsion - US
Gray scale non specific, depends on cause

Most suggestive: ipsilateral enlarged hypoechoic ovary (+/peripheral follicles)
Mass e.g. teratoma, functional cyst
Hemorrhagic infarction
Associated thickened fallopian tube
Ovarian Torsion: Enlarged Ovary [Figure 3-16-14]

Adnexal Torsion
Figure 3-16-14
Ovarian Torsion - US
Absence of flow in torsed left ovary

(bottom)
Doppler is extremely useful

Absence of arterial and venous flow
High resistance arterial flow
Loss of venous flow
Twisted vascular pedicle
Corkscrew vessels
Ovarian Torsion [Figure 3-16-15]

Figure 3-16-15
Asymmetric ovarian sizes in torsion

A-CT of enlarged right ovary with hemorrhagic infarction.
Paraovarian cyst (arrow) caused the torsion.
B-Low signal ovary on T2 with folllicles.
C-T1 shows high signal from hemorrhage.
D-Post contrast sat sat T1 shows no enhancement in
right ovary
657
Torsed Teratoma [Figure 3-16-16]
Figure 3-16-16
Adnexal Torsion: CT/MR
Deviation of uterus to affected side

Obliteration of fat planes
Enlarged displaced ovary
Beak sign with congested vessels
Lack of enhancement
Torsion of Cystadenoma*
Ovarian Torsion Twisted Pedicle
[Figure 3-16-17]
Ovarian torsion
caused by para-ovarian cyst
Twisted vascular pedicle on color Doppler of a torsed teratoma
Figure 3-16-17
Ovarian Torsion
Diagnostic difficulties
Dual ovarian arterial supply
Incomplete and intermittent torsion
False positives
Technical
Pathologic
High index of suspicion if symptomatic ovary enlarged
Rescan early
Hydrosalpinx [Figure 3-16-18]
Tubular fluid filled structure

Folding mimics multilocular lesion
Sequela of PID, endometriosis, surgery
Figure 3-16-18
Twisted pedicle (arrow): intra

operative image and CT
Dilated thin walled fallopian tube: US and CT
Hydrosalpinx/Pyosalpinx
Hydrosalpinx
658
Pelvic Inflammatory Disease
Figure 3-16-19
Imaging for:
Complications
Failure to respond to first line
treatment
Alternative diagnosis
US first line
CT or MR for difficult / severe cases
Thick walled tube
Cog wheel
Internal echoes
Tuboovarian complex or abscess
Complex adnexal mass with
pyosalpinx
Acute Salpingitis
Pyosalpinx [Figure 3-16-19]
Tuboovarian Abscess Aspiration
Tuboovarian abscesses
Ovarian Vein Thrombophlebitis
Septic thrombosis in ovarian veins

Post partum or post surgery/pelvic
inflammatory disease
Pain, fever and leucocytosis
My be occult
Treated with antibiotics and anticoagulants
Distended ovarian vein
Thrombus
Perivenous inflammatory changes
Edematous adnexa
a. Coronal and b. longitudinal ultrasound of a dilated

thickened tube with internal debris and endosalpingeal fold
thickening (arrow). c. longitudinal scan of a thickened dilated
tube. d. pathologic specimen showing endosalpingeal folds
(arrow)
Figure 3-16-20
Post Partum Thrombophlebitis [Figure 3-16-20]

Peritoneal Inclusion Cysts [Figures 3-16-21 and 3-16-22]
Benign cystic mesothelioma, multilocular peritoneal cyst

Loculated peritoneal fluid within adhesions
Previous pelvic surgery/endometrosis/PID
Pre or post menopausal
Treatment
Surgical (3050% recurrence)
OCP +/ TV US guided aspiration
May mimic a cystic ovarian neoplasm
Septated cystic peritoneal lesion surrounding normal ovary
Ovary suspended by adhesions spider in a web
Flow may be present in septations
Figure 3-16-21
Multilocular cystic lesion surrounding normal ovary (o)

659
MR Venogram. Distended right

ovarian vein with intra luminal
thrombus (arrow). Uterus (*) is
enlarged with central fluid/clot
Polycystic Ovary Syndrome
Figure 3-16-22
Infertility and hormonal disturbance

37% of women
Stein Leventhal Syndrome
Amenorrhea, Infertility, Hirsutism
Absence of mid cycle LH surge
Increased LH: FSH
Suspended follicular development
Androgen production
Polycystic Ovary Syndrome

[Figures 3-16-23 and 3-16-24]
Enlarged ovaries (>1012 cm3)

Multiple (>10) small (<810 mm)
peripheral follicles
Echogenic stroma
Normal ovaries (30%)
Figure 3-16-23
Large peritoneal inclusion cyst status post bilateral renal
transplants. The uterus (arrow) is displaced and compressed
by a large pocket of fluid which herniates into the perineum
Figure 3-16-24
Hirsutism and amenorrhea. Enlarged

ovaries with multiple peripheral
follicles
Enlarged ovaries with multiple small

follicles
660
Paraovarian/Paratubal Cysts
1020% of adnexal masses

Arise in broad ligament from mesothelial and paramesonephric remnants
Any age (3rd4th decades most common)
Complicated by
Hemorrhage
Torsion
Rupture
Neoplasm
Paraovarian Cysts
Simple unilocular adnexal cyst

Separate from ovary
Lack of change with time
Rarely bilateral or multiple or complex
Serous Inclusion Cysts
17% of asymptomatic post menopausal women

<3 cm, thin walled unilocular cysts
Cyclic variation
Secondary to remote ovulation with trapping of surface epithelium in ovarian
cortex
Majority resolve, follow-up sonography
Epithelial Inclusion Cysts

Gestational Trophoblastic Disease
Heterogenous group of disorders

Abnormal proliferation of chorionic tissues
Varying propensity to invade and metastasize
Elevated beta HCG
Hyperemesis, toxemia, bleeding
Benign
Hydatidiform mole
Malignant
Invasive mole
Choriocarcinoma
Placental site trophoblastic disease
Chorionic villi of blighted ovum persist

Hydropic change in placenta
Benign Hydatidiform Mole
Most common (80%)

1 in 12002000 pregnancies (US)
Risk factors
Extremes of reproductive life
Previous mole
Risk of recurrence 1% after 1 mole
23% after 2 molar pregnancies
Benign Mole
Classic complete mole

80%
46 XX
Complete molar change
No fetal tissue
Nuclear DNA paternal
10% malignant change
Partial mole
Triploid
69 XXY 80%
69 XXX
Hydropic placenta
0.5% malignant change
661
Complete Mole - Sonography
Enlarged uterus
Echogenic mass in
endometrial cavity
Small cystic spaces
Low impedance flow
Theca lutein cysts
(2050%)
May mimic incomplete
abortion, hydropic placenta
Figure 3-16-25
Transabdominal and transvaginal US of cystic endometrial mass.(left and

center) Theca lutein cysts in right ovary (right)
Hydatidiform Mole [Figure 3-16-25]

Complete Hydatidiform Mole [Figure 3-16-26]
Figure 3-16-26
Theca Lutein Cysts

Partial Mole
Triploid fetus
IUGR, anomalies
Hydropic placenta
Spontaneous abortion
Gestational
Trophoblastic Disease
Thickened endometrium with myometrial hypervascularity (*).

Arrows: theca lutein cysts
Management
D&C
Monitoring of beta HCG levels
Exponential drop (near zero by 1012 weeks)
US to exclude pregnancy
Invasive mole 10% Chemotherapy
Choriocarcinoma 5% Chemotherapy
Recurrent Complete Mole

Malignant GTD
Invasive mole
Locally invasive, non metastatic, <10%
Vesicular chorionic villi with myometrial invasion
Choriocarcinoma
5%, hematogenous metastases to lungs, brain, liver, etc.
May not necessary follow a gestation
No villous structure
Figure 3-14-28
Choriocarcinoma [Figures 3-14-27 and 3-16-28]
Figure 3-16-27
Infiltrating myometrial mass (top)

Lung metastases (bottom)
Infiltrating cystic mass in endometrial

cavity and myometrium
662
Summary
Characteristic sonographic features allow diagnosis of most benign adnexal

masses
MR useful for indeterminate adnexal mass
HCG and ultrasound for diagnosis and follow up of GTD
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
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16.
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18.
19.
20.
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Radiographics 2002;22:785-801.
Christensen JT, Boldsen JL, Westergaard JG. Functional ovarian cysts in premenopausal and gynecologically
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Green CL, Angtuaco TL, Shah HR, Parmley TH. Gestational trophoblastic disease: a spectrum of radiologic
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Hertzberg BS, Kliewer MA, Paulson EK. Ovarian cyst rupture causing hemoperitoneum: imaging features and the
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Jain KA. Imaging of peritoneal inclusion cysts. AJR Am J Roentgenol 2000;174:1559-1563.
Lee EJ, Kwon HC, Joo HJ, Suh JH, Fleischer AC. Diagnosis of ovarian torsion with color Doppler sonography:
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Levine D, Gosink BB, Wolf SI, Feldesman MR, Pretorius DH. Simple adnexal cysts: the natural history in
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Patel MD, Feldstein VA, Chen DC, Lipson SD, Filly RA. Endometriomas: diagnostic performance of US.
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Siegelman ES, Outwater EK. Tissue characterization in the female pelvis by means of MR imaging. Radiology
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Sohaey R, Gardner TL, Woodward PJ, Peterson CM. Sonographic diagnosis of peritoneal inclusion cysts. J
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Sugimura K, Okizuka H, Imaoka I, et al. Pelvic endometriosis: detection and diagnosis with chemical shift MR
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663
Imaging of Solid Organ Transplants

Transplantation
Higher success rates with better anti rejection therapy, patient selection and
surgical techniques
Rejection remains major cause of graft loss
Immunosuppression predisposes to infection and neoplasm
Symptoms and signs of infection subtle
Types of Transplants
Renal
Pancreas
Liver
Post-transplantation Imaging
Ultrasound -- primary modality

Parenchymal echotexture
Blood supply
Fluid collections
Specific complications
Guidance for interventional procedures
CT
Collections
Infection
Surgical Complications
Neoplasm
Guidance for procedures
MRI and MRA

Parenchyma
Vascular complications
Masses
Rejection?
Other
Scintigraphy
Cystography
Cholangiography
Arteriography and intervention
Renal Transplants
CRT: Cadaveric renal transplant

LRT: Living related renal transplant
LNRT: Living non-related renal transplant
Dual: En bloc pediatric or two adult
SPK: Simultaneous pancreas-kidney
664
Renal Transplantation: Surgical Technique
Iliac fossa extraperitoneal placement

Arterial anastomosis
End to side to external iliac artery
Venous anastomosis
End to side to external iliac vein
Ureteral anastomosis very variable
Renal Transplants: Sonography
Hydronephrosis, echotexture, collections

Color Doppler: identify vessels
Duplex Doppler of
MRA, MRV
Segmental, interlobar and arcuate arteries
Measure RI x3
Real time guidance for biopsy
Normal Renal Transplant

Resistive Index
RI= (PSVEDV)
PSV
Complications
Perinephric fluid collections (50%)

Rejection acute and chronic
Obstruction (110%)
Vascular Complications (10%)
Acute tubular necrosis (DGF)
Cyclosporine toxicity
PTLD (1%)
Torsion
Figure 3-17-1
Perinephric Fluid Collections
Early
Hematomas 9%
Seromas
Urinary leak 18% (12
weeks)
Later
Abscess 30%
Lymphoceles 43% (48
weeks)
Aspirate for diagnosis
Hematoma [Figure 3-17-1]
Appearance depends on age

Acute hyperechoic
Sub acute mixed
Chronic hypoechoic/anechoic
Contained or free
Sub acute perinephric hematoma (Left).

Acute subcaspular hemorrhage with bleeding vessel post biopsy
(Center).
Organizing hematoma (Right)
Figure 3-17-2
Hemorrhage [Figure 3-17-2]
Acute perinephric high density hematoma.(Left). Free

intraperitoneal hemorrhage (Right)
665
Urinoma [Figure 3-17-3]
Figure 3-17-3
Lymphocele [Figure 3-17-4]

Abscess
Hydronephrosis
Early: edema of UVJ

Late:
Compression by fluid
collections
Denervation (non
obstructive)
Full bladder (repeat with
empty bladder)
Ureteric ischemia,
surgical technique
Cystogram showing extra luminal contrast (arrow) from ureteral leak.(Left)
(kinks)
Renogram showing collection of radioactivity (arrow) inferomedial to
Rejection
transplant and non visualization of bladder (Right)
Intraluminal clot or
calculi
Figure 3-17-5
Ureteral Stricture [Figure 3-17-5]

Hydronephrosis
Dilatation does not equal

obstruction
RI not reliable in
differentiating dilatation
from obstruction
Figure 3-17-4
Echoes Within
Collecting System
Hemonephrosis
Low level echoes
Move with patient
Hematuria
Post biopsy: look for
AVF
Urinary infection
Hemonephrosis
[Figure 3-17-6]
Simple fluid collection causing some

mass effect on the transplant (Top)
Lymphocele (arrow) medial to
transplant (Bottom)
Hydonephrosis and hydroureter

(arrow) (top)
Antegrade pyelogram showing distal
ureteral stricture (arrow) (bottom)
Figure 3-17-6
a. Hydronephrosis with clot in collecting system. b. Clot in bladder. c. CT showing high density
blood in left transplant ureter (arrow) and d. clot in bladder (arrow) as well as Foley catheter
666
Candidiasis
Fungus balls
Highly echogenic, weakly shadowing
Candida in urine
Echoes Within Collecting System
Calculi or nepohrocalcinosis
Echogenic structures with acoustic shadowing
Nephrocalcinosis
Figure 3-17-7
Gas
Emphysematous pyelonephritis
Reflux from catheterization
Gas/Stent
Rejection
Non specific elevation of creatinine

Fever, white count, pain over transplant
Decreased urine outpout
Acute (> 5 days) reversible with treament
Chronic (months to years) irreversible
Edematous kidney with prominent pyramids
(arrow), increased cortical echogenicity and
thickened urothelium (*)
Acute Rejection Gray Scale
Non specific
Enlargement
Increased cortical echogenicity
Decreased echogenicity of central sinus
Loss of corticomedullary differentiation
Prominent pyramids
Thickening of collecting system
Figure 3-17-8
Acute Rejection
[Figure 3-17-7]
Thick Urothelium
[Figure 3-17-8]
Acute Rejection
[Figure 3-17-9]
Circumferentially thickened renal pelvis (arrow) in acute rejection

Vascular rejection results in
increased resistance with increase in resistive index
Correlation highly variable
Threshold? 0.7 or 0.9
Figure 3-17-9
Acute rejection
BIOPSY - only reliable method to

determine cause of renal dysfunction
Chronic Rejection
Small allograft
Echogenic from fibrosis
Fatty replacement
Calcification
Decreased blood flow
High resistance arterial waveforms with reversal of diastolic

flow in main renal artery and absence of diastolic flow in
interlobar arteries
667
Vascular Complications
Figure 3-17-10
Early (Surgical emergencies)

Renal vein thrombosis
Renal artery occlusion
Later
Renal artery stenosis (10%)
Post biopsy complications (AVF,
PSA)
Renal vein stenosis
Renal Vein Thrombosis [Figure 3-17-10]
Gray-scale: swollen hypoechoic

Doppler:
Absent venous flow
Reversed plateauing of diastole
High resistance
Renal Artery Occlusion
a. Power Doppler with minimal flow.b. Reversed arterial flow in

diastole. c. Absent venous flow. Beware of noise
Gray-scale: swollen kidney

Doppler
Absent intrarenal arterial
High resistance, high PSV, no
diastolic flow
Spiked preocclusive wave form
NB: Severe acute rejection can cause
diminished flow
Figure 3-17-11
Renal Artery Stenosis
Hypertension, graft dysfunction and

bruit
Conventional angiography
Reference standard (invasive
contrast)
Allows angioplasty
Sonography for screening
MR Angiography
MRA [Figure 3-17-11]
a. MRA of normal transplant renal artery. b. MRA showing

diffuse atherosclerosis with a mild stenosis (arrow) of the
proximal renal artery. c. MRA of a high grade iliac stenosis
(arrow) above a normal transplant renal artery
Sonographic Criteria
PSV >2 m/s

Velocity gradient >2:1
Post stenotic spectral broadening
Pulsus tardus-parvus( prolonged early acceleration, diminished amplitude
SAT >0.07s, AI <3 m/s2 , RI <0.56
Main Renal Artery Origin

Tardus Parvus
Intrarenal Arteriovenous Fistulae/Pseudoaneurysms
Secondary to percutaneous biopsy

Most clinically insignificant and resolve
Treated conservatively if small and asymptomatic
Embolized if large or causing ischemia and severe hematuria
668
Intrarenal Arteriovenous Fistulae
Gray scale
Usually invisible
Color Doppler
Flurry/perivascular bleeding
Feeding artery draining vein if large
Aliasing
Duplex
High velocity/low resistance
Arterialized venous flow
Figure 3-17-12
Arteriovenous Fistula
[Figure 3-17-12]
Embolization [Figure 3-17-13]
Pseudoaneurysms
[Figure 3-17-14]
Gray scale
Simple or complex cyst
Doppler
Yin yang swirling disorganized flow
To and fro (neck)
May rupture
Renal Vein Stenosis
a. Color; b. Power Doppler of perivascular thrill.

c. duplex of artery and d. of draining arterialized vein
Perivascular fibrosis
Compression by fluid collections
Doppler
Aliasing
Velocity increase (x34)
Figure 3-17-13
Pancreas Transplants
SPK: Simultaneous pancreas-kidney

PAK: Pancreas after kidney
PTA: Pancreas transplant alone
Pancreatic Transplantation:
Surgical Technique
Endocrine Drainage (Venous)

Systemic (iliac vein)
Portal vein
Exocrine Drainage
Bladder
Enteric
Arterial supply from common iliac artery
Selective renal arteriography. a. abnormal distal arterial branch

with early venous filling (arrow). b. Prompt filling of renal and
iliac veins. c. Post embolization, the av fistula is no longer
visualized
Figure 3-17-14
SPK
Systemic bladder drainage

Portal enteric drainage
Pancreatic Transplant
Complications
Rejection Acute and Chronic

Surgical complications
Infection
Anastomotic Leak
Vascular thrombosis Arterial / Venous
Pancreatitis
a. Gray scale; b. color and c. duplex Doppler of

pseudoaneurysm.
Note to and fro flow in neck of pseudoaneurysm (arrow)
669
Normal Pancreas Transplant [Figure 3-17-15]
Figure 3-17-15
Pancreas Transplant CT
[Figure 3-17-16]
Pancreatic Transplant MR
Pancreas Transplant MRA
Peripancreatic Collections
2-10%
Hematoma, seroma, anastomotic leak,
abscess
Nonspecific appearance
Aspiration needed for diagnosis
Anastomotic Leak with Abscess

[Figure 3-17-17]
Pancreatic Transplant Rejection
40% of graft loss

Gray scale, color and duplex of
Enlargement and heterogeneity of gland
normal pancreas transplant.
US Doppler RI no correlation
Arrow on pancreatic duct
Diagnosed by percutaneous US guided
biopsy
Pancreatic Transplant Vascular Thrombosis
610% of graft loss

Venous more common than arterial
US and MRA most useful
Swollen heterogenous gland
No flow or enhancement
Thrombosed vessels
Figure 3-17-16
Pancreatic Thrombosis
[Figure 3-17-18]
Liver Transplantation
Established or fulminant liver failure

(hepatitis C, PBC, PSC, alcolhol,
cryptogenic cirrhosis, etc.)
Cadaveric
Living or cadaveric split liver (right lobe)
Liver Transplantation
Reformatted coronal CT showing pancreas transplant * and

vessels (arrows)
Gray scale evaluation includes

Fluid collections
Free fluid (ascites or bile)
Biliary dilatation choledochojejunostomy or
choledocholedochostomy
Parenchyma
Figure 3-17-17
Liver transplantation
Doppler evaluation includes

MPV, LPV, RPV
CHA, LHA, RHA
HV x 3
IVC above and below anastomosis
Ultrasound (a and b) and CT(c) of a peripancreatic
fluid collection containing gas (arrow). P pancreas
670
Complications
Figure 3-17-18
Rejection
Vascular thrombosis or stenosis
Biliary obstruction or leak
Recurrent hepatitis
Fatty infiltration
Neoplasm
Hepatic Artery Thrombosis

[Figure 3-17-19]
MRA
Normal
Hepatic artery thrombosis
Hepatic Artery Thrombosis

[Figure 3-17-20]
Figure 3-17-19
a. Absence of color flow on color Doppler. b. Swollen pancreas

transplant. c. Non enhancement of pancreas.following contrast
d. Stump of graft artery
Figure 3-17-20
High resistance arterial flow secondary to thrombosis just distal

to site of sample. Proximal hepatic artery (arrow) with high
resistance pattern of flow
Digital subtraction arteriogram

demonstrating hepatic thrombosis.
The splenic artery (arrow) is patent.
Percutaneous cholangiogram shows
diffuse abnormality of the bile ducts
with strictures and filling defects
(arrow) resulting from ischemic bile
ducts
671
Bilomas after Arterial Thrombosis [Figure 3-17-21]
Figure 3-17-21
Portal Vein Thrombosis [Figure 3-17-22]

Cavernous Transformation
Biliary Tree
Collections
Post Transplant Lymphoproliferative
Disorder
Related to Epstein Barr virus

Any time (mean = 15 months)
Spectrum
Polyclonal diffuse B cell proliferation
Malignant monoclonal lymphoma
Treatment
Decreased immunosuppression
Antiviral agents
Chemotherapy
Radiology
Lymphadenopathy
Solid/hollow visceral
involvement
Liver
Lungs
Spleen
Bowel
Post Transplant
Lymphoproliferative
Disorder [Figure 3-17-23]
PTLD SBO [Figure 3-17-24]
Despite revascularization after hepatic artery thrombosis,

multiple bilomas (*) have developed in the liver on CT
and MR. Hepatic artery
Figure 3-17-22
Portal vein thrombosis after liver transplant

a and b. Coronal reformatted CT showing thrombus (arrow) within the
superior mesenteric and portal veins.
c. Color Doppler ultrasound showing echoes in the portal vein with lack of
color flow. The adjacent hepatic artery is patent
Figure 3-17-24
Figure 3-17-23
Multiple hepatic lesions on CT (a) and enhanced MR (b).

Mass in the transplant kidney (c) which was biopsied (d)
under ultrasound guidance
Soft tissue mass (arrows) causing
small bowel obstruction
(arrowheads)
672
PTLD Post Liver Transplant

Post Transplant Malignancy
Kaposis sarcoma
Lymphoma
Vulva/perineum
Lip
Skin (squamous)
Cervix
x 400500
x 20350
x 100
x 29
x 740
x 414
Lymphadenopathy
Summary
Ultrasound with color and duplex Doppler is an ideal first line modality for
renal, pancreas and liver transplants
Sensitive for vascular complications, fluid collections and hydronephrosis
Biopsy needed for diagnosis of rejection
CT for infection, fluid collections, procedures, malignancy
MR for evaluation of vascular and parenchymal abnormalities
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Baxter GM. Ultrasound of renal transplantation. Clin Radiol 2001; 56:802-818.

Boeve WJ, Kok T, Tegzess AM, et al. Comparison of contrast enhanced MR-angiography-MRI and digital subtraction
angiography in the evaluation of pancreas and/or kidney transplantation patients: initial experience. Magn Reson
Imaging 2001; 19:595-607.
Crossin JD, Muradali D, Wilson SR. US of liver transplants: normal and abnormal. Radiographics 2003; 23:10931114.
Dachman AH, Newmark GM, Thistlethwaite JR, Jr., Oto A, Bruce DS, Newell KA. Imaging of pancreatic
transplantation using portal venous and enteric exocrine drainage. AJR Am J Roentgenol 1998; 171:157-163.
Hohenwalter MD, Skowlund CJ, Erickson SJ, et al. Renal transplant evaluation with MR angiography and MR
imaging. Radiographics 2001; 21:1505-1517.
Kaushik S, Fulcher AS, Frable WJ, May DA. Posttransplantation lymphoproliferative disorder: osseous and hepatic
involvement. AJR Am J Roentgenol 2001; 177:1057-1059.
Krebs TL, Daly B, Wong JJ, Chow CC, Bartlett ST. Vascular complications of pancreatic transplantation: MR
evaluation. Radiology 1995; 196:793-798.
Linkowski GD, Warvariv V, Filly RA, Vincenti F. Sonography in the diagnosis of acute renal allograft rejection and
cyclosporine nephrotoxicity. AJR Am J Roentgenol 1987; 148:291-295.
Middleton WD, Erickson S, Melson GL. Perivascular color artifact: pathologic significance and appearance on color
Doppler US images. Radiology 1989; 171:647-652.
Middleton WD, Kellman GM, Melson GL, Madrazo BL. Postbiopsy renal transplant arteriovenous fistulas: color
Doppler US characteristics. Radiology 1989; 171:253-257.
Sebastia C, Quiroga S, Boye R, Cantarell C, Fernandez-Planas M, Alvarez A. Helical CT in renal transplantation:
normal findings and early and late complications. Radiographics 2001; 21:1103-1117.
Tobben PJ, Zajko AB, Sumkin JH, et al. Pseudoaneurysms complicating organ transplantation: roles of CT, duplex
sonography, and angiography. Radiology 1988; 169:65-70.
Vrachliotis TG, Vaswani KK, Davies EA, Elkahammas EA, Bennett WF, Bova JG. CT findings in posttransplantation
lymphoproliferative disorder of renal transplants. AJR Am J Roentgenol 2000; 175:183-188.
Wong JJ, Krebs TL, Klassen DK, et al. Sonographic evaluation of acute pancreatic transplant rejection: morphologyDoppler analysis versus guided percutaneous biopsy. AJR Am J Roentgenol 1996; 166:803-807.
673
The Neglected Nephrogram

Goals
Review normal nephrographic physiology

Present 6 abnormal patterns with the respective differential diagnosis
Urogram Does Not Equal Nephrogram

Nephrogram
Figure 3-18-1
Excretory Urography
CT
MR
Nuclear Medicine
Angiography
? Ultrasound
Normal - Nephrographic
- Physiology
Renal Vascularity
Normal CT enhancement. Left, enhanced CT in the vascular

Most flow is to the cortex
(corticomedullary phase); middle, nephrographic phase; right, pyelographic
Vasa recta to medulla
phase
Capsular vessels may
supply peripheral nephrons
#2,#3 important for rim NG
and reverse NG
Normal Nephrographic Pysiology
The main driving force for urine production is filtration pressure (blood
pressure)
Contrast is filtered. It is not excreted or reabsorbed by the tubules
Contrast which gets into the nephron will eventually get to the collecting
system
Density of the Nephrogram (3 Factors)
Figure 3-18-2
Iodine concentration
GFR
Transit time
Normal Nephrogram requires
Kidney
Blood in
Blood out
Urine out
Nephrons
Normal Pyelogram
[Figures 3-18-1 and 3-18-2]
Symmetric
3 minute film
Delayed side is the diseased side
Normal MR enhancement. Enhanced T1-weighted with fat

suppression dynamic scan of the left kidney
674
6 Nephrographic Patterns
Figure 3-18-3
Absent NG
Unilateral delayed pyelogram
Bilateral persistent NG
Rim NG
Reverse rim NG
Striated NG
Pattern #1 Absent [Figure 3-18-3]
Diagnosis:Renal agenesis with seminal

vesicle cyst
Pertinent Embryology
Ureteral bud comes off of the

mesonephric duct
Close association of mesonephric duct
and ureter
Renal agenesis with ipsilateral seminal vesicle cyst. Left,

enhanced CT: there is no left kidney in the left renal fossa
which is filled by large bowel. Right, CT at the level of the
bladder shows extrinsic impression of the left posterior bladder
by a cystic mass
Following nephrectomy the small bowel fills the renal fossa

[Figure 3-18-4]
17 Yo, Trauma: Global Infarction [Figure 3-18-5]

Nonfunction of the nonobstructed kidney
equals vascular occlusion
Figure 3-18-4
No Blood In
20 YO Woman, Rt flank pain, hematuria,
facial rash [Figure 3-18-6]
No Blood Out
Bowel in the left renal fossa. CT scans of 2
different patients. Left: congenital absence or
ectopia of the left kidney with large intestine filling
the renal fossa. Right: acquired absence of the
kidney (nephrectomy) with small bowel filling the
left renal fossa
Figure 3-18-5
Figure 3-18-6
Global infarction. Enhanced CT.

There is an absent left nephrogram
due to disruption of the left renal
artery
Right renal vein thrombosis. Left:
enhanced CT shows absent
nephrogram in the right kidney which
is enlarged. Right: IV cavogram
shows thrombus in the IVC which
had extended into the right renal vein
675
74 Year Old Man, BPH [Figure 3-18-7]
There was no dilated right ureter on lower images

Right UPJ with post obstructive atrophy
No Urine Out
Figure 3-18-7
22 YO man, Hematuria [Figure 3-18-8]
32 year old woman, RUTIS

[Figure 3-18-9]
Absent Left Nephrogram

[Figure 3-18-10]
Renal TB
Long standing right UPJ

obstruction with post
obstructive atrophy. Right,
IVP with non visualization of
the right kidney. Left,
enhanced CT shows a left
dilated pelvis and calyces
Figure 3-18-10
Figure 3-18-8
Multicystic dysplastic kidney.

Left, KUB with multiple
peripheral calcifications in the
left renal fossa. Right, IVP
shows absent function on the left
Autonephrectmy from
renal tuberculosis. Top,
KUB shows multiple illdefined calcifications in
the left kidney. Bottom,
IVP shows no function
No Nephrons
Congenital (MCK)
Acquired (XGP, TB)
Figure 3-18-9
Xanthogranulomatous pyelonephritis. Upper left, KUB shows a

left staghorn caclculus. Upper right, IVP shows left
nonfunction. Bottom, CT demonstrates replacement of the left
kidney by and inflammatory mass which extends into the
perirenal space
676
Pseudo-Absent Nephrogram [Figure 3-18-11]
Figure 3-18-12
Pelvic kidney
Figure 3-18-11
Pelvic kidney. Left, CT through the kidneys shows an absent

right kidney with colon in the right renal fossa. Right, lower
section shows a nonrotated right pelvic kidney (arrows)
Pattern #1 Absent Nephrogram
No kidney
No blood in
No blood out
No urine out
No nephrons
Pattern #2 Unilateral Delayed Pyelogram [Figure 3-18-12]
TCC left UVJ (slow urine out)
Slow Urine Out

10 year old boy hypertension [Figure 3-18-13]
Renal Artery Stenosis (Slow Blood In)
Delayed pyelogram
Small kidney
Hyperdense pyelogram
Slow Blood In
Diagnosis: Compression of Renal Vein by Pancreatic
Carcinoma (Slow Blood Out) [Figure 3-18-14]
TCC left UVJ. Upper left, IVP: there

is delay of the left pyelogram. Upper
right, left kidney: There is
hydronephrosis. Lower right,
bladder near the UVJ: there is a soft
tissue mass (arrow)
Figure 3-18-13
Figure 3-18-14
Renal vascular hypertension

(Takaysus). Left, axial CT:
the left kidney is small and the
pyelogram is delayed. There
is soft tissue around the aorta.
Right, sagital MR: there is
narrowing of the aorta (arrows)
Upper, axial CT: There is a mass (M) ventral to

the left kidney. The left kidney is in the
corticomedullary phase while the right kidney is in
the nephrographic phase. Lower, axial CT at the
level of the left renal vein. The left renal vein
arrow) is compressed by the mass
677
Slow Blood Out
Figure 3-18-15
75 Yo Woman Left Flank Pain

[Figure 3-18-15]
Acute pyelonephritis
Poor nephron function
Poor Nephron Function

Pattern #2 Delayed Pyelogram (Unilateral)
Slow urine out (OBST uropathy)

Slow blood in (RA stenosis)
Slow blood out (RV compress)
Poor nephron function (pyelonephritis)
Acute pyelonephritis. Axial CT: the

left kidney has a diminished
nephrogram and delayed pyelogram
In Cases with A Delayed Pyelogram There May Be An Ipsilateral

Hyperdense Nephrogram
Slow urine out

Slow blood in
Slow blood out
Figure 3-18-16
22 YO man, flank pain, hematuria [Figure 3-18-16]
Obstruction of renal pelvis by blood clot
Pyelonephritis
Rarely produces a unilateral hyperdense nephrogram (unless

there is tubular or pelvic obstruction with pus)
Pattern #3 Persistent Bilateral NG [Figure 3-18-17]
Diagnosis:Hypotension
Hyperdense nephrogram from pelvic

obstruction by clot. Upper, noncon
CT: there is hyperdense blood in the
right renal pelvis. Lower, enhanced
CT: there is a delayed right
pyelogram and a hyperdense right
nephrogram
Figure 3-18-17
Hypotension. Left, 10 min IVP: there are bilateral persistent

nephrograms with delayed pyelograms. Right, 20 min IVP with
correction of the hypotension: normal examination
Figure 3-18-18
Contrast 3 days ago [Figure 3-18-18]
Acute tubular necrosis (ATN)
ATN
Tubular damage and obstruction

Decrease blood flow
Acute vasomotor nephropathy
Renogram
ATN
Bilateral ureteral obsruction
Bilateral renal artery stenosis
Acute tubular necrosis. Axial CT

(contrast 3 days ago): There are
persistent bilateral nephrograms with
delayed pyelograms
678
Pattern #3 Persistent Bilateral Nephrogram
Hypotension
Intra renal obstruction
ATN
Urate
Protein
Myoglobin
Less likely
Bilateral ureteral obstruction
Bilateral renal Artery stenosis
Bilateral renal vein thrombosis
Figure 3-18-19
Pattern #4 Rim Nephrogram [Figure 3-18-19
21 yo man trauma 10 days ago
Pattern #5 Striated Nephrogram [Figure 3-18-20]

5 Patterns
Absent NG
Rim NG
Striated NG
Global infarction with rim

nephrogram. Enhanced CT: The
right kidney is normal. The left
kidney shows enhancement near the
coriticomedullary junction and in the
subcapsular area resulting in a rim
of enhancement. This is the same
case as figure 5, 10 days later
Figure 3-18-20
Acute Cortical Necrosis
Decreased blood flow to the cortex

Continued perfusion to the
subcapsular and juxtamedullary
cortex
Leads to renal failure
Late cortical nephrocalcinosis
#6 Striated Nephrogram
[Figure 3-18-21]
ARPCK
Acute Pyelo
Obstruction
RVT
Contusion
Hypotension
Tubular Obst
Normal
Figure 3-18-21
Acute cortical necrosis. Enhanced CT scans: There is no

cortical enhancement with selective enhancement of the
medulla
Case courtesy Dr. Parvi Ramchandai University of Pennsylvania
Acute pyelonephritis. The striated

nephrogram shows alternating bands
of density and lucency
679
6 Patterns
Figure 3-18-22
Absent NG
Rim NG
Reverse rim NG
Striated NG
Each Pattern May Be

Segmental or Subsegmental
[Figure 3-18-22]
Tubular obstruction
Shock
Pyelo
Infarct
Segmental abnormal nephrograms, 4 different cases. Upper left:

tubular obstruction, upper right: hypotension, lower left:
pyelonephritis, lower right: lobar infarction
References
1. Davidson AJ, Hartman DS, Choyke PL, Wagner BJ. Davidsons Radiology of the Kidney & Genitourinary Tract 3rd
Edition, W.B. Saunders Philadelphia 1999.
680
Problem Renal Masses

Learning Objective
To use radiological imaging for the characterization and management of the

problematic renal mass
Centennial Sounding Board
Personal Refelection on Growth of Diagnostic Imaging
As we accurately image and inspect the human body with thinner and more
detailed sections, we approach the 12 mm serial sections of the pathologist,
who can find evidence of disease in almost every organ and everyone.
The radiologist of the future will need to understand the implications of their
findings and know the natural history of each disease detected.
Robert J. Stanley, AJR 2000;174:609
Principles of Neoplasia
Small Renal Mass
Cystic Renal Mass
Principles of Neoplasia (4 Arbitrary Steps)
Carcinoma In Situ (CIS)

Angiogenesis
Vascular invasion
Metastasis
Cell Cycle
Proliferation
Programmed death (Apoptosis)
Normally Cell Proliferation and Apoptosis are Activated in

Parallel
Controlled by genes
Neoplasia
Results from disequilibrium of proliferation and cell death
Chromosomal Instability Pathway
Multi-step process
Numerous genetic events
Can stop at any point
Carcinoma In Situ
Confined by basement membrane

Stops expanding after reaching diffusion limit of the nearest vessel
No metastatic potential
Very, very common
Carcinoma In Situ
Most human tumors exist as in situ lesions

0.2 2 mm
Renal CIS is found in 22% of autopsies
Angiogenic Phenotype
Ability to recruit host blood supply

Penetrate basement membrane
May enlarge to become macroscopic
681
Virtually All Solid Tumors Which are Visible Are Angiogenesis

Dependent
Vascular Invasion
Tumor shedding and vascular invasion may occur relatively early

In animal models, tumors shed 3-6 million cells per Gram per 24 hours
Most cells which are shed do not progress to viable metastases
Metastasis
Very imprecise at knowing which, where and when RCC will metastasize
Mets require activation of genes
Each metastasis must become angiogenic to grow
Nonangiogenic Metastases May Remain Microscopic and

Dormant for Many Years
Figure 3-19-1
Principles of neoplasia
Small renal mass
Cystic renal mass
Rationale For Management Decisions

Best Way to Treat RCC Excision or Ablation
Chemotherapy, radiation and immunotherapy are

less effective
Critical Feature of RCC
Noncon
Enhanced
Cyst, AML and solid nonfatty mass in the right
kidney. Left, nonenhanced and right, enhanced
Renal Tumors <3 cm Uncommonly Have
axial
CT through the kidney. A is a 1.8 cm cyst. B
Detectable Metastases
is
a
2
cm AML. C is a 1.7 cm nonfatty mass which
The renal tumor doesnt know how large it is
most
likely
represents a renal epithelial tumor with
The larger the renal tumor, the more undifferentiated
a
low probability of metastasis
it may be
Metastases
The more undifferentiated, the greater the liklihood that a metastasis can
become angiogenic
Pathology / Radiology
The Small (< 3 cm) Renal Mass
1.5 - 3.0 cm
A . Cyst
B . Cystic
C . Solid
< 1.5 cm (often TSTC)
The Small Solid Renal Mass [Figure 3-19-1]
Fat = AML
No Fat
A . Renal Epithelial Tumor with low metastatic potential
B. Cannot diagnose Adenoma (Oncocytoma)
Management Options
Excise
Ablation
Follow
Biopsy
Nephrectomy
Ignore
682
The Small (< 3 cm) Renal Mass
1.5 - 3.0 cm
A . cyst
B . cystic
C . solid
< 1.5 cm (often TSTC)
Figure 3-19-2
Too Small To Characterize
No single algorithm for every case

Risks and benefits of any strategy
Always Consider
Pretest probability
Patients ability to tolerate uncertainty
Your ability to tolerate uncertainty
How Should The TSTC Mass Be Managed?
Enhanced CT scans of 2 different patients both of

which have a lesion which is too small to
characterize. Left, renal cell carcinoma; right,
renal cyst
There is no large, prospective, pathologically proven series which indicates

correct management
RCC / Cyst [Figure 3-19-2]

Too Small to Characterize (< 1.5 cm)
1. Ignore
2. Follow
3. Get another study
How Often Should Small Lesions Be Followed?
The smaller the lesion, the longer the followup interval

If following a lesion, compare oldest comparable study available
Figure 3-19-3
How Is Doubling Time Calculated?

http://www.chestx-ray.com/index.html
Small Lesion Considered Aggressive
Size >3cm
Doubling time faster than 6 months
Too Small to Characterize (< 1.5 cm)
1. Ignore
2. Follow
3. Get another study
T2 Fat Sat: Simple Cyst [Figure 3-19-3]
Value of getting a T2 fat sat MR when the CT is

equivocal
Get the Referring Doctor Involved
Simple cyst confirmed on MR. Upper CT scans

(unenhanced, corticomedullary and pyelographic
phases): the mass (arrow) is too small to
characterize. Below, T2 fat sat MR: the lesion is
homogeneously bright
Get the Patient Involved

Principles of neoplasia
Small renal mass
Cystic renal mass
683
Teaching Goal
To allow you to suggest the appropriate management for cystic renal

masses:
Ignore
Excise
Follow
Rationale For Management
10% of cases of renal carcinoma present as a fluid-filled mass

The simple cyst can be confidently diagnosed by ultrasound CT or MR
Rarely simple cysts become complicated
Hemorrhage
Infection
Ischemia
On gross examination, the complicated cyst may be indistinguishable from
cystic renal cell carcinoma
Differentiation is based upon histological diagnosis
The most effective treatment for renal cell carcinoma
Surgery or Ablation
A cyst not simple if it has any of the following

Renal Carcinoma Can Grow As A Fluid Filled Mass
Calcification
Hyperdense/high signal
Septations
Multiple locules
Enhancement
Nodularity
Thick wall
Although microscopic evaluation is required for precise

diagnosis, there are certain radiological findings which are
reliable in differentiating complicated cyst from cystic renal cell
carcinoma
Caveats
The portion of the cystic mass which is most worrisome should be used in
deciding appropriate management.
In cases with discordant imaging findings utilizing different radiological
examinations, the lesion should be managed based upon the most aggressive
imaging findings
What About Biopsy?
If there is a strong clinical suspicion that the mass is inflammatory, careful

puncture is acceptable. If there is evidence of infection, treat (antibiotics,
drainage, etc.) and follow
Renal neoplasia very rarely presents with infection
Guidelines
Ignore
Excise
Follow
Calcification
Ignore
Small amount
Smooth
Septal
Milk of calcium
No enhancement
684
Figure 3-19-4
Excise
Enhancement
Wall thickening
Nodularity
Follow
Thick
Nodularity
No enhancement
Hyperdense/High Signal [Figure 3-19-4]
CT: > 20 HU on unenhanced CT
Hyperdense/High Signal [Figure 3-19-5]
MR: higher signal intensity than water on T1-weighted images
Most Hyperdense Masses Are Cystic
Blood
Protein
Colloid
Hyperdense Masses May Be Solid [Figure 3-19-6]
Lymphoma
RCC (papillary)
AML (small amt of fat)
Mets
Hyperdense renal masses.

Unenhanced CT: there are two
masses (arrows) which are denser
than water. Without contrast, it is
impossible to state if they are cystic
or solid
Figure 3-19-6
Figure 3-19-5
Renal lymphoma (solid).

Unenhanced CT scan shows several
hyperdense masses some of which
are confluent (arrows)
High signal renal mass. T1-weighted

axial MR of the left kidney: the signal
of the mass is higher that that of
water
Hyperdense/High Signal
Ignore
Sharp margin
< 3 cm
Not completely intrarenal
Homogeneous or hematocrit
No enhancement
US: cyst or cystic
Significant Enhancement
CT
<10 H.U.=Beam hardening
10-15 H.U.=Indeterminate
>15 H.U.=Vascularity
685
MR
<15% relative enhancement = Benign
>15% relative enhancement =
Surgical
Hyperdense/High Signal
Excise
Poorly defined
Heterogeneous
Enhancement
US: solid
Follow
Totally intrarenal
> 3 cm
Septation
Ignore
Thin (< 1 mm)
Smooth
May calcify
No enhancement
Excise
Thick, irregular
Nodular
Enhancement
Follow
Only slightly greater than hairline
A cyst not simple if it has any of the following
Calcification
Hyperdense/high signal
Septations
Multiple locules
Enhancement
Nodularity
Thick wall
Figure 3-19-7
With More Than 3 or 4 Septa,

Multiloculated [Figure 3-19-7]
Multiloculated Masses
Excise
All multiloculated masses
Three multiloculated masses. Left: ultrasound; middle: enhanced CT;

right: T2-weighted axial MR
ML-RCC
Multilocular Cystic Nephroma (MLCN)
MLCN
Female
No Blood
Pelvic Herniation
Usually Benign
ML-RCC
Male
Blood
Venous Invasion
Always Malignant
Wall Thickening, Enhancement, Nodularity: Cannot Tell Benign

From Surgery
Enhancement
Excise
All enhancing masses
686
Nodularity
Figure 3-19-8
Excise
All masses with enhancing nodularity
Follow
Very small nonenhancing nodules
Wall Thickening
Excise
All noninfected masses with wall thickening
The Current Radiological Approach To Renal Cysts

Morton A. Bosniak, M.D., Radiology January 1986
Bosniak Classification - Ignore, Excise, Follow
I Simple cyst
II Min complic
IIF Probably benign
III Benign or malignant
IV Clearly malignant
Ignore
Ignore
Follow
Excise
Excise
Calcified cystic renal mass that can

be ignored. Top, unenhanced CT:
there is a small amount of
calcification at the 11 0clock position
as well as milk of calcium at the 6
oclock position. Bottom, enhanced
CT: there is no enhancement of the
mass
Communication is crucial!
Examples
Calcification that can be ignored [Figure 3-19-8]

Small amt of peripheral Ca++
Milk of calcium
No enhancement
Surgical Calcification [Figure 3-19-9]
Septal
Thick, irregular, nodular
Enhancement
Uncomplicated Cyst [Figure 3-19-10]
Figure 3-19-9
Figure 3-19-10
NON CON
NEPHROGRAPHIC
EXCRETORY
Calcific cystic mass which should be followed. Left,

unenhanced CT, middle early phase of enhancement, right,
excretory phase of enhancement. The mass is sharply
marginated and does not enhanced
687
Cystic renal mass which should be

excised. Top, unenhanced CT:
There is a thin septation which is
calcified (horizontal arrow). There is
an irregular nodular calcification at
the 1 oclock position. Bottom,
enhanced CT shows an area of
enhancement (vertical arrow)
adjacent to the calcific nodule
Figure 3-19-11
Follow-up Calcification [Figure 3-19-11]

Nodular
No enhancement
Hyperdense that can be ignored
[Figure 3-19-12]
Well defined, homogeneous

< 3 cm
No enhancement
Homogeneous with 7 OR 13.5 HU of
enhancement [Figure 3-19-13]
Surgical Hyperdense [Figure 3-19-14]
Papillary RCC
Solid on US
Follow-up Hyperdense [Figure 3-19-15]
Homogenous
No enhancement
Cystic
> 3 cm
NON CON
NEPHROGRAPHIC
EXCRETORY
Higher CT sections show several irregular nodules of

calcification. There is no enhancement
Figure 3-19-12
Figure 3-19-13
Hyperdense cyst that can be ignored. Left,

unenhanced CT: The mass is hyperdense (34
HU), homogeneous, well-defined and less than 3
cm. Right, enhanced CT: the mass does not
enhance (31 HU) and remains homogeneous
Figure 3-19-14
Because the enhancement was equivocal,

ultrasound was performed showing a solid mass
which was a papillary renal cell carcinoma
Figure 3-19-15
Minimally enhancing
hyperdense papillary renal cell
carcinoma. Top, unenhanced
CT scan: the mass measures
29 HU. Center, enhanced CT
scan, nephrographic phase:
the mass enhanced 13.5 HU to
42.5 Bottom, enhanced CT,
pyelogram: the mass deenhances to 36 HU
Hyperdense cyst which should be followed. Left, unenhanced

CT: the 6 cm mass is homogeneous and measures 68 HU.
Center, enhanced CT, the mass remains homogeneous and
does not enhance. Right, ultrasound: the mass is cystic with a
thin septation. Because the mass is greater than 3cm it should
be followed
688
Septations that can be ignored [Figure 3-19-16]

Thin <2mm, no nodularity
No enhancement
Figure 3-19-16
May calcify
Surgical Septations [Figure 3-19-17]
Thick > 2mm
Associated nodularity
Associated nodularity
Complicated cyst
Cystic RCC
MLCN / MLRCC [Figure 3-19-18]
Surgical [Figure 3-19-19]
Enhancing thick wall
Septations which can be ignored. Left, ultrasound: the septum
Surgical nodularity [Figure 3-19-20]
is thin without nodularity. Right, CT: the septum is thin and
Enhancing nodule
smooth without apparent enhancement
Case 10 [Figure 3-19-21]
Very small nonenhancing nodule
But follow very, very carefully
Baseline / 1 year later
[Figure 3-19-22]
Figure 3-19-17
Figure 3-19-18
Septations which should be excised. Left, ultrasound: the

septation is thick and has associated nodularity (horizontal
arrow). Right, CT: the septation is irregular and enhancing
Left: complicated cyst. Right: cystic renal cell carcinoma
Figure 3-19-19
2 different multiloculated
masses. Top: multilocular
cystic nephroma. Bottom:
multiloculated renal cell
carcinoma
Cystic renal cell carcinoma with an enhancing thick wall. Left,
unenhanced CT: the thick wall is difficult to appreciate. Right,
enhanced CT: the thick enhancing wall is easily visualized
689
Figure 3-19-20
Figure 3-19-21
2 different cases of cystic renal cell carcinoma. Enhancing

nodule (arrow) in the wall of each is clearly visualized
Figure 3-19-22
Cystic renal cell carcinoma with a small nodule which was

followed. Left, baseline T2 fat sat MR shows a small nodule in
the 8 o'clock position (arrow). Right, follow-up T2 fat sat MR
one year later shows increased nodularity and thickening
Very small nonenhancing nodule

which could be followed. Top,
ultrasound: there is a very small
nodule (vertical arrow). Bottom,
enhanced CT: there is a very small
nonenhancing nodule (horizontal
arrow)
References
1.
Hartman DS, Choyke PL, Hartman MS.A practical approach to cystic renal masses.RadioGraphics 2004;24: S101S115.
690
Genitourinary Seminar 1: MSAFP

All of the following scans were ordered following a routine
blood test.
What was the test?

Was it high or low?
Elevated Maternal Serum Alpha-Fetoprotein (MSAFP)

Fetal Alpha-fetoprotein
Glycoprotein produced by fetal liver, GI tract, and yolk sac

Excreted through the urinary tract into the amniotic fluid
Peaks at 1416 wks
Small amounts leak into maternal circulation
Maternal Serum Alpha-fetoprotein (MSAFP)
Screening in second trimester (1618 weeks)

Elevated if 2.5 MOM (multiples-of-the-median)
1015% risk of open neural tube defect
Case 1
Case 2
Elevated MSAFP
Incorrect dates
Twins
Fetal death
Open neural tube defect
Abdominal wall defect
Subchorionic hemorrhage
691
Seminar 1: MSAFP
Case 3
Case 4
Case 5
Placental hemorrhages
Elevated MSAFP
Can perform amniocetesis and measure direct AFP and ACE

Acetylcholinesterase (ACE) neural tissue specific
Elevated MSAFP
Inc AFP, inc ACE ONTD

Inc AFP, nl ACE abdominal wall defect
Nl AFP, nl ACE prior bleed
Decreased MSAFP
Trisomy 21,18
Combine with human chorionic gonadotropin (hCG) and estriol (uE3) for
increased specificity triple screen
Seminar 1: MSAFP
692
Genitourinary Seminar 2: Renal Calcifications

Renal Calcifications
Dystrophic calcification
Nephrocalcinosis
cortical
medullary
Nephrolithiasis
Dystrophic Calcification
Calcification of abnormal tissue

DDx
tumor
inflammatory mass (TB)
hematoma
cysts
66 yo with hematuria
Renal Tuberculosis
Hematogenous spread
Bacilli lodge in
corticomedullary jct.
Progress along nephron
into pelvo-calyceal
system
75% of active TB only in
one kidney
Symptoms
Asymptomatic
Frequency
Hematuria
Sterile pyuria
Papillary necrosis
693
Granuloma formation
Seminar 2: Renal Calcifications
Radiologic Findings
10% normal
Papillary irregularity
Papillary necrosis
Radiologic Findings
Infundibular stenosis
Amputated calyx
Parenchymal scarring
Tuberculomas
Calcifications
Present in 3050%
Variable appearance
punctate healed granulomas
amorphous granulomatous masses
extensive reniform autonephrectomy (putty kidney)
Ureter and bladder may also be involved
Diffuse renal calcification
45 yo woman from Mexico with pyuria
Medullary Nephrocalcinosis
Metastatic calcification calcification in normal tissue

Triangular deposition conforming to pyramids
Renal function usually not impaired
Often associated with nephrolithiasis
Hypercalcemic states
hyperparathyroidism, paraneoplastic, sarcoidosis, milk-alkali syndrome,
hyper-vitaminosis D
Medullary sponge kidney (renal tubular ectasia)
may be unilateral or focal
Renal tubular acidosis Type I (distal)

distal tubule can not secrete hydrogen ion, urine becomes alkaline
symmetric
Oxalosis
primary (children) -severe may also see cortical calcification
secondary distal small bowel resection
694
27 yo man with a history of stone

disease
5 yo boy with an inherited disorder
45 yo female with microscopic

hematuria and intermittent flank pain
Cortical Nephrocalcinosis
Egg-shell calcification
Generally small kidneys
Renal function usually impaired
Cortical Nephrocalcinosis
Chronic glomerulonephritis
Acute cortical necrosis
pregnancy, sepsis, trauma, nephrotoxins (ethylene glycol)
Chronic transplant rejection
Alports syndrome
nephritis, nerve deafness, hematuria, ocular abnormalities
695
33 yo male in an aircraft accident with

severe chest and skeletal trauma
47 yo male admitted for an abdominal

abscess. What is the renal disease?
696
Radiologic Pathology 2006-2007 - Volume 1 - Index

A. Israelii 187
Abdominal Angina 499
Abdominal Wall Defects 609
Abscess (Acute Pancreatitis) 463
Abscess (Crohn Disease) 388
Abscess (tuboovarian) 659
Absent Nephrogram 677
Accessory Spleen 532
ACCP 136
Achalasia 164
Actinomycosis 178
Acute Cholecystitis 439, 441
Acute Epididymitis 591
Acute Interstitial Pneumonia AIP 18
Hammon-Rich 18
Acute Mediastinitis 165
Acute Mesenteric Ischemia 488
Acute Pancreatitis 460
Acute Rejection (Kidney Transplants) 667
Acute Scrotum 591
Adenitis (Mesenteric) 430
Adenocarcinoma (bile ducts) 317
Adenocarcinoma (Gastric) 332
Advanced Gastric Carcinoma 333
Carmen Meniscus Sign 334
Early Gastric Carcinoma 333
WHO Classification 333
Adenocarcinoma (lung) 115
Adenocarcinoma (small intestine) 356
Adenoid Cystic Carcinoma 196
Adenoma (male Breast) 257
Adenoma (Oligocystic) 328
Adenoma (parathyroid) 162
Adenovirus 182
Adhesion (GI) 477
Adnexae (non neoplastic disorders) 653
Adnexal Torsion 658
ADPCKD 306
ADPKD 614
Adrenal Imaging 645
Adrenal Tumors 645
Adenoma 645
Carcinoma 646
Myelolipoma 647
Pheochromocytoma 647
Washout Calculation 648
AIDS Cholangiopathy 308
AIDS-Related Lymphoma (GI) 350
Airways Disease 26
Allergic Bronchopulmonary Aspergillosis 27
Diffuse Panbronchiolitis 27
Langherhans Cell Histiocytosis 27
Lymphangioleiomyomatosis 27
Mosaic density 27
AIUM Guidelines: First Trimester 597
Allergic Bronchopulmonary Aspergillosis 35
Alpha-1 Antitrypsin Deficiency 31
Alpha-fetoprotein (Fetal) 691
Alveolar Filling Pneumonias 178

Amebic Abscess (hepatic) 285
Anembryonic Pregnancy 598
Anencephaly 605
Aneurysm (mediastinum) 164
Angiodysplasia (GI Bleeding) 474
Angiomyolipoma (Kidney) 570
Angiosarcoma (intrahepatic) 280
Angiosarcoma (Spleen) 539
Anorectal Lymphoma 350
Anthrax 189
Appendagitis 430
Appendiceal Neoplasms 428
Appendicitis 427
ARPKD 616
Arteriovenous Fistulae/Pseudoaneurysms (post kidney
transplantation) 668
Asbestos 49
Asbestosis and Cigarette Smoking 52
Mesothelioma 50
Pleural Effusion 50
Pleural Plaques 50
Round Atelectasis 51
Rounded atelectasis 49
Ascariasis 188
Ascariasis lumbricoides 189
Aspergillus 77
Air Crescent 77
Halo-Sign 77
Aspiration (Nosocomial Pneumonia and) 186
Asplenia (Ivemark Syndrome) 534
Asthma 33
Atresia 609
Esophageal 609
Jejunal/Ileal 609
Atrial Septal Defect 133
Atypical Adenomatous Hyperplasia 117
B. Anthracis: Anthrax 189
BCG 71
Benign Hepatic Neoplasms 267
Bile Duct (Hepatic) Cyst 272
Biliary Cystadenoma 272
Focal Nodular Hyperplasia 268
Hemangioma 267
Hepatocellular Adenoma 270
Hepatocellular Adenomatosis 271
Lipomatous Tumors 273
Bilharziasis (see Schistosomasis) 288
Biliary Disease (benign) 303
Acute Pyogenic Cholangitis 309
AIDS Cholangiopathy 308
Caroli Disease 304
Choledochal Cyst 305
Polycystic Liver Disease 306
Primary Sclerosing Cholangitis 307
Recurrent Pyogenic Cholangitis 309
Biliary Parasites 288
Clonorchis sinensis 289
I1
Bilomas (liver transplantation) 672

Bladder Neoplasms 649
Blastomycosis 104
Pathology 105
Radiologic Manifestations 105
Bleeding - Gastrointestinal 468
Boerhaaves Syndrome 403
Bowel Disease (Idiopathic Inflammatory) 382
Bowel Ischemia 487
Brachytherapy (Prostate Cancer) 622
Breast 229
Angiosarcoma 236
Congenital Anomalies 230
Fibroadenoma 231
Fibrosarcoma 236
Invasive Ductal Cancer 233
Invasive Ductal Carcinoma 234
Invasive Lobular Cancer 235
Lobular Neoplasia 232
Mastitis 230
Medullary Carcinoma 234
Normal Anatomy 229
Pagets Disease 234
Papilloma 232
Phyllodes Tumor 231
Pregnancy Changes 230
Sarcoma 236
Spindle Cell Sarcoma 236
Tubular Carcinoma 235
Breast (Male) 257
Breast (Young Women) 246
Angiosarcoma 254
Benign Lesions 247
Diabetic Mastopathy 251
Fibroadenoma 247
Granular Cell Tumor 249
Granulomatous Mastitis 252
Hamartoma 250
Juvenile Hypertrophy 251
Juvenile Papillomatosis 250
Lactating Adenoma 250
Lymphoma 254
Phyllodes High Grade 254
Phyllodes Tumor 249
Pseudoangiomatous Stromal Hyperplasia (PASH) 251
Sarcoma 253
Secretory Carcinoma 253
Bronchial Adenoma 192
Bronchial Carcinoid 194
Bronchiectasis 190
Bronchiolitis Obliterans 80
Bronchioloalveolar Carcinoma 117
Bronchocentric Granulomatosis 71
Bronchogenic Cyst 159
Brunner Gland 354
Hamartoma 354
Hyperplasia 354
Budd-Chiari Syndrome 298
Burkitt Lymphoma 348
CA-125 638
Calcifications (benign - breast) 225

Fibroadenoma 226
Loa Loa 227
Lobular 226
Secretory 227
Skin 226
Sutural 226
Vascular 227
Cancer (Male Breast) 261
Carcinoid
Atypical 193
Typical 192
Duodenal, Jejunal, Ileal 358
Gastric 341
Thymic 154
Syndrome 358
Carcinoma - Adrenal 646
Carcinoma (Male Breast) 262
Lymphoma (Male Breast) 263
Metastasis (Male Breast) 262
Papillary Carcinoma 262
Carcinoma (Scirrhous - Stomach) 334
Carcinoma Colon, Rectum (see Colorectal Carcinoma) 361
Carcinosarcoma 197
Cardia (Carcinoma) 334
Carneys Triad 200
Caroli Disease 304
Castleman Disease 151, 346
Cavitary pneumonia 190
Cavitation 190
Cecal Volvulus 400
Cervix Carcinoma 559
Chest Wall 216
Neoplasms 218
Chiari II 604
Cholangiocarcinoma (intrahepatic) 279
Cholangitis 309
Acute Pyogenic 309
Recurrent Pyogenic 309
Cholecystitis 438, 441
Choledocholithiasis 442
Cholelithiasis 438
Cholescintigraphy 439
Chondroma 201
Chorionic Sac 594
Choroid Plexus Cysts 605
Chronic Idiopathic Intestinal Pseudo-obstruction 476
Chronic Liver Disease 293
Budd-Chiari Syndrome 298
Cirrhosis 293
Hemochromatosis 300
Hemosiderosis 300
Hepatocellular Carcinoma 295
Nonalcoholic Steatohepatitis 297
Primary Biliary Cirrhosis 296
Steatosis 296
Chronic Pancreatitis 460, 465
Chronic Thromboembolic Disease 133
Churg-Strauss Syndrome 67
Cirrhosis 293
CNS Malformations (Fetal) 602
I2
Coccidioidomycosis 106
Coccidioidoma 107
Pathology 106
Radiologic Manifestations 106
Colitis
Ischemic 398
Colon (Inflammatory Disease) 391
Cecal Diverticulitis 397
Diverticular Hemorrhage 397
Diverticulitis 395
Diverticulitis vs. Carcinoma 396
Giant Sigmoid Diverticulum 397
Ischemic Colitis 398
Colonic Lymphoma 350
Colonic Polyposis 519
Colorectal Carcinoma 361
Adenoma 363
Complications 366
EUS 368
Inflammatory Bowel Disease 366
Lymphatic Spread 369
Multiple 365
Obstructing 366
Rectal Adenocarcinoma: Lymphatic Drainage 369
Screening 362
Synchronous 365
Villous Adenoma 363
Community-acquired Pneumonia 179
Congenital CNS Malformations 602
Congenital Diaphragmatic Hernia 608
Constrictive Bronchiolitis 36
Cor Pulmonale 133
Corpus Callosum - Agenesis 605
Cowdens Syndrome 525
Crohn disease 382, 386
Gastric 410
Cronkite-Canada syndrome 526
Cryotherapy (Prostate Cancer) 622
Cryptorchidism 588
Cyst - Bile Duct (Hepatic) 272
Cyst (Breast) 223
Pneumocystography 223
Thickened Skin Pattern (breast) 224
Cyst (Bronchogenic) 159
Cyst (thymic) 161
Cystadenoma (Biliary) 272
Cystic Abdominal/Pelvic Collections 611
Cystic Adenomatoid Malformation 608
Cystic Disease of the Kidney 614
Cystic Fibrosis (P. aeruginosa) 185
Cystic Hygroma 607
Cystic Kidney Disease - Acquired 618
Cystic Lymphangioma (Male Breast) 258
Cystic Nephroma 686
Cystitis 650, 651
Cysts (Congenital - Mediastinum) 160
Cytomegalovirus 78
Cytomegalovirus Pneumonia 78
Dandy-Walker Malformation 604
Daughter cysts (E. granulosus) 287
Delayed Pyelogram 677, 678
Diabetic mastopathy 258
Diffuse Alveolar Damage 79

Diffuse Lung Disease 3
AIP 4
Asbestosis 3
Asthma 3
Bronchiectasis 6
Bronchoalveolar cell carcinoma 4
Constrictive bronchiolitis 3
Cor Pulmonale 11
DAD 4
DIP 4
Edema 6
Emphysema 3
Granuloma 7
Hypersensitivity pneumonitis 3
LAM 3
LCH 4
Lfgren syndrome 12
Lymphoma 4
NSIP 4
Organizing Pneumonia (BOOP) 4
Sarcoidosis 3, 6, 7
Differential Diagnosis 10
Mycetoma 12
Diffuse Panbronchiolitis 36
Diverticulitis 396
Cecal 397
Colovesical Fistula 396
CT 397
Pericolic Abscess 396
Ductal Carcinoma in Situ 238
Biopsy 243
Calcification 240
Classification 239
Ductal Plate 303
Duodenal Carcinoid 357
Duodenitis 455
Echinococcosis 188
Echinococcus granulosus 188, 286
Echinococcus multilocularis 286
Ectopic pregnancy 598
Eisenmenger Physiology 133
Emphysema 27
Emphysematous Cholecystitis 441
Emphysematous Cystitis 651
Encephalocele 605
Endometrial Carcinoma 558
FIGO Staging 559
Endometrioma 656
Endometriosis 655, 656
Endorectal Coil MRI 622
Enteric Cyst 374
Enteritis - Chronic Radiation 497
Enterography (MR - Crohn Disease) 389
Epidermal Inclusion Cyst (Male Breast) 260
Epidermoid Cyst(Testis) 588
Epididymal Masses 590
Adenomatoid Tumor 590
Lipoma (Paratesticular) 592
Papillary Cystadenoma 590
Polyorchidism 592
I3
Epididymis 586, 636

Epididymitis 591
Epiploic Appendagitis 430
Epithelial Inclusion Cysts 661
Esophageal Rupture (Causes) 403
Esophageal Varices 470
Esophagus - Inflammatory Diseases 444
Barretts Esophagus 445
Candida Esophagitis 446
CMV Esophagitis 447
Drug-Induced Esophagitis 448
Herpes Esophagitis 446
HIV Esophagitis 447
Intramural Pseudodiverticulosis 448
Reflux Esophagitis 444
Esophagus - Malignant Tumors 452
Adenocarcinoma 453
Early Squamous Cell Carcinoma 453
Spindle Cell Carcinoma 454
Squamous Cell Carcinoma 452
Esophagus Tumors 450
Duplication Cyst 452
Leiomyoma 450
Leiomyomatosis 451
Squamous Papilloma 450
Extralobar Sequestration 608
Extramedullary hematopoiesis 164
Extrarenal Cysts 615
Fetal Anomalies 607
Fibrolamellar Carcinoma 278
Fibrous Pseudotumor 592
Foramen Of Winslow Hernia 404
Fournier Gangrene 591
Galactocele 223
Gallbladder and Biliary Neoplasms 313
Gallbladder Empyema 442
Gallbladder Wall Thickening 440
Gallstone 438
Ganglion Cell Tumors 581
Ganglioneuroblastoma 158
Ganglioneuroma 158, 581
Gangrenous Cholecystitis 441
Gastric Lymphoma 335
Gastric Malignancies 332
Carcinoid 340
Carcinoma of the Cardia 334
Gastric Adenocarcinoma 332
Gastrointestinal Stromal Tumors (GIST) 338
Kaposi Sarcoma 341
Lymphoma 335
Mesenchymal Neoplasm 338
Metastases 341
Mucosa-Associated Lymphoid Tissue 335
Scirrhous Carcinoma 334
Gastric Ulcer Investigation 457
Gastric Volvulus 405
Bezoar (Gastric) 407
Zollinger-Ellison Syndrome 408
Gastritis 455
Gastrointestinal Bleeding 468
Angiography 470
Endoscopy 469
Gastritis 470
Lower GI 469
Nuclear Scintigraphy 469
Risk of Rebleeding 470
Upper GI 469
Gastrosplenic ligament 531
Germ Cell Neoplasms 155 (Chest)
Germ Cell Tumors (Retroperitoneum) 583
Gestation 596
Gestational Sac 594
Gestational Trophoblastic Disease 661
GIST (Gastrointestinal Stromal Tumors) 338
Glucagonoma 328
Goiter (mediastinal) 161
Graft-vs-Host Disease 79
Granular Cell Tumor (Male Breast) 260
Granuloma (lung) 205
Granulomatous Mastitis 261
Gut Hemangioma 471
Gynecomastia 257, 258
H.influenzae 178
H.Pylori 459
Hamartoma 199
Hamartoma (Breast) 222
Hemangioma 268
Hemangioma (Gut) 471
Hemangioma (Mediastinum) 163
Hemochromatosis 300
Hereditary 300
Secondary 301
Hemosiderosis 300
Hepatic Artery Thrombosis (liver transplantation) 671
Hepatic Cyst (Complex) - Differential Diagnosis 417
Hepatic Mass with a Scar - Differential Diagnosis 418
Hepatic Neoplasms 267
Hepatic Portal Venous Gas 402
Hepatocellular Adenomatosis (see Benign Hepatic
Neoplasms) 271
Hereditary GI Polyposis Syndromes 524
Herniations 164
Hiatus Hernia 164
Morgagni 164
Herpes viruses (respiratory) 182
Heterotopic Pregnancy 599
Histoplasmosis 100
Acute Radiology 101
Chronic 102
Disseminated 102
Fibrosing Mediastinitis 104
Histoplasmoma 103
Mediastinal granuloma 103
Pathology 101
Hodgkin Disease - Mediastinum 150
Holoprosencephaly 603
Hydranencephaly 602
Hydrocele 590
Hydrocephalus 603
Hydronephrosis 610
Hydronephrosis (post transplantation) 666
Hydrosalpinx 658
Hypoplastic Left Heart 608
I4
Idiopathic Inflammatory Bowel Disease 382

Immunocompromised Host (Hepatic Infections) 289
Candidiasis 289
Hepatosplenic Candidiasis 290
Pneumocystis jiroveci 291
Infiltrating Colloid Carcinoma 323
Inflammatory Carcinoma (breast) 225
Inflammatory Myofibroblastic Tumors 379
Inflammatory Pseudotumor (lung) 202
Influenzae 178
Influenzae A 181
Inguinal Hernia 634
Iniencephaly 607
Interstitial Pneumonia 182
Interstitial Pneumonias 14
Acute Interstitial Pneumonia (AIP) 14
Cryptogenic Organizing Pneumonia (COP) 14
Desquamative Interstitial Pneumonia (DIP) 14
Idiopathic Pulmonary Fibrosis (IPF) 14
NonSpecific Interstitial Pneumonia (NSIP) 14
Respiratory Bronchiolitis-Interstitial Lung Disease (RBILD) 14
Usual Interstitial Pneumonia (UIP) 14
Interstitial Pregnancy 599
Intracranial Aneurysms 615
Intraductal Papillary Mucinous Neoplasm 324
Intrahepatic Portal Venous Air 492
Intravenous Talcosis 134
Ischemia Mimic (Mesenteric) 500
Jejunal and Ileal Carcinoid 357
Juvenile Laryngeal Papillomatosis 201
Juvenile Polyposis: Syndrome 525
K. pneumoniae 178, 183
Kaposi Sarcoma (Gastric) 341
Kidney
Cystic Diseases 614
Neoplasms 561
Transplants 664
Trauma 576
KIT 338
Krukenberg Metastasis 335
L. pneumophila 183
Langerhans Cell Histiocytosis 29
Large Cell Carcinoma 114
Legionella 178
Leiomyoma 258, 261
Leiomyosarcoma (retroperitoneum) 582
Limb-Body-Wall Defect 610
Lipoma 258
Lipoma (breast) 222
Lipomatous Tumors (Liver)
Angiomyolipoma 273
Myelolipoma 273
Liposarcoma (breast) 222
Liposarcoma (retroperitoneum) 583
Liver Disease (chronic) 293
Liver Mass with Fat - Differential Diagnosis 420
Liver Neoplasms 267, 275
Liver Transplantation 670
Complications 671
Liver Transplants 664
Lung Cancer 111, 149
Cigarette smoking 111

Clinical Presentation 111
Paraneoplastic Syndromes 111
Lymphangioleiomyomatosis 38
Lymphangioma
Mediastinum 162
Lymphangioma
Retroperitoneum 582
Mesentery
Lymphangitic Carcinomatosis 143
Imaging Features 143
Lymphocele (post kidney transplantation) 666
Lymphoma 150
Lymphoma
Burkitt) 348
Lymphoma
Primary Gastric 336
Lymphoma
Testicular 588
Lymphomatoid Granulomatosis 70
Epstein-Barr Virus 70
M. pneumoniae 180
Malabsorption 505
Malakoplakia 650
Male Breast 257
Cancer 261
Malignant Fibrous Histiocytoma 582
Malignant Germ Cell Neoplasms (Non-Seminomatous) 156
Malignant Hepatic Neoplasms 275
Angiosarcoma 280
Epithelioid Hemangioendothelioma 280
Fibrolamellar Carcinoma 278
Intrahepatic Cholangiocarcinoma 279
Malignant Neoplasia (Chest)149
Malignant Peripheral Nerve Sheath Tumor 158
Mallory-Weiss Tear 471
MALT 335
Masaoka (Thymoma: Staging) 154
Mastitis 224
Mastitis (Granulomatous - Male Breast) 261
Mature Teratoma 155
Meckel Diverticulum (complications) 422
Diverticulitis 424
Diverticulitis with a Stone 425
Hemorrhage 424
Heterotopic Gastric Mucosa 423
Heterotopic Pancreatic Mucosa 423
Inverted 425
Meckels Diverticulum 473
Mediastinal
Compartments 148
Fibrosis 135, 151
Goiter 161
Masses 148
Mediastinitis 165
Mediastinum 148
Medullary Carcinoma (Kidney) 565
Menetrier Disease 409
Meningocele (Thoracic) 158
Mesenchymal Neoplasm of the Stomach 338
I5
Mesenteric Adenitis 430

Mesenteric Cyst 372
Mesenteric Fibromatosis (Desmoid Tumor) 346, 376
Mesenteric Ischemia 487
Mesenteric Ischemia - Etiologies 495
Embolus 495
Thrombosis 497
Mesenteric Masses (Differential Diagnosis) 346
Mesenteric Masses and Cysts 372
Benign Multicystic Mesothelioma 375
Diffuse Peritoneal Malignant Mesothelioma 375
Enteric Cyst and Mesothelial Cyst 374
Inflammatory Myofibroblastic Tumors (Inflammatory
Pseudotumor) 379
Lymphangioma 373
Nonpancreatic Pseudocyst 374
Sclerosing Mesenteritis 378
Mesothelioma
Malignant 215
Mesenteric 374
Benign Multicystic 375
Cystic Malignant 375
Diffuse Malignant 375
Scrotal 593
Metastases (pleural) 216
Metastases (pulmonary) 138
Calcification 142
Cannonball 140
Cavitation 141
Endobronchial 144
Lymphangitic Carcinomatosis 143
Micronodular 141
Parenchymal Nodules 139
Pathogenesis of Hematogenous Metastases 139
Pleural 144
Solitary 142
Tumor Embolism 143
Metastases Breast Young Women 254
Microcystic Adenoma 327
Microscopic Polyangiitis 67
Mole 661
Benign Hydatidiform 661
Choriocarcinoma 662
Complete 662
Partial 662
Morgagni (Herniation) 164
Mounier-Kuhn Syndrome 32
MR Enterography 389
MRSA 186
Mucinous Cystadenoma/Cystadenocarcinoma (Appendiceal)
428
Mucinous Cystic Neoplasm 326
Mucinous Noncystic Adenocarcinoma 324
Mucoepidermoid Carcinoma 196
Multilocular Cystic Nephroma 686
Multiple Lymphomatous Polyposis (Mantle Cell Lymphoma)
348
Myasthenia Gravis (Thymoma and) 152
Mycoplasma 178
Myelolipoma 647
Myofibroblastoma (Male Breast) 257, 260
N. Asteroides 188
Necrotizing Sarcoid Granulomatosis 69

Neoplasms (Germ Cell) 155
Neoplasms (Neurogenic) 157
Nephrocalcinosis 694
Nephrogram 674
Nephroma (Multilocular Cystic) 569
Nerve Sheath Tumor 158 (Chest)
Nerve sheath Tumors (Retroperitoneum) 581
Neural Tube Defects 605
Neuroblastoma (Mediastinal) 158
Neuroendocrine Tumors (pancreas) 328
Neurofibroma 157
Neurofibromatosis (NF1) 158
Neurogenic Neoplasms 157
Neurogenic Tumors (Retroperitoneum) 580
NF-1 (GI Neoplasms) 359
Nocardia 178
Nodes (NHL - Gastrointestinal) 345
Nodular Lymphoid Hyperplasia Colon 527
Non Hodgkin Lymphoma (abdominal) 344
Adenopathy 345
AIDS-Related Lymphoma 349
Burkitt Lymphoma 348
Enteropathy-Type T-cell Lymphoma 349
Gastrointestinal Lymphoma 346
Mantle Cell Lymphoma 348
Small Intestine 347
Small Intestine: Differential Diagnosis 349
Non Hodgkin lymphoma - Mediastinum 150
Non-Hereditary GI Polyposes 524
Non-Neoplastic Lymphadenopathy - Mediastinum 151
Non-Seminomatous Malignant Germ Cell Neoplasms 156
Nosocomial Pneumonia 185
Nosocomial Pneumonia and Aspiration 186
Ogilvie Syndrome 401
Oligohydramnios 609
Omental Infarction 431
Oncocytoma 569
Orchitis 591
Organ Transplantation 75
CMV 76
Fungal Infections 77
Graft-vs-Host 76
Pneumocystis carinii 76
Ovarian Cyst 611
Ovarian Cysts 653
Corpus Luteum 654
Follicular 653
Functional 653
Hemorrhagic 654, 655
Hyperstimulation 655
Rupture 655
Theca Lutein 655
Ovarian Neoplasms 637
Epithelial Ovarian Neoplasms: Endometrioid 639
Epithelial Ovarian Neoplasms: Mucinous 639
Epithelial Ovarian Neoplasms: Serous 638
Epithelial Ovarian Tumors: Clear Cell 639
Epithelial Tumors: Classification 637
Epithelial Tumors: Terminology 638
Mature Cystic Teratoma 640
I6
Ovarian Germ Cell Neoplasms 640

Ovarian Malignant Germ Cell Tumors 641
Sex-cord stromal tumors 641
Ovarian Torsion 657
Ovary
non neoplastic disorders 653
polycystic 660
Masses 637
Pancoast Tumor 113
Pancreas 412
Annular 412
Divisum 413
Pancreatitis, Chronic 414
Pancreas (Neoplasms) 321
Adenocarcinoma 321
Islet Cell Tumors 328
Metastatic 329
Microcystic Adenoma 327
Mucinous Cystic Neoplasm 326
Mucinous Noncystic Adenocarcinoma 323
Oligocystic Adenoma 328
Solid and Pseudopapillary Epithelial Neoplasm 325
Pancreas Transplants 664, 669
Complications 669
Rejection 670
Vascular Thrombosis 670
Pancreatic Adenocarcinoma 321
Resectability 323
Pancreatic Duct 411
Pancreatitis 460
Papilloma 201
Papilloma (Male Breast) 257
Papillomatosis (Biliary) 319
Paraganglioma 159
Paraganglioma (Extra-adrenal pheochromocytoma) 581
Paragonimiasis westermani 189
Paraovarian Cysts 661
Parathyroid Adenoma 162
Pelvic Inflammatory Disease 659
Pelvic MRI 553
Peptic Ulcer Disease 455
Peribronchial abscesses 188
Perinephric Fluid Collections 665
Peritoneal Inclusion Cysts 523, 659
Peritoneal Lymphoma (Primary) 350
Persistent Bilateral Nephrogram 679
Peutz Jeghers 525
Pleural Disease 204
Bacterial Pneumonia 208
Empyema 208
Pleural Effusion 208
Pleural Effusion: Asbestos Exposure 210
Pleural Effusion: Subpulmonic 209
Pleural Effusion: Tuberculosis 209
Pleural Fibrosis 211
Pneumothorax 211
Pulmonary Ligament 207
Round Atelectasis 210
Pleural Effusion 213
Malignant 213
Pleural Neoplasms 213
Localized Fibrous Tumor 213
Mesothelioma 215
Pneumatocele 190
Pneumatosis Intestinalis 493
Pneumocystis Jiroveci 78
Pneumocystis Jiroveci Pneumonia 78
Pneumonia 178
Pneumoperitoneum 403
Pneumothorax 211
Polycystic Kidney Disease (Autosomal Recessive) 616
Polycystic Liver Disease 306
Polycystic Ovary Syndrome 660
Polyhydramnios 609
Polymastia 221
Polyposis - Familial 519
Polysplenia 534
Polythelia 221
Post Transplant Lymphoproliferative Disorder 672
Post Transplant Malignancy 673
Posterior Urethral Valves 611
Post-transplantation Imaging 664
Post-transplantation Lymphoproliferative Disorder (GI) 349
Pregnancy 598
Primary Ciliary Dyskinesia 32
Prostate Cancer 620
Grading 620
Screening 620
Prostate Specific Antigen 620
Pseudoaneurysms (kidney transplantation) 669
Pseudocyst (Nonpancreatic) 374
Pseudocyst (Pancreatitis) 462
Pseudogynecomastia 257, 259
Pseudopapillary Epithelial Neoplasm (Solid and) 325
Pulmonary Blastoma 198
Pulmonary Circulation 131
Idiopathic 132
Pulmonary Embolism 82
Arterial Blood Gases 85
Chest X-Ray 84
Clinical Science Probability 85
Combined Pulmonary CTA and Venography 89
Common Radiographic Abnormalities 84
CT Angiography 86, 87
CT Findings 84
Diagnostic Algorithm 89
Pitfalls 88
Small Emboli 86
Ventilation/Perfusion Scanning 85
Pulmonary Gangrene 190
Pulmonary Hypertension 131
Pulmonary Lymphoid Disorders 55
B-Cell Lymphoma 58
Follicular bronchitis 55
Follicular Hyperplasia 55
Lymphoid Interstitial Pneumonia LIP 56
Lymphomatoid Granulomatosis 59
Nodular Lymphoid Hyperplasia 57
Posttransplantation Lymphoproliferative Disease 60
Pseudolymphoma 57
Pulmonary Lymphoid System 54
I7
BALT 54
Bronchus Associated Lymphoid Tissue 54
Pulmonary Thromboendarterectomy 134
Pulmonary Venous Hypertension 135
Pyelogram 674
Pyelonephritis 678
Pyogenic Hepatic Abscess 284
Pyosalpinx 659
Radioactive Ablation (Prostate Cancer) 622
Rejection (kidney transplants) 667
Renal Anomalies 610
Renal Artery (kidney transplantation) 668
Renal Calcifications 693
Dystrophic 693
Medullary 694
Renal Cancer in ACKD 619
Renal Cell Carcinoma 563
Renal Injuries 576
Renal Masses 681
Carcinoma In Situ 681
follow up interval 683
Small renal mass 682
Renal Neoplasms 561
Angiomyolipoma 570
Infiltrating Renal Cell 564
Juxtaglomerular Cell Tumor 570
Metastases 569
Oncocytoma 569
Renal Lymphoma 568
Robson Staging 565
Spontaneous Renal Hemorrhage 564
TNM Staging 565
Transitional Cell Carcinoma 567
Tuberous Sclerosis 570
Uroepithelial Neoplasms 567
Venous extension 562
Renal Transplants 664
Renal Vein Thrombosis (kidney transplantation) 668
Respiratory Bronchiolitis 29
Respiratory Viruses 181
Retroperitoneal Fibrosis 580
Retroperitoneal Masses 611
Retroperitoneal Masses (Fat containing) 584
Retroperitoneum 579
Ruvalcaba-Myhre-Smith 526
S. pneumoniae 178, 179
S.aureus 184
Saber Trachea 28
Sacrococcygeal Teratoma 612
Salpingitis 659
Sandwich Sign (NHL) 345
SARS 182
Scar Carcinoma 116
Schistosomasis (Bilharziasis) 288
Schistosomiasis (Urinary) 651
Schwannoma 157
Sclerosing Cholangitis (primary) 307
Cholangiocarcinoma 308
Sclerosing Mesenteritis 378
Scrotal Anatomy 630
Scrotal Calculi 590
Scrotum 585
Seminoma 156
Sequestration 608
Serous Inclusion Cysts 661
Serum Alpha-Fetoprotein (Elevated Maternal) 691
Severe Acute Respiratory Syndrome 182
Shock Bowel 500
Shwachman - Diamond Syndrome 513
Sigmoid Volvulus 401
Silicosis 46
Adenopathy 47
Alveolar Proteinosis 48
Calcification 47
Conglomeration 48
Scar emphysema 48
Small Bowel Bleeding 472
Small Bowel Lymphoma 347
Small Bowel Obstruction 475
Small Cell Lung Cancer 113
Small Intestinal Benign Neoplasms 353
Adenoma 355
Brunner Gland Hamartoma 354
Brunner Gland Hyperplasia 354
Differential Diagnosis: Duodenal Polyp 354
Periampullary Adenocarcinoma 355
Periampullary Duodenal Mass 355
Tubulovillous Adenoma 355
Small Intestine 347
Adjacent Mesenteric Disease 348
Cavitary Mass 348
Mural Infiltration 347
Small Intestine Malignant Neoplasms
Adenocarcinoma 355
Carcinoid 357
Carcinoid Syndrome 358
Differential Diagnosis 356, 359
Metastatic Disease 359
Small Intestine NHL - Differential Diagnosis 349
Smoking Related ILD 16
Macrophages 16
RB 16
Respiratory bronchiolitis 16
Spermatic cord 586
Spleen 531
Splenorenal ligament 531
Splenosis 533
Sprue 505
Steatosis 296
Stomach Malignancies 332
Stone Urinary 624
Striated Nephrogram 679
Strongyloides stercoralis 189
Strongyloidiasis 188
Swyer James Syndrome 38, 182
Sympathetic Ganglia Tumors 159
Tamoxifen 558
T-cell Lymphoma (Enteropathy-Type T-cell Lymphoma) 349
I8
Teratoma (Mature) 155

Teratoma (Retroperitoneum) 583
Testicular Carcinoma (Risk Factors) 588
Testicular Cysts 589
Testicular Ischemia 635
Testicular Masses (Bilateral) 589
Testicular Neoplasms 586
Germ Cell Neoplasms 587
Non Seminomatous Germ Cell Tumor 587
Seminoma 587
Testicular Torsion 630
Testis 586
Testis (torsion) 632
Thymic Carcinoid 154
Thymic Cyst 161
Thymic Hyperplasia 161
Thymolipoma 154
Thymoma 152
Torsion (Ovary) 657
Torsion (Testicle) 591
Transplants (Solid Organs) 664
Transverse Colon Volvulus 401
Trauma (Urinary Tract) (see Urinary Tract Trauma) 573
Trisomy 18 606
Trisomy 21 600
TRUS 621
Tuberculosis 93
Clinical features 95, 96
Hemoptysis 98
Lymphatic gradient 95
M. tuberculosis 93
Mycetoma 98
Mycobacteria 93
Pathogenesis 94
Radiologic features 95, 96
Rassmussen (pulmonary artery) aneurysm 98
Tuberculoma 97
Tuberculosis (renal) 693
Tuberculosis (scrotal) 591
Tuberous Sclerosis 39, 616
Renal 570
Tubular Ectasia (Testis) 589
Twins 596
Dizygotic 596
Monozygotic 597
Ulcerative Colitis 382, 384
CT Features 384
Imaging Features 384
Toxic Megacolon 385
Ulcers - Duodenal 458
Ulcers - Gastric 456
Benign 456
Equivocal 457
Malignant 457
UPJ disruption 578
Urachal Anomalies 649
Ureteral Injury 575
Urethra 649, 652
Urethral Trauma 573
Urethrography 652
Urinary Bladder 649
Urinary Stone Disease 624
Urinary Tract Trauma 573

Uroepithelial Neoplasms 567
Urothelial carcinoma 649
Usual Interstitial Pneumonia: Histology 15
Fibroblast foci 15
Uterine Disorders 551
Arcuate 555
Bicornuate 554
Diethylstilbestrol: DES Related 556
Mullerian Duct Anomalies 554
Septate 555
Unicornuate / Didelphys 554
Uterine Masses - Benign 556
Abnormal Uterine Bleeding 557
Adenomyosis 557
Endometrial Hyperplasia 558
Endometrial Polyps 558
Leiomyoma 556
Postmenopausal Bleeding 558
VACTERL Syndrome 610
Varicella Pneumonia 182
Varices (Esophageal) 164
Varicocele 592
Ventilator-associated Pneumonia 186
Viruses - Respiratory 181
Von Hippel Lindau 617
Water Lily Sign (E. granulosus) 287
Wegeners Granulomatosis 63
halo sign 66
Williams-Campbell 31
Yolk sac 596
Zollinger-Ellison Syndrome 328
I9
Radiologic
Pathology
Fifth Edition
VOLUME 2
Musculoskeletal
2006
2007
Editors

Mark D. Murphey, MD

Associate Editor
Illustrators
Heike Blum, MFA

Washington DC, USA

Washington, DC
20306-6000
_____________________________________
_____________________________________
987654321
ISBN 1-933477-00-8
Preface
Acknowledgements
Pathology,
iii
Faculty VOLUME 2
Musculoskeletal Radiology
Mark E. Schweitzer, MD
Mark D. Murphey, MD
Professor of Radiology and Orthopedic Surgery

Chief of Radiology - Hospital for Joint Diseases
Director, Musculoskeletal Radiology
New York University
New York, NY

Washington, DC
Christopher G. Fielding, COL, DC, USA

Department of Oral Maxillofacial Pathology
Washington, DC
Mark W. Anderson, MD
Associate Professor of Radiology and Orthopedic Surgery

Division Head, Division of Musculoskeletal Radiology
University of Virginia Health System
Charlottesville, VA
Donald J. Flemming, CAPT, MC, USN
G. Victor Rohrer Professor of Radiology Education

Penn State Hershey Medical Center
Hershey, PA
Mark J. Kransdorf, MD
Mayo Clinic College of Medicine
Rochester, MN
and
Consultant, Musculoskeletal Radiology
Mayo Clinic
Jacksonville, FL
William B. Morrison, MD

Director, Division of Musculoskeletal
and General Diagnostic Radiology
Thomas Jefferson University Hospital
Philadelphia, PA
Michael Mulligan, MD
Associate Professor of Diagnostic Radiology

Baltimore, MD
Thomas L. Pope, MD
Clinical Professor of Radiology/Orthopedics

Medical University of South Carolina
Charleston, SC
and
Washington, DC
Charles S. Resnik, MD
Professor of Diagnostic Radiology

Director, Section of Musculoskeletal Radiology
Director, Residency Program
Baltimore, MD
Timothy Sanders, MD

Bethesda, MD
iv
Mark D. Murphey, MD
Radiologic Assessment of Joint Replacement and Imaging of Bone Grafts . . . . . . . . . . . . . . . . . . . . . . . . .699

Musculoskeletal Manifestations of Chronic Renal Insufficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .711
Fundamental Concepts of Musculoskeletal Neoplasm: Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .720
Fundamental Concepts of Musculoskeletal Neoplasm: CT and MR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .733
Osteoid Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .743
Cartilaginous Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .757
Fibrous Lesions of the Musculoskeletal System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .771
Alphabet Soup and Cystic Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .784
Juxtaarticular Soft Tissue Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .792
Musculoskeletal Angiomatous Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .805
Paget Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .812
Musculoskeletal Infection I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .820
Musculoskeletal Infection II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .829
Imaging of Cervical Spine Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .839
Christopher J. Fielding, COL, DC, USA
Radiographic Differential Diagnosis of the Jaws . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .849
Mark W. Anderson, MD
MRI of the Knee: Part 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .858

MRI of the Knee: Part 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .865
MRI of the Wrist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .872
MRI of the Ankle and Foot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .879
Osseous Lesions: Unknown Histogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .887

Soft Tissue Lipomatous Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .893
Metabolic Bone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .900
Osteonecrosis and Related Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .906
Donald J. Flemming, CAPT, MC, USN
Approach to the Inflammatory Arthropathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .912

MRI of the Rotator Cuff . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .925
Timothy Sanders, MD
MR Arthrography of Glenohumeral Instability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .932

Imaging of Upper Extremity Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .941
Crystal Deposition Diseases and Neuropathic Osteoarthropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .949
Mark Schweitzer, MD / William Morrison, MD
MRI of the Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .955
Michael Mulligan, MD
Skeletal Metastases, Myeloma, Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .961
Thomas Lee Pope, MD
Imaging of Hematologic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .968

Generalized Musculoskeletal Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .976
Osseous Musculoskeletal Stress Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .987
Pelvis and Lower Extremity Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .995
Mark D. Murphey, MD
Musculoskeletal Seminar I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1005

Musculoskeletal Seminar II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1009
Musculoskeletal Seminar III . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1013
Musculoskeletal Seminar IV . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1018
Musculoskeletal Seminar V . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1025
697
698
Radiologic Assessment of Joint Replacement and Bone Grafts

Mark D. Murphey, MD
Current Materials For Total Joint Replacement
Metal components
Ultra-High Molecular Weight Polyethylene
Metallic Components
Cobalt-chromium-molybdenum alloy
Cobalt-chromium-tungsten alloy
Titanium-aluminum-vanadium alloy
Ultra-High Molecular Weight Polyethylene Component
Figure 4-1-1
Allows articulation of metallic components

Lowers friction and prolongs wear
Allows some plastic deformity improving joint
congruity
Radiolucent
Complications of Joint Arthroplasty
I. Loosening and/or infection

II. Small particle disease (osteolysis)
III. Dislocation and abnormal alignment
IV. Fractures and nonunion
V. Heterotopic bone formation
VI. Cement extrusion
VII. Polyethylene liner displacement and metal
arthropathy
Loosening and/or Infection
Aseptic loosening of both acetabular and femoral components

of a total hip arthroplasty with bone cement (arrows), and
cement-metal lucency (arrowheads) that progresses over
several years (right image). Cement fracture (open arrow) and
lateral migration of the femoral stem (curved arrow) are also
apparent
Most common complication historically

Difficult to differentiate
413% hip replacement
710% knee arthroplasty
Radiographic Signs: Loosening-Infection Cemented Arthroplasty [Figure 4-1-1]
Cement-bone lucency or cement metal lucency >2mm

Progressive widening of interfaces post-op
Component migration
Fracture of metal or cement
Periosteal reaction
Smooth endosteal scalloping with cement lucency
Air in soft tissues or joint
Figure 4-1-2
Bone Ingrowth (Porous Coated)

Joint Arthroplasty
Improved longevity
Beads sintered onto metal surface
Bone ingrowth into irregular surface (biologic
fixation)
Technically demanding
No motion to promote bone ingrowth
Normal Radiographic Appearance Ingrowth

Arthroplasty
[Figure 4-1-2]
Resorption of medial femoral cortex

Thin lucent rim with sclerotic margin about metal
(< 2 mm)
Endosteal sclerosis
Prosthetic subsidence (< 10 mm)
Periosteal reaction and cortical thickening
Not progress after 9 12 months post-op
Normal appearance of ingrowth hip arthroplasty with

superolateral lucency (arrowheads) (<2mm) and surrounding
sclerosis and resorption of medial cortex (arrow)
699
Joint Replacement and Bone Grafts
Loosening/Infection Radiographic Findings

With Ingrowth Arthroplasty
Figure 4-1-3
Prominent prosthetic subsidence (>8mm)

Bone destruction
Component migration or motion
Prominent lucent zone about metal (>2mm)
Increasing number of displaced beads
Infection of Total Joint Arthroplasty
The major long-term complication (1%-4%)

33% first 3 months post-op
33% 3 months 12 months post-op
33% > 12 months post-op
Usually associated loosening
Difficult to differentiate from aseptic loosening
Radiographic Signs Most Suspicious for

Infection [Figure 4-1-5]
Aseptic loosening of femoral component ingrowth hip

replacement with prominent lucency, lateral migration of
femoral stem (arrow), and subsidence medially (arrowhead)
Extensive bone destruction

Air in soft tissue and/or joint
Extensive or aggressive periosteal reaction
Wide or irregular lucent zone
Figure 4-1-4
Radionuclide Evaluation of Total Joint

Arthroplasty
Bone scintigraphy
Gallium scan
Indium WBC scan
PET (combined with bone scan)
Bone Scintigraphy
Normal increased activity post-op (69 months)

Increased activity subsequently suspicious for
loosening/infection
Nonspecific
Overall accuracy 50%-70%
Gallium (Ga-67) Scanning
In conjunction with bone scan

Incongruence with increased gallium uptake vs. bone
scan suspicious for infection
Not as accurate as bone scan WBC combination
Aseptic loosening of acetabular component ingrowth hip

replacement with prominent lucency (arrows) and bead
sheading (circles)
Figure 4-1-5
Infection of total knee arthroplasty with early prominent

development of lucency and bone destruction (arrows) about
both the femoral and tibial components soon following surgery
(two months- previous normal post-operative radiographs not
shown)
700
Indium-111 WBC Scanning
Figure 4-1-6
Increased sensitivity (50%-100%) and specificity (45%-100%) for

infection of TJR
Increased activity at tip of metal components can be normal for
up to 2 years post-op
Used in conjunction with bone and bone marrow scans
incongruity with more uptake on WBC scan > 90% accuracy
Sensitivity and the ability to correctly localize infection decreases
Infection becomes more chronic
Anatomic location more central
Arthrography of Joint Arthroplasty
Purpose
Obtain fluid for culture/sensitivity
Document intra-articular location
Confirm loosening
Detect other causes of pain
Hip Arthrography Technique
Preliminary fluoroscopy and radiographs

Anterolateral approach to metal at head neck junction 20 gauge
spinal needle
Aspiration for culture
If no aspirate inject saline reaspirate
Contrast injection with subtraction technique
Radionuclide injection
Pre and post exercise radiographs
Digital subtraction arthrogram with contrast in

the bone remnant interface of the acetabular
component (zone 2-arrowhead) and below the
intertrochanteric line (arrows) representing
aseptic loosening of both components
Arthrographic Criteria for Loosening/Infection

[Figure 4-1-6]
Acetabular Component
Contrast in bone cement or metal-cement interface all zones (90%)
Contrast in bone cement or metal-cement interface zones I & II or zones II
& III (90%)
Contrast in zones I & III with medium or large pseudocapsule bursa (57%)
Rim of contrast >2mm thick any zone (95%)
Lymphatic filling (?)
Femoral Component
Contrast in cement-bone interface distal to intertrochanteric line (98%)
Contrast in bone-metal interface below intertrochanteric line (98%)
Contrast at or below mid component long stemmed prosthesis (98%)
Knee Arthrography Technique
Preliminary fluoroscopy and radiographs

Lateral patellofemoral or anterior intercondylar approach 20
gauge spinal needle
Aspirate for culture
If no aspirate inject saline reaspirate
Contrast injection-subtraction technique
Pre and post exercise radiographs
Figure 4-1-7
Arthrography and Bursa

[Figure 4-1-7]
Greater trochanteric 50%

Supraacetabular 33%
Iliopsoas 17%
Can reduce accuracy of arthrography
II. Small Particle Disease

Granulomatous pseudotumor/histiocytic reaction/osteolysis

Previously unusual late sequelae of arthroplasty
Now may be most common cause failure
Large lobulated lucencies with cortical thinning
Prosthesis loosening
701
Septic loosening of the femoral component on

arthrography of a total hip replacement with
synovial nodularity in the joint (arrows) and
supraacetabular bursa formation (arrowhead).
Contrast extends below intertrochanteric line
Figure 4-1-8
Figure 4-1-9
Small particle disease (osteolysis or granulomatous

pseudotumor) as a cause for loosening of the tibial component
of a total knee replacement with large mass-like
radiolucency/low attenuation in the proximal tibia (arrowheads)
with associated fracture (arrow)
Figure 4-1-10
Small particle disease as a cause for
loosening of femoral and acetabular
components of a total hip replacement
with multiple largely intracortical areas of
radiolucency (arrowheads)
Dislocation / Abnormal Alignment

Normal Alignment
Acetabular angle: 40 degrees (+ or 10 degrees)

AP view
Acetabular anteversion
030 degrees lateral view
Knee:
Tibial plateau component parallel to floor
Tibia 57 degrees valgus
Abnormal Alignment
Predisposing to Subluxation
Dislocated noncemented total hip replacement, both femoral

(arrowhead) and acetabular components (arrows), with
increased inclination of the acetabular component as a
predisposition to this complication
[Figure 4-1-10]
Varus position of knee is unacceptable

Acetabular angle > 50/55 degrees AP view
Acetabular anteversion < 0 degrees or > 30 degrees lateral view
Exceeding extremes of motion
Interposed material
Greater trochanteric separation
Joint effusion
Loss of soft tissue support or imbalance (knee)
Figure 4-1-11
IV. Fractures and Nonunion Associated with

Arthroplasty
[Figures 4-1-11 and 4-1-12]
Bone
Metal
Cement
Polyethylene
Greater Trochanteric Nonunion After Total

Hip Arthroplasty
[Figure 4-1-11]
Improves exposure at surgery

Osseous union normally 6 12 weeks
Nonunion results in lack of abductor support
Bursitis predisposes to dislocation
Fracture of greater trochanteric wire mesh on follow up

radiograph (right image) with retraction due to the pull of the
abductors (arrow) about the total hip replacement.
702
Heterotopic Bone Formation After Total Joint Arthroplasty
Not infrequent 3 weeks post-op

2 years to mature
Hip: 21%40%; Knee: 10% anterior to femur
Predisposing conditions Ankylosing Spondylitis, DISH, prior
occurrence
Treatment radiation, steroids, diphosphonates, surgery, indocin
Figure 4-1-12
Brooker Classification
Heterotopic Bone
After hip replacement

Class I: Small islands of bone
Class II: Bone projection from acetabulum or femur with >1 cm
between osseous surfaces
Class III: <1cm between opposing bridge surfaces
Class IV: Osseous ankylosis bridging joint
Cement Extrusion
Usually clinically insignificant

Vein or lymphatic
Rarely nerve, vascular, bowel or bladder injury
Polyethylene Liner Displacement & Metal Arthropathy

[Figure 4-1-13]
Allows metal-metal friction

Inflammation and aseptic synovitis
Abnormal component position
Visualize radiolucent polyethylene
Metal line sign and debris
Prevented by early recognition
Ingrowth total hip replacement with fracture

(arrow) at the tip of the femoral component
transfixed by cerclage wires
Figure 4-1-13
VIII. Silicone Arthroplasty
Complications
Fracture
Dislocation
Infection
Silicone arthropathy
Displacement of polyethylene liner on follow-up radiograph of

total hip replacement (right image). Note widened medial joint
space compared to initial post-op radiograph (left image)
metal-line sign (arrowhead) and radiolucent rotated
polyethylene liner (*)
References
Joint Replacement
1.
2.
3.
4.
Bauer TW, Schils J. The pathology of total joint arthroplasty. I. Mechanisms of implant fixation. Skeletal Radiol.
1999 Aug;28(8):423-32. Review.
Keogh CF, Munk PL, Gee R, Chan LP, Marchinkow LO. Imaging of the painful hip arthroplasty. AJR Am J
Roentgenol. 2003 Jan;180(1):115-20.
Manaster BJ. From the RSNA refresher courses. Total hip arthroplasty: radiographic evaluation. Radiographics.
1996 May;16(3):645-60. Review.
Oswald SG, Van Nostrand D, Savory CG, Callaghan JJ. Three-phase bone scan and indium white blood cell
scintigraphy following porous coated hip arthroplasty: a prospective study of the prosthetic tip. J Nucl Med. 1989
Aug;30(8):1321-31.
703
History and Importance of Bone Graft Procedures
First performed 1688

Second most frequently transplanted tissue
Vital for orthopedic management
Figure 4-1-14
Radiologic Assessment: Important for

Patient Management
Normal bone graft incorporation

Abnormal alterations
Imaging Modalities
Radiographs
Conventional Tomography
Scintigraphy
Computed Tomography (CT)
Magnetic Resonance Imaging (MRI)
Bone Graft Indications
Delayed or nonunion
Pseudarthrosis
Fill osseous defects or cavities
Arthrodesis
Stabilize spinal segments
Bone stock in arthroplasty
Restore function in diseased articulations
Pictorial representation of onlay (A) and inlay (B and insert)

bone graft procedures
Figure 4-1-15
Classification of Bone Graft by Origin
Autograft
Allograft (homograft)
Xenograft (heterograft)
Bone graft substitute
Classification of Bone Graft by Structure
Cancellous
Cortical
Combination
Classification of Bone Graft by Technique [Figure 4-1-14]
Onlay
Inlay
Dowel
Muscle pedicle
Strut
Vascularized
Clothespin (H)
Radiographic Evaluation of the Donor Site

[Figures 4-1-15 and 4-1-16]
Iliac
Fibula
Rib
Distal radius
Calvarium
Femoral head
Tibia
Greater trochanter
Posterior spinal elements
Dowel graft placed across a scaphoid nonunion

(arrows) with progressive healing at both the
bone grafted site (arrows) and bone graft donor
site (arrowheads) on radiographs 3 months apart
704
Normal Radiographic Appearance Of Donor Site

[Figure 4-1-17]
Figure 4-1-16
Wedge or oval defect

Irregular margins after surgery
Initial increase in ill-defined margins
Subsequent marginal sclerosis
Complete regeneration
Donor Site Complications
Pain
Failure to recognize (radiologists)
Infection
Muscle herniation
Involvement of sacroiliac joint
Fracture
Normal Bone Graft: Repair/Incorporation
CT of normal iliac bone graft donor site with outer shell of bone
retrieved (arrowheads) and no violation of the SI joint (arrow)
[Figures 4-1-18 and 4-1-19]
Cancellous autograft
Cortical autograft
Vascularized autograft
Allograft
Figure 4-1-17
Figure 4-1-18
Fibula resection for use as bone graft with

regeneration over time on three sequential
radiographs
1 month post-op
2 months post-op
Figure 4-1-19
4 months post-op 6 months post-op

Progressive normal incorporation of cancellous
autograft (arrows) about post-traumatic site in the
lower leg on radiographs with individual osseous
fragments coalescing into a large ossific mass
Normal incorporation (arrows) of fibular cortical graft replacing a

humeral resection for Ewing sarcoma reconstruction. Three sequential
radiographs show progressive osseous bridging between fibular graft
and native humerus (arrowhead)
705
Important Terminology: Bone Graft Healing
Osteoconduction: Tissue ingrowth with prominent vascular and mesenchymal

components
Osteoinduction: Mesenchymal tissue differentiation into tissue capable of
osteogenesis
Osteogenesis: Bone formation
Vascularized Bone Autograft
Rib
Iliac
Fibula
Indications: Vascularized Autograft
Intercalary defects
Composite defects
Mandible reconstruction
Tumor resection
Conventional failure
Congenital pseudarthrosis
Normal Autograft Healing: Cancellous
Osseous union 1-1.5 years:

allografts successful in this pattern 70-80%
Normal Autograft Healing: Cortical
necrotic
osteoclastic
706

Characteristics
Fibula
Rib
Iliac Crest
Bone Length (Max)

Shape
Structure
22-26 cm
Straight
Cortico-Cancellous
30 cm
Curved
Membranous
10 cm
Slight Curve
Cortico-Cancellous
Vessels
Artery
Peroneal
Vein
2 Venae Comitnantes
Vascular Stalk
1.0-7.0 cm
Posterior Intercostal Deep circumflex-iliac

or Superficial
circumflex iliac
1 Intercostal
1 Vena comitante
3.0-5.0 cm
1.5-5.0 cm
Advantages
Graft remains viable
Promotes healing
Participates in osteogenesis
Improved strength
Disadvantages
Microvascular surgery expertise required
Increased time for surgery
Two surgical sites
Autograft Limitations
Insufficient volume
Postoperative morbidity risk
Inability to mold for function
Allograft
Particulate
Intercalary
Osteoarticular
Allograft Healing Depends on the Recognized Immunologic

Disparity
Allograft Source and Pretreatment
Trauma 1545 years of age

No history neoplasm or infection
No steroids or respirator
Freeze or freeze-drying
Osteoarticular Allograft
Osteochondral shell
Half-joint
Whole joint
707
Normal Allograft Healing

Histology
Radiographic Appearance
Prominent vascular and granulation

tissue ingrowth
Short initial osteoclastic phase (several Graft resorption with ill-defined and
weeks)
irregular margins
Improved osteoinduction and
osteogenesis owing to osteoprogenitor
cells improved survival (endosteum and
marrow elements)
Further healing
Further gradual decrease in graft

density and volume (first 1 month) until
bone production exceeds resorption
Gradual hematopoietic tissue ingrowth
w/graft density increasing to normal,
loss of margin between native bone and
graft w/trabecular continuity and
formation of cortex (2-6 months)
Initial increase in strength due to

osteoblastic activity
Osteoarticular Allograft
Low ratio bone: cartilage requires less pretreatment

Cartilage immuno-privileged tissue
Success depends on osseous component
Bone Graft Complications
Infection
Nonunion or pseudarthrosis
Fracture
Graft resorption
Joint instability
Infection of Bone Graft
Persistent tissue swelling

Periosteal reaction
Progressive bone destruction
Lucency about fixation and failure
Indium WBC scan may add specificity
Figure 4-1-20
Infection of Bone Graft

[Figure 4-1-20].
Autograft
Clinical evidence usually present
Vascularized Autograft
5%
Allograft
513% soft tissue swelling (beyond 6 months
post-op)
Increasing bone resorption (beyond 10 weeks
post-op)
Nonunion and Pseudarthrosis
Persistent lucency graft/host junction

Sclerosis at margins
Rounded osseous margins
Fracture or loosening of adjacent fixation
Stress views helpful
Allograft infection with progressive lack of osseous bridging

(arrowheads) and ultimately loosening and fracture of the
fixation device (arrow) on three sequential radiographs
708
Nonunion and Pseudarthrosis
Autograft
Failure to heal by 12 months
14% in segmental cortical bone grafts
7% questionable graft viability
Bone scan 1 week
Allograft
11% preventable with adequate osseous contact at host/graft junction
Treated with regrafting and/or fixation change
Fracture
Linear lucency through graft

Callus
Stress views helpful
Fracture
Autograft
Not infrequent
Often after healing with stress (68 months)
More common in longer grafts
Decreased incidence 3.5% due to improved strength
Allograft
16.5% most at weak points
Affected by pretreatment method
Bone Graft Resorption
Progressive graft loss

Graft decreases in size
Graft decreases in density
Difficult to distinguish from infection
Bone Graft Resorption
Autograft
Unopposed osteoclastic activity
Same as autograft
Allograft
Acute or chronic rejection
Graft replaced by fibrous tissue
Osteochondral Allograft: Joint Instability
2.9%5.5 % incidence
Causes: articular incongruity; lack of innervation and cartilage viability
AVN, neuropathic joint or rejection
Can be difficult to distinguish from infection or rejection
Joint Instability: Radiographic Findings
Joint narrowing and sclerosis

Osteophytes and subchondral cysts
Fragmentation with debris
Fracture and migration of fixation
Weight bearing views helpful
Xenograft
Supply-demand limitations of other graft

Calf and ox-bone
Treated to prevent rejection
Used as spacer prevents soft tissue ingrowth
Other graft material in combination
709
Bone Graft Substitutes
Hydroxyapatite (Ca10 [PO4] 6 [(OH) 2])

Tricalcium phosphate (Ca3 [Po4] 2)
Dense or porous ceramics
Osteoconductive but not osteoinductive
Figure 4-1-21
Porous Ceramics
Goniopora-cancellous bone
Porites-cortical bone
Approved human studies 1982
Initially weak mechanically
Strength increases after incorporation
More dense than native bone
Lucent peripheral band obliterated with ingrowth
Complications: Fractures, Implant failure, Infection
Spinal Bone Graft Assessment
Causes of Failed Back Surgery Syndrome (FBSS)

Recurrent disk
Arachnoiditis
Epidural scar
Infection
Facet subluxation
Spinal stenosis
Pseudarthrosis
Spine Bone Autograft: Normal Healing/Incorporation
Cervical - 3-4 months
Lumbar - by 9 months
Pseudoarthrosis of posterolateral
lumbar spine graft with horizontal
radiolucent clefts (arrows) and
surrounding sclerosis on conventional
tomography
Figure 4-1-22
Spine Bone Autografts: Normal Healing/Incorporation
Cervical 34 months
Lumbar by 9 months
Anterior Vertebral Interbody Fusion
Cervical and lumbar spine

Rib, iliac or fibular graft
Initial discrete graft-host junction obliterated
Radiologic Evaluation: Spine Pseudarthosis

[Figures 4-1-21 and 4-1-22]
Radiographs
Oblique views best; Radiolucent defect; Motion with bending increase
spine curve
Bone Scintigraphy
Wide range specificity and sensitivity Normal uptake <6 months post-op
Abnormal if increased uptake beyond 6 months post-op; Improved with
SPECT imaging; Asymptomatic patients may have focal uptake
Conventional Tomography
AP optimal plane (25 mm) best reported method 96% polydirectional
best; Limited availability and technically demanding
Interbody Fusion Complications
Nonunion
Graft fracture
Extrusion of graft fragments
Infection
References
Bone Graft
1. Murphey MD, Sartoris DJ, Bramble JM: "Radiographic Assessment of Bone Grafts"
In: Bone Grafts from Basic Science to Clinical Application, Habal MB, Reddi AH,
Editors. Philadelphia: W. B. Saunders, 1992, p. 9-36.
710
Sagittal T1 (upper image) and

T2-weighted (lower image) MR
images showing horizontal
clefts (arrowheads) in
posterolateral lumbar bone
graft with high signal on long
TR image representing two
sites of pseudoarthrosis
Musculoskeletal Manifestations of Chronic

Renal Insufficiency
Mark D. Murphey, MD
Hyperparathyroidism (HPT)
Primary
Secondary
Tertiary
Primary Hyperparathyroidism: Etiology
Adenoma
80%90%
Hyperplasia
10%15%
Carcinoma
2%4%
Nonparathyroid tumor
MEN Syndromes
Tertiary Hyperparathyroidism: Etiology
Autonomous gland function
End-Stage Renal Disease: Secondary Hyperparathyroidism
Multiple Causes
Most common cause glomerulonephritis
0.01% U.S. population
85,000 hemodialysis patients/year
8,000 renal transplantations/year
Involves all organ systems
Musculoskeletal manifestations common and increasingly recognized
Secondary Hyperparathyroidism: Etiology
Inability of renal excretion of phosphate

Resultant hyperphosphatemia
Hyperplasia of parathyroid chief cells and increased parathormone (PTH)
Also reduced degradation of PTH
Effects of PTH on Bone
Development of osteoclasts, osteoblasts, osteocytes

Osseous resorption
Brown tumors
Periosteal reaction
Renal Osteodystrophy
Secondary hyperparathyroidism
Osteosclerosis
Osteoporosis
Osteomalacia
Soft tissue and vascular calcification
Bone Resorption in Chronic Renal Insufficiency (CRI)
Caused by osteoclastic activity

10% early stages; 50%70% long-standing disease
Subperiosteal
Cortical
Subchondral
Trabecular
Endosteal
Subligamentous/Subtendinous
711
Musculoskeletal Manifestations of Chronic Renal Insufficiency
Subperiosteal Resorption
Figure 4-2-1
[Figures 4-2-1 to 4-2-3]
Initially described by Camp and Ochsner

Pathognomonic-hyperparathyroidism
Lacelike irregularity of cortical margin progress to
scalloping and spiculation
Earliest involvement middle phalanges/ terminal tufts-hand
Additional sites-upper medial tibia, femur and humerus,
ribs, lamina dura
Figure 4-2-2
Figure 4-2-3
Subperiosteal resorption involving the middle, proximal

and terminal phalanges (arrows) resulting from
secondary hyperparathyroidism and renal failure.
Similar features are noted on the clinical photograph as
well as clubbing
Figure 4-2-4
Subperiosteal resorption involving the

middle and terminal phalanges (solid
arrows) resulting from secondary
hyperparathyroidism and renal failure.
The terminal phalanx also shows
band-like acroosteolysis (open arrow)
resulting from subperiosteal resorption
Subperiosteal resorption involving

the lamina dura (arrows) caused
by secondary
hyperparathyroidism and renal
failure
Cortical Resorption
[Figure 4-2-4]
Caused by osteoclastic activity within haversian canal

Radiographs-intracortical tunneling with increased lucent
striations in cortex
Nonspecific finding
Endosteal Resorption
[Figure 4-2-5]
Intracortical resorption with areas of intracortical

tunneling (arrows) resulting from hyperparathyroidism
on radiography and matched histologic macrosection
showing resorption along preexisting Haversian canals
(arrowheads)
Causes scalloping of endosteum- hands

Osteopenia with loss of trabecular sharpness
Calvarium salt and pepper appearance with loss of distinction of
tables
Figure 4-2-5
Endosteal resorption in the skull on CT

causing loss of distinction of inner and
outer table cortices (salt and pepper
appearance-arrowheads) in secondary
hyperparathyroidism
712
Figure 4-2-6
Figure 4-2-7
Subchondral resorption of the distal clavicle and

acromion (arrows and arrowhead) from secondary
hyperparathyroidism on radiography
Subchondral resorption of the metacarpal heads

(arrows) from secondary hyperparathyroidism on
radiography simulating an erosive arthropathy
Figure 4-2-8
Subchondral Resorption
[Figures 4-2-6 to 4-2-8]
Common in appendicular and axial skeleton

Often in hands-single DIP joint (4th or 5th) also MCP and
PIP joints
More recently polyarticular involvement 40% of patients on
long-term hemodialysis IP and first CMC joints with
symmetry
Simulates erosions, often progress, 50% symptomatic
Other frequent sites-distal clavicle, AC joint (20%), SI joint,
SC joint, symphysis pubis, posterior patella
Pathologically-collapsed cortical bone and overlying
cartilage
Initiate an osteogenic synovitis
Accentuated by mechanical stress, joint incongruity and
intraarticular debris
Brown tumor (*), diffuse sclerosis and subchondral

resorption (arrows) about the SI joints are seen on this
CT of the pelvis in a patient with chronic renal failure
Subligamentous/Subtendinous Resorption
[Figure 4-2-9]
Patients usually asymptomatic

Radiographs-smooth and scalloped or irregular
Common sites
Inferior calcaneus
Greater and lesser trochanters
Anterior inferior iliac spine
Humeral greater tuberosity
Ischial tuberosity
Elbow
Figure 4-2-9
Brown Tumors (Osteoclastomas)
Caused by localized bone replacement by vascularized

fibrous tissue
May become cystic after necrosis and liquefaction
(osteitis fibrosa cystica) higher incidence in primary
hyperparathyroidism; 1.5%1.7% in secondary
Subligamentous/subtendinosis resorption in the pelvis
on radiography at the ischial tuberosity and anterior
inferior iliac spine (arrowheads) resulting from secondary
hyperparathyroidism and renal failure
713
Brown Tumors: Radiographic Findings
Figure 4-2-10
[Figures 4-2-8 and 4-2-10]
Often solitary; may be multiple

Well defined lytic lesions
Frequently eccentric or cortical (long bones)
May cause scalloping and bone expansion
Sites-ribs, pelvis, facial bones and femora, axial skeleton
can be involved
May heal after treatment with calcification, sclerosis and
lesion disappearance
Periosteal New Bone: Formation
Caused by osteoblastic activity

Prevalence 8%25% often with severe disease
Linear often with radiolucent zone separating it from cortex
Can be laminated and chronically thicken cortex
Most common-humeri, femora, tibiae, radii, ulnae,
metacarpals, metatarsals and phalanges
Osteosclerosis
[Figure 4-2-11]
Brown tumor of hyperparathyroidism involving the tibia
Cause unknown
with pathologic fracture (arrows) on radiography and
coronal macrosection. Cyst formation (*) is seen on
9%34%
the macrosection
Predilection for axial skeleton
Rugger Jersey spine
Figure 4-2-11
Other sites-pelvis, ribs and clavicles
Metaphyses and epiphyses can be involved
After renal transplant osteosclerosis may regress but more common to
further increase
Osteopenia
Accumulated effect of osteomalacia, bone resorption and osteoporosis

Contributory factors-acidosis, poor nutrition, azotemia, steroids,
hyperparathyroidism, and reduced vitamin D
After renal transplant osteopenia may worsen or bone mineral content
may increase
Predisposed to fractures (5%25%): vertebral body, pubic rami and ribs
Fracture healing-normal but delayed
Osteomalacia
Decreased active form of vitamin D

Renal tissue hydroxylates vitamin D to active form
Additional factors-hypocalcemia, inhibitors to calcification in uremia,
aluminum toxicity, hepatic dysfunction
Rickets
Common in children with chronic renal insufficiency

Normal vessels that invade zone of provisional calcification fail to
develop
Result-disorganized cartilage zone columns
Diffuse sclerosis of the cervical spine

on radiography in a patient with
secondary hyperparathyroidism
Figure 4-2-12
Radiographic Findings in Rickets

[Figure 4-2-12]
Widened growth plate

Epiphyseal irregularity; metaphyseal fraying and
cupping
Delayed bone age and osteopenia
Bowed long bones and scoliosis
Concave vertebral endplates
Basilar invagination
Triradiate pelvis and acetabuli protrusio
Rachitic rosary
Slipped epiphyses
Rickets in the distal femur resulting from renal failure on

radiography and coronal macrosection with metaphyseal
widening and cupping (arrows) caused by growth plate
disorganization (arrowheads)
714
Slipped Epiphyses in CRI Induced Rickets
Not uncommon -10%

Proximal femur and humerus, distal femur and radius,
metacarpal and metatarsal heads
Greatest risk-adolescent boys, uremia > 2 years,
treatment close to onset of puberty
Usually bilateral in chronic renal insufficiency (CRI)
and often asymptomatic initially
Figure 4-2-13
Radiographic Findings: SCFE in CRI

[Figure 4-2-13]
Medial femur subperiosteal resorption

Increase epiphyseal plate width
Decrease neck-shaft angle
Typical findings of slipped capital femoral epiphysis
(SCFE)
Radiographic Findings: Osteomalacia in

Adults
Osteopenia with ill defined trabeculae unlike

osteoporosis
Looser zones-pseudofractures
Uncommon - 1% CRI patients
Pubic rami, medial femoral neck, scapulae, ribs,
long bones, lesser trochanters, ischial tuberosity
Rickets of the proximal femora and bilateral slipped capital

femoral epiphyses (arrowheads) in a renal failure patient on
pelvic radiograph with hips abducted
Figure 4-2-14
Soft Tissue and Vascular Calcification
Increases calcium - phosphate product > 75mg/dL

Contributory factor - local tissue damage and
alkalosis (calcium salt precipitation)
Common sites - ocular tissue, arteries,
subcutaneous, periarticular and visceral
Periarticular Calcification
[Figures 4-2-14 and 4-2-15]
Asymptomatic or pain and joint limitation

Prevalence
7% after 1 year hemodialysis
55% after more than 4 years hemodialysis
Often regresses with treatment
Often multifocal and symmetric
Dense and cloudlike on radiographs
Hydroxyapatite - chalky paste-like material
Can extend into tenosynovial tissue and joints
Sites - phalangeal joints, wrists, elbows, shoulders,
hips, knees, ankles
Figure 4-2-15
Heterogeneous large soft tissue mass (*) about the right

shoulder on coronal STIR MR images in a patient with renal
failure initially sent to radiology for biopsy of suspected
sarcoma. Subsequent radiograph shows subtle calcification
and vascular catheters in the central vessels for hemodialysis.
Diagnosis of periarticular calcification related to renal failure
and no biopsy was necessary
Periarticular calcification about the left hip on CT with several
calcium fluid levels (arrows) in a patient with renal failure
715
Arterial Calcification
Occur in media and intimal tissue

Pipestem appearance radiographically
Can make shunts or fistula for hemodialysis difficult
Initially - dorsalis pedis artery also leg, hand and forearm
Prevalence 27% < 1 year therapy
83% with 8 years or more of therapy
Rare in children
Second type - nodular with luminal encroachment, obstruction with ulceration,
gangrene and cardiac failure
Calcification in shunt aneurysms
Visceral Calcification
Usually not apparent on radiographs

May detect on bone scintigraphy (poor looking bone scan)
Sites - heart, lungs, stomach, kidneys
Prevalence - 79%
In myocardial tissue important, can cause conduction defects and death
Musculoskeletal Abnormalities More Common After Treatment
Aluminum toxicity
Amyloidosis
Tendon rupture
Crystal deposition
Infection
Avascular necrosis (AVN)
Aluminum Toxicity
Prevalence 1%30% (rare today)

Results in osteomalacia previously responsible for most osseous abnormalities
in patients on long-term hemodialysis
Unknown mechanism
Clinically - low PTH, serum aluminum > 100ng/mL
Cause - ingestion of aluminum salts in phosphate - binding antacids to control
hyperphosphatemia
Cannot excrete alumina
Toxic effects
Cerebral (Encephalopathy)
Osseous system
Aluminum Toxicity: Radiographic Findings

[Figure 4-2-16]
Osteomalacia - osteopenia, looser zones, fractures, rickets

More than 3 atraumatic fractures (86%) - ribs, vertebrae, hips, pelvis, sternum,
clavicles, extremities
AVN
Lack of osteosclerosis
Limited subperiosteal resorption
Bone biopsy - histochemical stain for aluminum
Amyloidosis
Figure 4-2-16
Secondary due to chronic disease

B2 microglobulin
Areas of deposition bone, tenosynovium,
intervertebral disk, cartilage, capsule, ligament,
muscle
Stains with Congo red, characteristic under
polarized microscopy and immunoperoxidase
methods
Amyloidosis
Musculoskeletal Involvement
Carpal tunnel syndrome
Osseous and intraarticular deposition
Destructive spondyloarthropathy
Osteomalacia due to aluminum toxicity in a renal failure patient

with radiograph showing multiple nontraumatic fractures
(arrows) and acetabulae protrusio (curved arrows)
716
Carpal Tunnel Syndrome in CRI
Figure 4-2-17
Long-term hemodialysis prevalence 2%31 %

Equal sex distribution
No prevalence for dominant hand
Amyloid primary cause of median nerve compression
Other causes - venous stasis and edema related to
treatment fistula
Osseous and Intraarticular Involvement in

CRI
[Figures 4-2-17 and 4-2-18]
Focal lytic areas or less well defined intramedullary

lytic lesions
Most common - carpus - scaphoid, lunate and
capitate - can enlarge
Other sites - humeral head, knee (patella) and about
hip
Endosteal scalloping, fractures, extrinsic erosion
from soft tissue mass, neuropathic appearance
Intraarticular deposition - common in hip, knee,
shoulder-low intensity on T2W images
Amyloidosis in a patient with chronic renal insufficiency

causing multiple punched out lytic lesions in the wrist
(arrowheads)
Figure 4-2-18
Spontaneous Hemorrhage Associated with

CRI
In hemodialysis patients likely related to heparin

Most frequent in thigh
MRI best for evaluation; appearance varies with
hemorrhage age
Destructive Spondyloarthropathy
Described 1984 by Kuntz and colleagues

Usually in patients on long-term hemodialysis
(219 years)
Prevalence - 15%; symptoms - pain
Cervical and lumbar spine
Multiple levels >50% of patients
Rapid progression 33%; simulates infection
Initial postulated etiology - crystal and noncrystal
deposition, neuropathic and hyperparathyroidism
Amyloid now considered offending agent
Amyloid deposition in the hip joint of a renal failure patient on

hemodialysis on coronal T1 and T2-weighted MR images
showing low signal intensity material in the joint (*) with
extrinsic bone erosion (arrow)
Figure 4-2-19
Destructive Spondyloarthropathy: Radiographic

Findings
[Figure 4-2-19]
Discovertebral erosions with sclerosis

Vertebral body compression
Disk space narrowing with Schmorl nodes
Lack of osteophytes
Facet involvement with subluxation
Destructive spondyloarthropathy at L5-S1

caused by amyloid deposition related to renal
failure with disc narrowing and destruction
(arrows) on radiograph simulating
infectious spondylodiskitis
717
Distinction of Infection vs. Destructive Spondyloarthropathy

[Figure 4-2-20]
Clinical symptoms / laboratory evidence lacking

Multilevel involvement unusual for bacterial infection T1
Limited uptake on scintigraphy
CT - lack of paravertebral soft tissue mass (also
MRI)
MRI - disc / endplate marrow replaced T1W
No prominent increased intensity on T2W
Figure 4-2-20
T2
Tendon Rupture or Avulsion in CRI
Spontaneous; in patients on long- term dialysis

Solitary or multiple
Tendon sites - quadriceps, patellar, triceps, flexors
and extensors of fingers
Cause - PTH excess - increased joint laxity
Tendon calcification
Chronic acidosis
Result - decrease tendon tensile strength and
accelerated degeneration
Destructive spondyloarthropathy related to amyloid deposition

(same patient as previous radiograph) with marrow and disk
replacement remaining predominantly low signal intensity on all
pulse sequences (*)
Radiologic Findings in Tendon Rupture in CRI

[Figure 4-2-21]
Before rupture - may see subtendonous bone resorption

After rupture - focal soft tissue swelling, effusion, subluxation
CT, MRI or sonography to evaluate tendon integrity and disruption site
Crystal Deposition Disease in CRI
Calcium hydroxyapatite, CPPD, monosodium urate, calcium oxalate

Hemodialysis elbow - olecranon bursitis
Calcium hydroxyapatite - EM or X-ray diffraction
CPPD - chondrocalcinosis not common - knee, wrist, hip, shoulders
and symphysis
CPPD arthropathy rare in CRI
Gout - infrequent in CRI, radiographic findings same as those in
primary podagra except distribution
Oxalosis - chondrocalcinosis, calcified joints, disks and
periarticular, diffuse osseous sclerosis
Figure 4-2-21
Predisposition to Infection in CRI
Depressed host responsiveness

Steroids and immunosuppressive treatment
Entry site via arteriovenous fistula for hemodialysis
Secondary infection in osteonecrosis
Infection Associated with CRI
Spontaneous rupture of the quadriceps

Osteomyelitis and septic arthritis
tendon (arrows) in a renal failure patient on
Bacterial or fungal
sagittal T2-weighted MR image
Radiographic findings - deep soft tissue swelling, periosteal
reaction, bone destruction, and joint space narrowing - same as
other situations
Unusual syndrome - progressive peripheral ischemic ulcers usually after
treatment and secondary infection
Bone Scintigraphy in CRI
Diffuse increased activity Super bone scan

May use as index of severity
Cause of increased activity is combination of vitamin D deficiency and
hyperparathyroidism
Increase rate bone turnover and collagen metabolism
Excess immature collagen
High enzyme activity
Increased osseous surface area for binding
Increased focal or diffuse soft tissue uptake
718
Avascular Necrosis in CRI
Up to 40% of CRI patients after renal transplantation

Additional factors - structural weakening, fracture and collapse, excess PTH,
graft-host reaction
Most common site - femoral head
Other sites - humeral head, about knee, talar dome, humeral condyles, cuboid,
carpal bones, long bone diaphyses
Avascular Necrosis in CRI
Radiologic appearance identical to other causes AVN

MRI most sensitive however only 50% show early changes
Osseous malignancy can complicate osteonecrosis and general increased
malignancy rate after renal transplant
Hyperparathyroidism: Primary vs. Secondary
Brown Tumors
Osteosclerosis
Chondrocalcinosis
Periostitis
+++
Rare
+
Rare
++
+++
Rare
+
References
1.
2.
3.
4.
Camacho CR, Talegon Melendez A, Valenzuela A, Gonzalez Guirao MA, Gomez Benitez S, Gil L, palma Alvarez A,
Mateos Aguilar J. Radiological findings of amyloid arthropathy in long-term haemodialysis. European Radiology.
1992; 2:305-309.
Leone A, Sundaram M, Cerase A, Magnavita N, Tazza L, Marano P.. Destructive spondyloarthropathy of the cervical
spine in long-term hemodialyzed patients: a five-year clinical radiological prospective study. Skeletal Radiol. 2001
Aug;30(8):431-41.
Murphey MD, Sartoris DJ, Quale JL, Pathria MN, Martin NL. Musculoskeletal manifestations of chronic renal
insufficiency. Radiographics. 1993 Mar;13(2):357-79.
Slavotinek JP, Coates PT, McDonald SP, Disney AP, Sage MR.. Shoulder appearances at MR imaging in long-term
dialysis recipients. Radiology. 2000 Nov;217(2):539-43.
719
Fundamental Concepts of Musculoskeletal

Neoplasm: Radiographs
Mark D. Murphey, MD
Tumors are classified by their pattern of differentiation
Tumors are graded on their degree of anaplasia
Skeletal Components
(Derived from Embryonal Mesenchyme)
Bone and cartilage progenitor cells

Periosteal cells
Hematopoietic cells
Lipocytes
Nerve and Schwann cells
Fibroblasts
Osteoclasts and Osteoclast-like cells
Endothelial cells
Perithelial cells
Notochordal cells (rests)
Histiocytic cells
Epithelial cells (rests)
HISTOGENIC CLASSIFICATION
OSTEOID BONE TUMORS
BENIGN
MALIGNANT
ENOSTOSIS
OSTEOSARCOMA
OSTEOID OSTEOMA
OSTEOMA
OSTEOBLASTOMA
CARTILAGE BONE TUMORS
BENIGN
CHONDROBLASTOMA
MALIGNANT
CHONDROSARCOMA
CHONDROMYXOID FIBROMA
ENCHONDROMA
JUXTACORTICAL CHONDROMA
OSTEOCHONDROMA
MARROW BONE TUMORS
BENIGN
LIPOMA
MALIGNANT
LIPOSARCOMA
LYMPHOMA
MYELOMA/PLASMACYTOMA
Fundamental Concepts of MSK Neoplasm: Radiographs
720
FIBROUS BONE TUMORS
BENIGN
MALIGNANT
DESMOPLASTIC FIBROMA
FIBROSARCOMA
HISTIOCYTIC TUMORS
BENIGN
MALIGNANT
EOSINOPHILIC GRANULOMA
MALIGNANT FIBROUS HISTIOCYTOMA
NOTOCHORD BONE TUMORS
BENIGN
MALIGNANT
CHORDOMA (HISTOLOGICALLY BENIGN)
CHORDOMA
VASCULAR TUMORS
BENIGN
LOW GRADE MALIGNANT
GLOMUS
HEMANGIOENDOTHELIOMA
HEMANGIOMA
HEMANGIOPERICYTOMA
MALIGNANT
ANGIOSARCOMA
LYMPHANGIOMA
UNKNOWN ORIGIN TUMORS
BENIGN
MALIGNANT
GIANT CELL TUMOR
MALIGNANT GIANT CELL TUMOR

MALIGNANT FIBROUS HISTIOCYTOMA
EWING SARCOMA
ADAMANTINOMA
721
Incidence of Bone Tumors

[Figure 4-3-1]
Approximately 1 individual in 75000 develops a primary bone tumor that leads

to biopsy
About 4000 new cases per year
Figure 4-3-1
Figure 4-3-2
Incidence of Bone Tumors

[Figures 4-3-2 to 4-3-4]
Of biopsied primary bone tumors: malignant tumors are three times more
common as benign lesions
Metastatic lesions are biopsied about 35 times more frequently than primary
tumors
Primary Benign Bone Tumors

[Figure 4-3-3]
Primary Malignant Bone Tumors

[Figure 4-3-4]
Figure 4-3-3
Figure 4-3-4
5
15
5
722
Important Factors in the Diagnosis of Bone Tumors

Patient age and sex

Bone involved
Location in bone
Lesion margin
Matrix formation
Periosteal reaction
These radiologic characteristics reflect the pathologic process and its biologic
activity.
Primary Benign Bone Tumors: Age Distribution by Decade

[Figure 4-3-5]
Figure 4-3-5
Primary Malignant Bone Tumors: Age Distribution by Decade

[Figure 4-3-6]
Figure 4-3-6
723
The site frequency, peak age of incidence, and numerical frequency of bone
tumor indicate that they are not completely autonomous, but are subject
to the laws of field behavior and developmental anatomy of normal
bone...
Figure 4-3-7
Johnson L. 1953
Location in Bone: Longitudinal

Epiphysis
Metaphysis
Diaphysis
Figure 4-3-8
Chondroblastoma with lytic lesion in the

epiphysis
Ewing sarcoma involving the femoral diaphysis on radiograph, T1-weighted MR

and gross specimen
Location in Bone: Axial

[Figures 4-3-9 to 4-3-10]
Central
Eccentric
Cortical
Juxtacortical
Soft Tissue
Figure 4-3-10
Figure 4-3-9
724
Pattern of Bone Destruction and Lesion Margin

[Figure 4-3-11]
Figure 4-3-11
Type I: Geographic
A: Well-defined, sclerosis
B: Well-defined, no sclerosis
C: Ill-defined
Type II: Motheaten
Type III: Permeative
Transition Zone
Margin Reflects Biologic Activity
Figure 4-3-12
Aggressive versus Nonaggressive

Biologic Activity
Margin
Geographic IA
Geographic IB
Geographic IC
Motheaten
Permeative
Growth Rate
Slow
Slow to Intermediate
Intermediate
Intermediate
Fast
1A Margin
[Figure 4-3-12]
Geographic
Well-Defined
Sclerosis
Geographic 1A
Geographic 1A: Differential Diagnosis
Figure 4-3-13
[Figure 4-3-13]
Bone cyst
Brodie abscess [Figure 4-3-14]
Cartilage lesions
Chondroblastoma
Chondromyxoid Fibroma
Enchondroma
Fibroxanthoma
Fibrous Dysplasia
Figure 4-3-14
Nonossifying Fibroma /Fibroxanthoma with geographic 1A margin on

radiograph, gross specimen and macrosection
Brodie abscess with geographic 1A margin.

Note the channel like extension (arrow) representing a sinus
tract inferiorly on the conventional tomogram (right image)
725
1B Margin
Figure 4-3-15
[Figure 4-3-15]
Geographic
Well-Defined
No Sclerosis
Geographic IB: Differential Diagnosis

Giant Cell Tumor [Figure 4-3-16]
Bone Cyst
Cartilage Lesions
Chondroblastoma
Chondromyxoid Fibroma
Enchondroma
Fibrous Dysplasia
Myeloma/Metastasis
Figure 4-3-16
Geographic 1B
Figure 4-3-17
Giant cell tumor of the distal radius with geographic 1B margin

on radiograph and macrosection extending to subchondral bone
1C Margin
[Figure 4-3-17]
Geographic 1C
Geographic
Ill-Defined
Figure 4-3-18
Geographic IC: Differential Diagnosis
Chondrosarcoma
Enchondroma (Active)
MFH/Fibrosarcoma [Figure 4-3-18]
Giant Cell Tumor
Osteosarcoma
Metastasis/Myeloma
Tumor Margin
Motheaten [Figure 4-3-19]

Permeative[Figures 4-3-20 and 4-3-21]
Fibrosarcoma
726
Figure 4-3-19
Figure 4-3-20
Motheaten
Figure 4-3-21
Permeative
Motheaten: Differential Diagnosis
Ewing sarcoma
Round cell tumors
Malignant fibrous histiocytoma/Fibrosarcoma
Osteomyelitis
Osteosarcoma
Langerhans cell histiocytosis (LCH)
Metastasis/Myeloma
Permeative: Differential Diagnosis
Permeative
Ewing sarcoma
Round cell tumors
Malignant fibrous histiocytoma/Fibrosarcoma
Metabolic disorders [Figure 4-3-22]
Osteomyelitis (acute) [Figure 4-3-23]
Osteosarcoma
LCH
Myeloma/Metastasis
Figure 4-3-22
Figure 4-3-23
Hyperparathyroidism with variation of the permeative pattern of

bone lysis in the cortex on radiograph and macrosection
(multiple areas of resorption along Haversion canals arrows)
Multifocal acute bacterial osteomyelitis with motheaten to

permeative destructive pattern of bone lysis on radiographs
involving the tibia and femur.
Macrosection
(right image shows pus on either side of cortex-arrows)
727
Lytic Patterns
[Figure 4-3-24]
Figure 4-3-24
Invisible Margin
[Figure 4-3-25: Lymphoma]
Figure 4-3-25
Lymphoma (same patient) with extensive marrow replacement (*) on T1 weighted MR, not seen on
radiograph images.
728
Changing Margin [Figure 4-3-26]
Increased Biologic Activity
Figure 4-3-26
Changing Margin
Matrix Formation
I. Mineralized
Chondroid - rings, arcs,
honeycomb[Figure 4-3-27]
Osteoid - ivory or cloudlike
II. Nonmineralized
Fluid
Soft Tissue
Fat
Osteonecrosis (*) with malignant

transformation to malignant fibrous
histiocytoma (MFH) showing new lysis
(arrow) at periphery on specimen
radiograph representing the changing
margin
Osteonecrosis (*) with malignant

transformation to MFH on CT (same
patient as previous radiograph) with
new cortical destruction laterally and
soft tissue mass (arrow)
Coronally
sectioned gross
specimen and
macrosection
showing
osteonecrosis (*)
and MFH arising at
periphery (arrows)
(same patient as
previous
radiograph and CT)
[Figure 4-3-28]
Figure 4-3-27
Figure 4-3-28
Chondrosarcoma of the fibula on specimen

radiograph and gross specimen show ring and
arc matrix mineralization (arrows)
Osteosarcoma of the tibia with dense cloud-like matrix

mineralization (arrows)
729
Periosteal Reaction: Nonaggressive

[Figure 4-3-29]
Solid (a)
Buttressing (b)
Expansion (c)
Septation (d)
Figure 4-3-29
(a)
(b)
(c)
(d)
730
Periosteal Reaction: Aggressive

[Figures 4-3-30 and 4-3-31]
Codman triangle (a)

Sunburst (b)
Hair-On-End (c)
Laminated (d)
Figure 4-3-30
(a)
(b)
(c)
(d)
731
Polyostotic vs. Monostotic

Holes in Bone
Figure 4-3-31
Polyostotic Lesions: Benign
Langerhans Cell Histiocytosis (LCH) [Figure 4-3-32]

Enchondromatosis
Fibrous Dysplasia
Hereditary multiple exostoses (HME)
Osteomyelitis
Paget disease
Neurofibromatosis (type 1)
Angiomatous lesions
Polyostotic Lesions: Malignant
Metastases
Multiple Myeloma [Figure 4-3-33]
Hemangioendothelioma
Osteosarcoma with aggressive hairon- end periosteal reaction (arrows)
Figure 4-3-32
Figure 4-3-33
Langerhans cell histiocytosis with areas of calvarial lysis in the

frontal and occipital areas (arrows)
Multiple myeloma on lateral skull radiograph

with multiple areas of bone lysis
References
1.
2.
3.
4.
5.
6.
"General Considerations". In: Bone Tumors, ed Dorfman HD, Czerniak B, Mosby: St.
Louis 1998. p. 1-33.
Ghelman B. Radiology of bone tumors. Orthop Clin North Am. 1989 Jul;20(3):287312. Review.
Lodwick GS, Wilson AJ, Farrell C, Virtama P, Dittrich F. Determining growth rates of
focal lesions of bone from radiographs. Radiology. 1980 Mar;134(3):577-83.
Madewell JE, Ragsdale BD, Sweet DE. Radiologic and pathologic analysis of solitary
bone lesions. Part I: internal margins. Radiol Clin North Am. 1981 Dec;19(4):715-48.
Ragsdale BD, Madewell JE, Sweet DE. Radiologic and pathologic analysis of solitary
bone lesions. Part II: periosteal reactions. Radiol Clin North Am. 1981 Dec;19(4):74983.
Sweet DE, Madewell JE, Ragsdale BD. Radiologic and pathologic analysis of solitary
bone lesions. Part III: matrix patterns. Radiol Clin North Am. 1981 Dec;19(4):785814.
732
Fundamental Concepts of Musculoskeletal

Neoplasm: CT and MRI
Mark D. Murphey, MD
Important Features in Evaluation of Musculoskeletal Masses
Preoperative assessment and staging
Osseous Neoplasm [Figure 4-5-1]
Differential diagnosis of primary skeletal neoplasms is best determined by

radiographs!!
But...
MRI and/or CT are vital for delineating and staging osseous neoplasms prior to
surgery
Figure 4-5-1
Enchondroma vs.Chondrosarcoma on
radiograph due to chondroid
mineralization (arrow)
Chondrosarcoma (same patient as previous radiograph) on MR due to

associated cortical destruction and soft tissue mass (arrows)
Soft Tissue Neoplasm
Radiographs only occasionally helpful

CT and more often MRI can be tissue-specific
But...
MRI and/or CT are again vital for defining extent, staging and preoperative
evaluation. Clinical and radiologic characteristic often limit differential
diagnosis
Causes of Tissue Specific Diagnosis on CT/MRI in Evaluating

Soft Tissue Masses
20%50% cases
Contrast resolution, MRI > CT
Multiplanar imaging, MRI > CT
Location of mass
Growth pattern and history
Soft Tissue Masses Diagnosed with Imaging Alone

Lipomatous lesions
Angiomatous lesions
Neurogenic tumors
Elastofibroma and fibromatosis
PVNS and ganglion
733
Fundamental Concepts MSK Neoplasm: CT and MRI
Figure 4-5-2
T1
T2
Lipoma (coronal T1 and T2-weighted images) isointense to fat

on all pulse sequences (*) with single thin septation (arrows)
Figure 4-5-3
Malignant peripheral nerve sheath tumor (arrow) in patient with

neurofibromatosis type 1 (note second small subcutaneous
neurofibroma- curved arrow)
Staging of Musculoskeletal Tumors: Benign (G-O) [Figure 4-5-4]
Stage 1 Unchanged or healing lesion; well-encapsulated; indolent clinical

course
Stage 2 Active growth; symptomatic, remains intracapsular but may be
deforming
Stage 3 Aggressive local growth; may penetrate cortex or compartment;
higher recurrence rate
Staging of Musculoskeletal Tumors: Malignant [Figure 4-5-5]
Stage 1 (G1): Low Grade, well differentiated, few mitoses; tend to recur locally
1A Intraosseous / Intracompartmental
1B Extraosseous / Extracompartmental
Stage 2 (G2) High Grade, poorly differentiated, many mitoses; high
incidence of metastases
2A Intraosseous / Intracompartmental
2B Extraosseous / Extracompartmental
Stage 3 Metastases; regional or remote (visceral, lymphatic or osseous)
734
Figure 4-5-4
Staging of Musculoskeletal Neoplasm: Histologically Benign (G-O)
STAGE
Bone
Soft Tissue
II
III
735
Figure 4-5-5
Staging of Musculoskeletal Neoplasm: Histologically Malignant
Low grade histo: IA
Low grade histo: IB
Bone
Soft Tissue
High grade histo: IIA
High grade histo: IIB
BONE
BONE
SOFT TISSUE
SOFT TISSUE
Stage III
736
American Joint Commission Staging

Protocol for Sarcoma of Soft Tissue
Histologic grade (G)

G1 well differentiated
G2 moderately well differentiated
G3-4 poorly differentiated, undifferentiated
Primary Tumor (T)
T1 tumor 5cm or less in greatest dimension
T2 tumor more than 5cm in greatest dimension
Regional lymph nodes (N)
N0 no regional lymph node metastasis
N1 regional lymph node metastasis
Distant metastasis (M)
M0 no distant metastasis
M1 distant metastasis
Staging of Musculoskeletal Neoplasm Has

Implication on Surgical Treatment
Figure 4-5-6
Intracapsular excision
Marginal excision
Wide excision
Radical resection
Amputation
Figure 4-5-7
LIMB SALVAGE PROCEDURES
AMPUTATIONS
737
Important Factors on Imaging for Staging

Musculoskeletal Neoplasm
Figure 4-5-8
Intramedullary extent
Extent of soft tissue component
Lesion matrix
Cortical involvement
Neurovascular involvement
Joint involvement
Intramedullary and Soft Tissue: Extent of

Musculoskeletal Neoplasm [Figures 4-5-8 and 4-5-9]
MRI superior to CT
Superior contrast resolution
Multiplanar imaging capability
Regional Metastases osseous/lymph node
Can be helpful to direct biopsy
Always perform in consultation with orthopod
Done in institution of definitive procedure
Give orthopod anatomic landmarks !
Osteosarcoma with spread across physeal plate (arrows) not

seen on radiograph
Figure 4-5-9
MRI may overestimate musculoskeletal

neoplasm extent because of surrounding
edema (reactive zone)
Musculoskeletal Neoplasm: Lesion Matrix
Evaluation
I. Mineralized CT > MRI

A. Chondroid rings and arcs [Figure 4-5-10]
B. Osteoid cloudlike, ivory-like
C. Other calcification phlebolith, synovial sarcoma
II. Nonmineralized MRI > CT
A. Fluid, necrosis, hemorrhage [Figure 4-5-11]
B. Fat
C. Soft tissue nonspecific
T1
T2
Ewing sarcoma following chemotherapy with prominent

reactive zone (*) around the low signal intensity
pseudocapsule (arrows)
Figure 4-5-10
Figure 4-5-11
Chondrosarcoma with chondroid matrix mineralization

not seen on radiographs or MRI
Aneurysmal bone cyst with fluid levels on T2-weighted

MRI reflecting cystic spaces on gross specimen
738
Musculoskeletal Neoplasm: Cortical

Involvement [Figure 4-5-12]
Figure 4-5-12
CT > MRI (my opinion)

MRI=CT (literature)
CT better spatial resolution
Important in differential diagnosis of osseous lesions
Important for surgical resection/staging
Musculoskeletal Neoplasm: Neurovascular

Involvement
[Figures 4-5-13 and 4-5-14]
Vital information for surgical resection

MRI > CT (post-contrast if use CT)
Improved contrast resolution
Multiplanar MR images often helpful
Axial plane usually best
Look for intact fat plane
Osteoid osteoma (arrow) and lesion was difficult to detect on
MRI (right image) compared to CT (left image)
Best on T1W images
If fat plane lost cannot exclude involvement
Soft tissue mass encase vessels definite involvement
Figure 4-5-13
Figure 4-5-14
Osteosarcoma with displaced but nonencased

neurovascular bundle (arrows)
Musculoskeletal Neoplasm: Ligament and

Tendon Involvement
Important for surgical reconstruction

MRI > CT; best on T2W image
Tendons/ligaments low intensity vs. tumor high signal
On CT tendon/ligament similar to tumor attenuation
Also multiplanar imaging of MR helpful
Musculoskeletal Neoplasm: Joint

Involvement
Osteosarcoma with encased neurovascular bundle (arrows)

with tumor replacing normal fat seen about vessels
Figure 4-5-15
[Figure 4-5-15]
Dramatically changes surgery from:

Limb salvage; intraarticular resection
Extraarticular limb salvage/amputation
MRI superior to CT multiplanar imaging
Coronal or sagittal plane best
Three routes of spread into joint
Through bone/cartilage (transarticular)
Around joint margin (periarticular)
Along ligaments/tendons, or hematogenous
Presence of joint effusion suggestive
Absence of joint effusion excludes
Osteosarcoma invading the knee joint with effusion (arrows)

and tumor (*) along ACL (arrowheads) on sagittal T2 MR
and gross specimen
739
Overall Delineation of Musculoskeletal Masses: All Features

(56 Cases, N=189)
MRI > CT 60%

MRI = CT 16%
CT > MRI 24%
Intramedullary Extent
Soft Tissue Mass
Mineralized Matrix
Cortical Involvement
Neurovascular Involv.
Joint Involvement
MRI (N=56)
81%
89%
0%
7%
78%
73%
CT (N=56)
5%
0%
92%
72%
0%
3%
CT Indications
Cannot perform MRI

Matrix producing neoplasm not adequately evaluated on radiographs
Unusual location
Ribs, sternoclavicular region, scapula
Abdominal/chest wall
Fibula
Imaging Characteristics Suggesting Benign Soft Tissue Mass
Small size
Well marginated
Homogeneous signal intensity
No neurovascular encasement
Enhancement pattern dynamic MRI (late, slow, diffuse/none)
Imaging Characteristics Suggesting Malignant Soft Tissue Mass
Large size
Poor margin definition with edema
Heterogeneous signal intensity
Neurovascular encasement
Enhancement pattern dynamic MRI (early, rapid, peripheral)
Distinction of Benign vs. Malignant Soft Tissue Mass by MRI
Totty Radiology 1986; 160:135141 (N=32)

Sundaram MRI 1988; 6:237248 (N=53)
Kransdorf AJR 1989; 153:541547 (N=112)
Berquist AJR 1990; 155:12511255 (N=95)
Crim Radiology 1992; 185:581586 (N=83)
740
Soft Tissue Masses Misinterpreted on MRI:

Benign vs. Malignant [Figures 4-5-16 to 4-5-18]
Figure 4-5-16
Diabetic muscle ischemia

Hematoma
Fibromatosis
Reactive lymph node, abscess, bursitis
Myositis ossificans
Synovial sarcoma
Myxoid liposarcoma
General consensus is that in an individual case, MRI is not accurate

enough to predict whether a nonspecific solid soft tissue mass is
benign or malignant
Musculoskeletal Neoplasm Follow-Up
Myositis ossificans with aggressive

appearance on coronal T2-weighted MRI
Pre-operative-post therapy
Post-operative-recurrence
MRI superior to CT
Figure 4-5-17
Post Therapy Imaging
Increasing ossification Osteosarcoma, Ewing sarcoma

Radiographs/CT
Change in size and extent MRI
Increasing peritumoral edema
Tumor necrosis and hemorrhage
> 90% required for a pathologic good response
Post-Operative Imaging: Radiographs
Comparison to previous studies

Findings of recurrence
New bone destruction
New areas matrix formation
Myositis ossificans with early rim of ossification

(arrow) on CT (same patient as previous MRI)
Post-Operative Imaging: MRI/CT: Normal
MRI > CT improved contrast resolution

Comparison to baseline study (first 2-3 months)
Recognize normal changes
Post-op edema/myositis
Radiation necrosis
Muscle flap
Fluid collections subfascial, lymphocele/seroma
Figure 4-5-18
Post-Operative Imaging: MRI/CT: Abnormal

[Figures 4-5-19 and 4-5-20]
New bone destruction/marrow replacement

Any recurrent or residual nodular region
Tumor until proven otherwise
Texture sign
Regardless of signal characteristics unless low all
sequences representing fibrosis or fluid collection
Contrast studies can be helpful
Synovial sarcoma with homogeneous appearance and

defined margins suggesting an indolent lesion. Intrinsically
the lesion has nonspecific features of a solid mass
Figure 4-5-19
Post operative lymphocoele/seroma (*)

in patient with previous MFH resection
with homogeneous low (T1) and high
(T2) signal intensity as expected for a
fluid collection
741
Musculoskeletal Neoplasm: Use of MRI with

Gadolinium
Figure 4-5-20
[Figure 4-5-21]
Increase lesion conspicuity (usually not needed)

Tumor, edema, inflammation and fibrosis all
enhance
Help differentiate cyst/hemorrhage
Helpful in post-op cases to show nodular
enhancement with recurrence
Vanel/Bloem - dynamic subtraction MRI early
enhancement of recurrent tumor and response to
therapy
Musculoskeletal Masses: Imaging Goals
Delineate precise extent of lesion

Diagnosis/exclude metastases
Give most likely tissue type and differential
diagnosis
Recurrent MFH (arrows) adjacent to residual

lymphocoele/seroma (*) (same patient as previous MRI but 2
years later). Note the tumor staining on angiogram (far right
image-arrowhead) versus vessels draped about fluid collection
(curved arrow)
Figure 4-5-21
Myxoid MFH with enhancing peripheral solid nodular tissue

(arrows). These enhancing areas represent viable tumor
regions and biopsy should be directed toward these regions as
they harbor diagnostic tissue and were only detected after
contrast administration
References
1.
2.
3.
4.
5.
6.
Berquist TH. Magnetic resonance imaging of musculoskeletal neoplasms. Clin Orthop Relat Res. 1989
Jul;(244):101-18. Review.
Sundaram M, McGuire MH. Computed tomography or magnetic resonance for evaluating the solitary tumor or
tumor-like lesion of bone? Skeletal Radiol. 1988;17(6):393-401.
Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop Relat Res. 1986 Mar;(204):9-24.
Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin
Orthop Relat Res. 1980 Nov-Dec;(153):106-20.
Stacy SG, Mahal RS, Peabody TD. Staging of Bone Tumors: A Review with Illustrative Examples. Am. J.
Roentgenol., Apr 2006; 186: 967 - 976.
Murphy WA Jr. Imaging bone tumors in the 1990s. Cancer. 1991 Feb 15; 67(4 Suppl):1169-76. Review.
742
Osteoid Lesions of Bone

Mark D. Murphey, MD
Figure 4-5-1
Enostosis and associated conditions

Osteoma
Osteoid osteoma
Osteoblastoma
Osteosarcoma
Enostosis (Bone Island): Clinical

Characteristics
Stieda 1905 - Kompakten Knochenkerne

Usually incidental finding
Patient asymptomatic
Common lesions-unknown frequency
(0.43%14% rib, pelvis and spine)
Rare in children
Enostosis (Bone Island): Histology [Figure 4-5-1]
Enostosis histologically with pink cortical bone (*) and irregular

thorn-like medullary margins (arrows)
Intramedullary location
Composed of normal appearing compact lamellar bone with haversian
canals
Blends with surrounding trabecular bone creating
irregular margin
Likely developmental - can be considered a
hamartoma
Figure 4-5-2
Enostosis (Bone Island): Radiology

Round to oval (0.22.0 cm) osteoblastic area

Often epiphyseal or metaphyseal
Thorny, radiating spicules at margin but well
defined
Vast majority (>95%) no need for further radiologic
evaluation following radiograph
Bone scan (if needed) usually normal (may show
Enostosis with thorn-like margins (arrow) in the fifth
minimal activity)
metacarpal head
May slowly increase or decrease in size
Differential diagnosis: osteoblastic metastasis, osteoma, osteoid osteoma, low
grade osteosarcoma
Follow-up 1, 3, 6 and 12 months
Figure 4-5-3
Biopsy if increase in size too rapidly
> 25% over 6 months
Giant Bone Island [Figure 4-5-4]
> 2-3 cm in size

Most often in pelvis
More likely to have increased activity (25%) on bone scan
(but usually mild; < ant. iliac crest)
Most difficult to differentiate from sclerosing low grade
intramedullary osteosarcoma (on histology look for
entrapped host lamellar bone)
Identical on histology to other bone islands
Enostosis with thorn-like margins (arrow)

743
Possible Diseases Related to Enostosis
Figure 4-5-4
Osteopoikilosis
Osteopathia striata
Melorheostosis
Osteopoikilosis (Osteopathia Disseminata)

Clinical Characteristics
Autosomal dominant inheritance; asymptomatic

Dermatofibrosis lenticularis disseminata
Keloid formation
May have mild arthralgias (15%-20%)
Osteopoikilosis:
Radiology and Pathology
Giant bone island with irregular thorn-like

margins (*)
Numerous circular or ovoid radiodensities

Often symmetric - no increased activity on bone scan
Predilection epiphyses and metaphyses
Also carpus, tarsus, pelvis
May increase or decrease in size
Pathology - same as solitary enostosis
Figure 4-5-5
Osteopathia Striata (Voorhoeve Disease):

Osteopoikilosis variant - 1924

Autosomal dominant?
Usually asymptomatic or mild arthralgias
Associated syndromes - Goltz syndrome, cranial sclerosis
Osteopathia Striata: Radiology [Figures 4-5-5 to 4-5-7]
Linear bands of sclerosis from metaphysis in long bones

Fan-like bands of sclerosis in flat bones (iliac)
Could simulate heavy metal poisoning
Sometimes associated with osteopoikilosis
Bone scan-normal
Mixture of osteopoikilosis (circular areas of

sclerosis-arrow) and osteopathia striata (linear
areas of sclerosis-arrowhead)
Figure 4-5-6
Melorheostosis: Clinical Data
Described 1922: Leri and Joanny

Only 50% evident before age of 20 years
Equal sex distribution
Often symptomatic - pain, decreased range of motion (ROM),
contractures; limb swelling/length discrepancy and bowing
Scleroderma like skin lesions over osseous changes
Melorheostosis: Pathology
Figure 4-5-7
Thickened and enlarged cortical

bone
Haversian canals normal with
irregular arrangement
Marrow space may show
increased cellularity
Soft tissue may contain mass of
fibrous tissue with or without
ossification
Mixture of osteopoikilosis (arrowhead)

and osteopathia striata (arrow) showing
low signal intensity on MRI
Osteopathia striata with linear bands of

sclerosis in the tibia and femur
744
Melorheostosis: Radiology [Figures 4-5-8 to 4-5-10]
Distribution - single limb - more common lower extremity

One or more bonessclerotome (skeleton supplied by spinal sensory
nerves) pattern
Osseous excrescences often exuberant and lobulated along bone
surface -candle wax
Also endosteal involvement may extend into marrow space
Can extend into soft tissue with ossification, often periarticular
May lead to joint ankylosis
Intense activity on bone scan
Figure 4-5-10
Figure 4-5-8
Figure 4-5-9
Melorheostosis
involving foot
with increased
uptake on bone
scan (same
patient as
previous two
images)
Melorheostosis with mineralized

inguinal soft tissue mass
(arrowhead)
Figure 4-5-11
Melorheostosis classic dripping candle wax

appearance (arrow)
Osteoma: Clinical
Characteristics
A benign, slow growing tumor, composed of osteoid tissue

Found in cranial vault, sinuses, mandible and (rarely) long bones
Represent protruding mass of dense periosteal intramembranous bone
on surface of host bone
Signs/symptoms depend on size/location
Sinus lesions may lead to sinusitis, headache, or can grow into cranial
vault
Orbital lesion may cause exophthalmos, displacement of globe, diplopia
0.42% patients with sinus radiographs
Osteoma: Pathology
Nodules of dense osseous tissue

Differences from bone island:
Often a mix of woven & lamellar bone
May/may not contain haversian system
Arises from cortex rather than intramedullary
Do not blend with trabecular bone
Most frequent in the skull
In craniofacial area often in spectrum of fibrosseous
lesions
Calvarial osteoma (*)
Figure 4-5-12
Osteoma: Radiology [Figure 4-5-11 and 4-5-12]
Sharply defined, homogeneous, bone mass arising

from surface of bone
Most frequently diagnosed incidentally on radiographs
Frontoethmoid sinus region - 75%
Sphenoid 1%4%
745
Gross specimen calvarial osteoma (*) on surface of outer

table (arrow) but not extending into the diploic space (DS)
Osteoma: Gardner Syndrome
Multiple osteomas are almost always associated with Gardner syndrome

Familial autosomal dominant
Intestinal polyposis
Multiple osteomas
Dental lesions
Soft tissue desmoid tumors
Skin lesions
Sebaceous cysts and fibromas
Osteoid Osteoma: History
Described in 1935 by Jaffe as an osteoblastic tumor composed of osteoid and

atypical bone
Established by Jaffe as a distinct clinical and pathologic entity
Controversy remains as to true nature: inflammatory, traumatic, vascular, viral
Osteoid Osteoma: Clinical Characteristics
11% of all bone lesions that come to biopsy (3% of primary bone tumors)
Spinal lesions commonly present with painful scoliosis, without neurologic
dysfunction
Intraarticular lesions often present with nonspecific vague joint pain
Young patients usually 1025 years
Male : Female approximately 23:1
Rare in blacks
Pain almost invariably present complaint (1.6% painless - 50% of these in the
hand)
Pain relief with aspirin/nonsteroidals
Inhibit prostaglandin E2, aggravated by ETOH
Osteoid Osteoma: Skeletal Distribution [Figure 4-5-13]
Figure 4-5-13
Femur/tibia - 50% - 60% of lesions

Most frequent in long bone diaphysis (70% - 80%)
Spine - 10% (90% posterior elements; 10% vertebral body)
Hand/foot - 10% - 20% - proximal phalanx, metacarpal, scaphoid,
navicular, calcaneus
Epiphyseal lesions - rare
Osteoid Osteoma: Pathology
The nidus is the lesion-yellowish to red pea

Composed of osteoid and woven bone with interconnected
trabeculae
Background and rim of highly vascularized fibrous connective tissue
Extensive reactive bone may surround the lesion
No malignant potential
Osteoid Osteoma: Classification
Cortical (70% - 75%): long bone shaft; intense fusiform sclerosis; central nidus
Cancellous (25%) intermediate frequency; usually femoral neck, hand/foot;
often limited surrounding sclerosis
Intraarticular lesions (cortical/cancellous may have limited sclerosis/periosteal
reaction and can be distant from nidus
Subperiosteal; rare, arises adjacent to bone; usually femoral neck, talar neck,
hand, foot; bone may show pressure erosion
746
Osteoid Osteoma: Radiology: Cortical Lesions

[Figures 4-5-14 to 4-5-19]
Figure 4-5-14
Dense fusiform sclerosis

Periosteal bone solid, rarely lamilated
Nidus usually central, rarely > 1.5 - 2cm
Hot on scintigraphy - double density sign
Nidus well-defined on CT with a smooth peripheral
margin, +/- central mineralization
Figure 4-5-15
CT of osteoid osteoma with central

focus of calcification
Humeral osteoid osteoma in the cortex with bone scan showing

double uptake sign
Figure 4-5-16
Figure 4-5-17
MR imaging of osteoid osteoma with intermediate signal

intensity on T1 [left] and T2 [right] -weighting (arrow).
The surrounding marrow edema is prominent (*)
and can obscure the nidus
Osteoid osteoma specimen radiograph shows central

calcification and entire nidus within
the bivalved gross specimen
Osteoid Osteoma: MR Imaging
Low to intermediate intensity T1-images

Intermediate to high intensity T2-images
Low intensity all pulse sequences if totally
mineralized nidus
May detect synovitis/joint effusion/soft tissue
edema that can be very prominent/confusing
imaging feature
Reactive marrow edema may obscure lesion
CT more helpful in majority, however, MRI may be
very helpful in difficult cases
Figure 4-5-18
Figure 4-5-19
Radiograph and bone scan of spinal osteoid osteoma in facet

with a sclerotic pedicle (arrow) and increased uptake on the
radionuclide bone scan (arrowhead)
CT of spinal osteoid osteoma in facet

with central calcification
747
Differential Diagnosis of Osseous Lesions with Sequestra-Like

Appearance
Osteomyelitis-pyogenic
Subacute osteomyelitis or unusual organism
Osteoid osteoma/osteoblastoma
Metastases
Fibrosarcoma/MFH
Lymphoma
Figure 4-5-20
Cortical Osteoid Osteoma: Differential

Diagnosis
Brodie abscess
Stress fracture (linear not circular)
Osteoid Osteoma Radiology:

Intraarticular/Cancellous Lesions
Reactive osteosclerosis/periosteal reaction often

mild/absent and may be distant from lesion
Associated joint effusion/lymphofollicular synovitis
Regional osteoporosis-disuse
May have associated periostitis
May be diffusely hot on scintigraphy
Subperiosteal lesions present as juxtacortical
masses
Subtle intraarticular osteoid osteoma (arrow) with central

calcification causing effusion and hip joint widening simulating
septic arthritis
Figure 4-5-21
Intraarticular Osteoid Osteoma:

[Figures 4-5-20 and 4-5-21]
Rheumatoid arthritis, JRA

Tuberculous arthritis
Nonspecific synovitis
Septic arthritis
Osteoblastoma (especially spine)
Osteoid Osteoma: Classic Treatment
Surgical excision - curative with complete nidus removal - post op biopsy

radiographs
Dramatic relief of symptoms
Recurrence due to incomplete excision can then have multiple nidi
Problems
Locating lesion at surgery
Tetracycline and radionuclide labeling
Osteoid Osteoma: Other Treatment Methods
Medical- spontaneous healing

Percutaneous removal
Percutaneous ablation (radiofrequency)
Embolization single feeding artery (potentially) ?
Osteoblastoma
Rare benign osteoid producing tumor characterized by osteoid and woven

bone production
Subtle intraarticular osteoid osteoma
Synonyms include: giant osteoid osteoma and osteogenic fibroma
(arrow) with central calcification
About 1.0% of excised primary osseous tumors
causing effusion and hip joint widening
Osteosarcoma 20x more common, osteoid osteoma 4x more frequent
simulating septic arthritis on CT and
MR. The CT shows typical nidus with
central calcification (arrow) that is
difficult to perceive on the MR
748
Osteoblastoma: Clinical Features
Patients are young, median age 18, 80% are between 1030 years
Males : Females ratio 23:1
Pain is most common symptom, less severe than osteoid osteoma
Less often at night and may or may not be relieved by aspirin
Figure 4-5-22
Osteoblastoma: Skeletal Distribution
Spine, (40%) equally distributed cervical through sacrum

About 30% occur in long bones, most commonly femur and tibia
Skull, mandible, maxilla (15%)
Also small bones of hand & feet (10%) and pelvis (5%)
Osteoblastoma: Pathology
Micro: large number of osteoblasts producing trabeculae, osteoid or

bone
Virtually indistinguishable from osteoid osteoma on high-power
histologic examination
At times minor microscopic differences from osteoid osteoma but may
rely on radiology
Osteoblastoma: Radiology - General
Described as having 3 radiologic patterns

1) Similar to but larger than osteoid osteoma (>2cm)
Osteoblastoma of C3 spinous process
and lamina
2) Expansile lytic lesion with mineralization
3) More aggressive appearance marked bone destruction, scattered
calcification and soft tissue mass
Osteoblastoma: Radiology - Specific
Radiologic features are not always distinctive

Lytic lesion with varying bone production
Cortex thinned with expanded contour, may be destroyed, and have a soft
tissue mass
Mineralization may appear like chondroid tissue -arcs and rings but no
chondroid tissue pathologically
May have surrounding edema but less common than osteoid osteoma
Up to 16% secondary ABC (Aneurysmal Bone Cyst) component
Solid elements often low/intermediate signal T2 MR
Figure 4-5-23
Osteoblastoma: Radiology Spine [Figures 4-5-22 and 4-5-23]
Posterior elements alone most common (>60%)

Posterior elements extending into vertebral body (25%)
Vertebral body alone (<15%)
More likely to contain ossification and soft tissue mass
Less likely to elicit sclerosis
Scoliosis variably present less characteristic than osteoid osteoma
Osteoid Osteoma/Osteoblastoma
Differential Diagnosis: Spine
Osteoblastoma
Size > 1.52.0cm
Growth and soft tissue mass
Matrix - multifocal - noncentral
Scoliosis and classic symptoms absent
Pedicle sclerosis - lymphoma, mets, spondylolysis, congenital
absence/ hypoplasia posterior elements, malaligned apophyseal joints,
unusual infection
749
CT of C3 osteoblastoma with extensive

mineralization
Osteoblastoma Radiology Long Bones
Figure 4-5-24
[Figure 4-5-24]
Usually eccentric, metaphyseal (25%)

or diaphyseal (75%)
Intramedullary or cortical, rarely subperiosteal
Solid periostitis (50%)
May appear as a blister lesion in hand or foot, may
also have osteoporosis
Correct diagnosis usually not suggested
prospectively
Osteoblastoma: Natural History
The lesion usually grows slowly

Treatment is curettage or excision
Recurrence rate is 10%15%
Aggressive Malignant Osteoblastoma
Osteoblastoma of proximal femur with calcification on CT
Initially described 1967- Mayer

Considerable controversy and definition not clearly established
Problems in distinction from osteoblastoma like osteosarcoma and rare reports
of osteoblastoma recurring as osteosarcoma
Aggressive Osteoblastoma: Pathology
Figure 4-5-25
Similar to conventional osteoblastoma

Wider more irregular trabeculae
Lace-like osteoid
Variable mitoses but no atypical figures
Epithelioid osteoblast
Aggressive Osteoblastoma:
Clinical and Radiologic Characteristics
[Figure 4-5-25]
Older patients average 33 years of age

Similar locations
Larger more aggressive on radiologic examination
with soft tissue mass
Local recurrence rate up to 50%
Usually no metastases
Aggressive osteoblastoma with large soft tissue mass (arrow)

and multilevel involvement
Osteosarcoma: Definitions
A mesenchymal malignancy that differentiates to produce osteoid

...If only 1% of a tumor manifests osteoid and/or bone production by
malignant cells, it is by general convention an osteosarcoma
No matter how meager the osseous component
Only true for intraosseous neoplasm
Mirra Bone Tumors. Lee & Febiger 1989
Osteosarcoma is the second most common primary malignant

bone tumor - 15% of all biopsied primary bone tumors
Osteosarcoma (OS): Additional Definitions
Primary OS: lesion in absence of a benign precursor lesion or treatment

Secondary OS: lesion that has a benign precursor or is metastatic from
primary OS
Synchronous OS: lesions discovered within 6 months of each other
Metachronous OS: lesions discovered more than 6 months apart
Osteosarcoma: Pathology-General
Osteoid and/or immature bone production by tumor cells

Malignant stromal cells graded on their degree of anaplasia I-IV
750
Osteosarcoma: Histologic Pattern
Figure 4-5-26
Types
Osteoblastic (mixed and sclerosing) 82%
Fibroblastic
(Fibrosarcoma and MFH like)
7%
Chondroblastic
5%
Telangiectatic
3%
Small Cell
1%
Other
2%
Symptoms usually pain and swelling
Primary Osteosarcoma: Classification
Intramedullary-high grade
75%
Juxtacortical
7%-10%
Gnathic
6%
Low grade sclerosing
4% - 5%
Soft tissue
4%
Osteosarcomatosis - multifocal 1% - 2%
Intracortical
0.2%
Intramedullary osteosarcoma with osteoid matrix (arrows) and

aggressive bone lysis (arrowheads)
Secondary Osteosarcoma: Classification
Paget disease (67% - 90%)

Figure
Radiation induced (6% - 22%)
Osteonecrosis
Others - fibrous dysplasia, prosthesis, osteogenesis imperfecta, chronic
osteomyelitis, retinoblastoma
About half of osteosarcoma over age 50 are secondary, 67% over age 60
4-5-27
Osteosarcoma: Primary, High-Grade, Intramedullary
About 75% of all osteosarcomas

Most patients are between 15 and 25 years, rare younger than 6 or older than
60 years
Male > Females 1.52:1
About 70% are in the long bones, more than 50% about the bones of the knee
90% are metaphyseal, 5%10% diaphyseal
Osteosarcoma Intramedullary: Radiology

[Figures 4-5-26 to 4-5-29]
Usually mixed sclerosis and lysis

Soft tissue mass (80%)
Periosteal reaction (80%) - Codman triangle, lamination,
perpendicular (sunburst, hair on end)
Osteoid matrix fluffy/ cloud-like (90%)
Extend across epiphyseal plate
(75% - 90%)
MRI/CT essential for staging and preoperative
planning
Bone scan of intramedullary osteosarcoma of

tibia with intense radionuclide uptake. The
femur and ankle also show increased uptake
(arrowheads) from hypermia and disuse
Figure 4-5-28
Osteosarcoma Telangiectatic
Tumor largely composed of cystic cavities containing

necrosis and hemorrhage ( > 90%)
ABC like misdiagnosed on radiographs
Distribution similar to other intramedullary
osteosarcomas
Femur, tibia, humerus
Metaphyseal (90%), diaphyseal (10%)
Coronal STIR MR image and gross specimen of tibial

intramedullary osteosarcoma show identical lesion extent (*)
751
Osteosarcoma Telangiectatic
Figure 4-5-29
[Figures 4-5-30 to 4-5-33]
Largely osteolytic and expansile

Look for small areas of osteoid (58% X-rays, 85%
CT)
Fluid-fluid levels (CT 48% / MRI 89%)
Pathologic fracture (25% - 61%)
Donut sign on bone scan (65%)
Previously worse prognosis, now may actually be
better than other intramedullary osteosarcomas
(68% 5 year survival)
Juxtacortical Osteosarcoma
Parosteal
(65%)
Periosteal
(25%)
High grade surface
(10%)
Prognosis varies with grade and extent
Intramedullary osteosarcoma with skip metastases (*) on
coronal STIR MR image and gross specimen with distal
primary lesion (arrow) and intervening normal marrow (M)
Figure 4-5-30
Figure 4-5-31
Telangiectatic osteosarcoma of
scapula with thick peripheral
mineralization (arrow)
Telangiectatic osteosarcoma of scapula with thick enhancing

nodular wall containing calcification (arrowheads) and central
hemorrhage/necrosis (*) on CT
Figure 4-5-33
Figure 4-5-32
Telangiectatic osteosarcoma of distal femur on sagittal T1weighted post-contrast MR image and gross specimen showing
thick nodular enhancement peripherally in viable tumor
(arrowheads) correlating to gross specimen with central
nonenhancing cystic/necrotic regions (*)
Gross specimen of scapular telangiectatic

osteosarcoma with cystic spaces (*) surrounded
by solid viable tissue (arrowheads)
752
Parosteal Osteosarcoma: Clinical and Pathology
Figure 4-5-34
Arise from outer layer of periosteum

Usually a low grade tumor fibroblastic stroma and
streamers of woven bone
Patients about a decade older than intramedullary
osteosarcoma; F>M
Location - femur (65%), humerus (15%), tibia (10%),
fibula (3%), forearm (3%)
Most common posterior distal femur metaphysis
Parosteal Osteosarcoma: Radiology

[Figures 4-5-34 to 4-5-37]
Initially an exophytic sclerotic mass

Cauliflower-like with lucent cleavage plane between
lesion and cortex
Radiodense centrally with growth may reattach to cortex
and obliterate cleavage plane
MRI/CT to evaluate intramedullary invasion important for
surgical resection
Long term survival 80%90%
Periosteal Osteosarcoma: Clinical and Pathology
Parosteal osteosarcoma with specimen radiograph

and gross specimen showing surface mass with
central dense stalk of attachment to the cortex (*)
and lucent cleavage plane (arrows)
Periosteal sarcoma is usually chondroblastic (>90% of

tumor) and intermediate grade
Arise from inner layer of periosteum
More than 85% are in the diaphysis of the femur and tibia; ulna and humerus
(10%)
Similar age to conventional osteosarcoma and sex distribution (M>F)
Better prognosis but 15% rate of metastasis
Figure 4-5-35
Figure 4-5-36
Figure 4-5-.37
Parosteal osteosarcoma of the

distal femoral metaphysis with
radiograph, CT and gross
specimen and specimen
radiograph showing surface
mass (*), lucent cleavage plane
(arrows) and medullary
backgrowth (arrowhead)
Parosteal osteosarcoma of the distal femoral metaphysis with

radiograph, CT and gross specimen and specimen radiograph
showing surface mass (*), lucent cleavage plane (arrows) and
medullary backgrowth (arrowhead)
753
Periosteal Osteosarcoma: Radiology
Figure 4-5-38
[Figure 4-5-38]
Saucerized cortex with chondroblastic soft tissue

mass on imaging in area of erosion
Cortical thickening at margins of erosion (40%)
May have Codman triangle
Spiculated periosteal reaction
Only rarely intramedullary invasion reported
Osteosarcoma: High - Grade Surface

[Figures 4-5-39 and 4-5-40]
Histology high-grade similar to a conventional

osteosarcoma as is prognosis with same potential for
metastasis
Sites femur (45%), humerus (26%), fibula (10%),
ulna (6%)
Radiologic changes: broad based lesion with osteoid
arising on osseous surface
Similar appearance to periosteal osteosarcoma but
often more aggressive
Periosteal osteosarcoma or radiograph and coronal CT

reconstruction with diaphyseal broad based soft tissue
mass causing erosion of uderlying thickened cortex and
hair-on-end periosteal rection
Figure 4-5-39
Figure 4-5-40
High-grade surface osteosarcoma
Gnathic Osteosarcoma [Figure 4-5-41]
About 6% of all osteosarcomas

Patients are usually older
Lesions are usually lower grade
About half are chondroblastic
Prognosis is better dont tend to metastasize but
locally invasion
High-grade surface osteosarcoma (*) on sagittal T1-weighted

MR and sagittally sectioned gross specimen. Note the surface
location and sparing of the medullary canal (M)
Figure 4-5-41
Osteosarcoma: Low Grade Intramedullary
Described in 1977 by Unni (27 cases)

Also called sclerosing osteosarcoma being recognized with
increasing frequency
Patients average about a decade older than conventional
osteosarcoma
Most patients present with pain (85%), or swelling (15%), 5%
are incidental findings
Four histologic patterns simulating FD (50%), NOF (25%),
chondroblastoma (15%), CMF (10%)
754
Gnathic osteosarcoma with an aggressive

mixed lytic and sclerotic (arrows) lesion
destroying the alveolar ridge (arrowhead)
Osteosarcoma: Low Grade Intramedullary
Figure 4-5-42
[Figures 4-5-42 and 4-5-43]
Not uncommonly metaepiphyseal

Location same as intramedullary conventional
Central sclerosis with expansile remodeling
Ground glass density and trabeculation within
Not as aggressive appearance on radiographs
and lack soft tissue mass
ISS 2003 (Skel Rad 2004, 33:373-379)
MR/CT all 17 cases had a soft tissue mass
Better prognosis with 10% or less metastatic rate
Osteosarcoma: Soft Tissue

(Extraskeletal)
Rare same histology

Middle aged to older patients (mean 55 years)
Location-deep soft tissues of extremities-thighs
and shoulders also retroperitoneum
Not uncommonly history of trauma (10%15%)
Relationship to myositis ossificans?
Radiology soft tissue mass with calcification or
ossification
Low-grade intramedullary osteosarcoma

simulating fibrous dyplasia on radiograph and CT.
Note soft tissue mass medially (*)
Osteosarcomatosis:
Multifocal Osteosarcoma [Figures 4-5-44 and 4-5-45]
Figure 4-5-43
Rare
Classified into types in 1969 by Amstutz:
1-Synchronous, young patients < 18 years of age
2-Synchronous, adults
3-Metachronous
Type 3 likely represents metastatic disease
Types 1 and 2 usually demonstrate a radiologically dominant lesion
Amstutz. Cancer 1969;24:923
Low-grade intramedullary
osteosarcoma simulating fibrous
dyplasia on radiograph and CT.
Note soft tissue mass medially (*)
Figure 4-5-44
Figure 4-5-45
Osteosarcomatosis with multifocal areas of metaohyseal

sclerosis (*)
with primary dominant sclerotic focus in the tibia ( *)
[Figure 4-5-45]
755
Osteosarcoma : Intracortical
Figure 4-5-46
[Figure 4-5-46]
Very rare, only a handful of cases

Almost all in the femur and tibia
diaphysis
The lesion is dominantly or exclusively
intracortical with no
(or only minimal) intramedullary
involvement
Usually lytic/surrounding sclerosis
Intracortical osteosarcoma with matrix

mineralization and location within the
cortex (arrow) on radiograph and CT
Osteosarcoma: Treatment and

Prognosis
Complete removal of the primary tumor

limb salvage
Preoperative chemotherapy look for >
90% tumor necrosis most important
predictor of prognosis (90% 5 year
survival; 14% <90% necrosis)
Post operative chemotherapy
Overall 5 year survival 41%64%
5 year survival 60%-70% no metastases
at presentation and surgical resection
References
1.
2.
3.
4.
5.
6.
Greenspan A, Stadalnik RC.. Bone island: scintigraphic findings and their clinical application. Can Assoc Radiol J. 1995
Oct;46(5):368-79.
Judkiewicz AM, Murphey MD, Resnik CS, Newberg AH, Temple HT, Smith WS. Advanced imaging of melorheostosis
with emphasis on MRI. Skeletal Radiol. 2001 Aug;30(8):447-53.
Klein MH, Shankman S. Osteoid osteoma: radiologic and pathologic correlation.
Skeletal Radiol. 1992;21(1):23-31. Review.
Kroon HM, Schurmans J. Osteoblastoma: clinical and radiologic findings in 98 new cases. Radiology. 1990
Jun;175(3):783-90.
Murphey MD, Robbin MR, McRae GA, Flemming DJ, Temple HT, Kransdorf MJ. The many faces of osteosarcoma.
Radiographics. 1997 Sep-Oct;17(5):1205-31.
Sundaram M, Falbo S, McDonald D, Janney C. Surface osteomas of the appendicular skeleton. AJR Am J Roentgenol.
1996 Dec;167(6):1529-33.
756
Cartilaginous Lesions of Bone

Mark D. Murphey, MD
Figure 4-6-1
Cartilaginous Lesions
Osteochondroma
Enchondroma
Juxtacortical chondroma
Chondromyxoid fibroma (CMF)
Chondroblastoma
Chondrosarcoma
Osteochondroma
The most common benign neoplasm of bone that

leads to biopsy
Osteochondroma: Types
Osteochondromas with marrow (*) and cortical (arrows)

continuity on radiography and histology. Note undertubulation
on macrosection in patent with hereditary multiple exostoses
(HME) with other small lesions identified by
hyaline cartilage caps (arrowheads)
Solitary osteocartilaginous exostosis

Hereditary multiple exostoses (HME)
Diaphyseal aclasis
Multiple osteochondromas
Osteochondromatosis
Osteochondroma: Radiographic Subtypes
Figure 4-6-2
Pedunculated
Sessile
Osteochondroma: Clinical Data
No sex predilection
Young patients - 75% < 20 years old
Present as a mass: responsible for symptoms
Mechanical, cosmetic, affect on adjacent
structures (tendon, muscles, nerve, vessel),
fracture
Location: femur (30%), tibia (20%), humerus
(20%), hand and foot (10%), pelvis (5%), scapula
(4%)
Symptoms dependent on size/location
Bursa formation
Solitary < 1%
Treatment-Individualized-Resection
Dependent on symptoms/size/location
Sessile and pedunculated osteochondromas with marrow (*)

and cortical (arrows) continuity
Figure 4-6-3
Osteochondroma: Pathology
Medullary and cortical continuity with underlying

bone
Hyaline cartilage cap
Cartilage cap involutes after growth (skeletal
maturity)
Only benign skeletal neoplasm associated with
radiation
Can be induced by implanting epiphyseal tissue
Traumatic osteochondroma
Pelvic osteochondroma revealing only sclerosis on radiograph
with cortical and medullary continuity revealed on CT (arrow)
and gross specimen (*)
757
Osteochondroma: Imaging [Figures 4-6-1 to 4-6-3]
Mature bone-cortex and marrow

Hyaline cartilage cap-calcification
Cortical and marrow continuity with underlying bone
Long bones radiographs to diagnose
Metaphyseal
Grows away from epiphysis
May be associated with failure of tubulation (particularly
HME)
Flat bones often need CT/MR to diagnose
Tend to be larger and sessile
More variable appearance
Osteochondroma
Figure 4-6-4
Osteochondroma on CT with marrow and

cortical continuity
Figure 4-6-5
The cartilage cap deserves the most consideration in

radiologic interpretation
Osteochondroma: Cartilage Cap

[Figures 4-6-4 to 4-6-9]
Radiographs - chondroid calcification

Increasing destruction or change in appearance
Worrisome for malignancy
Ultrasound - good for cap and bursae
CT - soft tissue with calcification
Can be difficult to distinguish from muscle
BS - increased uptake
MRI - Intermediate signal T1W images
High signal T2W images
Cap thickness - benign vs malignant
Benign < 1.5 cm (0.1- 3.0 cm; ave. 0.6-0.9 cm)
Malignant > 1.5 cm (1.5 -12 cm; ave. 6 cm)
Depends on skeletal maturity
Osteochondroma on radiograph and sagittal T1weighted and axial T2-weighted MR images with thin
cartilage cap showing high signal on long TR MR
(arrowhead)
Figure 4-6-8
Figure 4-6-6
Gross specimen and macrosection of resected

osteochondroma with thin bluish cartilage cap (*) correlating
with the imaging (same patient as 4-6-5)
Axial T1-weighted MR images show cortical and marrow

((arrowheads) continuity and thick cartilage cap (*) (15 year old
boy; same patient as previous radiographs)
Figure 4-6-7
12 years old
Figure 4-6-9
15 years old
Sagittal STIR MR and gross specimen of resected

osteochondroma with high signal, thick cartilage cap (15 year
old boy with thick cartilage cap simulating malignancy but only
represented growth due to young age; same patient as
previous radiographs and axial T1-weighted MR)
Lateral ankle radiographs at 3 year interval shows growth

of the osteochondroma in this 12 year old boy. The same
radiographic appearance would represent malignant
transformation in an adult
758
Subungual Exostosis: Dupuytren Exostosis

[Figure 4-6-10]
Figure 4-6-11
Osteochondroma variant
Females > Males (2:1)
Often painful and associated with trauma and infection
Great toe (77%-80%); Fingers (10%-14%)
Fibrocartilage cap
Located away from physis
Figure 4-6-10
Dysplasia epiphysealis hemimelica

(Trevor disease) with early genu varus
deformity caused by the epiphyseal
osteochondroma (arrow)
Subungual exostosis (arrow)

with clinical photograph
Figure 4-6-12
Dysplasia Epiphysealis
Hemimelica: Trevor Disease
[Figures 4-6-11 to 4-6-13]
Male predominance (3:1)

Rare
Swelling, pain and deformity
Usually lower extremity, unilateral
65% multiple bone involvement talus, distal femur,
tibia
Ankle and knee most common
Medial joint 2x lateral
Lobular epiphyseal mass
Histologically identical to an osteochondroma
May produce deformity and secondary
osteoarthritis
Figure 4-6-13
Dysplasia epiphysealis hemimelica (Trevor disease) with the

epiphyseal osteochondroma (*) arising from the posterior
femur on lateral radiograph, and sagittal T2-weighted MR and
coronal 3D CT reconstruction
Dysplasia epiphysealis hemimelica (Trevor

disease) with the epiphyseal osteochondroma
(*) arising from the posterior femur on lateral
radiograph, sagittal T2-weighted MR and
coronal 3D CT reconstruction
(same patient as 4-6-12)
759
Hereditary Multiple Exostoses: Clinical Data
Figure 4-6-14
[Figures 4-6-14 to 4-6-16]
Male predominance (3:1)

Autosomal dominant inheritance
Variability in size and number
Any portion of the skeleton preformed in cartilage may be
involved
Present in childhood
May be bilaterally symmetric
One side may predominate
Increased incidence of malignant transformation
(10%20%)
Newer literature 3%5%
Figure 4-6-15
Hereditary multiple exostoses (HME) with associated

undertubulation of bone (Erlenmeyer flask deformity)
Figure 4-6-16
HME with typical pelvic and proximal femoral deformity
Enchondroma: The most common tumor

encountered in the phalanx
Enchondroma: Types
Solitary enchondroma
Multiple enchondromatosis
Ollier disease
Maffucci syndrome
Enchondroma: Clinical Data
HME with typical pelvic and proximal femoral deformity. Bone
scan shows a left pelvic lesion to reveal more intense

3%5% all biopsied primary bone lesions; 1% all
radionuclide uptake(*) and this area demonstartes a very thick
bone tumors
hyaline cartilage cap (>3 cm) and soft tissue mass with
No sex predilection
chondroid mineralization (rings and arcs) on radiograph, CT
Peak incidence 3rd decade (1030 years old)
and T1/T2 weighted MR images (arrows) resulting from
Hands and feet (40%65%), long tubular bones
malignant transformation to chondrosarcoma. This is also
shown on the gross specimen
(25%)
Phalanges and metacarpals most common locations
May be incidental finding or present with pathologic fracture
Enchondroma: Pathology
Rests of hyaline cartilage

Hyaline cartilage often with myxoid areas
Variable amorphous calcification and enchondral ossification
May cause expansile remodeling and cortical thinning
760
Enchondroma: Imaging [Figures 4-6-17 to 4-6-19]
Figure 4-6-17
Geographic lytic lesion IA-IB

Central often metaphyseal
Expansile remodeling with prominent thinned
cortex (short tubular bones only)
Chondroid matrix in majority 17% limited or none
(radiographs); all by CT
MRI Lobulated margin
Marked increase intensity T2W images
Calcified chondroid-low intensity
Figure 4-6-18
Enchondroma of the phalanx with typical ring and arc

chondroid mineralization and deep endosteal scalloping
Figure 4-6-19
Enchondroma of tibia on coronal T1-weighted (left) and

T2-weighted (right) MR images. Note lobular margin
and no endosteal scalloping and high signal on long TR
image resulting from high water content of
nonmineralized hyaline cartilage
Multiple Enchondromatosis: Clinical Data
Variable severity
May be predominantly unilateral
(Ollier disease-1899)
May become stable at puberty
Increase malignant transformation to
chondrosarcoma (5%50%)
Marked skeletal deformity
Not hereditary
Mild male predilection
Presents in childhood
Enchondroma of the distal femur on radiograph, sagittal T1weighted and T2-weighted MR images and histology. Note
lobular margin (arrows), ring and arc mineralization
(arrowhead), no endosteal scalloping and high signal on long
TR image resulting from high water content of hyaline cartilage
Maffucci Syndrome
Described in 1881 by Maffucci as enchondromatosis with hemangiomas
Figure 4-6-20
Maffucci Syndrome: Clinical Data
Multiple enchondromas
Cavernous hemangiomas
Very rare; nonhereditary
Mild male predilection
Hands and feet greatest involvement
Complications of hemangiomas
Chondrosarcoma (15%20%)
Vascular sarcoma (3%5%)
Ovarian malignancy
Glioma and carcinoma
Enchondromatosis on radiograph and coronal T2* (GRE)

MR image which show diagnostic columns of cartilage
extending into metaphysis from epiphyseal plate (arrows)
761
Maffucci and Ollier Syndromes: Imaging Characteristics

[Figures 4-6-20 and 4-6-21]
Figure 4-6-21
Lesions seen in infancy

Lytic columns from epiphyseal plate extending into
the metaphysis
Later typical enchondromata
Geographic IA-IB margin with expansion
Chondroid matrix calcification
Growth disturbance and bowing
Enchondroma growth slows after plate closure
Soft tissue masses with phleboliths
Chondrosarcomatous transformation
New bone destruction with ST mass
New periosteal reaction
Disorganized or destroyed matrix calcification
Juxtacortical Chondroma
Arise adjacent to cortex beneath periosteum

Metaphyseal
Proximal humerus (50%), femur and tibia also
hands and feet (25%)
< 30 years old, M > F (2:1)
Often more cellular than enchondroma
Juxtacortical Chondroma: Imaging

[Figure 4-6-22]
Cortical saucerization (13 cm)

Variable sclerosis/periosteal reaction
Soft tissue mass with chondroid calcification
(50%)
High intensity T2W MR images
Difficult to differentiate chondrosarcoma
Enchondromatosis on bone scan and extensive deformity of

the upper extremity on radiograph. Note predominance on one
side of the body on bone scan. MR, CT and gross specimen
reveal malignant transformation to chondrosarcoma with small
associated soft tissue mass laterally (arrows)
Figure 4-6-22
Juxtacortical chondroma with extrinsic erosion or

saucerization (arrowheads) of metacarpal
Chondromyxoid Fibroma (CMF): Fibromyxoid Chondroma
Rare, least common cartilage tumor

Usually lower extremity
55% around knee, 20%25% in foot
Young adults, 60% < 30 years old
Rarely malignant transformation
762
CMF: Pathology
Figure 4-6-23
Myxoid, fibrous and chondroid tissue in various

proportions
Myxoid areas-central in lobules
Cellular areas peripheral in lobules
Foci of calcification 5%27%
Lobulated tumor mass
CMF: Radiology [Figure 4-6-23]
Geographic lytic lesion IA -IC

Eccentric metaphyseal location; often cortical (long
bone lesions)
Expansile remodeling simulate cortical permeation
Flat/short tubular bone lesions central
Rare matrix mineralization (CT/tomography)
MRI similar to slightly lower intensity than muscle
T1W images
Very high signal intensity T2W images
Chondroblastoma: Codman Tumor - History
Cartilage containing GCT

Kolodney 1927
Calcifying GCT
Ewing 1928
Epiphyseal chondromatous GCT
Codman 1931
Chondromyxoid fibroma with intracortical location in the

tibia and outer margin appearing aggressive on
radiograph (arrowhead) but intact on CT (arrow) and low
attenuation (*) resulting from high water content hyaline
cartilage (note the lack of matrix mineralization)
Figure 4-6-24
Chondroblastoma: Clinical Data
Uncommon; 1%2% all bone tumors

Male > Female (2:1)
Children and young adults; 90% between ages 5
and 25 years
Chondroblastoma: Location
Epiphysis/apophysis only 40%

Epiphysis and metaphysis 55%
Metaphysis only 4%
Epiphyseal/Apophyseal Lesions:
Chondroblastoma centered in the epiphysis but extending into

the metaphysis, matrix mineralization and periosteal reaction
extending into the diaphysis (arrowhead) is also seen
Chondroblastoma
GCT
Subchondral cyst/intraosseous ganglion
Infection
Clear cell chondrosarcoma
Figure 4-6-25
Chondroblastoma: Location
Proximal femur
Head and neck
Trochanter
Distal femur
Proximal tibia
Proximal humerus
Hands and feet
23%
16%
7%
20%
17%
17%
10%
Chondroblastoma on coronal T1-weighted and T2-weighted

MR image with the lesion showing low to intermediate signal
intensity on long TR image (arrowheads) and extensive
surrounding marrow edema (*)
(same patient as previous image)
763
Chondroblastoma: Histology
Chondroblasts - can be confused with

chondro/osteosarcoma
Multinucleated giant cells
Chondroid 1% - 15%
ABC component 5% - 15%
Initially cellular; later necrosis, fibrosis, maturation
Figure 4-6-26
Chondroblastoma: Imaging
[Figures 4-6-24 to 4-6-28]
Geographic lytic lesion IA / IB

Eccentric > central; rarely expansile
Calcified chondroid matrix 30% - 50%
Periosteal reaction 30% - 50%
Adjacent diaphysis/metaphysis
CT/MRI-fluid/fluid levels
MRI not typical chondroid characteristics
BEWARE!! - low/intermediate T2W ( 95%)
Extensive surrounding edema
Joint effusion (30% - 50%)
Chondroblastoma of greater trochanter (apophysis =

epiphyseal equivalent) on coronal T2-weighted MR image
with typical low signal intensity lesion (arrow)
and surrounding edema (*)
Figure 4-6-27
Chondroblastoma: Treatment
Curettage and cryosurgery or en bloc resection

and bone graft
Radiofrequency ablation
Local recurrence 5%-10%
Malignant chondroblastoma very rare
Chondrosarcoma Types: Primary
Intramedullary
Periosteal/juxtacortical
Clear Cell
Mesenchymal
Myxoid
Extraskeletal
Dedifferentiated
Chondrosarcoma Types: Secondary
Enchondroma
Osteochondroma
Paget Disease
Radiation induced
Miscellaneous
Figure 4-6-28
Chondroblastoma with ABC component in the patella
presenting as a pathologic fracture. Radiograph and sagitally
sectioned gross specimen and macrosection show the lytic
expansile lesion with fracture, largely composed of cystic areas
(*) and small solid component of chondroblastoma inferiorly
(arrow)
Chondroblastoma with ABC component in the greater

trochanter. Multiple MR image show the lesion largely
composed of cystic areas (*) and small solid component of
chondrobstoma medially (arrows)
764
Chondrosarcoma: Pathology
Figure 4-6-29
Malignant tumor of cartilage often with myxoid

changes
Grades I (30%), II (40%), III (30%)
Grade I difficult to differentiate from benign
Diagnosis based on histologic and growth
features, symptoms and tumor size/location
Intramedullary Chondrosarcoma:
Clinical Data
Symptoms pain (95%-99%) and mass (82%)

Male > Female (3:2)
Average age 4045 years; metaphysis
Location femur (25%), pelvis (30%), shoulder
(15%), ribs/sternum (10%), vertebrae (7%),
scapula (5%)
8%-17% all biopsied primary bone tumors
Intramedullary Chondrosarcoma: Imaging

[Figures 4-6-29 to 4-6-37]
Geographic IA -IC to permeative

Often predominantly sclerotic
Deep endosteal scalloping
Cortical thickening/periosteal reaction
Expansile remodeling
Soft tissue mass (20% - 76%)
Chondroid matrix (78% by X-ray; 94% by CT)
CT/conventional tomography if matrix subtle
MRI - similar to muscle T1W images
Lobulated high intensity T2W images
Matrix calcification low intensity
Peripheral/Septal contrast enhancement
Low grade chondrosarcoma of the humerus with typical

features on multiple imaging modalities. Radiograph shows
typical ring and arc mineralization of a chondroid lesion (white
arrows) with deep endosteal scallop (black arrow).
Bone scan reveals marked increased radionuclide uptake
Figure 4-6-30
Low grade chondrosarcoma of the humerus with typical features

on multiple imaging modalities. CT and axial MR images show
matrix mineralization on the CT (white arrows) and typical septal
and peripheral enhancement of cartilage lesions on the post
contrast MR (white arrows)
(same patient as previous and next images)
Figure 4-6-31
Figure 4-6-32
Low grade chondrosarcoma of the humerus with typical

features on multiple imaging modalities. Coronal T2-weighted
MR and coronally sectioned gross specimen reveal marrow
replacement (large white arrows) and deep scalloping with
early extension into the soft tissues (small white arrows)
Intramedullary
chondrosarcoma of femur with
chondroid mineralization
superiorly (arrow) and deep
area of scalloping laterally
(arrowhead)
765
Figure 4-6-33
Figure 4-6-34
Axial proton density MR images show cortical

breakthrough and soft tissue mass (arrowheads) in this
femoral intramedullary chondrosarcoma
(same patient as previous radiograph)
Acetabular intramedullary
chondrosarcoma shows subtle bone
destruction (arrow) and matrix
mineralization in this
complex area of anatomy
Figure 4-6-35
Figure 4-6-36
CT of acetabular intramedullary chondrosarcoma shows

matrix mineralization (arrowhead)
and large associated soft tissue mass (*)
Figure 4-6-37
Coronal T2-weighted MR image of acetabular intramedullary
chondrosarcoma shows large high signal intensity mass (*)
(same patient as previous CT)
Intramedullary chondrosarcoma of anterior rib on CT with low

attenuation mass and matrix mineralization (arrow)
766
Juxtacortical/Periosteal: Chondrosarcoma
[Figures 4-6-38 and 4-6-39]
Figure 4-6-38
Similar to juxtacortical chondroma

Periosteal lesion cortical erosion
Similar in appearance to periosteal OGS (but no
hair on end periosteal reaction)
Chondroid matrix calcification
Larger size than juxtacortical chondroma
(>34cm)
Intramedullary canal spared
Clear Cell Chondrosarcoma: Clinical Data
2% of chondrosarcomas
Slightly younger age
75%80% lesions proximal femur or humerus
Flat bones involved 10%
Propensity for epiphysis, > 90%
Better prognosis
Juxtacortical chondrosarcoma seen on multiple imaging

modalities. Radiograph, CT and axial T1-weighted MR show
the juxtacortical mass (M) with chondroid matrix mineralization
(rings and arcs). There is extrinsic erosion of the fibular cortex
(black arrows on radiograph/CT and curved arrow on MR) and
low attenuation of the nonmineralized components on CT (*)
Clear Cell Chondrosarcoma: Pathology
Clear cell chondrocytes

Osteoblastoma like osseous metaplasia
Areas of conventional chondrosarcoma 50%
Chondroblastoma like areas
Osteoclastic giant cells
Figure 4-6-39
Clear Cell Chondrosarcoma: Imaging

[Figures 4-6-40 and 4-6-41]
Geographic lysis IA to IC
Totally lytic (50%); Calcified chondroid matrix 33%
Rind of sclerosis (20%) simulates nonaggressive
lesion
Soft-tissue mass less common 10%
MRI often high signal T2W images but may have
areas of low signal as well
Figure 4-6-40
Juxtacortical chondrosarcoma seen on multiple imaging

modalities. Sagittal T2-weighted MR shows high signal
intensity of the mass (arrows and black M). The coronally
sectioned gross specimen reveals the lobular chondroid growth
(C), extrinsic erosion of the cortex (arrows), Normal marrow
space (white M) and the periosteal elevation (P)
(same patient as figure 4-6-38)
Figure 4-6-41
Clear cell chondrosarcoma of humerus extending to

subchondral region simulating a giant cell tumor
Clear cell chondrosarcoma with high signal intensity (unlike

giant cell tumor) on coronal STIR MR images
767
Mesenchymal Chondrosarcoma: Clinical Data
Less than 10% chondrosarcomas

Younger age averages 25 years
Males = Females
Osseous location femur (15%), ribs (15%), spine
(10%), craniofacial (20%), pelvis (10%)
Figure 4-6-42
Extraskeletal Chondrosarcoma:
Mesenchymal Type
Young patients 23 44 years

20%30% soft tissue
Location meninges and thigh
High grade malignancy
Mesenchymal cells with islands of cartilage
Large soft tissue masses may have chondroid
matrix calcification
Metastases lymph node, lung
Mesenchymal chondrosarcoma showing very aggressive bone

destruction with large soft tissue mass and chondroid matrix
mineralization (arrows) on radiograph and CT
Figure 4-6-43
Mesenchymal Chondrosarcoma: Pathology
Undifferentiated mesenchymal cells

Multifocal islands of malignant cartilage
Hemangiopericytoma like areas
Aggressive high grade lesions
Poor prognosis
Mesenchymal Chondrosarcoma: Imaging

[Figures 4-6-42 and 4-6-43]
Aggressive osseous destruction motheaten to

permeative
Chondroid matrix calcification less prominent small
foci (60%70%)
Soft tissue mass (near 100%)
Masses show lower water content (CT/MR) and
enhance diffusely; may see high flow vessels (MR)
Myxoid Chondrosarcoma
Figure 4-6-44
Figure 4-6-45
Rare in bone 12% chondrosarcomas

More aggressive radiologic appearance
Worse prognosis
Look for myxoid areas
Low attenuation or signal intensity T1W
images; may show areas of
hemorrhage
Very high signal intensity T2W images
Contrast enhancement Peripheral /
diffuse
Extraskeletal Chondrosarcoma:
Myxoid Type (Chordotic)
[Figures 4-6-44 and 4-6-45]
Mesenchymal chondrosarcoma on MR images shows marrow

replacement (M) and diffuse contrast enhancement as well as
small serpentine vessels (arrows). This is not the enhancement
pattern of conventional chondrosarcoma (peripheral/septal)
Myxoid chondrosarcoma of
proximal femur shows bone
destruction with intramedullary
chondroid mineralization
(arrowhead) and large posterior
soft tissue mass with marked low
attenuation (*)
Middle aged patients average age 50 years

Deep musculature tissue extremities
Thigh/Popliteal fossa (70%)
Low grade malignancy may recur late
Hemorrhage and myxoid areas can be seen with imaging
Typical chondroid regions radiographs
Metastases lymph node, lung
768
Axial T2-weighted MR image

reveals marked high signal
intensity resulting from very
high water content of myxoid
chondrosarcoma (same patient
as previous image)
Dedifferentiated Chondrosarcoma: Clinical Data
Older patients average 60 years

10%20% chondrosarcomas
Often associated with secondary chondrosarcoma
(> 50%)
Location femur (20%), humerus (15%), pelvis
(30%), ribs and scapula (12%)
Figure 4-6-46
Dedifferentiated Chondrosarcoma:
Pathology
Low grade chondrosarcoma

Small foci higher grade chondrosarcoma
Spindle cell component
MFH/ fibrosarcoma, osteosarcoma,
rhabdomyosarcoma, GCT
Collision of two tumors
Dedifferentiated Chondrosarcoma: Imaging

[Figures 4-6-46 and 4-6-47]
Dedifferentiated chondrosarcoma with radiographs showing

typical chondroid mineralization (rings and arcs-arrows). There
is anterior cortical destruction with a small soft tissue mass
(arrowheads)
Radiology emulates pathology: beware the dual

characteristic
One region chondrosarcoma
Second area aggressive bone destruction
Cortical permeation and soft tissue mass (70%)
Biopsy of anaplastic region confusing
Dedifferentiated component compared to chondroid component
Different intrinsic characteristics
Different contrast enhancement (diffuse)
Figure 4-6-47
Radiologic Differential of
Chondrosarcomatous Lesions
Aggressive chondroid lesion with soft tissue mass

Higher grade conventional chondrosarcoma
Dedifferentiated chondrosarcoma
Mesenchymal chondrosarcoma
Large fluid component bone or soft tissue
Myxoid chondrosarcoma
Change in appearance or foci of more aggressive
nature
Low-Grade Chondroid Lesion:

Enchondroma
Low-grade chondrosarcoma
Bone infarct
Dedifferentiated chondrosarcoma with post contrast fat

suppressed T1-weighted MR image showing typical peripheral
and septal enhancement in the cartilaginous portion of the
lesion (arrows) and diffuse enhancement in the dedifferentiated
anterior soft tissue component (*) correlating with the sagitally
sectioned gross specimen
Bone Infarct: Osteonecrosis [Figure: 4-6-48 and 4-6-49]
Ischemic area may undergo mineralization

Can have chondroid-like matrix
Look for peripheral rim of calcification
No cortical thickening
Prominent areas of endosteal scalloping or mass exclude osteonecrosis
Except malignant degeneration
Diagnostic Dilemma Long Bone:

Enchondroma vs. Chondrosarcoma
Enchondroma
Common in hand/foot
Rare in axial skeleton
Common in long bones (1.7% distal femur)
769
Figure 4-6-48
Chondrosarcoma
Common in axial skeleton
Common in long bones
Rare in hand/foot
Enchondroma vs. Low-Grade Chondrosarcoma:

Clinical Data
Pain (95%99%) and mass (20%76%) favor chondrosarcoma

Pain in enchondroma 40%
Often related to activity
Stress microfracture
Vague longer duration
Is pain referable to lesion?
Radiologic consultation
Enchondroma vs. Low-Grade Chondrosarcoma:

Pathology
Permeation of chondroid tissue

Permeation of cortex
Soft tissue mass
Fibrous bands separating cartilage
Invasion of marrow fat
Long Bone Enchondroma : Imaging
Size < 67cm (X-ray); < 5cm (CT/MRI)

Bone scan =/< AIC* 79% (70% homogeneous)
Majority in diaphysis
Endosteal scalloping depth < 2/3 cortex (90% - 95%)
No cortical thickening (17%); periosteal reaction (3%)
No cortical destruction/soft tissue mass
MRI peripheral enhancement?
Multiple areas of osteonecrosis with serpentine

peripheral calcification (arrows) and simulating
chondroid (ring and arc) mineralization
Murphey, Radiographics 98; 18: 1213 * *AIC = Anterior Iliac Crest
Figure 4-6-49
Long Bone Chondrosarcoma: Imaging
Majority in the metaphysis

Size > 67cm (X-ray); > 5cm (CT/MRI)
Bone scan =/ > AIC* 82% (63% heterogeneous)
Endosteal scalloping depth > 2/3 cortex (75% 90%)
Cortical thickening (47%); periosteal reaction
(51%)
MRI peripheral and septal enhancement?
CT of osteonecrosis shows peripheral rim of serpentine

calcification. Simulation of chondroid mineralization is an
artifact of radiographs in looking at a three dimensional
structure with a one dimensional image
Murphey, Radiographics 98; 18: 1213 * *AIC =

Anterior Iliac Crest
References
1.
2.
3.
4.
5.
6.
Bloem JL, Mulder JD. Radiol. 1985;14(1):1-9. Chondroblastoma: a clinical and radiological study of 104 cases.
Skeletal Radiol. 1985;14(1):1-9.
Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and
complications with radiologic-pathologic correlation. Radiographics. 2000 Sep-Oct;20(5):1407-34. Review.
Murphey MD, Flemming DJ, Boyea SR, Bojescul JA, Sweet DE, Temple HT. Enchondroma versus chondrosarcoma
in the appendicular skeleton: differentiating features. Radiographics. 1998 Sep-Oct;18(5):1213-37; quiz 1244-5.
Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH. From the archives of the AFIP:
imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 2003 Sep-Oct;23(5):124578. Review.
Robinson P, White LM, Sundaram M, Kandel R, Wunder J, McDonald DJ, Janney C, Bell RS. Periosteal chondroid
tumors: radiologic evaluation with pathologic correlation. AJR Am J Roentgenol. 2001 Nov;177(5):1183-8.
Wilson AJ, Kyriakos M, Ackerman LV. Chondromyxoid fibroma: radiographic appearance in 38 cases and in a review
of the literature. Radiology. 1991 May;179(2):513-8. Review. Erratum in: Radiology 1991 Aug;180(2):586.
770
Fibrous Lesions of the Musculoskeletal System

Mark D. Murphey, MD
Fibrous Lesions
Figure 4-7-1
Fibroxanthoma (Nonossifying fibroma)

Fibrous dysplasia
Osteofibrous dysplasia / Adamantinoma
Desmoplastic fibroma
Fibromatosis
Malignant fibrous fistiocytoma / fibrosarcoma
Dermatofibrosarcoma Protuberans (DFSP)
Fibroxanthoma: Other Terms
Fibrous cortical defect

Nonossifying fibroma (NOF)
Fibrous medullary defect
Nonosteogenic fibroma
Fibroxanthoma [Figures 4-7-1 and 4-7-2]
...If the lesion has attained a fairly large size and has penetrated into
and continues to grow in the medullary cavity, it ceases to be a mere
fibrous cortical defect and is then known as a nonossifying fibroma
(Jaffe 1958)
Fibroxanthoma
Very common 20% F, 50% M, older than 2 years of age

Children and adolescents; M > F
Usually asymptomatic: only 2% of biopsied primary bone tumors
Heal spontaneously with average life span 29 months
Typical nonossifying
fibroma/fibroxanthoma in the tibia
(arrow)
Figure 4-7-2
Fibroxanthoma: Pathology
Whorls/bundles of fibrous tissue

Variable cellularity
Giant cells
Foam or xanthoma cells
Areas hemorrhage/hemosiderin
Fibroxanthoma: Skeletal Location
Metaphyseal origin can migrate to diaphysis

Long tubular bones 90%
Lesions around knee 55%
Tibia 43%, femur 38%, fibula 8%
Upper extremity uncommon 8%, humerus 5%
Fibroxanthoma: Radiology [Figures 4-7-3 to 4-7-5]
Eccentric cortically based lesion

Longitudinal growth pattern
Can extend or primarily involve medullary cavity
Lobulated contour
Expansile remodeling with trabeculation
Cortex may appear permeated focally but no soft tissue mass
Usually a rim of sclerosis
Bone scan minimal to mild uptake
MR can be low or high intensity on T2W images
771
Nonossifying fibroma/fibroxanthoma
with healing by extensive ossification
(arrow)
Figure 4-7-3
Figure 4-7-5
Nonossifying fibroma/fibroxanthoma in the distal femur on radiograph, gross

specimen and histology
Figure 4-7-4
Nonossifying
fibroma/fibroxanthoma in the
fibula with medullary location
(arrow) as is typical for lesions
in this location
Figure 4-7-6
Nonossifying fibroma/fibroxanthoma in the distal femur on radiograph and

multiple MR images. Note intracortical location, heterogeneous signal intensity
on T2 with areas of high signal and enhancement
Interval growth of nonossifying

fibromas/fibroxanthomas and multiple lesions (arrows)
between two radiographs
Figure 4-7-7
Fibroxanthoma: Natural History
Often heal with residual sclerosis start from diaphyseal

side
May persist or grow
Pathologic fracture greater likelihood in lesions > 3
cm and with >50% bone width involved and weight
bearing bones
Fractures through three nonossifying

fibromas/fibroxanthomas
772
Fibroxanthoma: Types and Associations [Figure 4-7-8]
Figure 4-7-8
Multiple with neurofibromatosis type 1
Multiple with cafe-au-lait spots
Jaffe-Campanacci syndrome
Oncogenic osteomalacia
Benign Fibrous Histiocytoma [Figure 4-7-9]
Use of this terminology controversial

Patient often symptomatic
Radiographic appearance larger lesions, more
expansion, medullary involvement, older patient
Pathology identical to fibroxanthoma
Fibrous Dysplasia: Clinical

Characteristics
Multiple nonossifying fibromas/fibroxanthomas associated with

neurofibromatosis 1
Developmental anomaly of bone formation

Osteoblasts fail to develop
Marrow replaced by fibrosseous tissue
Usually diagnosed < age 30 but > age 2 years
Males and females equally affected
Monostotic (70%80%)
Polyostotic (15%30%)
Cafe-au-lait spots irregular serrated borders
(coast of Maine)
1% of biopsied primary bone tumors
Figure 4-7-9
Fibrous Dysplasia: Monostotic
Smaller sized lesions

Often asymptomatic
Cafe-au-lait spots less common
Distribution femur (35%40%), tibia (20%),
skull and facial bones (10%25%), ribs (10%)
Uncommon sites hands and feet, spine,
clavicle
Fibrous Dysplasia: Polyostotic
Larger lesions, symptomatic at earlier age

70% present before age 10 limp, pain, fracture or
deformity
Cafe-au-lait spots >50% patients
Involvement variable two to >75% skeleton; propensity to
involve one side of body more extensively
Common sites skull and facial bone (>50%), long bones,
ribs, pelvis
Fibrous Dysplasia Polyostotic: Associations
Endocrinopathies: 2%3% patients

McCune Albright syndrome bone lesions, cutaneous
pigmentation, precocious puberty 20%50% of females
(only 1 in 30 to 40 have complete triad)
Others hyperthyroidism, hyperparathyroidism,
acromegaly, diabetes m., Cushing syndrome
Soft tissue myxoma Mazabraud syndrome
Fibrous Dysplasia: Pathologic Characteristics
Benign fibrous histiocytoma in the tibia with prominent

sclerotic margin (unusual for GCT).
On MR, prominent low signal intensity on both pulse
sequences
This lesion is identical pathologically to nonossifying
fibroma/fibroxanthoma but patients are symptomatic
and older age at presentation
Fibrosseous metaplasia
Stroma may have cystic or myxoid elements
Trabeculae are pure woven bone with alphabet soup appearance
Occasional osteoblastic rimming and chondroid foci
May have ABC component
773
Fibrous Dysplasia
Radiology: Appendicular Skeleton [Figures 4-7-10 to 4-7-12]
Medullary diaphyseal lesions

Radiolucent with woven bone in marrow
creating ground glass appearance
Expansile remodeling
Usually well defined and may have sclerotic rind
(monostotic lesions)
May have multiloculated appearance caused
by subperiosteal reinforcement
Areas of sclerosis (most common in skull)
Skeletal deformity fracture, bowing
(Shepherds Crook), growth disturbance (more
common polyostotic disease)
Figure 4-7-10
Figure 4-7-11
Typical "ground glass" appearance in several patents with fibrous
dysplasia
Figure 4-7-12
Typical monostotic fibrous dysplasia in

intertrochanteric femur with thick rind of
sclerosis (arrow)
Fibrous dysplasia of rib on radiograph and macrosection showing

elongated involvement (*) of a prominent osseous extent
Fibrous Dysplasia Radiology:

Craniofacial Skeleton [Figures 4-7-13 to 4-7-15]
Commonly involved frontal, sphenoid, ethmoid, maxilla, zygoma, parietal,

occipital and temporal
Often mixed lucency and sclerosis
Sclerosis often marked at skull base can impinge on cranial nerves
Calvarium expanded with greater involvement
Figure 4-7-14
outer table
Figure 4-7-15
Figure 4-7-13
Fibrous dysplasia of the calvarium with skull base

sclerosis (*) and expansion of the occipital outer table
(arrow)
Coronal CT reconstruction of fibrous

dysplasia shows mixed lysis and
sclerosis and outer table expansion
774
Fibrous dysplasia on T2-weighted

MR with prominent low signal
intensity in the frontal bone with
expansile remodeling (*)
Fibrous Dysplasia Radiology: Other Studies
Figure 4-7-16
[Figures 4-7-16 and 4-7-17]
Bone scan usually increased activity probably more

variable than recognized
CT especially helpful in skull
MRI
20% low intensity T2W images
20% same as fat T2W images
60% high intensity T2W images
Figure 4-7-17a
Fibrous dysplasia of the humerus with typical intense

uptake of radionuclide bone scan
Figure 4-7-17b
Fibrous dysplasia of femoral diaphysis with

nonspecific marrow replacement (*) on coronal T1weighted MR image
Fibrous Dysplasia: Complications [Figure 4-7-18]
Malignant transformation 0.5%

Osteosarcoma most frequently but also
MFH/fibrosarcoma and chondrosarcoma
Both polyostotic and monostotic
Prior radiation in 30%
Fibrous dysplasia of femoral diaphysis with

nonspecific marrow replacement and high signal
intensity on coronal T2-weighted MR image (*)
(same patient as previous MR)
Fibrous Dysplasia: Differential Diagnosis
Bone cyst
Fibroxanthoma (medullary)
Meningioma
Osteoblastoma (long bone)
Enchondromatosis
Paget disease
Neurofibromatosis
Figure 4-7-18
Osteofibrous Dysplasia: Previous Terms
Cortical fibrous dysplasia

Intracortical fibrous dysplasia
Ossifying fibroma dont confuse with facial
lesion
Juvenile adamantinoma
Osteofibrous Dysplasia:
Unusual lesions 0.2% of biopsied primary bone

tumors
Patients <10 years age; rare after 16 years
Tibia alone (75% - 80%) or also fibula (12%); fibula
only (7%), both tibiae (3%), rarely radius/ulna
775
Monostotic fibrous dysplasia of the proximal femur with

malignant transformation to MFH on radiograph and gross
specimen. Note ground glass appearance distally (*) and more
aggressive bone destruction proximal with extension through
lesser trochanter proximally (arrows)
Osteofibrous Dysplasia: Pathology
Figure 4-7-19
Vascularized fibrous stroma like fibrous dysplasia

Prominent osteoblastic rimming
No alphabet soup of woven bone
Can be weakly keratin positive but no epithelial
nests
Osteofibrous Dysplasia: Radiology

[Figures 4-7-19 and 4-7-20]
Lytic lesion anterior cortex mid tibial diaphysis

May involve medullary canal
No soft tissue mass
Expansile remodeling and sclerotic component
Causes bowing, fracture, pseudarthrosis and may
progress to involve entire tibia
Homogeneous intermediate on T1 and high on T2
Homogeneous mild to moderate enhancement
Adamantinoma: Clinical Characteristics
Typical osteofibrous dysplasia with elongated/multifocal

intracortical tibial involvement on radiograph and matched
macrosection (*)
Figure 4-7-20
Present with pain/swelling; often history of trauma

Dont confuse with mandibular ameloblastoma
Rare low grade malignancy 0.1% biopsied
primary bone tumors
Also previously called angioblastoma
Male to female ratio 1.3:1, average age 35 years
Adamantinoma: Pathology and Location
Epithelial nests / prominent keratin staining

Background of bland fibrous stroma
May have foci of Ewing-like areas worse
prognosis
Tibia (80% - 85%), tibia and fibula (5%), femur
(5%), humerus (4%), ulna (3%), fibula (1%)
Typical osteofibrous dysplasia on MR imaging with elongated

intracortical tibial involvement and homogeneous intermediate
signa l intensity on T1-weighting and high signal on T2weighting (arrows).
Adamantinoma: Radiology
Diaphyseal to metadiaphysis anterior tibial cortex

Mixed lytic and sclerotic
May be multifocal with medullary involvement and soft tissue
mass
Expansile remodeling with cortical thickening
Figure 4-7-21
Adamantinoma:
Radiology and Prognosis [Figures 4-7-21 to 4-7-24]
MRI
Very heterogeneous high intensity T2W
Vascularity with prominent enhancement
Locally aggressive
10 year survival: 10% - 65%
15% patients die with metastases
Osteofibrous Dysplasia:
Relationship to Adamantinoma
Differentiation - patient age

Multiple recurrence
MRI heterogeneous, intense enhancement
Epithelial nests; both can be keratin positive
Several cases reported of foci of adamantinoma in osteofibrous
dysplasia and progression to adamantinoma
Adamantinoma of the tibia on radiograph with
mixed lytic and sclerotic lesion centered in the
cortex and an elongated lesion
776
Figure 4-7-22
Figure 4-7-23
Adamantinoma of the tibia on multiple MR images (same

patient as previous radiograph and CT) with elongated lesion
centered in the cortex (arrows). There is prominent
heterogeneity on STIR
Adamantinoma of the tibia on several CT images (same patient

as previous radiograph) with mixed lytic and sclerotic lesion
centered in the cortex (arrows)
Desmoplastic Fibroma: Clinical Characteristics
Rare fibrous lesion of bone

0.2% - 0.3% biopsied primary bone neoplasms
M=F or slight female predilection
70% between ages 15 and 40 years
Desmoid tumor of bone (510 times less common than soft
tissue lesion)
Figure 4-7-24
Desmoplastic Fibroma: Clinical Characteristics
Location femur, tibia, humerus, radius, mandible, pelvis

Metaphyseal central
Pain and swelling (90%)
Pathologic fracture (15%)
Desmoplastic Fibroma: Pathology
Intraoperative photograph of adamantinoma of

the tibia with intracortical curretage (same
patient as previous radiograph, MR and CT)
Figure 4-7-25
Histology identical to soft tissue desmoid

Gross - lobular firm white to gray mass
Fibroblasts producing well-formed collagen
Nuclear monotony, variable cellularity, rare
mitosis
Rarely associated with fibrous dysplasia
Desmoplastic Fibroma: Radiology

[Figure 4-7-25]
Lytic lesion with expansile remodeling

May have sclerotic margin
Internal trabeculae - subperiosteal reinforcement
May have more aggressive appearance
May be low intensity T2W MR images
Cortical Desmoid [Figures 4-7-26 and 4-7-27 overleaf]
Desmoplastic fibroma of the iliac bone with prominent

multilocular appearance caused by internal trabeculation
Avulsive cortical injury (chronic)

Posteromedial distal femur metaphysis
Stress related at attachment
Adductor magnus
Medial head gastrocnemius
Pathology simulates aggressive lesion
Children 1st decade (35%)
More frequent in boys, often bilateral
Surface irregularity/lucency
CT looks like NOF no soft tissue mass
MRI may see surrounding inflammation
777
Figure 4-7-26
Figure 4-7-27
Two patients with cortical desmoids (chronic avulsive injury) of

the distal femur (arrows)
Sagittal CT reconstruction showing medial head of
gastrocnemius muscle extending into cortical desmoid (arrow)
and macrosection of reparative tissue (*) in a different patient
Fibromatosis
Deep grow rapidly, larger, more aggressive

Superficial slow growing, small, arise from fascia/aponeurosis
Can be multifocal 5%-20%
Fibromatosis: Types
Extra-abdominal desmoid (deep)

Aggressive infantile fibromatosis (deep)
Juvenile aponeurotic fibroma (sup.)
Infantile dermal/digital fibromatosis (sup.)
Adult palmar and plantar (sup.)
Infantile myofibromatosis (both)
Figure 4-7-28
Fibromatosis: Pathology
Gross - glistening white, variable cellularity

Spindle shaped fibrous cells
Abundant collagen, can see mitoses
Infiltrative growth common
Post-contrast axial T1-weighted MR image showing

extraabdominal desmoid (fibromatosis) with an enhancing
paraspinal mass (*) and ill-defined margins
Fibromatosis: Radiology
Soft tissue mass, unusual to calcify

Can erode adjacent bone
CT-soft tissue mass may show attenuation greater than muscle
MRI
T1W image low / intermediate signal
T2W image variable signal
Fascial tail sign
Low signal bands
Enhance with contrast
Figure 4-7-29
Axial STIR MR image showing extraabdominal desmoid

(fibromatosis) with a soft tissue mass (*) and ill-defined margins
and linear extension (fascial tail sign) laterally (arrow). These
features are also shown on a gross specimen froma different
patient
778
Fibromatosis: Extraabdominal Desmoid [Figures 4-7-28 and 4-7-29]
Painless growing deep mass (2535 years)

Rarely familial
Shoulder (20%), chest wall/back (15%), thigh
(12%), mesentery (10%), neck (8%), knee (7%),
buttock (6%)
Intermuscular along fascia/aponeurosis, infiltrative
Figure 4-7-30
Fibromatosis: Aggressive Infantile

[Figures 4-7-30 to 4-7-32]
Painless soft tissue mass, M>F

Discovered first two years of life
Intermuscular head/neck, shoulder, thigh, foot
Can erode bone
Multifocal (10%-15%)
No metastasis but locally aggressive and recur
Fibromatosis:
Juvenile Aponeurotic Fibroma
Children/adolescents; M>F
Hands (77%), feet (13%) palms and soles
Painless slowly growing mass
Calcification and local recurrence (50%) common
Attached to tendon/aponeurosis
Aggressive infantile fibromatosis on sagittal T1-weighted MR

image with large mass eroding bone (*) which ultimately led to
amputation following multiple recurrences.Note low intensity
bands (arrowheads)
Figure 4-7-31
Fibromatosis: Infantile Dermal/Digital
Birth to age 2 years; F>M

Fingers > toes; dorsum, spare thumb/great toe
Bone erosion rare, can have contractures
Pathology intracellular inclusion bodies
Recurrence local 60%
Adult Palmar Fibromatosis
Palmar (Dupuytren contracture) 1%2%

population (1/5 people older than 65)
M>F (45:1); ulnar side, thumb and index finger
spared
Fibrous nodules - cords (40%60% bilateral)
Other fibromatosis 5%20%
Contractures/recurrence common
Figure 4-7-32
Same patient as previous image showing multicentric

involvement with second site (*) in lower calf demonstrating
high signal intensity on axial T2-weighted MR image. The
patient ultimately required amputation as shown on the gross
specimen with the large recurrent mass (*)
Axial T2-weighted MR image in patient with aggressive

infantile fibromatosis after radiation shows marked low signal
(arrows) resulting from collagenization following successful
nonoperative treatment
779
Adult Plantar Fibromatosis
Figure 4-7-33
Ledderhose disease
Less common than palmar
M>F (2:1); wider age range 55% < age 30
Starts as single nodule middle to medial sole
Often leads to early excision, contractures rare
Associated with palmar disease 5%20%
Infantile Myofibromatosis
Discovered at birth or within weeks

Solitary form (good prognosis)
Multifocal (poor prognosis) soft tissue, muscle,
viscera
Bone lesions common but involute
Lesions grow in perinatal period
Myoblastic and fibroblastic lesion
Osseous MFH with radiograph showing solitary geographic

lytic lesion with wide zone of transition (arrows)
Fibromatosis: Treatment and Prognosis
Surgical excision
Recurrence common
High signal on T2 corresponds to more cellular regions increasing
recurrence
Re-excision may use radiation therapy
Can follow with MRI
Can ultimately require amputation
Figure 4-7-34
Malignant Fibrous Histiocytoma (MFH) and Fibrosarcoma
Osseous
Soft tissue (S.T.)
Malignant Fibrous Histiocytoma: Pathology
Described 1964 Stout and coworkers

CT of osseous MFH shows no matrix
Three cell types present
mineralization and cortical penetration
Fibroblastic spindle cells
anteriorly with soft tissue mass (*)
Plump histiocytic cell (from marrow monocytes?)
(same patient as previous image)
Giant cells benign and malignant
Histologic typesstorioform (pleomorphic 50%60%), myxoid (25%), giant cell
(10%), inflammatory (10%), angiomatoid (5%)
No malignant matrix; diagnosis of exclusion
Figure 4-7-35
WHO 2002 Undifferentiated high grade
pleomorphic sarcoma (soft tissue lesions only)
Fibrosarcoma: Pathology
Malignant collagen producing spindle cells

Herringbone pattern lower grade lesions (I-II)
Higher grade (III-IV) lesions more anaplasia
No matrix or malignant giant cells
Osseous MFH and Fibrosarcoma: Clinical

Features
Age 4070 years

Pain, swelling and pathologic fracture (30%50%)
Slight male predilection
Metaphyseal around knee (40%80%), humerus
(10%), pelvis (10%)
Secondary lesions Paget disease, osteonecrosis,
fibrous dysplasia, chronic osteomyelitis
780
Coronal T1-weighted MR image and coronal gross specimen

shows distal femoral MFH (same patient as previous two
images) not extending to subchondral bone (arrowhead) (as
would be seen with giant cell tumor)
Osseous MFH and Fibrosarcoma: Radiology
Figure 4-7-36
[Figures 4-7-33 to 4-7-38]
Lytic lesions from geographic IB to

motheaten/permeative reflects tumor grade
With geographic IB - IC lesions GCT like; look for
diaphyseal > epiphyseal extension
Little periosteal reaction or sclerosis
May not show increased intensity on T2W MR
images
Soft Tissue MFH: Clinical Features
Most common adult S.T. sarcoma

Accounting for 15%30% all S.T. sarcomas
Age 5070 years: M>F 2:1
Location deep soft tissues lower extremity
(50%), upper extremity (20%), retroperitoneum
(15%), head and neck (5%)
Musculoskeletal Soft Tissue

Sarcoma Incidence
Osseous MFH showing aggressive solitary lytic lesion (arrows)

in supraacetabular region with lateral cortical destruction
difficult to detect
Figure 4-7-37
Figure 4-7-38
Malignant fibrous histiocytoma and

Fibrosarcoma 20%30%
Liposarcoma 16%19%
Rhabdomyosarcoma 10%19%
Nonspecific spindle cell sarcoma 5%15%
Leiomyosarcoma 5%10%
Dermatofibrosarcoma protuberans (DFSP)
5%10%
Synovial sarcoma 5%10%
Soft Tissue Fibrosarcoma: Clinical

Features
Palpable mass deep soft tissues

Lower extremity knee and thigh (45%); upper
extremity (28%), trunk (17%), head and neck
(10%)
Age 30 to 55 years, no sex predilection
Osseous MFH (same patient as

previous image) with
intraosseous lesion and large
associated soft tissue mass (*)
but no matrix mineralization
Axial T1-weighted MR image of

osseous MFH (same patient as
previous two images) shows
marrow replacement and
associated soft tissue mass (*)
Soft Tissue MFH and Fibrosarcoma: Radiology

[Figures 4-7-39 to 4-7-42]
Deep soft tissue mass MRI > CT for evaluating extent prior to surgery
MRI
Usually similar to muscle T1W images
High intensity T2W images
May not show increased intensity on T2W images
Pseudocapsule low intensity all sequences
Calcification/ossification: 5%20% MFH
May involve underlying bone
MFH hemorrhage high intensity T1W
images
Can differentiate myxoid lesions look like fluid
with nodular peripheral contrast enhancement
Figure 4-7-39
Soft tissue MFH with mass replacing vastus lateralis muscle (*) on
axial T1-weighted MR image [left]; with high signal intensity mass (*)
on axial T2-weighted MR image [right]
781
Figure 4-7-40
Figure 4-7-41
T1
Sagittal T1-weighted MR images before and after contrast

show enhancement of the solid component (arrowheads) of the
MFH and nonenhancing hemorrhagic areas (*)
Largely hemorrhagic (*) soft tissue MFH in

the anterior thigh on CT with the only solid
component adjacent to the anteromedial
femur (arrowhead)
Figure 4-7-42
Fibrosarcoma and MFH:

T1 GD
Treatment - wide local resection/amputation

Local recurrence common (50%)
follow up imaging
Metastasis (40%) common
hematogenous - lung, lymph nodes, liver and
bone
Dermatofibrosarcoma Protuberans
(DFSP): Clinical Features
6% all soft tissue tissue sarcomas

Third to fifth decades of life
Reddish brown to bluish superficial nodule
May be multiple
Most common to affect trunk (50%)
Remainder head/neck, upper/lower extremities
Soft tissue MFH with soft tissue mass (*) causing extrinsic
erosion of adjacent femur (arrowheads)
Dermatofibrosarcoma Protuberans (DFSP): Pathology
Uniform fibroblasts
Storiform pattern (may be myxoid)
May have areas of higher-grade sarcoma
Usually fibrosarcoma (17% - 27%)
Dermatofibrosarcoma Protuberans (DFSP):

Radiologic Characteristics [Figure 4-7-39]
Subcutaneous mass - no calcifications

Usually centered on skin and protuberant
Nonspecific solid intrinsic features CT/MRI
Enhance with contrast, ST attenuation
Intermediate T1; high signal T2
May have hemorrhage
Look for linear extension (skin/fascial tail sign)
Satellite nodules
782
Figure 4-7-43
T1
DFSP on sagital T1, axial STIR and gross

specimen showing protuberant subcutaneous
mass (*) involving the skin with linear extensions
along the skin surface (arrows)
Dermatofibrosarcoma Protuberans (DFSP):

Treatment and Prognosis
Surgical excision (wide with 3cm margin)

Local recurrence 20%-55% (within 3 years)
Higher with head/neck lesions (50%-75%)
Metastases
Lungs (5%-6%)
Lymph nodes up to 25% of metastases
Higher incidence with high-grade component (21%)
Fibrous Lesions References

1.
2.
3.
4.
5.
6.
7.
8.
Fitzpatrick KA, Taljanovic MS, Speer DP, Graham AR, Jacobson JA, Barnes GR, Hunter TB. Imaging findings of
fibrous dysplasia with histopathologic and intraoperative correlation. AJR Am J Roentgenol. 2004 Jun;182(6):138998. No abstract available.
Jee WH, Choe BY, Kang HS, Suh KJ, Suh JS, Ryu KN, Lee YS, Ok IY, Kim JM, Choi KH, Shinn KS. Nonossifying
fibroma: characteristics at MR imaging with pathologic correlation. Radiology. 1998 Oct;209(1):197-202.
Jee WH, Choi KH, Choe BY, Park JM, Shinn KS. Fibrous dysplasia: MR imaging characteristics with radiopathologic
correlation. AJR Am J Roentgenol. 1996 Dec;167(6):1523-7.
Murphey MD, Gross TM, Rosenthal HG. Musculoskeletal malignant fibrous histiocytoma: radiologic-pathologic
correlation. RadioGraphics 1994; 14:807-826.
Ritschl P, Karnel F, Hajek P. Fibrous metaphyseal defects--determination of their origin and natural history using a
radiomorphological study. Skeletal Radiol. 1988;17(1):8-15.
Robbin MR, Murphey MD, Temple HT, Kransdorf MJ, Choi JJ. Imaging of Musculoskeletal Fibromatosis.
Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O'Connell JX, Knowling MA. Dermatofibrosarcoma protuberans:
MR imaging features. AJR Am J Roentgenol. 2002 Apr;178(4):989-93.
Van der Woude HJ, Hazelbag HM, Bloem JL, Taminiau AH, Hogendoorn PC. MRI of adamantinoma of long bones
in correlation with histopathology. AJR Am J Roentgenol. 2004 Dec;183(6):1737-44.
783
Alphabet Soup and Cystic Lesions

of The Bone
Mark D. Murphey, MD
Alphabet Soup and Cystic Lesions of the Bone
Giant cell tumor (GCT)

Unicameral bone cyst (UBC)
Aneurysmal bone cyst (ABC)
Epidermoid inclusion cyst
Subchondral cyst
Intraosseous ganglion
Post-traumatic cyst
Giant Cell Tumor (GCT): Clinical Features
Approximately 5% of all biopsied primary bone tumors; 18%-23% of

benign bone neoplasms
Symptoms pain and swelling often relieved by decreased
activity
Pathologic fracture 10%35%
Adults 80% between 2050 Years
Rare in children 1%3% (<14 years)
Sex distribution
F-M ratio 3:2 benign GCT
M-F ratio 3:1 aggressive GCT
Figure 4-8-1
Giant Cell Tumor: Location
Originate metaphyseal side of growth plate and grow to

subchondral bone (84%99%)
Long tubular bones 75%90%
About the knee 50%65%; distal femur 23%-30%; proximal
tibia 20%-25%
Radius (10%-12%); humerus (4%-8%)
Spine (7%-15%) - vertebral body sacrum-thoracic-cervicallumbar
Pelvis (4%); hands/feet (5%)
Multifocal (0.5%1%) skull and face (Paget disease), Goltz
syndrome
Giant cell tumor (GCT) of the proximal tibia with

geographic lytic lesion with a mild rim of partial
sclerosis (arrows-unusual in GCT), mild
expansile remodeling and increased
radionuclide uptake on bone scan (right image)
Figure 4-8-2
Giant Cell Tumor: Pathology
Osteoclast like giant cells (90%)

Mononuclear spindle cell stromal component
Hemorrhage, necrosis and hemosiderin
ABC like areas 10%15%
Osseous Lesions Containing Giant Cells
GCT/ABC/UBC
NOF/CMF/OGS
Brown tumor HPT/chondroblastoma
Fibrous dysplasia and variants
Osteoblastoma
Giant cell reparative granuloma
Giant Cell Tumor: Radiology [Figures 4-8-1 to 4-8-10]
CT of giant cell tumor shows no mineralized

matrix (*) (same patient as previous
radiograph)
Solitary eccentric geographic lytic lesion extending into

subchondral bone
Center of lesion-metaepiphysis
Margin IB (80%-85%), IC (10%-20%), IA (1%-2% but up to 20% by CT)
No mineralized matrix
Alphabet Soup and Cystic Lesions of Bone
784
Expansile remodeling (47%-60%) with apparent cortical permeation

(33% - 50%)
Septations - subperiosteal new bone
Periosteal reaction unusual 10%30%
Radiologic characteristic do not reflect clinical
behavior of GCT
Bone scan - doughnut sign (57%)
Usually a vascular lesion (75%-90%)
MRI>CT for evaluation of extent
Fluid-fluid levels
Low to intermediate intensity usually
predominates on T2W images (90%95%)
Figure 4-8-3
Figure 4-8-4
Giant cell tumor of distal femur shows well defined geographic (1B
margin-arrow)) lysis extending to subchondral bone. Bone scan
reveals marked increased uptake in femur. Bone scan reveals
marked increased uptake in the femoral GCT but also in the
adjacent tibia and patella. The increased uptake in the tibia and
patella are due to hyperemia and disuse, not tumor involvement
Figure 4-8-5
Axial T1-weighted MR image shows marrow

replacement and small anterior soft tissue mass
(*) resulting from this benign giant cell tumor
(same patient as previous radiograph and bone
scan).
Figure 4-8-6
Sagittally sectioned gross specimen and macrosection

show identical findings as on the previous images of this
benign giant cell tumor including extension to subchondral
bone (arrows) and anterior soft tissue component (*)
Figure 4-8-8
Figure 4-8-7
Giant cell tumor of the fibula

with marked expansile
remodeling of bone
Coronal T2-weighted MR image

shows intermediate to low (*) signal
intensity tissue typical of giant cell
tumor
785
Giant cell tumor of the patella

(sesamoids and apophysis are
epiphyseal equivalents for the
differential diagnosis of lytic lesions)
Sacral Lesions: Differential Diagnosis
Figure 4-8-9
GCT/ABC
Metastasis
Myeloma/plasmacytoma
Chordoma
Neurogenic tumor
Giant Cell Tumor: Treatment and Prognosis
Curettage and cryosurgery or en bloc resection and bone graft

Local recurrence rate 40%60% historically
Current recurrence rate 2%25%
Recurrence does not correspond to radiologic or microscopic
appearance
Osseous recurrence - new bone destruction
Soft tissue recurrence - mass and may calcify/ossify about
periphery
May metastasize - 2%-5% (50% benign histology)
Malignant GCT 10%15% (much <5% in our experience) (more
common with radiation)
Giant cell tumor of the sacrum with

predominantly low to intermediate signal
intensity (*) on the axial T2-weighted MR image
Figure 4-8-10
Giant Cell (Reparative) Granuloma
Rare benign lesion described in 1953 by Jaffe

Mandible/maxilla and hands/feet
Phalanges > metacarpal > metatarsal > carpus >
tarsus
Women > men (jaw), age to 10 - 50 years
May have history trauma
Giant Cell (Reparative) Granuloma:

Pathology
Granuloma - like arrangement of fibroblastic

stroma and osteoid on micro
Metadiaphyseal lytic lesion
Expansile remodeling and trabeculation
Recurrence only if incompletely excised
Giant cell tumor of the sacrum with predominantly intermediate

signal intensity (*) on the sagittal T1-weighted MR image and
large associated soft tissue mass (*) correlating identically with
the sagittally sectioned gross specimen
Giant Cell (Reparative) Granuloma: Radiology [Figure 4-8-11]
Similar to GCT
May not extend to subchondral bone (hand)
Expansile remodeling and trabeculation
May detect small amount of mineralization
Figure 4-8-11
Unicameral Bone Cyst: Simple Bone Cyst
A fluid - containing lesion lined by mesothelial (epithelial-like)

cells usually arising in metaphysis of long bone adjacent to
physis
Simple Bone Cyst: Clinical Features
3% of all biopsied primary osseous neoplasms

Young patients 85% < 20 years
M>F; 2:1
Pathologic fracture 50%
Simple Bone Cyst: Pathology
Clear, straw - colored fluid filled cyst

Cyst lining - thin flat epithelial - like cells - mesothelial origin
Complicated cysts - hemorrhage, fibro-osseous repair tissue
Giant cell (reparative) granuloma in the second

metacarpal
786
Simple Bone Cyst: Location and Etiology
Under age 20 - humerus (55%65%), femur (25%30%), tibia, fibula, radius

and ulna rare
Over age 20 - iliac bone and calcaneus
Cause - lymphatic or venous obstruction vs. synovial origin
Figure 4-8-12
Simple Bone Cyst: Radiology [Figures 4-8-12 to 4-8-14]
Geographic IA lesion - originate in central metaphysis (active)

Can migrate into the diaphysis (latent)
Mild expansile remodeling
Not infrequently multilocular
Pathologic fracture
Fallen fragment sign (5%)
CT/MRI- noncomplicated see simple fluid
Thin wall and septal enhancement beware delayed MR imaging with
diffusion
CT/MRI- complicated case
Solid components, thick walls, fluid level
Simple Bone Cysts: Treatment and Course
Spontaneous regression or heal after fracture

Curettage and bone grafting
Intralesional steroids (70%95% effective)
Recurrence 20%40%
Extremely rare-malignant transformation
Simple bone cyst with fracture

(arrow) and fallen fragment
sign (curved arrow)
Figure 4-8-13
Aneurysmal Bone Cyst (ABC): Definition
The so called aneurysmal bone cyst is neither a cyst nor a

neoplasm; rather it is probably a periosteal to intraosseous
arteriovenous malformation not uncommonly seen in association
with other well known benign and even malignant lesions.
Mirra JM. Bone Tumors. Lea & Febiger 1989
Aneurysmal Bone Cyst: Clinical Features
1% of all biopsied primary osseous neoplasms

80% between ages 5 and 20 years
Patients present with pain, swelling, and pathologic fracture
(10%20%)
May be associated with trauma
Slightly more common in women
Aneurysmal Bone Cyst: Secondary Lesion
Simple bone cyst in the calcaneus (arrow)
Figure 4-8-14
1%32% of cases
Benign lesions - chondroblastoma, CMF, NOF, GCT, fibrous dysplasia,
UBC, brown tumor, hemangioma, giant cell reparative granuloma
Malignant lesions - hemangioendothelioma, telangiectatic
osteosarcoma, chondrosarcoma
Osseous Lesions with Prominent Fluid Levels

Aneurysmal bone cyst (only fluid levels)

Giant cell tumor (to bone end,metaphyseal center)
Chondroblastoma (epiphyseal center)
Osteoblastoma (posterior elements spine)
Telangiectatic Osteosarcoma (thick walls, osteoid on CT)
Fibrous dysplasia (diaphysis, ground glass)
Aneurysmal Bone Cyst: Pathology
Gross - Blood-filled sponge

Cavernous blood filled spaces line by fibrous walls
May see chondrosseous tissue indicating repair
787
Simple bone cyst in the calcaneus

on CT with septation (arrowheads)
Aneurysmal Bone Cyst: Location
Long tubular bone 70%80%

Spine posterior elements - 15% (thoracic, lumbar, cervical, sacral)
Pelvis 5%10%
Hands 10%15%
Aneurysmal Bone Cyst: Radiology [Figures 4-8-15 to 4-8-21]
Only osseous neoplasm named for its radiologic appearance

Metaphysis (80%90%), eccentric medullary geographic lytic lesion
Can appear central with expansion
Diaphysis (10%20%), often surface lesions
Expansile remodeling uneven in distribution creating one aggressive margin
Spine - expansion can lead to neurologic deficits
Periosteal membrane intact on CT/MRI
Bone scan - peripheral activity (65%)
Fluid-fluid levels (CT/MRI)-nonspecific representing sedimentation of blood
Angiography-hypovascular lesion with localized areas of increased vascularity
Figure 4-8-16
Figure 4-8-15
Aneurysmal bone cyst (primary) with donut sign (increased

uptake peripherally and photopenia centrally) on bone
scintigraphy (same patient as previous radiographs)
Aneurysmal bone cyst (primary) with more prominent expansile

remodeling of bone posteriorly (more aggressive appearancearrow) versus rim of sclerosis in other areas (indolent
appearance-arrowheads)
Figure 4-8-18
Figure 4-8-17
T2
Aneurysmal bone cyst (primary) on axial T2-weighted MR image

shows fluid levels (arrows) from hemorrhage in all parts of the
lesion (same patient as previous bone scan)
Aneurysmal bone cyst (primary) with sagittal

gross specimen showing blood filled spaces (*)
lined by thin septae (arrows) (same patient as
previous MRI)
788
Figure 4-8-19
Figure 4-8-20
T1
T1 GD
Coronal pre and post contrast T1-weighted MR images showing

thin enhancing periphery and septae (arrows) typical of a primary
aneurysmal bone cyst
T1
T2
Coronal T1-weighted and sagittal T2-weighted MR images of a

secondary (chondroblastoma) aneurysmal bone cyst with cystic
areas containing fluid levels (arrows) and anterior solid
component (*)
Figure 4-8-21
Giant cell tumor with ABC component on various MR pulse

sequences with diffuse enhancement and intermediate signal
intensity of the solid component (*) and rim enhancement, high
signal and fluid level in the cystic component (arrows)
Aneurysmal Bone Cyst: Treatment and Prognosis
Rarely spontaneous regression

Curettage, cryosurgery and bone grafting
Recurrence 10%20%
Radiotherapy
Radiofrequency ablation with methylmethacrylate placement
Embolotherapy*
Cory DA et al. AJR 1989; 153:369
Aneurysmal Bone Cyst: Solid Variant?
Recently described (1983); controversial

Radiography - similar to other ABCs but more often aggressive and axial
location
Histology-fibrous tissue proliferation, osteoid production, osteoclastic giant
cells, sinusoids
789
Epidermoid Inclusion Cyst [Figure 4-8-22]
Two types
Hand - distal phalanx-traumatic origin
Radiographs - punched out lesion with surrounding sclerosis,
dorsal cortex often absent
Skull-intraosseous-frontal and temporal bone-congenital origin
Radiographs - well defined lytic lesion sclerotic margin and can
cause expansile remodeling of bone
Pathology - stratified squamous epithelium
Figure 4-8-22
Subchondral Cyst
Other terms - geodes and synovial cyst; no true epithelial or

synovial lining
Middle to older aged patients
Around joints and associated with other arthritic changes
Etiology - synovial fluid intrusion vs. osseous contusion
Can be large/solitary, articular damage subtle simulating neoplasm
(GCT)
Intraosseous Ganglion [Figures 4-8-23 and 4-8-24]
Uncommon lesion; middle aged adults

Pain increases with activity
Periarticular, eccentric, geographic IA-B lytic lesion
Tibia (medial malleolus), femur, about wrist (>65%
of lesions)
Pathology - same as soft tissue ganglion
Epidermoid inclusion cyst with welldefined terminal phalangeal lytic lesion

(arrows)
Figure 4-8-23
Post-Traumatic Cyst [Figure 4-8-25]
Occurs as complication of fracture in children

Usually forearm - radius/ulna
Caused by hemorrhage then fibrosis
Radiolucent lesion well defined, may heal or
persist
Figure 4-8-24
Intraosseous ganglion in the medial malleolus with geographic

lysis (arrows) and thin sclerotic margin (1A)
Figure 4-8-25
Intraosseous ganglion in the subchondral region of the medial

malleolus with intermediate signal intensity on T1-weighting
and high signal intensity on T2-weighting (*) and septation
Post traumatic cyst in the radius subsequent to

a fracture (arrows)
790
References
1.
2.
3.
4.
Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am
J Roentgenol. 1995 Mar;164(3):573-80. Review.
Martinez V, Sissons HA. Aneurysmal bone cyst. A review of 123 cases including primary lesions and those secondary
to other bone pathology. Cancer. 1988 Jun 1;61(11):2291-304.
Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ. From the archives of AFIP.
Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation.
Radiographics. 2001 Sep-Oct; 21(5):1283-309. Review.
Parman LM, Murphey MD. Alphabet Soup: Cystic Lesions of Bone. Seminars in Musculoskeletal Radiology 2000;
4(1):89-101.
791
Juxtaarticular Soft Tissue Masses

Mark D. Murphey, MD
Soft Tissue Masses In and About Joints
Tumor like-tumoral calcinosis, PVNS, ganglion, synovial cyst, myositis

ossificans
Benign - synovial lipoma, myxoma, synovial chondromatosis/chondroma,
nodular fasciitis
Malignant-synovial sarcoma, clear cell sarcoma
Figure 4-9-1
Tumoral Calcinosis: Clinical Features
Usually children/young adults

Increased incidence in blacks
Familial tendency (33% of cases)
Large calcified paraarticular mass, hip, shoulder, elbows, and feet
Can be associated with CPPD arthropathy, pseudoxanthoma elasticum like syndrome
Also skin ulceration, marrow and dental changes
Etiology - metabolic (hyperphosphatemia and increased Vitamin D), trauma,
idiopathic
Tumoral Calcinosis: Pathology
Gross-encapsulated multilocular mass, filled with viscous calcium

hydroxyapatite
Fibrous septations
May have inflammatory elements
Tumoral calcinosis about the

shoulder with large calcified
periarticular mass (*) and
radiolucent septations
(arrowheads)
Figure 4-9-2
Tumoral Calcinosis: Radiology

[Figures 4-9-1 to 4-9-6]
Calcified paraarticular mass

Extensor surface
Radiolucent septations (chicken wire)
Extraarticular (bursae); no loss ROM
Average 3 lesions/individual*
CT/MRI: fluid-fluid levels (liquefied calcium)
More active disease
Bone scan-best for detection, and localization
MRI low signal T1W images
Variable low to high signal T2W images
Pseudoxanthoma elasticum-skin/vascular calcification,
retina angioid streaks
CPPD arthropathy
Dental abnormalities-root enlargement, intrapulp calcification
Marrow involvement - calcific myelitis
Tumoral calcinosis about the knee
Figure 4-9-3
*Martinez, Radiology 1990;174:215
Clinical photograph in patient with

tumoral calcinosis about the knee
showing cosmetic deformity but no
decreased range of motion
Juxtaarticular Masses
792
Figure 4-9-4
Figure 4-9-5
Tumoral calcinosis about the elbow extensor surface.

Contralateral elbow revealed identical findings (not
shown)
Tumoral calcinosis about the hip

with calcium fluid levels (arrowheads) on CT
Figure 4-9-6
Tumoral calcinosis about the shoulder with large calcified

periarticular mass (*) showing peripheral and septal
enhancement after contrast (arrowheads)
Periarticular Calcification: Differential Diagnosis
Scleroderma
Other collagen vascular diseases
Chronic renal failure (secondary tumoral calcinosis)
Milk - Alkali syndrome
Synovial sarcoma
Tumoral Calcinosis: Treatment
Phosphate depletion therapy

Surgical excision
Pigmented Villonodular Synovitis (PVNS): Clinical Features
Proliferative disorder of synovium of joint, tendon or bursa

Young adults 3rd and 4th decades
Two types diffuse (15%25%) and localized (75%85%)
Symptoms - pain, swelling, ROM loss
P V N S: Pathology
Etiology unknown - inflammatory/neoplasm/trauma

Variable degree of villous/nodular synovial proliferation and pigmentation
(hemosiderin) and inflammation components
Giant cells, fibrous tissue, xanthoma cells
793
P V N S: Location
Localized form usually extraarticular

Giant cell tumor tendon sheath (GCT-TS)
Hand (80%), feet, knee (12%)
Diffuse form - knee (60%80%) hip, ankle, shoulder, elbow
Figure 4-9-7
GCT-TS: Radiology
Second most common mass hand/wrist

Lobulated soft tissue mass < 2 cm
More common volar surface
Osseous erosion uncommon 10%15%
P V N S - Diffuse Form: Radiology
Erosive bone lesions - 50% : hip (93%), shoulder (75%), knee (26%)
Geographic IA lytic lesion - extrinsic erosion
Joint effusion
Arthrography - brownish or chocolate fluid, multinodular filling defects
P V N S: Radiology [Figures 4-9-7 to 4-9-11]
Bone scan - mild increase activity (statics)

Angiography - can show impressive vascularity
CT - soft tissue mass, increased attenuation
MRI T1W image low intensity mass T2W image variable usually
prominent low intensity regions
Figure 4-9-8
Localized form of PVNS (giant

cell tumor of tendon sheath)
with volar thumb mass (*) on
sagittal T1-weighted MR image
Figure 4-9-9
Same patient as previous MR image with intermediate signal

intensity in the giant cell tumor of tendon sheath (*) of the
thumb on axial T2-weighted MR
PVNS of the hip (diffuse type) with radiograph showing

erosions on both sides of the joint (arrows) and maintained
joint space
Figure 4-9-10
PVNS hip (same patient as previous radiograph) with marked

low signal intensity tissue on coronal T2-weighted MR image (*)
794
Figure 4-9-11
Synovial Based Lesion:

PVNS/Synovial chondromatosis
Arthritis - inflammatory
Infection - unusual low-grade
Amyloid
Synovial sarcoma
P V N S: Treatment and Results
Surgical resection/synovectomy
Recurrence rate
GCT - TS (10%20%)
Diffuse form (40%50%)
Radiation - internal synovectomy - yttrium 90 and
dysprosium 165
Soft Tissue Ganglion: Clinical Features
PVNS knee (diffuse type) with large amount of low

signal intensity intraarticular hemosiderin laden
tissue (*)
Young adults (2545 years old)

Most common mass hand/wrist (60% of masses)
Pain, tenderness or functional impairment (50%), rarely nerve palsy
Figure 4-9-12
Soft Tissue Ganglion: Pathology
Etiology - unknown - neoplasm, inflammation, trauma

Thick walled unilocular/multilocular cystic spaces
Gelatinous - mucinous fluid rich in hyaluronic acid and
mucopolysaccharides
Soft Tissue Ganglion: Radiology

[Figures 4-9-12 to 4-9-16]
Soft tissue mass (1.52.5 cm) - dorsum hand/wrist

Attached to tendon sheaths usually no communication with
joint
Rarely cause adjacent bone erosion; periosteal reaction, wall
calcification
CT/Sono/MRI - cystic mass
May have higher attenuation on CT or signal T1W MR image
high protein mucin
Wall/septae may show mild enhancement
Large ganglion in the most frequent location
dorsal to the proximal carpal row with low to
intermediate signal intensity mass (*) on axial
T1-weighted MR image and homogeneous
high signal intensity on T2-weighting
Figure 4-9-13
Figure 4-9-14
Large ganglion in the most frequent location

dorsal to the proximal carpal row with anechoic
appearance on sonography (*)
Ganglion in Guyon canal with intermediate signal
intensity mass (*) on axial T1-weighted MR image
[upper image] and marked high signal intensity on
axial T2-weighted MR image (*) [lower image]
causing ulnar nerve entrapment symptoms
795
Myxoma: Clinical
Features
Figure 4-9-15
Figure 4-9-16
Location - heart,
subcutaneous,
intramuscular,
juxtaarticular
Adults 4070 years of
age
Slightly more common
in women
Painless palpable mass
Myxoma: Pathology
Ovoid/globular whitish
appearance
Intraarticular ganglion (*) in the knee on sagittal
Contain gelatinous
T2-weighted MR image with septations and
marked high signal intensity (same patient as
material
Intraarticular ganglion in the knee on CT
previous CT)
with low attenuation and multiple
Unusual to have cystic
septations
(*)
spaces
No fibrous capsule, but edema and muscle atrophy surround mass
Myxoma: Radiology [Figures 4-9-17 to 4-9-19]
Soft tissue mass - location - thigh, shoulder, buttock, upper arm

Fluid characteristics CT/MRI
Not simple fluid on sonography
High protein material may increase CT attenuation or signal on T1W MR
image
Peripheral rim may enhance with contrast or mild diffuse (57%) pattern
Septations (43%) thick and mildly nodular regions
Small rim of fat-like tissue CT (25%), MR (71%)
Edema surrounding mass MR (79%)
Rare to recur after removal (partial or complete)
Figure 4-9-17
Myxoma: Differential Diagnosis
Abscess
Chronic hematoma
Ganglion/synovial cyst/bursa
Other myxomatous neoplasms MFH/liposarcoma/neural tumors
Figure 4-9-18
Intramuscular myxoma in paraspinal

location on CT with low attenuation
mass (*) simulating a cyst
Figure 4-9-19
Paraspinal intramuscular myxoma (same

patient as previous CT) with low
echogenicity mass but some internal echoes
(*) are present suggesting that the lesion is
not cystic
Intramuscular myxoma in the forearm on sagittal MR imaging
with low signal intensity on T1-weighting and high signal
intensity on T2-weighting (*) simulating a cyst. However, the
intramuscular location, subtle rim of fat (arrowheads on T1)
and surrounding edema (arrows on T2) exclude a cyst as a
reasonable diagnostic consideration
796
Synovial Cyst: Definition
Figure 4-9-20
A herniation or continuation of the synovial membrane through the

joint capsule
Synovial Cyst: Location and Etiology
Most commonly recognized - popliteal

Shoulder, elbow, hip, hand, foot and ankle
Types
Primary - unknown cause - children
Secondary - any cause joint distention
Adults - rheumatoid arthritis
Synovial Cyst: Pathology
Fluid filled may be multilocular

Dense fibrous wall
Lined by synovium
Popliteal Cyst - Baker Cyst
Synovial Cyst: Radiology
[Figure 4-9-20]
Often asymptomatic or pain from other causes

Uncommon to present as mass
May dissect in calf simulate DVT
Imaging shows infiltration of calf (long fusiform lesion) caused by
extension of cyst with dissection and surrounding edema
Results from communication between knee joint and gastrocnemius
semimembranosus bursa
Increase incidence with age 50% autopsy series
Incidence varies arthrography (7% 42%), sonography (15%),
MRI (5%)
[Figures 4-9-21 to 4-9-25 continues overleaf]
Arthrogram showing the morphology of a

ruptured popliteal cyst with long fusiform
shape and irregular margins caused by
infiltration into surrounding muscle
Fluid filled mass - Sono/CT/MRI

May have septations
Arthrography can show joint communication
Can have solid components if complicated (rupture) with hemorrhage,
dissection or superimposed infection
Contrast enhancement of rim/septae
Noncomplicated thin walls
Contrast enhancement more complex in complicated/ruptured popliteal
cysts
Complicated cysts difficult to exclude other causes of mass; must look at
morphology
Imaging shows infiltration of calf (long fusiform lesion) caused by
extension of cyst with dissection and surrounding edema
Figure 4-9-22
Figure 4-9-21
Figure 4-9-23
Synovial cyst (popliteal) on CT with

low attenuation (*) and single
septation (arrowhead) in typical
location with neck between the
semimembranosus and
gastrocnemius tendons
Synovial cyst (popliteal) on axial T1weighted MR image with low signal

intensity mass (*) and typical location
(neck between gastrocnemius and
semimembranosis tendons-arrowheads)
as described in previous CT
Synovial cyst (popliteal - same patient as

previous MR) on axial T1-weighted postcontrast MR image with peripheral/septal but
nonnodular enhancement (arrowheads)
797
Figure 4-9-24
Figure 4-9-25
Synovial cyst (popliteal - same patient as previous two MRs)

on axial T2-weighted post-contrast MR image
with diffuse high signal intensity (*)
and neck extending into joint (arrowhead)
Ruptured popliteal cyst on sagittal T1 and T2-weighted MR

image with evidence of hemorrhage (*) and extensive
surrounding edema both superiorly and inferiorly (arrowheads)
Meniscal Cyst: Clinical Features
Adults (2040 years); M>F ratio 2:1

Cystic masses related to meniscal tears (1%2% incidence)
Fluid accumulates from joint through tear
Pain at night or after exercise
Figure 4-9-26
Meniscal Cyst: Radiology
Radiographs - soft tissue mass

CT/SONO/MRI - fluid collection adjacent to meniscus
Lateral > Medial 310:1 now more equal
Medial - small cystic mass within or adjacent to meniscus
Lateral - larger fluid collection filling potential space between
meniscus and collateral ligament
MRI - best to evaluate meniscal tear and extension into cyst
Must repair tear and resect cyst
Synovial Lipoma
Two types
Localized form
Diffuse form - lipoma arborescens
Lipoma arborescens with villonodular fronds of

fatty tissue (arrows) extending into the knee joint
on sagittal T1-weighted MR image
Synovial Lipoma: Localized
Figure 4-9-27
Rare - knee most frequent

Solid fatty intraarticular mass
Filling defect on arthrogram
CT/MRI - lipomatous tissue
Lipoma Arborescens: Clinical Features
Diffuse infiltration of synovium by fat

Monoarticular - knee most common
Often secondary (but can be primary) to chronic arthritis from
trauma or inflammatory disease
Lipoma Arborescens: Radiology

[Figures 4-9-26 and 4-9-27]
Radiographs - soft tissue swelling

Arthrography - multiple filling defects
CT fatty infiltration
MRI best to identify frond-like fatty projections
Lipoma arborescens (same patient as previous

MR ) showing high signal intensity fluid
surrounding fatty nodules (arrows) on sagittal
T2-weighted MR image
798
Synovial Chondromatosis: Clinical Features
Figure 4-9-28
Formerly synovial osteochondromatosis

Cartilage metaplasia in synovium
Knee (50%), hip, elbow, any joint can be involved
M > F 2:1; 3rd to 6th decade
Joint pain, decrease range of motion
Synovial Chondromatosis: Pathology
Hyaline cartilage metaplasia in synovium

Cartilage nodules (23 cm) can break away into joint, grow, reattach
to synovium
Hypercellularity and nuclear atypia simulate cartilage malignancy
Synovial Chondromatosis: Radiology [Figures 4-9-28 to 4-9-32]
Radiographs - calcified bodies (70%75%), may ossify, extrinsic

erosions, joint widened, OA changes
Bone scan - mild increased activity
Arthrography - filling defects
CT thickening about joint, effusion often small if present,
calcification/ossification
MRI - variable depending on degree of mineralization, some
hyperintensity T2W images
Can also involve tendons and bursa
Secondary chondromatosis - trauma, OA, RA, AVN, osteochondritis
dissecans
Figure 4-9-29
Synovial chondromatosis wit multiple

round filling defects on hip
arthrography. No calcification was
seen on pre-arthrography radiographs
(not shown)
Figure 4-9-30
Synovial chondromatosis of right hip with subtle

calcifications (arrowhead) difficult to detect on
radiograph, although joint is widened (arrow)
Synovial chondromatosis of the shoulder with

innumerable calcified intraarticular
osteochondral bodies all similar in size and
shape on radiograph
Figure 4-9-32
Figure 4-9-31
Synovial chondromatosis of right hip (same patient as previous

radiograph) with multiple calcifications (arrowheads) about hip on CT
799
Synovial chondromatosis of right hip (same patient

as previous radiograph and CT) with extensive high
signal intensity intraarticular tissue (*) but
calcification is not apparent on T2-weighted MR
image
Synovial Chondromatosis: Treatment and Prognosis
Surgical synovectomy
Recurrence common
External radiation therapy
Internal RT - nuclear medicine synovectomy?
Rare degeneration into chondrosarcoma
Figure 4-9-33
Soft Tissue Chondroma: Clinical Features
Less common than synovial chondromatosis

3rd and 4th decades, M>F
Slow growing masses, painless
Fingers (80%), hands, toes, feet, trunk
Soft Tissue Chondroma: Pathology
Usually < 3 cm, often attached to tendon

Mature hyaline cartilage lobular pattern
Can show ossification
Fibrous capsule not tenosynovium unlike synovial
chondromatosis
Soft tissue chondroma of the finger on

radiographs with large calcified mass
(arrowheads)
Soft Tissue Chondroma: Radiology

[Figures 4-9-33 and 4-9-34]
Nonspecific soft tissue mass related to IP joint

Also common in infrapatellar fat
Chondroid matrix, can ossify
Unusual to erode underlying bone
Heterotopic Bone Formation: Myositis Ossificans
Young adults, M>F, usually trauma history

No history trauma 25%50%; also paraplegics
Can involve muscles, fascia, tendons, subcutaneous fat
Initially pain/tenderness and localized mass; pain decreases
with time
Heterotopic Bone Formation: Location
Same patient: CT with large calcified mass

(arrowheads). Noncalcified portion is low
attenuation consistent with a chondroid lesion
Extremities - 80%, anterior compartments

Lower extremity - quadriceps muscle
Upper extremity - brachialis muscle
Subcutaneous fat - 30% of cases
Figure 4-9-34
Same patient: MR imaging with high water content soft tissue

mass (arrowheads) consistent with a chondroid lesion
Soft tissue chondroma in Hoffa fat pad

on radiograph with large calcified mass
800
Heterotopic Bone Formation: Pathology
Zonal pattern of maturation

Central immature osteoid/fibroblastic tissue
Periphery calcifying osteoid to mature lamellar bone
Cortical bone with further maturation
Figure 4-9-35
Heterotopic Bone Formation: Radiology

[Figure 4-9-35]
Early soft tissue mass and edema

Calcification 24 weeks then matures (zonal phenomena)
to central trabecular and peripheral cortical bone
Usually separable from cortex but may be attached
Bone scan marked increased activity
Angiography - staining and neovascularity early
CT best to see early ossification pattern with peripheral
rim enhances with contrast
Heterotopic Bone Formation: MR Imaging

[Figure 4-9-35]
Early to intermediate
Normal with displaced fascial planes (T1W)
Increased intensity mass with prominent edema (T2W
image)
Late - heterogeneous well defined mass marrow fat on
T1W/T2W MR images, no edema, low intensity rim
Often misinterpreted as malignant tumor
Heterotopic bone formation (myositis

ossificans) with peripheral rim of
calcification on radiograph (arrow)
Heterotopic Bone Formation: Treatment and

Prognosis
May resorb or be asymptomatic

Resect after maturation (1218 months)
Premature resection - recurrence with vengeance
Rare report malignant transformation
Malignant myositis (mucinous carcinoma )
Nodular Fasciitis: Clinical Features
Very common; most frequent tumor-like lesion fibrous

tissue
Rapidly growing mass 12 weeks duration
Young adults (2035 years), M<F
History trauma (10%15%)
Same patient: peripheral rim of

calcification (arrow) separated from
femoral cortex on CT
Nodular Fasciitis: Location
Upper extremity (50%) volar forearm

Trunk - chest wall and back
Head and neck in children
Rare hand/feet/lower extremity
Nodular Fasciitis: Pathology
Subcutaneous type (70%) - soft tissue nodule

Intramuscular type (15%) - not circumscribed multinodular
Immature fibroblasts in irregular fascicles
Reticulin meshwork, collagen minimal, inflammatory and
mucoid component
801
Same patient: axial T2-weighted MR

image with heterogeneous mass
(arrow) suggesting a more aggressive
neoplastic process as peripheral
calcification is less apparent
Nodular Fasciitis: Radiology and Treatment

Figure 4-9-36]
Figure 4-9-36
Nonspecific soft tissue mass; may show fascial extensions

CT/MRI-mass with irregular margins and heterogeneous on MRI,
surrounding edema
Suggests malignancy imaging and pathology
Surgical resection-recurrence rare (1%2%) even if incomplete
Synovial Sarcoma: Clinical Features
Malignant mesenchymal tumor

Young adults 1540 years of age
Fourth to fifth most common soft tissue sarcoma
Painful deep soft tissue mass
Often indolent slow growing mass (4 years to diagnosis)
Musculoskeletal Soft Tissue Sarcoma: Incidence
MFH/Fibrosarcoma 20%30%
Liposarcoma 16%19%
Rhabdomyosarcoma 10%19%
Nonspecific spindle cell sarcoma 5%15%
Leiomyosarcoma 5%-10%
Dermatofibrosarcoma protuberans (DFSP) 5%-10%
Synovial sarcoma 5%10%
Nodular fasciitis of the forearm on

coronal STIR MR image with a
high signal intensity subcutaneous
mass (*) with linear fascial
extensions (fascial tail sign
arrows) both superiorly and
inferiorly
Figure 4-9-37
Synovial Sarcoma: Location
Extraarticular adjacent to tendons bursa, ligaments > 90%

Intraarticular < 10%
Lower extremity 60% - around knee
Upper extremity 25% - around wrist
Synovial Sarcoma: Pathology
Two cell lines

Epithelial (keratin positive)
Spindle cells
Monophasic / biphasic
Variable - calcification, hemorrhage
Synovial Sarcoma: Radiology [Figures 4-9-37 to 4-9-40]
Radiographs - normal (50%) or nonspecific soft tissue mass near joint

Bone erosion or periosteal reaction (11%20%), bone invasion (5%)
Soft tissue calcification up to 30% - best by CT
Bone scan - increased activity
MRI
T1W images - similar to muscle
T2W images - usually high intensity
Triple sign on T2W MR (35-57%) (nonspecific)
Very heterogeneous (bowl of grapes)
Not uncommonly well defined with pseudocapsule - simulates benign
characteristics
Fluid - fluid level 10%25% (hemorrhage) worse prognosis in highly
vascular lesions
Synovial sarcoma of the foot with

indolent appearing extrinsic
erosions on radiograph (arrow)
Figure 4-9-38
Synovial Sarcoma: Treatment and Prognosis
Surgical resection/amputation
Radiation therapy/chemotherapy
Local recurrence 30% 50%
5 year survival 36% 76%; 10 year survival 20% 63%
Metastases (16%-25%) lung (94%), lymph node (10%), marrow
Synovial sarcoma about elbow with
calcification (arrowhead) in mass near
but not in the joint on radiograph
802
Clear Cell Sarcoma: Clinical Features
Figure 4-9-39
Malignant melanoma of soft parts

Arise in tendons/aponeurosis
Deep tissue without skin involvement
Foot/ankle (43%), knee, thigh, hand
Adults 2040 years; F>M
Clear Cell Sarcoma: Pathology
Cells with clear cytoplasm

Framework of fibrocollagenous tissue
Intracellular melanin 60%75%
Hemosiderin also present
Clear Cell Sarcoma: Radiology [Figure 4-9-41]
Soft tissue mass at/in tendon/aponeurosis

Bone erosion/destruction
CT/MRI - infiltrative soft tissue mass
MRI
T1W image - intermediate intensity
T2W image - may be low intensity
Clear Cell Sarcoma: Treatment and Prognosis
Surgical resection/radiation/chemotherapy
Poor prognosis
Local recurrence and metastases
Mets - lungs, lymph nodes, bone
Synovial sarcoma about the ankle on coronal T1weighted MR image with heterogeneous
hemorrhagic mass (*) invading bone (arrowhead)
Figure 4-9-40
Figure 4-9-41
Synovial sarcoma about the ankle (same

patient as previous MR) on axial T2 weighted MR image with heterogeneous
multicompartment mass
Clear cell sarcoma on MR imaging with

origin in the quadriceps tendon as
evidenced on the axial image with low signal
intensity both anterior and posterior to the
mass (arrowheads). Sagittal T2-weighted
MR shows nonspecific intermediate signal
intensity
803
Noncalcified Juxta/Intraarticular Soft Tissue Masses

Synovial/Meniscal cyst
Ganglion/myxoma
Gouty tophus
Hemangioma/PVNS
Lipoma
Synovial sarcoma
Calcified juxta/Intraarticular Soft Tissue Mass

Myositis ossificans
Aneurysm
Gouty tophus
Hyperparathyroidism/hemangioma
Osteochondromatosis (synovial)
Synovial sarcoma
Tumoral calcinosis
Soft tissue sarcoma
References
1.
Al-Nakshabandi NA, Ryan AG, Choudur H, Torreggiani W, Nicoloau S, Munk PL, Al-Ismail K. Pigmented villonodular
synovitis. Clin Radiol. 2004 May;59(5):414-20. Review.
2. Kransdorf MJ, Meis JM, Jelinek JS. Myositis ossificans: MR appearance with radiologic-pathologic correlation.
AJR Am J Roentgenol. 1991 Dec;157(6):1243-8.
3. Martinez S, Vogler JB 3rd, Harrelson JM, Lyles KW. Imaging of tumoral calcinosis: new observations. Radiology. 1990
Jan;174(1):215-22.
4. Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and
complications with radiologic-pathologic correlation. Radiographics. 2000 Sep-Oct;20(5):1407-34. Review.
5. Murphey MD, McRae GA, Fanburg-Smith JC, Temple HT, Levine AM, Aboulafia AJ. Imaging of Soft Tissue Myxoma
with Emphasis on CT and MRI and Comparison of Radiologic and Pathologic. Radiology 2002; 225:215-224.
6. Ortega R, Fessell DP, Jacobson JA, Lin J, Van Holsbeeck MT, Hayes CW. Sonography of ankle Ganglia with
pathologic correlation in 10 pediatric and adult patients. AJR Am J Roentgenol. 2002 Jun;178(6):1445-9.
7. Robinson P, White LM, Kandel R, Bell RS, Wunder JS. Primary synovial osteochondromatosis of the hip: extracapsular
patterns of spread. Skeletal Radiol. 2004 Apr;33(4):210-5. Epub 2004 Feb 18.
8. Steinbach LS, Johnston JO, Tepper EF, Honda GD, Martel W. Tumoral calcinosis: radiologic-pathologic correlation.
Skeletal Radiol. 1995 Nov;24(8):573-8.
9. Tschirch FT, Schmid MR, Pfirrmann CW, Romero J, Hodler J, Zanetti M. Prevalence and size of meniscal cysts,
ganglionic cysts, synovial cysts of the popliteal space, fluid-filled bursae, and other fluid collections in asymptomatic
knees on MR imaging. AJR Am J Roentgenol. 2003 May;180(5):1431-6.
10. Valenzuela RF, Kim EE, Seo JG, Patel S, Yasko AW. A revisit of MRI analysis for synovial sarcoma. Clin Imaging.
2000 Jul-Aug;24(4):231-5.
804
Musculoskeletal Angiomatous Lesions

Mark D. Murphey, MD
Angiomatous Lesions
Hemangioma
Lymphangioma
Glomus Tumor
Angiomatosis and associated syndromes
Hemangiopericytoma
Angiosarcoma
Osseous Hemangioma: Clinical Characteristics
M > F (2:1); 4th 5th decade

Majority asymptomatic
May have soft tissue components
Common sites : vertebral body (11% of spines), calvarium
Soft Tissue Hemangioma: Clinical Characteristics
7% of all benign S.T. neoplasms

1.5% of the general population
Most frequent S.T. neoplasm in children
More common in young women may increase in
size with pregnancy
Can be subcutaneous, intramuscular or synovial
Figure 4-10-1
Hemangioma: Pathology
Subtype based on predominant vascular

component but usually mixed tumor
Capillary most common first years of life
skin, subcutaneous, vertebrae (low flow)
Cavernous childhood larger and deeper
(low flow)
Arteriovenous deep or superficial persistent
fetal capillary bed (high flow)
Venous adults deep involvement
retroperitoneum, mesentery or extremities
(low flow)
Epithelioid dermis/subcutis
Vertebral hemangiomas with thickened vertical trabeculae

(arrows-corduroy appearance) on radiograph and coronally
sectioned gross specimen (different patients)
Osseous Hemangioma: Radiology
Figure 4-10-2
[Figures 4-10-1 to 4-10-5]
Vertebrae focal or diffuse vertical striations

(corduroy or polka dot) posterior element
involvement more likely symptomatic
Calvarium/mandible radiating web-like trabecular
pattern
Long bone - multifocal lytic honeycomb pattern,
cortical lesions/erosions in diaphysis
Bone overgrowth
Arthritis from intraarticular bleeding
Vertebral hemangioma (asymptomatic) of lumbar spine with

"polka dot" appearance and fat between trabeculae on CT
805
Figure 4-10-3
Figure 4-10-4
Vertebral hemangioma (symptomatic patient) with "polka dot"

appearance on CT (arrowheads) and soft tissue extension.
Sagittal T1 and T2-weighted MR images show vertebral
fracture and spinal canal compromise caused by anterior
epidural soft tissue component (arrows) but diagnostic
trabecular thickening is difficult to appreciate
S.T. Hemangioma Radiology:

S.T. Changes [Figure 4-10-6]
Calcification curvilinear or amorphous, phlebolith

(30%-50% of lesions)
Angiography irregular enlarged feeding arteries,
contrast pooling, arteriovenous shunting
Venous lesions seen only with venography
Calvarial hemangioma with spoke wheel pattern of trabecular

thickening (arrows) on radiograph, CT and vascular
channels/spaces on gross specimen (arrowheads)
Figure 4-10-5
S.T. Hemangioma: Imaging

[Figures 4-10-7 to 4-7-12]
T1W images low to intermediate heterogeneous

mass; look for fat overgrowth
Very high intensity T2W images (low flow)
Serpentine vessels / cavernous spaces may help
distinguish types; high vs. low flow
Phleboliths CT > MRI
Bone scan often only limited activity
Sonography solid mass Doppler may show low
resistance flow
Femoral hemangioma on coronal T1-weighted MR image

before and after contrast shows multifocal round areas of
marrow replacement (arrowheads) representing vascular
channels with enhancement and serpentine feeding vessels
Figure 4-10-6
Figure 4-10-7
Soft tissue hemangioma of the hand with phleboliths on

radiograph (arrows) and intraoperative photograph
Soft tissue hemangioma of axilla (intramuscular and

cavernous) on CT showing enhancing vascular channels
(large arrows), fat overgrowth (small arrows), and phlebolith
(arrowhead)
806
Figure 4-10-8
Figure 4-10-9
Soft tissue hemangioma of forearm (intramuscular and cavernous) on

sagittal T1-weighted MR images before and after gadolinium showing
intermediate signal intensity serpentine vascular channels and spaces
(arrows) that enhance following contrast and fat overgrowth
(arrowheads). Axial T2-weighted MR reveals multiple circular high
signal areas corresponding to slow flow cavernous spaces (*)
Figure 4-10-10
Soft tissue hemangioma of the thigh on sagittal T1-weighted

(right image) and axial STIR (left image) MR images with
associated fat atrophy (arrowheads) and slow flow circular
vascular spaces (arrow) corresponding to the gross specimen
and histology [4-10-10]
Figure 4-10-11
Soft tissue hemangioma
(high flow arteriovenous)
about knee with low
signal intensity
serpentine vessels
(arrows) on coronal T2weighted MR image
Figure 4-10-12
Soft tissue hemangioma (arrows) with

associated fat atrophy (arrowheads) in
surrounding thigh muscle on gross specimen
(left image). Histology (right image) reveals
phleboliths (*) with calcification peripherally
(arrows) and fat atrophy of muscle
(arrowheads)
Capillary hemangioma
on T2-weighted MR
image (arrow) with
nonspecific high signal
intensity in the face
with typical extensive
strawberry nevus
clinically. No
characteristic features
of fat overgrowth or
serpentine vascular
structures are seen to
suggest hemangioma as the vessels in this type of lesion are to small
(capillary) to discern on imaging as demonstrated on the histology
807
Hemangioma: Treatment
Surgical resection / laser therapy

Embolization
Radiation in symptomatic unresectable lesions spine
Vertebroplasty
Recurrence (15% 30%) large lesions
Figure 4-10-13
Lymphangioma:
Rare lesion in bone, usually S.T.

Often present at birth (50% 65%)
90% apparent by age 2 years
Head, neck, axilla 75% of cases
Soft fluctuant mass
Lymphangioma of the neck on CT with a
homogeneously low attenuation mass (*)
Lymphangioma: Pathology
Sequestrated noncommunicating lymphoid tissue

Large multiloculated cystic spaces
Lined by lymphatic endothelium
Filled with proteinaceous material
Figure 4-10-14
Lymphangioma: Radiology [Figures 4-10-13 and 4-10-14]
Radiographs soft tissue mass

Imaging large cystic spaces less common serpentine
component may appear complex solid components (high
signal on T1 25%)
Cystic hygroma hydrops fetalis, Turner syndrome
Glomus Tumor [Figure 4-10-15]
Patients 4th to 5th decade

Tumor of neuromyoarterial glomus
Almost all terminal phalanx soft tissue
Bone erosion/invasion 15%65%
Angiomatosis
Multifocal or diffuse infiltration of bone by hemangiomatous or

lymphangiomatous lesions with or without soft tissue
involvement
Angiomatosis: Clinical Characteristics
Young patients first 3 decades

M > F (2:1)
Osseous involvement only benign course
Visceral involvement poor prognosis
Lymphangioma of the neck in an infant on

coronal T1-weighted MR image with
heterogeneous mass (arrow) showing both
high and low signal intensity areas extending
along the chest wall (arrowhead)
Figure 4-10-15
Angiomatosis: Pathology
Capillary or cavernous hemangiomas

Lymphangiomas lymphatic backflow
Mixed vascular lesion difficult to distinguish
Glomus tumor with erosion of the terminal phalanx of the long

finger on radiograph (arrows) and sagittal macrosection (*)
808
Angiomatosis: Radiology [Figure 410-16]
MR imaging/CT some as solitary angiomatous lesions more extensive

Imaging to evaluate visceral involvement/extent
Lymphangioma proven with lymphangiography
and contrast in lesion
Diffuse round/oval medullary lytic lesions
May have sclerotic margins
Location: femur, ribs, spine, pelvis, humerus,
scapula, other long bones, clavicle
Figure 4-10-16
Angiomatous Syndromes
Maffucci syndrome
Osler Weber Rendu
Klippel Trenaunay Weber
Massive osteolysis of Gorham
Associated osteomalacia and thrombocytopenia
Maffucci Syndrome
Angiomatosis (lymphangiomatosis) with extensive infiltration of

the entire lower extremity causing elephantiasis on coronal T1weighted MR image and clinical photograph
Multiple enchondromata
Cavernous soft tissue hemangiomata
Often hands/feet, unilateral predominance
Malignant potential both lesions and viscera
Osler-Weber-Rendu
Figure 4-10-17
Hereditary hemorrhagic
telangiectasia
Dilated capillaries and veins
Autosomal dominant
GI, GU, lung, spinal; bone rare
Klippel-Trenaunay-Weber
[Figure 4-10-17]
Nonhereditary, lower extremity

Unilateral cutaneous capillary
hemangioma
Varicose veins and local gigantism
Can have arteriovenous component
Massive Osteolysis of Gorham:

Vanishing Bone Disease
Klippel-Trenaunay-Weber syndrome on clinical photograph and coronal T1weighted and T2-weighted MR images showing classic triad of
hemihypertrophy,varicose veins and extensive predominantly slow flow
angiomatous lesion (arrowheads). Smaller high flow component is also seen on
T2-weighted image (arrows)
[Figure 4-10-18]
Patients < age 40 years

History trauma 50%
Upper extremity favored, may extend across joint
Progressive bone resorption and fragmentation
(simulate neuropathic)
Pathology - proliferating vascular channels
Figure 4-10-18
Osteomalacia and Thrombocytopenia
Tumor induced osteomalacia: most frequent

vascular lesions
Hemangioma / hemangiopericytoma
Kasabach - Merritt syndrome - hemangioma/
hemangiopericytoma associated with
thrombocytopenia and purpura
Gorham vanishing bone disease involving the foot with

radiograph and gross specimen showing extensive sharply
defined bone resorption (arrowheads and arrow)
809
Intermediate to Malignant Musculoskeletal Angiomatous

Lesions
Figure 4-10-19
Hemangiopericytoma
Angiosarcoma
Hemangioendothelioma (HE)
Intermediate benign or malignant

Composed of vascular endothelial cells
Often in young patients
Bone or soft tissue
Locally aggressive, unusual to metastasize
Hemangiopericytoma (HPC)
Intermediate benign or malignant

Tumor of cells around vessels pericytes
Tumor of middle-aged adults
Sites soft tissue of thigh, pelvis and retroperitoneum
Rare in bone
Angiosarcoma (ASC)
Malignant; M > F (2:1)

Composed of hemangiosarcoma or lymphangiosarcoma cellular
elements
Location: skin, muscular, viscera, bone
Associated with lymphedema post-mastectomy (Stewart-Treve
syndrome)
Osseous HE, HPC, ASC: Skeletal Location
Hemangioendothelioma: skull, vertebrae, lower extremity

Hemangiopericytoma (rare): pelvis, proximal long bones,
vertebrae, mandible
Angiosarcoma: long tubular bone lower extremity
Figure 4-10-20
Osseous HE, HPC, ASC :

T1
[Figure 4-10-19]
Malignant hemangioendothelioma of bone with

multifocal lytic lesions (arrowheads) in the tibia
and fibula on radiograph and osseous
replacement by hemorrhagic tissue (*) on
photograph of coronally sectioned gross
specimen
Multifocal lytic lesions honeycomb appearance

Aggressive bone destruction with expansion and
soft tissue mass
T2
Radiology of HE, HPC, ASC :

Advanced Imaging
T1 GD
[Figures 4-10-20 to 4-10-21]
Angiography intensely vascular with peripheral

vessels displaced by tumor early, dense blush late
Sonography hypo or hyperechoic mass
Doppler arteriovenous shunting
MRI
T1W usually similar to muscle
Can be high intensity hemorrhage
Look for prominent serpentine vessels
Fluid - fluid levels, contrast enhancement
Dominant skin mass in chronic lymphedema (ASC)
Angiosarcoma developing in a patient with chronic leg
lymphedema. Axial T1-weighted, T1-weighted post contrast
and T2-weighted MR images show the enlarged leg with
subcutaneous edema (arrows) and dominant skin mass
(arrowheads) representing the angiosarcoma.
The superficial angiosarcoma is also seen on the clinical
photograph (grey arrows)
810
Figure 4-10-21
T1
T2
Hemangiopericytoma in of the thigh showing high flow vessels (arrows) in the soft tissue mass (*) and
feeding the lesion on both axial T1-weighted and coronal T2-weighted MR images. Photograph of the
sectioned gross specimen also shows the soft tissue mass (*) and the high flow vessels (arrowheads)
Cannot distinguish HE, HPC or ASC from other soft tissue masses
if prominent serpentine vessels are not recognized
Hemangioendothelioma, Hemangiopericytoma and Angiosarcoma
cannot be differentiated from each other radiologically
Distinction of HE, HPC and ASC from Hemangioma
Large masses
Aggressive characteristics with infiltration
No fat overgrowth
Treatment and Prognosis: HE, HPC and ASC
Surgical resection
Malignant lesions radiation and chemotherapy
Local recurrence common
Metastases common to lung in ASC
Summary:
Osseous Multifocal bone lysis Honeycomb appearance

Look for serpentine vascular pattern MRI
Overgrowth of fat MRI
Multiple associated syndromes and angiomatosis
Higher grade lesions HE, HPC, ASC
Larger aggressive lesions
Infiltrative characteristics
References
1.
2.
3.
4.
5.
6.
Baudrez V, Galant C, Vande Berg BC. Benign vertebral hemangioma: MR-histological correlation. Skeletal Radiol.
2001 Aug;30(8):442-6.
Coldwell DM, Baron RL, Charnsangavej C. Angiosarcoma. Diagnosis and clinical course. Acta Radiol. 1989 NovDec;30(6):627-31.
Fayad L, Hazirolan T, Bluemke D, Mitchell S. Vascular malformations in the extremities: emphasis on MR imaging
features that guide treatment options. Skeletal Radiol 2006; 35:127-137.
Laredo JD, Assouline E, Gelbert F, Wybier M, Merland JJ, Tubiana JM. Vertebral hemangiomas: fat content as a sign
of aggressiveness. Radiology. 1990 Nov;177(2):467-72.
Lorigan JG, David CL, Evans HL, Wallace S. The clinical and radiologic manifestations of hemangiopericytoma.
AJR Am J Roentgenol. 1989 Aug;153(2):345-9.
Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. From the archives of the AFIP.
Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 1995 Jul;15(4):893-917.
811
Paget Disease
Mark D. Murphey, MD
Figure 4-11-1
Paget Disease: Clinical Characteristics
Described 1877 by Sir James Paget

Osteitis deformans
Common disease
3% over age 40
10% over age 80
Slightly more common in men
Common in Great Britain and descendents (USA,
Australia)
Continental Europe
Uncommon in Asia
Many patients asymptomatic (20%)
Paget Disease: Clinical Presentation
Pain
Osseous bowing and enlargement
Neurologic symptoms
High output congestive failure
Lab
Serum alkaline phosphatase (blastic phase)
Urinary and blood hydroxyproline (lytic phase)
Marrow replacement by fibrovascular tissue (*) in active Paget

disease (left image) versus fat (F) in marrow with inactive
disease (right image)
Figure 4-11-2
Paget Disease: Etiology
Unknown
Possible etiologies
Infection; viral; intranuclear inclusions; paramyxovirus
(measles)
Autoimmune
Neoplastic
Paget Disease: Pathology [Figure 4-11-1]
Initially osteoclastic resorption

Subsequently osteoblastic response (active)
Excessive and disorganized
Mosaic or jigsaw pattern
Marrow: fibrovascular reaction
Marrow: fat (inactive)
Paget Disease: Skeletal Distribution [Figure 4-11-2]
Calvarium, spine (lumbosacral) and pelvis: 25%-75%

Proximal long bones: 25%30%
Humerus (31%), scapula (24%), clavicle (11%)
Initially monostotic 10%35%: most polyostotic
Paget Disease: Radiologic Evaluation
Radiographs diagnosis
Bone Scan assess areas involved
CT/MRI: to assess complications or unusual cases
Paget Disease
812
Paget Disease: Radiologic Stages
Active
Lytic - osteoclastic activity
Mixed - majority of cases
Inactive
Blastic - osteoblastic activity
Usually progresses through these phases but not always
Recrudescent lytic phase in patients at rest; simulates tumor
Paget Disease: Radiologic Lytic Phase [Figures 4-11-3 to 4-11-5 ]
Skull: large well-defined areas; involve both inner and outer tables of
frontal/occipital bones (osteoporosis circumscripta)
Long Bones: subchondral location with advancing wedge/V shape blade of
grass/candle flame
Figure 4-11-3
Figure 4-11-4
Figure 4-11-5
Lytic phase of Paget disease with sharp margins and subchondral extension
Osteoporosis circumscripta with sharply

marginated large area of bone lysis
813
Paget Disease
Paget Disease: Radiographs Mixed/Blastic Disease
Sclerosis and lucency

Trabecular and cortical thickening
Along the lines of stress
But some disorganization
Bone enlargement
Figure 4-11-6
Paget Disease: Radiographs: Mixed/Blastic Disease

[Figures 4-11-6 to 4-11-9]
Skull: cotton wool appearance obscures inner and outer

tables, often spares facial bones
Spine: vertebral body (picture frame); ivory vertebral body;
posterior elements may be involved
Figure 4-11-7
Cotton wool appearance on radiograph with

multifocal areas of sclerosis and thickening of
the diploic space anteriorly
Figure 4-11-8
Picture frame appearance of Paget disease of the spine,

multiple levels, on radiograph and coronal macrosection
Figure 4-11-9
Mixed lytic/blastic Paget disease in skull with

diploic space expansion and hyperemic bone
on CT, gross specimen and histology
Ivory Vertebral Body:

Blastic metastasis: breast,

prostate, adenocarcinoma GI
tract, carcinoid, transitional cell
carcinoma bladder
Lymphoma
Chronic infection
Chordoma
Ivory vertebral body in Paget disease on radiograph and

intense uptake on radionuclide bone scan
Paget Disease: Radiographs Mixed/Blastic Phase

[Figures 4-11-10 and 4-11-11]
Pelvis: asymmetric involvement

Thickened iliopubic and ischial lines
Enlarged pubic rami and ischium
Long Bones: epiphyseal involvement
Rarely diaphyseal (tibia)
Enlarged bone
Paget Disease
814
Figure 4-11-10
Figure 4-11-11
Figure 4-11-12
Paget disease mixed lytic and blastic in left

pubic rami
Paget disease (noncomplicated) in right

iliac crest with trabecular and cortical
thickening (arrowheads)
Paget disease with coarsened

trabecular pattern and thickening of
the iliopectineal line involving the
entire hemipelvis
Paget Disease: Bone Scintigraphy
Figure 4-11-13
Active disease marked uptake

Dynamic and static images
Abnormal before radiographs
Overview of disease look at distribution
Monitor disease and therapy
T1
Paget Disease: CT/MRI Noncomplicated

Diseases
[Figure 4-11-12]
Not usually needed for diagnosis

CT- thickened trabeculae
Bone enlargement
Lytic areas
Paget disease involving the calcaneus (noncomplicated),

mixed lytic/blastic on radiograph, with maintained yellow
marrow on T1- weighted MRI
Paget Disease: MRI - Noncomplicated Cases

[Figures 4-11-13 to 4-11-16]
T1-weighted images
Cortically trabecular thickening
Enlarged bone
Low signal (sclerosis)
Yellow marrow/fat (inactive disease)
Heterogeneous signal (active disease)
Marrow replacement non-masslike
T2-weighted images
Low signal (sclerosis)
Fat signal intensity (inactive disease)
Heterogeneous intermediate/high signal (fibrovascular marrow active disease)
No focal mass
Figure 4-11-14
Paget disease mixed lytic/blastic

with cortical thickening (arrow)
involving the tibia
815
Paget Disease
Figure 4-11-16
Figure 4-11-15
T1
T1 GD
Coronal T1-weighted (noncontrast) and axial T1-weighted (after contrast) MR

images show speckled marrow pattern with enhancement in more active Paget
disease (noncomplicated) (*) and more intense enhancement of the intracortical
component that is most active
Paget Disease: Complications
T2
Axial T2-weighted MR image shows

speckled pattern of increased intensity (*)
in more active Paget disease
(noncomplicated-same patient as previous
MRI and radiograph)
Figure 4-11-17
Osseous deformity
Fractures
Neurologic symptoms
Arthropathy
Neoplasm
Paget Disease: Osseous Deformity

[Figure 4-11-17]
Effects of bone softening

Bowing common in long bones
Acetabulae protrusio
Basilar invagination 30% of patients with skull involvement
Paget Disease: Fractures [Figure 4-11-18]
Partial or complete (insufficiency)

True acute fractures
Horizontal lucencies (banana fracture)
Convex surface or bone
Single or multiple
Often symptomatic
Sites: femur, humerus, pelvis
Spine: central compressions
May heal but high nonunion rate
At risk for sarcoma (biopsy?)
Acetabulae protrusio on radiograph and coxa

varus deformity of the femur on the coronally
sectioned macrosection in patients with Paget
disease. Note the axial narrowing of the hip joints
Figure 4-11-18
Paget Disease: Neurologic Symptoms

[Figure 4-11-19]
Symptoms impingement of cranial and spinal nerves

Caused by skull and spine involvement
Bone enlargement, fractures, bone softening with basilar invagination
and increased osseous vascularity with cord hypoxia
CT/MRI for evaluation
Paget disease of femur with complete

and incomplete fractures (arrowheads)
Paget Disease
816
Paget Disease: Arthropathy
Rheumatic diseases with poor association: rheumatoid arthritis,

calcium pyrophosphate deposition (CPPD),
ankylosing spondylitis
Gout higher incidence hyperuricemia (40%) from increased
cell turnover
Osteoarthritis
Hip and knee most common
Abnormal mechanics from deformity
Bone weakening
Hip narrowing can be axial
Soft tissue calcification
Tendinitis and with vitamin D treatment
Figure 4-11-19
Paget Disease: Neoplasm
Sarcomatous transformation
1% patients with Paget disease
5% - 10% patients with extensive disease
Patients 55 to 80 years old
Common sites femur, pelvis, humerus
Osteosarcoma (50% - 60%)
MFH/fibrosarcoma (20% - 25%)
Chondrosarcoma (10%)
GCT
Metastasis, myeloma, lymphoma
Paget disease at C2 (*) with marked osseous

enlargement
Figure 4-11-20
Paget Disease: Neoplasm Radiology

[Figures 4-11-20 to 4-11-22]
Bone destruction predominates

Cortical involvement and soft tissue mass
No periosteal reaction
Persistent nonhealing fracture
Compare with old radiographs
Bone scan: photopenic area
Gallium scan: increased uptake
MRI
T1W: masslike marrow replacement
T2W: focal mass of high intensity
Soft tissue mass
Figure 4-11-21
Pictoral representation of malignant

transformation in Paget disease
Paget disease with malignant transformation to

osteosarcoma in the proximal tibia where there
is mass-like marrow replacement (*) and soft
tissue extension (arrow) on radiograph and
multiple sagittal T1-weighted MR images
817
Paget Disease
Paget Disease: Neoplasm [Figure 4-11-23]
Figure 4-11-22
GCT skull or facial bones

More often benign
Rarely multiple (familial)
Lytic expansile lesion
Metastasis likely related to
hyperemia
Paget Disease:
Diffuse sclerosis chronic renal

failure (CRF), myelofibrosis,
metastasis, lymphoma, sickle cell
anemia
Trabecular thickening hemangioma,
chronic infection, osteomalacia,
fluorosis
Polyostotic lesions CRF
(hyperparathyroidism), Langerhans
cell histiocytosis, unusual infection,
metastasis, fibrous dysplasia,
lymphoma, Gaucher, mastocytosis
Paget disease with malignant transformation to osteosarcoma in the humeral

midshaft where there is mass-like marrow replacement (*) and soft tissue
extension (arrows) on radiograph and multiple sagittal T1-weighted MR
images, CT and gross specimen
Figure 4-11-23
Paget Disease: Treatment
Calcitonin inhibits resorption

Biphoshonates inhibits bone
resorption and production
Mithramycin cytotoxic antibiotic
Often relieve pain
Paget Disease:
Radiology Post-Treatment
Often subtle or no change

Occasionally improved radiographs
Watch for fractures: may increase
with diphosphonates
Bone scans best treatment indicator
Hereditary Hyperphosphatasia:
Juvenile Paget Disease
Paget disease with benign giant cell tumor of the clavicle associated with
Described 1956: Bakwin/Elger
pathologic
fracture showing cortical thickening (arrows) and destructive lesion
Autosomal recessive
(*) on CT scans, gross specimen radiograph and gross specimen
Disorder of infants/children
Elevated alkaline and acid phosphatase, uric acid
Juvenile Paget Disease: Radiographic Findings
Figure 4-11-24
[Figure 4-11-24]
Generalized cortical thickening

All bones including skull involved
Osteopenia and bowing
Epiphyses may be spared
Patients severely deformed
Juvenile Paget disease (hereditary

hyperphosphatasia) on radiographs with
osseous bowing and shortening as well as
trabecular thickening
Paget Disease
818
Paget Disease: Summary
Common disease: 3% - 4%
Diagnosis: Radiographs
Lytic: well defined, subchondral (v/wedge shape candle flame)
Thickened trabeculae and cortex
Osseous enlargement
Bone scan overview
Complications fractures, osseous deformity, neurologic symptoms, arthritis, neoplasm
CT/MRI to evaluate complications
CT/MRI: noncomplicated case
Bone enlargement
T1W: low signal (sclerosis), yellow marrow, heterogeneous non-masslike marrow replacement
T2W: low signal (sclerosis), yellow marrow, heterogeneous high signal, no focal mass
CT/MRI: complicated case
CT: focal bone destruction / soft tissue mass
T1W: masslike marrow replacement
T2W: focal mass in marrow with high signal and soft tissue mass
References
1.
2.
3.
4.
5.
6.
Boutin RD, Spitz DJ, Newman JS, Lenchik L, Steinbach LS.. "Complications in Paget disease at MR imaging."
Radiology. 1998 Dec;209(3):641-51.
Cooper C, Dennison E, Schafheutle K, Kellingray S, Guyer P, Barker D.. "Epidemiology of Paget's disease of bone."
Bone. 1999 May;24(5 Suppl):3S-5S.
Moore TE, Kathol MH, el-Khoury GY, Walker CW, Gendall PW, Whitten CG.. "Unusual radiological features in
Paget's disease of bone." Skeletal Radiol. 1994 May;23(4):257-60.
Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnik CS, Gannon FH.. "From the archives of the AFIP.
Radiologic spectrum of Paget disease of bone and its complications with pathologic correlation." Radiographics.
2002 Sep-Oct;22(5):1191-216.
Vande Berg BC, Malghem J, Lecouvet FE, Maldague B.. "Magnetic resonance appearance of uncomplicated Paget's
disease of bone." Semin Musculoskelet Radiol. 2001;5(1):69-77.
Vellenga CJ, Bijvoet OL, Pauwels EK.. "Bone scintigraphy and radiology in Paget's disease of bone: a review." Am
J Physiol Imaging. 1988;3(3):154-68.
819
Paget Disease
Musculoskeletal Infection: Part I

Mark D. Murphey, MD
Musculoskeletal Infection: Routes of Inoculation
Hematogenous
Contiguous spread
Direct implantation/post-surgical
Hematogenous Vascular Supply

Tubular Bones
Figure 4-12-1
[Figure 4-12-1]
Diaphyseal
Enter through cortex
Ascending/descending branches
Metaphyseal
Sharp turns beneath growth plate
Epiphyseal
Artery from epiphysis/metaphysis
Periosteal
From diaphyseal/muscle/soft tissue vessels
Synovial
From epiphysis/surrounding vessels
Hematogenous Vascular Supply

Tubular Bones
[Figure 4-12-2]
Vascular supply to long bone and joint
Age dependent
Infant vessels penetrate growth plate
Child vessels dont extend across plate
Adult vessels cross closed growth plate
Figure 4-12-2
CHILD
INFANT
ADULT
Vascular supply of tubular bone by patient age
Pathophysiology of Acute Osteomyelitis [Figure 4-12-3, opposite]
Inoculation/colonization/immunologic response
Marrow and soft tissue edema
Bone destruction trabecular/cortical
Subperiosteal/soft tissue/medullary abscess
Vascular thrombosis infarction (sequestrum)
Periosteal new bone (involucrum)
Musculoskeletal Infection I
820
Figure 4-12-3
a) Deposition
b) Extension
d) Superiosteal Lift
e) Stripping
c) Lateral Spread
Progression of osteomyelitis through the cortex into the subperiosteal space (*)
Radiographic Abnormality: Acute Osteomyelitis
Deep soft tissue swelling (within 3 days)

Osteoporosis to focal bone lysis (714 days); magnification views
Periosteal reaction (1014 days)
Increased blood flow: scintigraphy (early)
Decreased blood flow: scintigraphy (later)
Other Radiologic Studies: MSK Infection
Nuclear Medicine BS: 3 phase studies

Gallium: with BS, active infection
WBC: increased specificity
Sonography fluid collections/effusions
CT bone destruction/sequestra/abscess
Sinography sinus tract extent
MRI: marrow involvement, abscess very sensitive (STIR, GADO)
Osteomyelitis: Organisms
Staph aureus (80% - 90% of pyogenic cases)

H. flu, strep (shoulder, trauma from delivery)
Gram negatives, uncommon except enterics (25% of infections)
Pseudomonas penetrating trauma/IVDA
Salmonella sickle cell
Blood cultures positive 50%
821
Hematogenous Osteomyelitis: Infant (Up to 12 Years)

[Figures 4-12-4 to 4-12-7]
Figure 4-12-4
May be clinically silent (NICU/catheters)

Group B strep more common than other ages
Metaphysis/epiphysis location
Extend into joint
Most prominent sequestrum/involucrum
Common soft tissue/subperiosteal abscess
Fracture/sinus tracts uncommon
May lead to late sequelae
Figure 4-12-5
Pictorial
representation of
infection
deposition sites
in an infant long
bone
Figure 4-12-6
Osteomyelitis in an infant with soft tissue swelling (circle and

arrow) as the only initial finding. Compare to normal left arm
(right image)
Figure 4-12-7
Subsequent radiographs 1 week (left image) and two
weeks (right image) later reveal development of
periosteal reaction initially (arrows) followed by extensive
involucrum (arrowheads) and sequestrum (*) formation
Figure 4-12-8
Continued follow-up radiographs 1 month (left image), 3

months (middle image) and 1 year (right image) later show
progressive resorption of sequestra representing the majority of
the humerus (*-bone within bone appearance) and
subsequent remodeling to nearly normal appearance
Hematogenous Osteomyelitis: Child (116 Years)

[Figures 4-12-8 to 4-12-13]
Tubular bones 75%

Metaphysis/lower extremity
Can involve joint (hip /shoulder)
Sequestrum/involucrum common
Soft tissue/subperiosteal abscess common
Fracture uncommon
Sinus tracts can occur
Pictorial representation of infection

deposition sites in a child long
bone
822
Figure 4-12-9
Figure 4-12-10
Osteomyelitis in the distal femoral metaphysis of the

femur with bone destruction (arrows).
Aggressive bone destruction (arrow) and periosteal reaction

(arrowheads) in the distal ulnar metaphysis on radiograph and
marked uptake of radionuclide on bone scan resulting from
acute bacterial osteomyelitis in a child
Figure 4-12-12
Figure 4-12-11
There is marrow replacement on the T1-weighted MR image with focal rim enhancing subperiosteal abscess (arrowheads) posteriorly
showing high signal on T2-weighting (*)
Figure 4-12-13
Subperiosteal Abscess
[Figure 4-12-14]
Common in children/infants
Loose periosteum
Femur/tibia long extension
Adults sinuses/orbit
Often dont recognize on Figure 4-12-14
radiographs
Nuclear Medicine rim
with photopenia (BS,
WBC)
CT/MR/SONO fluid
collection bone surface
Pictorial presentation of
subperiosteal abscess
Civil war gross specimen and radiograph showing

extensive involucrum (arrows) and sequestrum (*)
resulting from war injury induced osteomyelitis
823
Hematogenous Osteomyelitis: Adult (Over 16 Years)

[Figures 4-12-15 to 4-12-17]
Tubular bones less common

Spine/pelvis/small bones more common: subchondral focus
Joint involvement/sinus tracts common
Involucrum/sequestrum/abscess uncommon
Fracture uncommon except neglected cases
Figure 4-12-15
Figure 4-12-17
Figure 4-12-16
Acute bacterial osteomyelitis in an adult. Initial radiograph reveals only soft tissue swelling
posterior to olecranon (*). Subsequent radiographs two weeks (right images) later show motheaten to permeative bone destruction resulting from staph aureus osteomyelitis
Pictorial representation of infection

deposition sites in an adult long
bone
Subacute Osteomyelitis: Brodie Abscess

[Figures 4-12-18 and 4-12-19]
Described in 1832 chronic/subacute

Walled-off with central fluid, often sterile (staph)
Children (M>F), metaphysis, tibia
Intramedullary - channel-like lucencies
May cross growth plate or be cortical
Periosteal reaction/sequestra may be seen
Figure 4-12-18
Figure 4-12-19
Brodie abscess with sequestra (arrows) in the cortex of

the proximal femur on radiograph, axial CT and coronal
CT reconstruction. Debrided sequestered fragment is
also demonstrated (*)
824
Chronic Osteomyelitis [Figure 4-12-20]
Bone formation results in sclerosis

Sclerosing osteomyelitis of Garre
No pus, may have mixed radiolucency
Active infection: new bone destruction, sequestra, aggressive periosteal
reaction on radiographs
MRI, scintigraphy (bone, gallium, WBC)
Figure 4-12-20
Osteomyelitis/Septic Arthritis
Contiguous Spread: Source
Soft tissue infection

Trauma
Human/animal bites
Puncture wounds
Ulcers
Surgery
Contiguous Spread: Radiographs
Soft tissue swelling/air

Periosteal reaction
Cortical destruction into marrow
Joint space loss
Chronic osteomyelitis of the ulna

with diffuse sclerosis on
radiograph
Figure 4-12-21
Contiguous Spread: Hand
Tendon sheaths, fascial planes, lymphatics

Felon-volar tuft destruction
Paronychia-dorsal tuft destruction
Bites
Human: MCP joint with fight (Staph/strep)
Animal: dog (90%), 5% infected;
cat (10%), 20%50% infected
(Pasteurella multocida)
Contiguous Spread: Other Sites [Figure 4-12-21]
Foot pathways: medial, intermediate, lateral

Puncture: pseudomonas
Diabetes: multiple organisms
Aerobic/anaerobic
Pelvis decubitus ulcers (paraplegics)
Ischial tuberosity chronic osteomyelitis
Post-operative: ring sequestra
Diabetic patient with ulcer lateral to fifth MTP joint and

radiograph shows underlying destruction of distal fifth
metatarsal head and proximal phalanx resulting from
osteomyelitis and pyarthrosis. Amputation specimen shows
similar findings
Osteomyelitis: Diabetes Mellitus
Bone destruction/periosteal reaction

Bone scan normal no osteomyelitis
WBC scan good predictive value
MRI marrow replacement geographic area T1 and
increased signal STIR, post gado
Normal on T1 or subtle/subcortical only; then
probably marrow reaction not osteomyelitis
(Collins et al AJR 185:2005)
Focal fluid collections
Figure 4-12-22
Osteomyelitis vs Neuropathic
Diabetes Mellitus
Factors favoring infection: Sinus tract (84%/0%); ST

replacement (68%/32%); Fluid collection
(95%/48%); Extensive marrow abnormality
(41%/12%)
Factors favoring neuropathic: Thin rim enhancement
of effusion (68%/21%); Presence of subchondral
cysts (76%/2%); Intraarticular bodies (53%/12%)
Ahmadi et al, Radiology 238; 622-631, 2006
825
Chronic osteomyelitis with sinus tract and secondary

epidermoid carcinoma. Aggressive bone destruction is seen
distally and medially corresponding to malignancy (arrow
and *) as sinus tract enters bone
Complications of Osteomyelitis [Figure 4-12-22]
Figure 4-12-23
Sequestra discharged through sinus tracts

Avascular necrosis
Fracture/slipped epiphysis
Growth plate disturbance
Osteolysis
Systemic amyloidosis (rare)
Epidermoid carcinoma
0.5% long term draining sinus
M>F, 2040 year latent period
Tibia/femur
Arise deep in sinus tract
Septic Arthritis: Bacterial
Cause
Hematogenous, contiguous spread, direct implantation, postsurgical
Polyarticular 20%
Organisms
H. flu leading cause up to age 2 years
Staph, alpha/beta hemolytic strep.
Pneumococcus, E.coli, Pseudomonas
Figure 4-12-24
Septic Arthritis: Pathology [Figure 4-12-23]
Pictorial representation of septic arthritis
Synovial inflammation /hyperemia/fluid production

Fibrin deposits inhibit cartilage nutrition
Attract WBCs release enzymes (collagenase)
Pannus formation
Cartilage destruction/bone erosion
Septic Arthritis: Radiology [Figure 4-12-24]
Soft tissue swelling / joint effusion (sonography)

Periarticular osteopenia
Increased vascularity scintigraphy
Pannus arthrography gadolinium enhanced
MRI
Septic arthritis and osteomyelitis of the fourth MTP joint with joint
narrowing and bone destruction (arrowhead)
Figure 4-12-25
Septic Arthritis:
Hip Infant/Childhood [Figure 4-12-25]
Staphylococcus aureus
Spread from adjacent osteomyelitis
Radiologic signs
Widened hip joint/effusion (sonography)
Displaced pericapsular fat planes
Surgical emergency
Septic Arthritis:
Complications/Sequelae [Figure 4-12-26]
Avascular necrosis
Slipped epiphysis
Growth disturbance
Osteomyelitis
Secondary osteoarthritis
Synovial cyst, tendon/capsule injury
Septic arthritis in the right hip of a young child with joint widening
indicating a joint effusion on radiography
Figure 4-12-26
Soft Tissue Infection
Septic bursitis usually injury

Staph/prepatellar (child)
Septic tenosynovitis
Cellulitis
Necrotizing fasciitis
Soft tissue abscess pyomyositis
Staph (90%)
Complication of septic arthritis with right hip
osseous ankylosis on radiography
826
Soft Tissue Infection: Radiology [Figure 4-12-27]
Figure 4-12-27
Radiographs
Soft tissue swelling
Air (rare): clostridia, E. Coli (coliform), bacteroides
CT/MRI/Sonography
To detect focal abscess
Contrast enhancement of rim
Cellulitis
Acute inflammatory process of deep subcutaneous tissues

Location
Extremities, thorax, abdomen, neck
Organisms
Streptococci, staphylococci, H. influenza
Necrotizing Fasciitis: Clinical
Infection and necrosis of fascia (Staph/Strep)

Important to distinguish from cellulitis
Systemic severe toxicity - IVDA
Extremities, neck, face, perineum
High mortality (>70% if not treated)
Need prompt aggressive treatment
TreatmentFasciotomy, debridement,
antibiotics
Necrotizing Fasciitis: Imaging
Skin thickening
Subcutaneous edema and air
Involves deeper tissue unlike cellulitis
Focal fluid collections (abscess)
Gadolinium enhancement
Chronic Granulomatous Disease

of Childhood
X-linked recessive (males)

WBCs cannot kill organisms
Skin lesions, lymphadenitis,
Soft tissue abscess in the buttock on CT and MR showing focal fluid collection
hepatosplenomegaly
(*) with internal foreign material (arrows) after accident
Pneumonias, chronic osteomyelitis
(25%35%)
Fatal 40% cases low virulent organisms
Symptoms of osteomyelitis limited
Bone destruction/limited sclerosis
Small bones hands/feet, chest wall, spine
Figure 4-12-28
Chronic Recurrent Multifocal Osteomyelitis

(CRMO) [Figure 4-12-28]
Chronic symmetric plasma cell osteomyelitis

Indolent/recurrent bone infection
Age 515 years; knee metaphyses, clavicle, face
Mixed lysis/sclerosis
May be sterile, plasma cells, lymphs and histiocytes
SAPHO (Synovitis, Acne, Pustular rash palms/soles,
Hyperostosis, Osteitis)
Musculoskeletal Infectionn Drug Abusers
Altered WBC function and infected needles

S joints spine, sacroiliac, and sternal joints
AC joint, symphysis, ischial tuberosity
Pseudomonas, Klebsiella, Serratia
Candida in heroin addicts
CRMO with patchy areas of lucency and sclerosis in the distal

tibial metaphysis on radiography. Contralateral ankle, ipsilateral
knee and iliac bone showed similar findings (not shown)
827
Spine Infections: Routes of Contamination
Hematogenous
Arterial
Venous: Batson Plexus
Contiguous source
Direct implantation/post-operative
Discography (-ectomy), biopsy, laminectomy
Spine Infections: Clinical Characteristics
Spondylodiskitis
2%4% of all osteomyelitis cases
M>F (1.53:1); age 4060 years
Lumbar > thoracic > cervical/sacrum
Vertebral body
History: recent primary infection
Symptoms: back pain, fever, neurologic
Figure 4-12-29
Spine Infections: Pathology
Staph aureus (55%90%)

Localizes to anterior subchondral bone
Rapidly extends into disc (13 weeks)
Can extend into paravertebral soft tissue
Spine Infections: Radiography
Initially radiographs normal or subtle

subchondral destruction
Usually seen radiologically after disc involved
Rapid disc narrowing with irregular endplate
destruction
Later osteosclerosis (1012 weeks)
Treatment: diffuse sclerosis, DDD, disc ankylosis
Bacterial spondylodiskitis with rapid disk space loss and endplate

destruction over two weeks (right image) due to staph aureus
infection. Left image initial radiograph
Spine Infections: CT/MR Imaging [Figure 4-12-29]
MR imaging optimal
Bone destruction/marrow replacement (T1W)
High signal T2W MR images
Disc and adjacent vertebra
Post gadolinium images helpful
Focal abscess detection
Paravertebral soft tissue masses (20% pyogenic cases)
Anterior and lateral (psoas)
Epidural
Spine Infections: Differential Diagnosis
Amyloid in CRF
Low signal T2W MR images
Tumor-crossing disc
Chordoma, lymphoma, myeloma, GCT
Other causes disc narrowing (well-defined bone margins)
DDD, CPPD, neuropathic, RA, trauma, sarcoid
Invertebral Discitis:
Hematogenous children (116 years)

Disc vascularized
Symptoms mild after primary infection
Cultures negative (50%90%); staph aureus
Antibiotics given empirically
Intervertebral Discitis: Radiology
Scintigraphy positive early

Late disc narrowing and erosion
MRI findings similar to adults
Paravertebral soft tissue changes unusual
Disc often reconstitutes after therapy but may remain deformed with sequelae
828
Musculoskeletal Infection
Part II: Atypical Organisms
Mark D. Murphey, MD
Unusual Bacterial Musculoskeletal Infection
Brucella
Mycobacteria (acid-fast bacilli)
Tuberculosis
Atypical
Leprosy
Actinomycosis
Brucellosis
Malta fever undulant fever

WHO 500,000 cases annually
B. abortis, melitensis, suis, canis
Endemic Midwest USA, Saudi Arabia, South America, Spain, and Italy
Ingested milk/meat reticuloendothelial system (marrow)
Brucellosis [Figure 4-13-1]
Musculoskeletal infections 30%85%

Septic arthritis knee, sacroiliac joint, shoulder
Prepatellar bursitis
Spine 53% lumbar (lower L4)
Focal: subchondral anterior superior endplate (parrot beak phyte)
Diffuse: vertebral body maintained, less disc and paravertebral
involvement, disc gas (25%30%)
Al-Shahed, Radiographics 94:14:333-348
Figure 4-13-1
Brucellosis spondylodiscitis on radiograph,

CT and sagittal T1- and T1- weighted postcontrast MR images shows multilevel
involvement of the discs and vertebral
bodies (arrows,open arrows,arrowheads
and *) and paraspinal and anterior epidural
inflammatory changes (curved arrows and
arrows on MR). Note the lack of vertebral
collapse and parrot beak
(open arrows on radiographs) osteophytes
Musculoskeletal Tuberculosis: Clinical

Characteristics
Increasing immunocompromised patients

1%3% of TB patients MSK involvement
Pain, swelling, stiffness long delay to diagnosis
Mortality still significant
Negative skin test MSK TB unlikely
Negative CXR (child) MSK TB unlikely
829
Musculoskeletal Infection II
Musculoskeletal Tuberculosis: Pathology

Hematogenous, pulmonary changes 50%
Tubercles: central giant and epithelioid cells
Central caseating necrosis (may calcify)
Peripheral: lymphocyte mantle
Synovial tissue/fluid: 80%90% positive culture
Musculoskeletal Tuberculosis: Sites of Involvement
Spine: 25%60% of MSK cases

Other osseous sites unusual
Dactylitis
Arthritis knee, hip
Bursitis/tenosynovitis hand/wrist
BCG related
Tuberculous Spondylodiscitis
May result in neurologic symptoms

Hematogenous venous (Batson) plexus
L1 most common: decreases above/below
More than one level frequent
Usually contiguous
Separate foci: 1%4%
Tuberculous Spondylodiscitis: Radiology

[Figures 4-13-2 to 4-13-7]
Subchondral vertebral body (25 months)

Anterior 82%, posterior 18%
Disc extension
Less common involvement posterior elements, isolated vertebral body (ivory)
Subligamentous extension gouge defects
Kyphosis (gibbus) thoracic
Paraspinal extension psoas (L1-L5)
Calcification: amorphous/teardrop
Abscess (5% psoas)
MR imaging usually optimal
Assess extent/relationship to canal
Abscess shows rim enhancement
Tuberculous Spondylodiscitis: Differential Diagnosis Pyogenic
Can be difficult
Findings favoring tuberculosis
Multilevel involvement
Slow vertebral/disc destruction
Calcified paraspinal mass
Lack of sclerosis
Figure 4-13-2
Tuberculous Osteomyelitis
Not common, usually with arthritis

Often epiphyseal, any bone, joint
Children metaphyseal cross plate
Cystic variety multifocal defined lytic foci
Dactylitis < age 5 (0.5%14% cases)
Multifocal (25%35%)
Spina (spike-like) ventosa (puffed full of air)
Pictoral representation of tuberculous

spondylodiscitis
830
Figure 4-13-3
b
Tuberculous spondylodiscitis:
a) Radiographs show myelographic block (arrowhead) with
endplate destruction, collapse, and disc involvement (arrow);
b) CT reveals large paraspinal mass (*);
c) Sagittal T1-weighted MR images show marrow replacement
and disc involvement at two levels (arrowheads) as well as
anterior paraspinal and posterior epidural masses (arrows) ;
d) Sagittal and axial post-contrast T1-weighted MR images
reveal rim enhancement about anterior paraspinal and
posterior epidural abscesses (arrows);
e) High signal intensity is seen on the axial and sagittal T2weighted MR images in the involved vertebrae, discs and
paraspinal abscesses (*)
Figure 4-13-5
Figure 4-13-4
Anterior gouge defects on sagittal T2-weighted MR image and

sagittal macrosection resulting from tuberculous
spondylodiscitis extending under the anterior longitudinal
ligament (arrows) and invading other vertebral segments
(arrowheads)
Sagittal dried bone specimen and gross specimen show

effects of tuberculous spondylodiscitis with gibbus deformities
(arrows) and anterior epidural inflammatory mass (*)
displacing the spinal cord (c) posteriorly
831
Figure 4-13-7
Figure 4-13-6
Teardrop paraspinal calcification (*) in

a tuberculous paraspinal abscess
related to spondylodiscitis
CT of the spine in a patient with tuberculous spondylodiscitis and

calcified paraspinal abscesses (arrows)
Figure 4-13-8
Tuberculous Arthritis [Figure 4-13-8]
Large joints (knee/hip); monoarticular

Synovial thickening covered by fibrin
Granulation tissue erodes cartilage bone
Slow process/lack proteases
Areas of cartilage contact spared
Tuberculous Arthritis: Radiology

[Figures 4-13-9 and 4-13-10]
Phemister triad
Juxtaarticular osteopenia
Slow joint space loss
Peripheral erosions
Joint effusion
MRI nodular synovial thickening
Less reactive bone, periostitis, osseous ankylosis
Figure 4-13-9
Pictorial representation of tuberculous arthritis (right

image, left image normal joint)
Figure 4-13-10
Pelvis radiographs over a
two month interval show
slow pancompartmental
loss of the right hip joint
resulting from tuberculous
arthritis
Radiograph of tuberculous arthritis with diffuse joint space loss,

marginal erosions (arrows) and osteopenia (Phemister triad) and
sagittal macrosection showing fibrinous exudate (*)
throughout the joint
832
Atypical Mycobacterium [Figure 4-13-11]
Early diagnosis/treatment important skin/pulmonary
Types photochromogens (M. Kansasii),
nonchromogens (M. avium); rapid growers
Bone/joint multiple lesions, less osteopenia, hand/wrist,
metaphysis/diaphysis lysis/sclerosis, abscess/sinus tracts common
Figure 4-13-11
Leprosy: Mycobacterium Leprae
Africa, South America, Asia

USA Texas, Louisiana, Hawaii, Florida
Long incubation 36 years
Infection through skin/mucous membranes
M>F; usually detected before age 20
Leprosy: Pathology Types
Lepromatous many bacilli more severe/generalized

Tuberculoid more reaction/less bacilli/skin & nerve involvement
Dimorphous features of both
Indeterminate
Leprosy Direct: Specific Signs [Figures 4-13-12 and 4-13-13]
Related to bacilli presence

Uncommon 1%2% findings
Small bones hands/feet (direct spread)
Punched out or lacelike osseous destruction phalanges, nasal bone
Periosteal reaction, fragmentation, arthritis
Figure 4-13-12
Sagittal T1-weighted and axial T2-weighted

MR images reveal marked thickening about
the second flexor tendon (arrowheads)
related to atypical mycobacteria
tenosynovitis. Chronic fibrosis causes the
intermediate signal on T2-weighting (arrow)
Figure 4-13-13
Lattice-like lucencies in the phalanges representing a direct
sign of leprosy infection on radiography
Actinomycosis: Clinical Characteristics
Anaerobic higher bacteria; acid fast-like

Actinomyces (Israelii), nocardia (asteroides)
Normal flora in mouth
Trauma often results in inoculation
Xeroradiography shows medial distal arm calcification that is

located in the ulnar nerve (*) on the resected gross specimen
resulting from leprosy
833
Actinomycosis: Radiology [Figure 4-13-14]
Figure 4-13-14
Sites mandible, axial skeleton (rib, spine, pelvis),

large joints
Lysis and sclerosis (bone proliferation)
Spine (posterior elements) and rib
Sinus tracts/abscesses (no calcification) common
Spirochetes
Syphilis
Yaws
Lyme disease
Tropical ulcer
Bejel, rat bite, fever, leptospirosis
Actinomyces osteomyelitis in the mandible on radiograph

showing patchy areas of destruction and sclerosis (arrowheads)
Congenital Syphilis: Early Changes [Figure 4-13-15]
Toxic effects degenerating spirochetes/infection

Osteochondritis metaphyseal lucent
bands/irregularity
Long bone (tibia: Wimberger sign), rib, spine, sternum
Epiphyseal widening/separation
Heal quickly with treatment
Osteomyelitis diaphyseal lysis/sclerosis/periostitis
Diffuse periostitis
Figure 4-13-15
Congenital Syphilis: Late Changes
Congenital syphilis 75% diagnosed after age 10

Hutchinson triad (Hutchinson teeth, interstitial keratitis,
nerve deafness)
May be similar to early changes
Usually more like acquired findings
Dactylitis fingers more common
Painless knee effusions (Clutton joints)
Congenital Syphilis: Early Changes
Congenital syphilis of the lower extremity showing

periosteal reaction (arrows) and osteochondritis
(arrowheads) of the proximal tibiae (Wimberger sign)
Bone changes in up to 20% (early as 6 weeks)

Proliferative periostitis
Most common finding
Tibia (saber shin), skull, ribs, sternum
Solid/laminated (rarely perpendicular)
Osteomyelitis/osteitis/septic arthritis
Much less common
Skull: outer table aggressive lysis
Nasal bone collapse saddle nose
Congenital Syphilis: Late Changes
Periostitis/osteomyelitis/osteitis
Gumma any bone caseous necrosis related to degenerating spirochete
Bone resorption: Carries sicca
Bone lysis/reactive sclerosis
Arthritis uncommon ankles, MTP, elbows, knees
Swelling, effusion, narrowing, destruction
Neuropathic (5%10%) knee, hip, ankle, spine
Yaws
Treponema pertenue
Africa, South America, South Pacific
Very similar to syphilis less nose changes, more phalanges (spares distally)
Exostosis maxilla goundou
Tropical Ulcer
Central/East Africa
Lower leg ulcer destroys muscle/tendon
To bone focal osseous production tibia/fibula
Multiple organisms including spirochetes
Epidermoid carcinoma 25% (>10 years latency)
834
Lyme Disease: Clinical Characteristics
Figure 4-13-16
Recognized 1975; 23,763 new cases (2002)

Most common vector borne illness in USA
USA (NE/MidAtlantic), Europe, Far East, Australia
Tick bite - ixodes dammini (transmissiom)
Spirochete - Borrelia burgdorferi (deer, mice)
Skin lesions (erythemia chronicum migrans 20%)
Joint symptoms (usually 2-6 mths; 2 weeks - 2 yrs)
80% cases reported May-August
Lyme Disease: Radiology [Figure 4-13-16]
Knee (80%), shoulder, elbow, temporomandibular, ankle, wrist,

hip, hand/foot
Monoarticular, oligo, or polyarticular
Soft tissue swelling/effusion (MRI synovitis)
Adenopathy, myositis, lack subcutaneous edema
Chronic changes (10%) osteopenia, joint loss (25%),
erosions, secondary carcinoma
Musculoskeletal Infection: Fungal [Figures 4-13-17 to 4-13-20]
Aspergillosis
Blastomycosis
Candidiasis
Cryptococcosis
Histoplasmosis
Mucormycosis
Sporotrichosis
Madura Foot
Fungal Musculoskeletal Infection:

Common Changes
Frequently in immunocompromised
Osteomyelitis large punched out lytic lesions
May have surrounding sclerosis
Often multifocal/may involve bone protuberances
Joint involvement slow progressive destruction
MRI: nodular synovial thickening
Aspergillosis
Lyme arthritis of the knee on lateral radiograph with effusion

(* ) and MR revealing synovitis (arrows), myositis (M) and
adenitis (A)
A. Fumigatus normal URT organism

Sites related to pulmonary changes (children) or
Hematogenous (adult): rib, sternum, spine
Arthritis rare
Figure 4-13-17
Blastomycosis
B. Dermatiditis (N. American)

Ohio, Miss. River Valleys, Mid-Atlantic
B. Brasiliensis Mexico, Central/South America
Skin/pulmonary entry from soil
Bone involved in up to 60% patients
Ribs, spine, tibia, carpus, tarsus, skull
Arthritis <10% patients
Candidiasis (Moniliasis)
C. Albicans but many other species

Patients on hyperal, antibiotics, intraarticular steroids
Bone involvement rare (1%2%)
Monostotic/monoarticular long bone, sternum, spine, knee
Arthritis more often precedes osteomyelitis
Coccidioidomycosis
C. Immitis soil- inhalation

SW USA, Mexico
<1% disseminated; (10%50% MSK changes)
Metaphyseal, may be symmetric
Joints ankle, knee, also migratory arthritis
Aspergillus infection with pulmonary

and rib (arrowheads) involvement
835
Cryptococcosis
Figure 4-13-18
Torulosis, C. Neoformans
Soil inhalation
Disseminated disease, 5%10% MSK changes
Arthritis unusual
Histoplasmosis
H. Capsulatum USA (Miss. River Valley)

H. Dubosii Africa MSK changes 80% cases
Soil inhalation; most common fungal in USA
Pelvis, skull, ribs, small tubular bones
Arthritis knee, ankle, wrist, hand
Mucormycosis
Phymycetes rhizopus, mucor, absidia

Diabetes, uremia, burns
Entry via sinuses
Skull/face (maxillary/ethmoid sinuses)
Bone destruction
Sporotrichosis
Blastomycosis of the humerus with

Sporothrix schenckii
extensive involvement demonstrating
Saprophyte on vegetation
mixed lysis and sclerosis
Inhalation/skin wound (rose thorn)
Disseminated form 80% MSK changes
Arthritis (monoarticular) common knee (66%) hand wrist (52%) ankle, elbow
Osteopenia often not prominent
Osteomyelitis due to arthritis
Figure 4-13-19
Figure 4-13-20
Sporotrichosis of
the knee with CT
(post-arthrogram)
and MR showing
enhancing
nonspecific
nodular synovial
thickening
(arrowheads) after
intravenous
contrast
Histoplasmosis with multifocal area of lysis, many of which

involve tuberosities and trochanteric regions ("lumps and
bumps" of bone)
836
Madura Foot: Mycetoma [Figure 4-13-21]
Figure 4-13-21
Chronic granulomatous infection

Foot (65%70%), hand, legs, back/head
Many organisms can be cause Eumycetes
(Madurella), Actinomycetes, Monosporium
Apiospermum (USA)
Tropics India, Africa, Arabia, Latin America
Tarsals/metatarsals most involved
Viral/Protozoan Musculoskeletal Infection
Torch metaphyseal lucent bands (celery stalking)

Cat-scratch disease viral like bacteria AFIPIA felis
(R. Hensalae and Bartanella species)
Look for adenitis (epitrochlear)
Aids: Musculoskeletal Changes [Figure 4-13-22]
Seronegative arthropathy
Osteomyelitis, septic joint/bursitis any organism
Pyomyositis (staph); lower extremity (95%), multiple
(50%)
Bacillary angiomatosis (rochalimaea hensalae,
quintana) skin lesion/bone destruction (cortex
prominent)
Neoplasm Kaposi sarcoma, lymphoma
Helminths/Worms
Musculoskeletal changes usually soft tissue

calcification
Loa loa - Africa subcutaneous calcification (fine
lace-like or thicker bead-like)
Filariasis lymphatic obstruction (elephantiasis)
Guinea worm (dracunculosis) long calcification
female worm lower extremity hand can cause
arthritis
Cysticercosis linear/oval rice grain calcification
along axis of muscle (up to 2025 mm length)
Echinococcus: Musculoskeletal Changes

[Figure 4-13-23]
Echinococcus (E. multilocularis/granulosis)

Bone disease: 0.5%4% (E. granulosis)
Spine, long bone epiphysis,iIlium, skull, rib
Multiloculated (bundle of grapes) lysis/expansion
May be soft tissue loculated, cyst margins may
calcify
Mycetoma (Madura foot) on radiographs, sagittal T1-and T2weighted MR images and gross specimen show extensive
multifocal destruction with sclerosis/fibrosis representing
chronicity
Figure 4-13-22
Sarcoid: Musculoskeletal
Usually have chest changes (80%90%)

Muscle myositis (50%80% patients)
MRI low signal central scar
Subcutaneous soft tissue nodules (5%)
Arthritis (10%35%) acute/chronic
Hand, wrist, ankle, knee, elbow
Sarcoid - Musculoskeletal:
Osseous Changes [Figures 4-13-24 and 4-13-25]
1%13% of patients; may be asymptomatic

Lattice like lysis (hands)
Punched out lytic lesions
May appear aggressive
Acroosteolysis, acrosclerosis (30%50%)
Areas of sclerosis
Marrow replacement creating mottled appearance
(MRI)
837
Coronal STIR MR shows a focal fluid collection (*)

representing pyomyositis in an HIV patient
Figure 4-13-23
Figure 4-13-24
Typical lattice like multifocal lucencies of several phalanges

resulting from sarcoid
Paraspinal echinococus infection with multiloculated fluid filled

cysts (*) on axial T1- and T2-weighted MR images and gross
specimen
Figure 4-13-25
Sarcoid marrow involvement showing heterogeneous or mottled

marrow signal intensity diffusely on sagittal T1-weighted cervical
spine MR images
References (Musculoskeletal Infection Parts 1 and 2)

1.
2.
3.
4.
5.
6.
7.
Erdman WA, Tamburro F, Jayson HT, Weatherall PT, Ferry KB, Peshock RM. Osteomyelitis: characteristics and
pitfalls of diagnosis with MR imaging. Radiology. 1991 Aug;180(2):533-9.
Jung NY, Jee WH, Ha KY, Park CK, Byun JY. Discrimination of tuberculous spondylitis from pyogenic spondylitis
on MRI. AJR Am J Roentgenol. 2004 Jun;182(6):1405-10.
Lawson JP, Rahn DW. Lyme disease and radiologic findings in Lyme arthritis. AJR Am J Roentgenol. 1992
May;158(5):1065-9. Review.
Palestro CJ, Kipper SL, Weiland FL, Love C, Tomas MB. Osteomyelitis: diagnosis with (99m)Tc-labeled
antigranulocyte antibodies compared with diagnosis with (111)In-labeled leukocytes--initial experience. Radiology.
2002 Jun; 223(3):758-64.
Sharif HS, Aideyan OA, Clark DC, Madkour MM, Aabed MY, Mattsson TA, al-Deeb SM, Moutaery KR. Brucellar
and tuberculous spondylitis: comparative imaging features. Radiology. 1989 May;171(2):419-25.
Sharma P. MR features of tuberculous osteomyelitis. Skeletal Radiol. 2003 May; 32(5):279-85. Epub 2003 Mar 25.
Unger E, Moldofsky P, Gatenby R, Hartz W, Broder G. Diagnosis of osteomyelitis by MR imaging. AJR Am J
Roentgenol. 1988 Mar;150(3):605-10.
838
Imaging of Cervical Spine Trauma

Mark D. Murphey, MD
Cervical Spine Trauma: Demographics
Most common portion of spine injured

Responsible for 65% of all spinal injuries
Mechanism: MVA/Fall/Sports injury
Spinal cord injury: 40% (10,000 annually)
Cervical Spine Trauma: Patterns
Areas most commonly involved

C1 2 (particularly in children)
C5 7
Other fractures 20%
Particular association of low cervical fracture with high thoracic and
thoracolumbar injury
Cervical Spine Trauma: Radiographic Signs - Normal
ABCS - alignment, bone integrity, cartilage (joint/disc space), soft tissues

Lateral view - anterior/posterior vertebral body arcs
Spinolaminal arc (except childhood pseudosubluxation C2-3)
AP view-spinous and lateral mass arcs
Cervical Spine: Normal Measurements
Lateral atlantoaxial offset

(open mouth view) - 2 mm
Predental space - 3 mm adult; 5 mm child
Anterior vertebral height vs. posterior
2 mm (except C5)
Pretracheal space at C6 - 22 mm adult,
14 mm child
Facet width 2 mm
Listhesis with flexion/extension - 2 mm
Retropharyngeal space at C2 - 7-8 mm
Exceptions: ET/NG tubes; inflammatory process/crying child
Interspinolaminar space - 2 mm
Between 3 continuous levels
Cervical Spine Trauma: Radiographic Evaluation
Standard 3 view series

AP, lateral, open mouth
Kasabach view modified oblique open mouth
Oblique views
Trauma oblique views
Swimmer (Twining) views
Upright, lateral, flexion, extension
Trauma Oblique: Radiographs
Developed by Gehweiler and Abel

X-ray tube angled 30o - 40o from horizontal
Add 15o cranial tube tilt
Better than Swimmer view for cervico-thoracic junction
Flexion and Extension: Radiographs
Use upright lateral first to evaluate cervical spine straightening/reversal

To evaluate abnormal alignment/stability
False negative from muscle spasm
Repeat after delay
Performed under physician guidance
Passive motion
839
Cervical Spine Trauma: CT
Indispensable, widely available, rapid study

1-3 mm sections, coronal/sagittal reconstructions
Spiral/Multichannel/Holography
3D helpful to depict spatial relationships
Cervical Spine Trauma: MR Imaging/Myelography

MRI indications
Post traumatic cervical myelopathy/radiculopathy
Clinical symptoms unexplained by other
radiologic studies
Assess ligamentous injury
Myelography (CT) largely replaced by MRI
CSF obstruction
Nerve root avulsion, dural tear
Figure 4-14-1
Cervical Spine Trauma: Stability
Mechanical ability to not deform under physiologic

stress
Neurologic potential to produce new or increase
previous deficit
Acute/Chronic
Cervical Spine Trauma:

Radiographics Signs Instability
Widened interspinous spaces (>2 mm)

Widened apophyseal joints (>2 mm)
Anterior listhesis > 3.5 mm
Narrowed/widened disc space
Focal angulation of >11o
Vertebral compression > 25%
Pictoral representation of flexion sprain cervical injury with

distraction forces posteriorly causing interspinous widening or
fanning (*)

Classification by Mechanism
Hyperflexion
Modified by rotation/lateral flexion
Hyperextension
Modified by rotation
Axial loading burst
Complex, poorly understood mechanism
Figure 4-14-2

Hyperflexion Injuries
Account for 50% - 80% of injuries

Flexion forces maximal at C4 C7 anterior;
distraction posterior
Sprain; compression fracture
Facet fracture/subluxation/dislocation
Flexion teardrop fracture
Clay (coal) shovelers fracture
Lateral flexion fractures unilateral occipital
condyle/lateral mass C1
Uncinate or transverse process
Cervical Spine Trauma: Hyperflexion Sprain

[Figures 4-14-1 and 4-14-2]
Lateral radiograph of flexion sprain cervical injury with

distraction forces posteriorly causing interspinous widening or
fanning at C6-7 (arrows)
Disrupted one-level posterior ligaments by distraction

Acute focal pain/limited ROM
Delayed instability (30% - 50%) - lack symptoms (delayed flexion/extension
views)
Radiographic findings
Focal kyphosis, mild anterolisthesis
Widened facet, interspinous/interlaminar spaces
Widened posterior, narrowed anterior disc
Compression fracture often associated
All findings accentuated with flexion; MRI to confirm ligament injury
840
Cervical Spine Trauma: Compression Fracture

[Figure 4-14-3]
Figure 4-14-3
Associated hyperflexion sprain common

Usually stable unless > 25% compression
Radiographs
Loss of height superior endplate
Focal cortical angulation
Band of increased density from impaction

Unilateral Facet Injury
Hyperflexion and rotation

Common 13% of cervical injuries
Radicular symptoms common
Most frequent C4 C6
Often mechanically stable, PLL partially intact
Unstable with prominent articular mass/laminar
fractures
Unilateral Facet Injury:

Radiologic Characteristics
[Figures 4-14-4 and 4-14-5]
Anterolisthesis < 50% vertebral width

Dislocated facet anterior (oblique view in foramen)
Abnormal spinolaminar space/facet rotation
Bow-tie sign
Spinous process rotation toward side of dislocation
Sagittal MR (proton density and T2) of flexion sprain cervical

injury with superior endplate fracture (arrow) and distraction
forces posteriorly causing interspinous widening or fanning at
C6-7 (*)
Figure 4-14-4
Unilateral Facet Injury:

CT
Naked facet (may be subtle and partial)
Contralateral facet subluxation common
Articular mass fracture (73%) isolating pillar (17%), posterior
vertebral body fracture (25%)
MRI/MRA disc herniation and vertebral artery injury not
uncommon
Figure 4-14-5
Pictoral representation of a unilateral facet

injury (circle)
Figure 4-14-6
Radiographs (AP and lateral) of a unilateral facet injury (circle)

with subluxation. Note that on the AP film the spinous
processes above the level of injury are shifted to the left. Also
on the lateral film the facets below the level of injury are
projected as in a lateral projection whereas above the level of
injury they are obliqued consistent with the rotational
component of force
Sagittal CT of a unilateral facet injury with

locked facets (IF-inferior facet of the level
above; SF-superior facet of the level below)
841
Cervical Spine Trauma

Facet Injury: Bilateral [Figures 4-14-7 and 4-14-8]
Figure 4-14-7
Hyperflexion may be some rotation

At least as common as unilateral injury
Disrupted PLL, disc, and often ALL
Unstable injury
High incidence of cord damage
(72% quadriplegia)
Bilateral facet dislocation may be partial or complete
Bilateral Facet Dislocation:

Radiologic Characteristics [Figures 4-14-9 and 4-14-10]
Anterolisthesis > 50% vertebral body diameter

Dislocated inferior facets, anterior to superior facets
Dislocated facets in foramen oblique views
Findings of hyperflexion fanning, focal kyphosis, disc
narrowing
Spinous processes not rotated
CT naked facets, small fracture fragments often not
seen on radiographs
Pictoral representation of a bilateral facet injury with perched

facets
Figure 4-14-8
Figure 4-14-9
Pictoral representation of a bilateral facet injury with locked

facets
Figure 4-14-10
Lateral radiograph of a bilateral facet dislocation

with anterolisthesis (arrow) at C4-5. Also on the
lateral film the inferior facets (IF) of the level
above is anterior to the superior facets (SF) of
the level below
Sagittal CTs of bilateral facet dislocation in two different

patients with the inferior facets (IF) of the level above anterior to
the superior facets (SF) of the level below
842
Cervical Spine Trauma: Flexion Teardrop Fracture

[Figure 4-14-11]
Figure 4-14-11
Most severe devastating flexion injury

Usually lower cervical spine C5-6 (70% of cases)
Diving accident shallow pool common cause
Immediate, complete and permanent quadriplegia
(90% of cases)
Acute anterior cord syndrome loss pain,
temperature, and touch
Retention position, motion, vibration (posterior
column senses)
Flexion Teardrop Fracture:

Involved vertebrae and levels above in severe

flexion
Vertebral body fracture with triangular fragment from
anteroinferior corner
Central vertebral body not severely involved but
posteriorly displaced
Bilateral facet subluxation/dislocation
MRI/MRA disc herniation and vertebral artery injury
not uncommon
Pictoral representation of a flexion teardrop injury
Figure 4-14-12
Cervical Spine Fracture:

Clay Shoveler Fracture
Avulsion C7, C6, T1 spinous process

Result of abrupt flexion against opposing
interspinous ligament
Stable injury
Oblique fracture spinous process
May see double spinous process sign
(AP radiograph)
Spinous process fractures can also result from
extension/direct trauma

Hyperextension Injuries
[Figure 4-14-13]
Usually caused by force to face/forehead

Compression posteriorly, distraction anterior
Less common than hyperflexion injuries (19% - 38%)
Atlas and laminar fractures
Hyperextension teardrop fracture
Hangman fracture
Pillar fracture
Radiographs (AP and lateral) of a flexion teardrop injury with

facet widening (circles and solid arrows), interspinous fanning
(*) and vertebral fracture with teardrop fragment anteriorly
(open arrow)
Figure 4-14-13
Atlas Fractures
Avulsion of anterior arch C1

Rare stable injury
Results from anterior atlantoaxial ligament
Horizontal cleft in anterior arch (difficult on CT)
Posterior C1 arch fracture
Bilateral posterior fractures (no anterior
component)
No anterior soft tissue swelling; stable
Distinguish from normal congenital cleft
Laminar Fractures
Lamina crushed on extension from above/below

Pictoral representation of an extension injury
Often in older patients with spondylosis
Usually C5 to C7
Difficult to detect on radiographs
CT optimal
Mechanically stable (Intact anterior column/facets)
Neurologically unstable due to cord impingement by fragments
843
Hyperextension Dislocation
Common in older patients with spondylosis

Also bone forming diatheses AS, DISH
Rupture of ALL, disc and stripping of PLL (unstable)
Patients usually severe neurologic symptoms
Acute central cord syndrome
Spinal cord impinged by subluxation and intact posterior
elements
Often recoils back to relatively normal position
Figure 4-14-14
Hyperextension Dislocation: Radiographic

Characteristic [Figure 4-14-14]
Relatively normal cervical alignment in quadriplegic patient

Soft tissue swelling (100%)
Only finding 33%
Avulsed fragment anteroinferior vertebrae (65%)
Longer horizontally (unlike extension teardrop fracture)
In young patients ring apophysis, no neurologic deficit
Widened disc anteriorly and vacuum (15%)
Hyperextension Fracture/Dislocation:
Pedicolaminar Fracture-Separation
Combined hyperextension, compression and rotation

Fractures of pillar, lamina, pedicles and spinous process opposite
side of translation
Vertebral body often mildly (3 6 mm) anteriorly displaced
Spinous process not rotated
Lateral radiograph of an extension fracture

subluxation at C5-6 (arrow) in a patient with
ankylosing spondylitis (syndesmophytesarrowhead)
Hyperextension Fracture/Dislocation:
Pedicolaminar Fracture-Separation
Disc narrowing and vertebral rotation above injury

Opposite facet may be widened/dislocated
Commonly involve foramen transversarium
Vertebral artery (MRA)
Important to distinguish from flexion injury
Pillar Fracture
Not common, 3% - 11% of cervical injuries (C6 C7)

Hyperextension and rotation
Articular mass compressed on side of rotation
Stable, radiculopathy common without cord damage
Pillar Fracture: Radiologic Characteristics [Figure 4-14-15]

Subtle on radiographs
Disrupted lateral cortical margin (AP view)
Visualize facets on AP radiographs
Loss of posterior articular mass overlap
Lateral radiograph (double outline sign)
CT optimal degree of fragmentation and
additional other fractures
Pedicle, transverse process, lamina
Figure 4-14-15
Radiograph, AP tomogram and axial CT of an articular pillar fracture

with offset at the facet (solid arrows) and fracture (open arrow)
seperating the articular pillar from the remaninder of the vertebrae
844
Hyperextension:
Teardrop Fracture
Figure 4-14-16
[Figures 4-14-16 and 4-14-17]
Often occur in older osteoporotic patients

Avulsion by ALL of triangular fragment
Anterioinferior vertebral body (usually C2)
Fragment vertical height same or larger than length
Unlike avulsion with hyperextension dislocation
Soft tissue swelling more prominent in younger
patients
Unstable in extension
Traumatic Spondylolisithes:
Hangman Fracture (Hangee Fracture)
Common 5% of all cervical spine injuries
Hyperextension is probably transient modified by
flexion/compression/distraction
Unstable injury
Neurologic symptoms unusual unless distraction
Large canal relative to cord at C2
Autodecompression from bilateral
posterior fractures
Pictoral representation of an extension teardrop fracture
Figure 4-14-17
Traumatic Spondylosithes:
[Figures 4-14-18 and 4-14-19]
Effendi classification
I: Minimally displaced fracture
II: More displacement, involves C2 3 disc (widening)
III: Bilateral facet dislocation
Oblique C2 pedicle fracture lateral view
Mild anterolisthesis, posteriorly displaced
spinolaminar line
Associated injuries-anterior corner
fractures C2/C3
C1/high thoracic fractures (10%)
Vertebral artery injuries
Figure 4-14-18
Lateral radiograph and tomogram of an extension teardrop fracture with

avulsed fragment (arrow) from the attached anterior longitudinal ligament (ALL)
Figure 4-14-19
Pictoral representation of the different types of traumatic
spondylolistheses
Lateral radiograph and axial CT of a type 1 traumatic spondylolisthesis

showing the linear nondisplaced fracture (arrows)
845
Axial Compression Injury:

Burst Fracture
Figure 4-14-20
Not common, 4% of cervical injuries

Only occurs where cervical spine in neutral
position
C1 Jefferson fracture
Lower cervical burst fracture C3 - 7
Jefferson Fracture [Figure 4-14-20]
Axial compression drives occipital condyles

toward atlas
Bilateral fractures anterior/posterior
Lateral displacement
Unstable, neurologic symptoms unusual
Large neural canal
Outward displacement of fragments
Jefferson Fracture:
[Figure 4-14-21]
Pictoral representation of a Jefferson fracture
Open mouth view best

Laterally displaced lateral masses
Lateral radiograph may only show soft tissue swelling; look
for posterior fractures
CT optimal for bilateral fractures
Jefferson variants
Lateral mass displacement > 7 mm / predental space > 6
mm = ruptured transverse atlantal ligament
Small nondisplaced fragment medial to articular mass
intact ligament
Figure 4-14-21
Cervical Burst Fracture
Caused by vertical force driving nucleus pulposis through

endplate with body exploding from within
Mechanically stable unless posterior ligament injury
Neurologically unstable deficit may progress
Fragments change position
Symptoms transient paresthesias to quadraplegia
Axial CT of a Jefferson fracture with four components

(arrows)
Cervical Burst Fracture:

Soft tissue swelling with straightening (but no kyphosis)

Retropulsed fragments disrupted posterior vertebral body line
Degree of vertebral body comminution variable
Vertical fracture midline/eccentric
Disrupted joints of Lushka (facets)
Figure 4-14-22
Lateral radiograph, axial CT and sagittal CT of burst type fractures in different patients showing the comminuted fracture (circle),
retropulsed fragments (solid arrows) and fracture at the junction of the lamina and spinous process (open arrow)
846
Indeterminate Mechanism: Cervical Injuries
Odontoid fractures
Occipitoatlantal dissociation
Torticollis
Rotary atlantoaxial subluxation/dislocation
Figure 4-14-23
Odontoid Fracture
Most common of C2 fractures (41%)

11% - 13% of all cervical spine injuries
Mechanism flexion and or extension
Other fractures (13%) face, mandible,
posterior arch C1, extension teardrop,
hangman, atlantoaxial dissociation
Anderson/DAlonzo classification
Odontoid Fracture: Radiologic

Characteristics [Figures 4-14-23 to 4-14-26]
Prevertebral soft tissue swelling(may be only

finding)
Pictoral representation of different types of odontoid fractures
Type I
Rare (if occurs) avulsion at tip from alar
Figure 4-14-24
ligaments
Type II
At base (60%-70%)
May miss on CT
High nonunion rate (72%), higher if displacement
> 5 mm
Open mouth view (simulated by Mach effect);
atlantoaxial instability
Os odontodeum distinguished by sclerotic
margins
Type III (30%-40%)
C2 body
Disruption of Harris ring
Fat C2 sign, invariably heal
Figure 4-14-25
Lateral radiograph of a type 2 odontoid fracture (arrows)
Figure 4-14-26
Open mouth radiograph of a type 3 odontoid fracture (arrows)
Coronal and sagittal CT of a type 3 odontoid fracture (arrows)

847
Cervical Spine Trauma: Role of MR Imaging

Thecal sac/spinal cord impingement
Disc herniation/extrusion: 20% - 40% patients
Highest (100%) in patients with anterior cord
syndrome
Epidural hematoma (1% - 2%); spinal cord
edema/hematoma
Ligamentous disruption; cervical spondylosis
Subsequent complications
Syringomyelia, myelomalacia
Figure 4-14-27
T1
T2
MR Imaging: Spinal Cord Injury

Intramedullary swelling
T1W increased cord caliber
T2W increased signal
Intramedullary edema
T2W increased signal
Intramedullary hemorrhage
Variable MR appearance (often
heterogeneous)
Poor prognostic sign
Sagittal T1 and T2-weighted MR images show high signal

intensity in the spinal cord (arrows) that subsequently reveals
low signal intensity on T2-weighting on a follow-up MR all
indicative of hemorrhage as seen on the gross specimen
T2
MR Imaging: Intramedullary
Hemorrhage [Figure 4-14-27]
Oxyhemoglobin
Hyperacute (minutes hours)
Intermediate signal T1W
High signal T2W
Deoxyhemoglobin
Usually first 24 hours
Can be up to 8 days with hypoxia
Intermediate signal T1W
Low signal T2W
Methemoglobin
Usually after 24 hours
High signal T1W (begins peripherally)
Low signal T2W (early subacute
intracellular)
High signal T2W (late subacute
extracellular)
References
1.
2.
3.
4.
Blackmore CC, Mann FA, Wilson AJ.. "Helical CT in the primary trauma evaluation of the cervical spine: an
evidence-based approach." Skeletal Radiol. 2000 Nov;29(11):632-9. Review.
Jarolimek AM, Coffey ECC, Sandler CM, West OC. "Imaging of uppercervical spine injuries -- Part III: C2
below the dens." Applied Eadiology. 2004 July; 9-21.
Murphey MD, Batnitzky S, Bramble JM. "Diagnostic imaging of spinal trauma." Radiol Clin North Am. 1989
Sep;27(5):855-72.
Stabler A, Eck J, Penning R, Milz SP, Bartl R, Resnick D, Reiser M.. "Cervical spine: postmortem assessment of
accident injuries--comparison of radiographic, MR imaging, anatomic, and pathologic findings." Radiology. 2001
Nov;221(2):340-6.
848
Radiographic Differential Diagnosis of the

Jaws
Christopher G. Fielding, COL, DC, USA
Generalities
Dentists like plain films

Act as radiologist
Oral & Maxillofacial Surgery
Oral & Maxillofacial Radiology
Dental Anatomy Review
Primary dentition (deciduous)

20 teeth
Eruption starts at 6 months
Completed eruption sequence 3 years
Permanent dentition
32 teeth
Eruption starts at 6 years
Completed eruption sequence 12 years
Except 3rd molars (wisdom teeth)
Primary Dentition
Eruption/Exfoliation
Tooth Numbering
Primary Tooth Numbering
Primary tooth numbering
Tooth Numbering
Imaging Techniques
Intraoral
Bitewing
Periapical
Occlusal
Extraoral
Panoramic
AP, PA, lateral, oblique, Waters, Townes
CT, MRI, technetium scan
Bitewing
Radiographic Description
Size : in centimeters
Border: well circumscribed, poorly circumscribed, illdefined
Shape: unilocular, multilocular, uniform, irregular
Number: focal, multifocal
Color: radiolucent, radiopaque, mixed;
buzzwords: ground glass, cotton wool
Location: exact location within the maxilla or
mandible; location in relation to adjacent structures
(periapical, interradicular, pericoronal, etc)
Teeth
Impaction, displacement, or resorption
Periodontal supporting structures
Periodontal ligament space enlargement or loss
of the lamina dura
849
Tooth numbering (adult)

Radiographic Differential Diagnosis of the Jaws
Features Unique to Dental Radiographs
Dental anatomy
Crown of tooth
Periodontal ligament
Lamina dura
Artifactual thin white line around roots of teeth
Root canal
Apex of root
Inferior alveolar canal
Above - think odontogenic
Below - think fibro-osseous & developmental
Figure 4-15-1
Overview
Radiolucent lesions
Periapical
Pericoronal
Multilocular
Radiopaque/mixed density lesions
Periapical
Interradicular
Multifocal confluent
Target lesion
Osteosarcoma of the gnathic skeleton
Periapical cyst/periapical granuloma
Radiolucent Lesions: Periapical
Periapical granuloma
Periapical cyst
Traumatic bone cyst
Nasopalatine duct cyst
Early focal cemento-osseous dysplasia
Figure 4-15-2
Periapical Granuloma and Periapical Cyst

[Figure 4-15-1]
Inflammatory lesion progressing from nonvital pulp as a

sequelae of caries or trauma
Pain with or without swelling and drainage
Radiographic
Circumscribed or diffuse radiolucency which destroys the
periodontal ligament space and lamina dura
Usually limited in size
Differential diagnosis:
Immature periapical cemental dysplasia (tooth is vital)
Immature cementoblastoma (vital tooth, usually mandibular
molars)
Traumatic bone cyst (vital teeth, intact lamina dura)
Treatment
Endodontic (root canal therapy)
Extraction
Nasopalatine Duct Cyst [Figure 4-15-2]
Aka incisive canal cyst

Nasopalatine duct cyst (must exceed 6 mm in
diameter, the upper limit for the normal incisive
Most common non-odontogenic cyst in the oral cavity
canal)
(developmental cyst)
Swelling of anterior palate, pain, drainage
4th 6th decades
Well-circumscribed ULRL midline of palate, apical to central incisors
Treatment
Surgical enucleation
Recurrence is rare
850
Fibro-osseous lesions
Figure 4-15-3
Cemento-osseous dysplasia
Periapical
Focal
Florid
Ossifying fibroma
Fibrous dysplasia
Cemento-osseous Dysplasia
Focal
Single site
90% occur in F
Whites > blacks
Posterior mandible most common site
Many occur in extraction sites
Most lesions smaller than 1.5 cm in diameter
Well defined
RL ---> mixed density ---> RO
Early lesions RL
Calcification with maturation of lesion
Cemento-osseous Dysplasia [Figures 4-15-3 and 4-15-4]
Periapical
Anterior mandible
F predilection (10:1, 14:1)
70% occur in blacks
Pulps are vital
Teeth are usually unrestored
Asymptomatic
Incidental finding
RL --->mixed density --->RO
Early lesions RL
Periapical cemento-osseous dysplasia (early)
Figure 4-15-4
Cemento-osseous Dysplasia [Figures 4-15-5 and 4-15-6]
Florid
Multifocal, not limited to anterior mandible
Black F
Usually middle-aged
Pulps are vital
Asymptomatic
Dull pain
Occasional expansion or sinus tract
Incidental finding
RL --->mixed density --->RO
Early lesions RL
Periapical cemento-osseous dysplasia

(late/mature)
Figure 4-15-5
Figure 4-15-6
Florid cemento-osseous dysplasia
851
Gardner Syndrome
Figure 4-15-7
Cemento-osseous Dysplasia
Treatment/prognosis
No treatment required
Clinical & radiographic diagnosis in most cases
Surgery, extraction, biopsy of sclerotic lesions
Chronic osteomyelitis
Radiolucent Lesions: Pericoronal
Dentigerous cyst
Ameloblastoma
Ameloblastic fibroma
Odontogenic keratocyst
Dentigerous Cyst [Figure 4-15-7]
The most common developmental odontogenic cyst

Derived from reduced enamel epithelium of the dental follicle
Radiographic
Well demarcated radiolucency that may be extensive
Encompasses the crown of an unerupted or impacted tooth
Third molars and cuspids of young adults most commonly
involved
Rarely may give rise to:
Ameloblastoma
Mucoepidermoid carcinoma
Treated by enucleation
Dentigerous cyst involving an impacted

mandibular 3rd molar
Figure 4-15-8
Radiolucent Lesions: Multilocular (Macho-Macho)
Central giant cell granuloma
Ameloblastoma
Cherubism
Odontogenic myxoma
Hemangioma/AV malformation
Odontogenic myxoma
Botryoid odontogenic cyst
Hyperparathyroidism
Multilocular RL of the Mandible
Multilocular
Aneurysmal bone cyst
Cherubism
Hyperparathyroidism
Odontogenic fibroma
Can Cross Midline

Myxoma (odontogenic)
Ameloblastoma
Central giant cell lesion
Hemangioma/A-V malformation
Odontogenic Keratocyst [Figure 4-15-8]
The most common multilocular radiolucency

Usually presents in adults
Up to 3/4 in posterior mandible and ramus
Radiographic
Well-defined unilocular, or more commonly multilocular
radiolucency with sclerotic borders
Usually grows in a relatively linear direction
852
Odontogenic keratocyst involving the posterior

mandible and ramus
Obvious expansion may NOT be seen, even with large cysts

Often associated with an impacted tooth
May grow aggressively with:
Cortical perforation
Tooth displacement and/or resorption
Soft tissue extension
Odontogenic Keratocyst
Treated by thorough enucleation and curettage

Occasionally, en bloc resection required
Up to 1/3 will recur
Long term radiographic follow-up requiredfollow-up quiredfo
If multiple, evaluate for nevoid basal cell carcinoma syndrome
(Gorlin syndrome)
Figure 4-15-9
Nevoid Basal Cell Carcinoma Syndrome

(Basal Cell Nevus Bifid Rib Syndrome; Gorlin
Syndrome) [Figure 4-15-9]
Teenagers, both male and female

Autosomal dominant disease
Symptom complex characterized by:
Numerous basal carcinomas of the skin
One or more bifid ribs
Multiple odontogenic keratocysts
The keratocysts are identical to the solitary ones
Recurrence of OKCs is the rule in this syndrome
Multiple odontogenic keratocysts associated with nevoid

basal cell carcinoma syndrome (Gorlin Syndrome)
Bifid Rib Basal Cell Carcinoma Syndrome

(Nevoid Basal Cell Carcinoma Syndrome; Gorlins Syndrome)
Other findings include:

Palmer/planter pitting pitting
Frontal bossing
Calcified falx cerebri
Tendency to develop medulloblastomas
Many others
Long-term periodic radiographic follow-up is needed
Figure 4-15-10
Central Giant Cell Granuloma [Figure 4-15-10]
Aggressive reactive process more often in mandible

Young adults with female predilection
Radiographic
Unilocular, or more commonly multilocular
radiolucency with well demarcated margins
The most common anteriorly located multilocular
lucency
May cross the midline
May cause expansion of the involved bone with
thinning of the cortex
Root divergence and smooth, concave root resorption is common
Central giant cell granuloma
Central Giant Cell Granuloma
Histology
Highly cellular fibrovascular stroma with
Dispersed multinucleated giant cells
Osteoid or osseous trabeculae
Extravasated blood and hemosiderin
Brown tumor of hyperparathyroidism is histologically indistinguishable
Serum calcium determination required
Surgical enucleation with extraction(s) if necessary
May also try hormonal therapy with calcitonin
May recur if incompletely removed
853
Ameloblastoma [Figure 4-15-11]
Figure 4-15-11
Most common odontogenic neoplasm

Average age is 34 with no sex predilection
Presents as a painless expansile lesion
Most common location is posterior mandible and
ramus
Maxillary lesions may extend to nasal cavity and base
of skull
Radiographic
Classically a soap-bubble multilocular
radiolucency with clearly demarcated borders
Unilocular (unicystic) ameloblastomas possible
Most are unassociated with impacted teeth
May displace and/or resorb teeth
Centrifugal growth
May reach gigantic proportions
Ameloblastoma
Treatment:
Curettage or en bloc resection for mandibular lesions
Resection for maxillary lesions
Recurrence rate is high
Unilocular ameloblastomas have a better prognosis with rare
recurrence and requiring only simple enucleation
Rare malignant transformation has been reported
Cherubism [Figure 4-15-12]
Rare developmental jaw condition that is generally inherited as an

AD trait
Usually occurs between ages 2-5 YO
Bilateral involvement of the posterior mandible developing
characteristic cherub-like facies
Maxillary involvement can occur
Expansile multilocular radiolucency
Ameloblastoma
May cause tooth displacement, eruption failure,
impair mastication, speech difficulties
Treatment:
Prognosis in any given case is unpredictable. Most cases demonstrate
varying degrees of remission and involution after puberty.
Radiotherapy contraindicated
Radiopaque and Mixed Lesions: Periapical
Periapical cemento-osseous dysplasia

Cementoblastoma
Erupting teeth
Endodontic procedures
Hypercementosis
Idiopatic osteosclerosis
Focal scerosing osteomyelitis
Figure 4-15-12
Cherubism (bilateral multilocular radiolucencies)
854
Cementoblastoma [Figure 4-15-13]
Benign cemento-osseous tumor, most commonly associated with

a root of a mandibular molar
Usually 1st molar
Some authorities consider this a simple variant of
osteoblastoma
M=F; < 25y of age
Slow growth with possible expansion
Sometimes painful
Radiographic
Calcified, highly radiodense mass intimately associated with
the root
Root outlines obscured
Usually surrounded by radiolucent rim
Tx/Prognosis
Excision or root amputation/endodontics
May continue growing if incompletely excised, otherwise no recurrence
Idiopathic Osteosclerosis [Figure 4-15-14]
Focal area of increased radiodensity of unknown cause

Vital pulp
M=F; arise 1st 2nd decade
May remain static or slowly increase in size
Once skeletal growth stops, the lesions become static
90% in mandible, usually 1st molar region
Usually involving a root apex
Multifocal in some cases
XR
Well-defined, rounded or elliptic radiodense mass
No RL rim
3 mm 2 cm
Figure 4-15-13
Cementoblastoma
Figure 4-15-14
Idiopathic osteosclerosis
Focal Sclerosing Osteomyelitis [Figure 4-15-15]
Aka condensing osteitis

Localized increased radiodensity
Apex
Widened PDL space or periapical RL
Molar-premolar areas of mandible commonly
Figure 4-15-15
Condensing Osteitis
Tx/prognosis
Endodontic/ext
85% regress or resolve
Bone scar
Residual lesion
Radiopaque and Mixed Lesions: Interradicular
Ossifying fibroma
Active ossifying fibroma
Focal cemento-osseous dysplasia
Osteoblastoma
Adenomatoid odontogenic tumor
Odontoma
855
Focal sclerosing osteomyelitis (condensing

osteitis)
Ossifying Fibroma [Figure 4-15-16]
Only true neoplasm in the benign fibro-osseous lesion

category
Seen more often in teenagers and young adults
No sex predilection
Radiographic
Well defined mixed lesion with sclerotic borders
Density will vary with the maturity of the lesion
Tooth bearing and non-tooth bearing areas, especially
posterior mandible
Growth is radial instead of linear (as in fibrous dysplasia)
Expansion buccally and lingually will thin cortex
Bowing of inferior border is characteristic
May expand alveolar crest
Treatment
Thorough excision of mass
Mass tends to shell out easily from the surrounding bone
Up to 1/3 may recur
Figure 4-15-16
Radiopaque and Mixed Lesions: Multifocal

Confluent
Florid cemento-osseous dysplasia

Pagets disease of bone
Chronic sclerosing osteomyelitis
Gardner syndrome
Multiple tori and exostoses
Radiopaque and Mixed Lesions: Ground Glass
Fibrous dysplasia
Hyperparathyroidism
Osteopetrosis
Fibrous Dysplasia
Occurs in children, teenagers and young adults of both sexes

Four main forms: monostotic, polyostotic, McCune-Albrights,
and craniofacial
Craniofacial FD may involve multiple contiguous bones in
the midface and cranium
McCune-Albrights disease includes polyostotic FD, focal
skin hyperpigmentation (caf-au-lait spots) and endocrine
disturbances (usually precocious puberty and/or
hyperthyroidism)
Painless expansile process of osteoprogenitor tissue
Slow growth with facial deformity
More common in the maxilla
Ossifying fibroma
Figure 4-15-17
Fibrous dysplasia (ground glass

appearance)
Fibrous Dysplasia [Figure 4-15-17]
Radiographic
Mature maxillary lesions are homogeneous, ground glass or peau
d'orange
No margination or borders and blends with adjacent trabecular bone
May obliterate the maxillary sinus
Mandibular lesions more likely mottled or multicystic
Skeletal survey to rule out polyostotic disease
Use plain films or CT as MRI does not demonstrate the traditional
radiographic findings
Defer surgical treatment (cosmetic recontouring) until skeletal maturity
Growth may cease, continue, or resume after periods of quiescence
Quarterly follow-up
Small chance of sarcomatous transformation, usually osteosarcoma or
fibrosarcoma
Especially in patients with a history of radiotherapy
856
Radiopaque and Mixed Lesions: Target Lesion, Dense
Odontoma
Osteoma
Focal cemento-osseous dysplasia
Ameloblastic fibro-odontoma
Ossifying fibroma
Osteoblastoma
Figure 4-15-18
Odontoma [Figure 4-15-18]
Equal sex predilection

More common in teenagers and young adults
A mixed odontogenic tumor / hamartoma
Asymptomatic but may prevent tooth eruption
Types:
Compound
Target lesion with central tooth-like structures
More common in anterior jaws between teeth
Complex
Well demarcated opacity with frayed margins
May have a radiolucent rim
More common in posterior jaws (pericoronal)
Treated by enucleation
Does not recur
Compound odontoma
Osteosarcoma [Figure 4-15-19]
Malignancy of mesenchymal cells

M>F
Extragnathic
Bimodal (2nd 3rd decades, 6th decade)
Distal femur/proximal tibia
Axial skeleton/flat bones
Gnathic
3rd 4th decades
Maxilla = mandible
XR
RL, mixed, RO
Ill-defined periphery
Sunburst or sunray
25% of gnathic lesions
Spiking root resorption
Widening of PDL space
Treatment/prognosis
Gnathic lesions low-grade??
Radical surgical extension
Best hope for cure
Preoperative chemotherapy
Local uncontrolled dz
Leading cause of death
Usually within 2 years of initial tx
Risk factors
Pagets disease
H/O radiation
Figure 4-15-19
Osteosarcoma (widening of the periodontal

ligament space)
857
MRI of the Knee: Part 1

Mark Anderson, MD
Lecture Outline
Part 1
Technique
Menisci
Articular cartilage
Part 2
Bones
Stabilizers
Miscellaneous
Technique
Surface coil
High resolution
T1, T2, fat suppression
Sagittal, coronal, axial planes
Technique: Pulse Sequences
T1
Overall anatomy
Menisci
Bones (marrow)
Fat/hemorrhage
Muscle
T2
Fluid / edema
Tendons, ligaments
Soft tissue injury
Fast Spin Echo-T2
T2 contrast faster acquisition
Caution:
Bright fat (marrow pathology)
Blurring effect proton density (meniscal
tears)
Gradient Echo (T2*)
Menisci
Articular cartilage (3D)
Susceptibility effects
Caution: Marrow Pathology
STIR (Fat suppressed T2)
Marrow pathology
Soft tissue injury
Articular cartilage
Figure 4-16-1
Cadaveric specimen of the menisci
Figure 4-16-2
Summary: Pulse Sequences
Menisci
Short TE (T1, PD, GRE) - caution with FSE
Bone Marrow
Fat saturation (STIR, Fat Sat FSE T2) - not GRE
T1W in one plane
Other soft tissues (ligaments, tendons)
T2W with fat saturation (STIR, Fat Sat FSE T2)
Cartilage
Contrast between fluid and cartilage
Schematic diagram of a meniscus cut in cross-section (upper);
normal sagittal image of posterior horn of the medial mensicus
(lower); normal meniscus at arthroscopy (right)
858
Technique: Imaging Planes
Figure 4-16-3
Sagittal
Menisci
Cruciate ligaments
Extensor tendons
Articular cartilage
Bones
Coronal
Collateral ligaments
Menisci
Articular cartilage
Bones
Axial
Patellofemoral joint
Muscles / tendons
Popliteal fossa
Sagittal gradient
echo images
(corresponding
to lines on
diagram) through
the medial
meniscus
Figure 4-16-4
Menisci [Figures 4-16-1 and 4-16-2]
Fibrocartilage
Medial/lateral
Functions:
Joint congruity
Shock absorption
Load transmission
Sagittal gradient
echo images
(corresponding
to lines on
diagram) through
the lateral
meniscus
Medial Meniscus [Figure 4-16-3]
Larger C
Posterior horn > anterior horn
Attached more tightly to the capsule
Covers 1/2 contact surface of tibial plateau
Lateral Meniscus
[Figure 4-16-4]
Tighter C
Posterior horn = anterior horn
Attached more loosely to the capsule
Covers contact surface of tibial plateau
Popliteus tendon (fascicles)
Figure 4-16-5
Menisci: Attachments
[Figures 4-16-5 and 4-16-6]
Tibia
Capsule
Ligaments
Transverse
Meniscofemoral
Humphrey
Wrisberg
Oblique meniscomeniscal
Figure 4-16-6
Normal transverse intermeniscal ligament
Diagram of the knee (posterior view)

demonstrating the meniscofemoral ligament of
Wrisberg (arrow).
859
Menisci: Variants
Figure 4-16-7
[Figures 4-16-7]
Discoid
Enlarged meniscus
Embryologic, congenital?
Lateral > medial
Prone to tear
Complete / incomplete
Wrisberg variant
Buckled
Lax, Flounce
Medial meniscus
Ligament injury/laxity
Positional
Ossicle
Vestigial, post-traumatic?
May be symptomatic
PHMM most common
Variable MRI signal intensity
A. Diagram illustrating a discoid lateral meniscus (L) from above.

B. Normal sized medial meniscus (thin arrow) and enlarged,
discoid lateral meniscus (large arrow)
Figure 4-16-8
Meniscus
Microstructure
Collagen bundles
Circumferential
Transverse (tie fibers)
Resist longitudinal loading
hoop stresses
Menisci: Pathology
Degeneration
Tear
traumatic vs. degenerative
20% asymptomatic pts > 50 y.o. show MR evidence of tear
Surgical Considerations
Primary goal
Preserve as much meniscal tissue as possible
A. Diagram of a meniscus cut in cross-section

revealing an oblique undersurface tear.
B. Similar tear in the posterior horn of the
medial meniscus on sagittal image
Meniscal Tear: MRI
Abnormal
Signal intensity -Morphology
Meniscal Tear: MRI [Figure 4-16-8]
Signal intensity (Grades)

1 Globular
2 Linear
3 Contact with articular surface ==> tear
Meniscal Tear: Close
Kaplan
13/20 (65%) no tear
DeSmet
1 image only 30%55%
> 1 image.... 90%
Dont overcall be descriptive
Meniscal Tear: MRI
Morphology
Blunted, truncated
Size
860
Meniscal Tears [Figure 4-16-9]
Figure 4-16-9
Vertical
Radial
Longitudinal
Traumatic
Divides into ant/post or med / lat fragments
Horizontal
Degenerative
Divides into sup / inf fragments
Meniscal Tears [Figures 4-16-10 and 4-16-11]
Radial
Perpendicular to axis
Vertical
Traumatic or degenerative
Meniscal subluxation
Irreparable?
Figure 4-16-10
Diagram of radial tear
Figure 4-16-11
Radial tear involving the body of the lateral meniscus (arrow)
Longitudinal
Vertical
Along axis of meniscus
Bucket handle
displaced fragment
medial meniscus
locking
Peripheral
potentially reparable
outer 1/3 (red zone)
Peripheral Tears [Figure 4-16-13]
Figure 4-16-13
Figure 4-16-12
Longitudinal tear
Outer 1/3 of meniscus

Vascular region
(red/red zone)
Tend to heal
primary repair
conservative therapy
A. Radial tear.
B. Longitudinal tear.
C. Horizontal tear.
A. Diagram of a peripheral tear.

B. Peripheral tear involving the posterior horn
of the medial meniscus (arrow).
861
Meniscocapsular Separation
Figure 4-16-14
PHMM
Fluid at meniscocaps interface
Poor sensitivity/PPV
Figure 4-16-15
Parrot Beak Tear

Combination
Radial + longitudinal
Oblique to long axis
Meniscal Flap
Parrott beak
tearParrott beak tear
Horizontal
Often degenerative
May be asymptomatic
Meniscal cysts
Meniscal cyst [Figure 4-16-16]
Parameniscal/intrameniscal
Lateral > medial
Horizontal tear
A. Diagram of a horizontal tear.

B. Horizontal tear of the body of the meniscus
(white arrow) and an associated parameniscal
cyst (black arrow)
Displaced Meniscal Tears [Figures 4-16-17 to 4-16-21]
Bucket Handle (medial)

Flipped (lateral)
Gutter
Extruded
Figure 4-16-16
Figure 4-16-17
Bucket Handle Tear

[Figures 4-16-17 to 4-16-19]
Coronal
Sensitivity
Displaced fragment
Blunted body
Sagittal
Too few bowties
Double PCL
(94%)
(64%)
(97%)
(30%)
Helms AJR 1998
Diagram of a bucket
handle tear of the
meniscus
Figure 4-16-18
Figure 4-16-19
Sagittal image corresponding to the dashed line shows the

large bucket handle fragment within the notch creating the
double PCL sign (P = PCL)
Coronal image corresponding to the dashed line

demonstrates the displaced meniscal fragment of this bucket
handle tear (large arrow) and irregular, truncated body of the
meniscus (thin arrow)
Horizontal tear with associated parameniscal

cyst
862
Figure 4-16-20
Figure 4-16-21
A. Diagram of a flipped (longitudinal) meniscal tear.

B. Sagittal image at the level of the dashed line shows the
flipped fragment (short arrow) adjacent to the anterior horn
(long arrow) creating the double anterior horn sign. Note also
the small residual posterior horn (circle).
Menisci: Post-surgical
Truncated, absent, normal

Healed tear can look like new tear
MR arthography
0.2 cc Gd + 20 cc saline
T1W with fat-saturation
Gd extending into tear
White et al. Radiology, February 2002

364 patients
Conventional MR
Indirect MR arthrography
Direct MR arthrography
Direct slightly more accurate, but no significant difference
A. Coronal image demonstrating a displaced

meniscal fragment along the medial joint line
(arrow).
B. Diagram illustrating the horizontal tear and
flipped fragment
Signs of new or recurrent tear?

Fluid in tear
Displaced fragment
Tear in new area
Meniscal Tear: MRI
Accuracy > 90%

50% - sagittal only
3% - coronals only
FSE ~ 80% (?)
Problem areas:
Free edge
PHLM
Meniscal Tear: Pitfalls
Anatomy
Transverse ligament
Meniscofemoral ligaments
Oblique meniscomeniscal lig
Lateral inf geniculate artery
Popliteus tendon
Edge artifact
Meniscal Tear: Pitfalls
Artifacts
Patient motion
Phase artifact (artery)
Magic angle
Gas/hemosiderin
Chondrocalcinosis
863
Take Home Points
Meniscal tear?
Signal intensity and morphology
Small meniscus?
Find the missing fragment
Healed tear?
Can look just like a new tear
True pathology or pitfall?
To most easily identify pathology, know the normal anatomy
References
1.
Helms CA, Laorr A, Cannon WD, Jr. The absent bow tie sign in bucket-handle tears of the menisci in the knee.
864

Mark Anderson, MD
Lecture Outline
Figure 4-17-1
Part 1
Technique
Menisci
Articular Cartilage
Part 2
Bones
Stabilizers
Miscellaneous
Bones
Femur, tibia, patella, fibula

Cortical
compact
subchondral plate
Cancellous
trabecular
Spectrum of acute osseous injuries
Figure 4-17-2
Trabecular Bone
10 x load-bearing capacity of cortical bone

Dissipates forces
Support for subchondral plate
Bones: Injuries
Acute
Impaction (contusion, occult fracture)
Avulsion
Chronic
Fatigue, insufficiency fracture
Spontaneous osteonecrosis
Osteochondritis dissecans
Acute Impaction Injuries: Spectrum [Figure 4-17-1]

Focal contusion involving the posterolateral
tibial plateau
Acute Trauma: Impaction
Contusion, bone bruise

Edema, hemorrhage
Trabecular fx
Detection
Explains symptoms
Avoids unnecess arthroscopy
Mechanism of injury
May change Rx plan
Acute Trauma: Contusion [Figure 4-17-2]
MRI
Reticular
Ill-defined margins
T1, + STIR
100% heal; 24 months
865
Geographic Contusion [Figure 4-17-3]
Figure 4-17-3
Subchondral
Cartilage damage
softening, fissuring,
chondral fx
proteoglycans*
Long term sequelae?
Protect during healing
*Johnson, AJSM, 1998
Focal subchondral contusion along the

posterior weight-bearing portion of the lateral
femoral condyle
Figure 4-17-4
Contusion Patterns: ACL Tear [Figure 4-17-4]
Lat. femoral condyle (sulcus terminalis)

Post-lateral tibial plateau
Specific
95% (adults)
75% (children)
Contrecoup
Post-medial tibial plateau
Peripheral injury
Patterns: Patellar Dislocation [Figure 4-17-5]
Lateral dislocation
Contusions
Lat. femoral condyle
Anterior / non-wgt bearing
Medial patella
Medial retinacular injury
Cartilage injury
Avulsion fracture
ACL contusion pattern (lateral femoral

condyle and posterolateral tibial plateau)
Figure 4-17-5
Acute Trauma: Fracture [Figure 4-17-6]
Linear
T1
signal intensity
STIR
+ or signal intensity
Figure 4-17-6
Patellar dislocation contusion pattern (lateral

margin of lateral femoral condyle
and medial patella)
Occult fracture of the lateral tibial plateau on sagittal T1weighted and coronal fat-saturated T2-weighted images
866
Knee Stabilizers
Figure 4-17-7
Central: ACL, PCL

Medial: MCL
Lateral:
Iliotibial band
Fibular collateral lig.
Biceps femoris tendon
Anterior: Patellar Retinacula
Cruciate Ligaments [Figure 4-17-7]
Intracapsular, extrasynovial
Intercondylar notch
Anterior (lateral)
Posterior (medial)
ACL [Figure 4-17-8]
LFC --> anterior tibia

Restricts ant displacement of tibia
MRI
low SI
usually striated
taut
Diagram of the knee (frontal view)

demonstrating the anterior (A) and posterior (P)
cruciate ligaments
Figure 4-17-8
ACL Tear
75% of all ligament injuries

Twisting + valgus force
Hyperextension
Associated injuries
Meniscal tear (40%70%)
ODonoghues Triad
ACL, MCL, medial meniscus (?)
(Lateral / Medial about equal)
70% mid substance; 20% proximal; 10% distal
ACL Tear: MRI [Figure 4-17-9]
MRI 90%95% accurate

Primary Signs
Edematous mass (48%)
Non-visualization (empty notch) (18%)
Disruption (11%)
Irregular, wavy, horizontal contour
Focal + SI
Normal anterior cruciate ligament
Figure 4-17-9
ACL Tear: MRI
Secondary Signs
Bone contusions
Deep notch LFC
Segond fracture
10% ACL tears fx
75100% fx - ACL tear
Anterior drawer
(uncovered PHLM)
Chronic ACL Tear: MRI
Non-visualization
Focal angulation
Fragments
Normal (scar)
Without Edema
Complete ACL rupture
867
Post-Op ACL [Figure 4-17-10]
Figure 4-17-10
Graft
Integrity
Signal Intensity
Variable, especially early
Roof Impingement
Cyclops lesion
Anterior arthrofibrosis
PCL [Figure 4-17-11]
MFC -> posterior tibia

Restricts post tibial displacement
MRI
Low signal intensity
Arched
Left: Normal ACL graft (dashed arrow).

Right: Cyclops lesion (arthrofibrosis) along ventral margin of
ACL graft (arrow).
Figure 4-17-11
PCL Tear [Figure 4-17-12]
Force -> ant tibia flexed knee

Dashboard injury
Complete Tear (45%)
Midsubstance
Partial Tear (47%)
Avulsion (8%)
Medial Collateral Ligament

[Figure 4-17-13]
Superficial component
Bursa
Deep component
Meniscofemoral
Meniscotibial
Normal PCL
MCL Injuries: MRI [Figure 4-17-14]

Grade Clinical
1
Sprain
2
3
MRI
Thickened
Irregular
Adjacent edema
Partial Tear
Focal SI
Complete Tear Discontinuity
Figure 4-17-12
Figure 4-17-13
Full thickness PCL tear (midsubstance)
Normal MCL
868
Lateral Stabilizers [Figure 4-17-15]
Figure 4-17-14
Biceps femoris tendon

Fibular (lateral)
collateral ligament
Iliotibial tract
Posterolateral Corner Injuries
Hyperextension with varus force

Isolated injuries rare
often with cruciate injuries
PCL most common
Urgent exploration indicated
within 3 days 3 weeks
reconstruct with cruciate(s)
ITB Friction Syndrome
Lateral pain
Abnormal contact ITB/LFC
Bursa develops
Fluid collection/edema
Lateral recess?
Tendons
Medial
Semimembranosus
Sartorius
Gracilis
Semitendinosus
Lateral
Biceps femoris
Iliotibial tract
Posterior
Gastrocnemius (med/lat)
Anterior
Quadriceps tendon
Patellar tendon
Patellar retinacula
Extensor Mechanism
Partial tear of proximal MCL (arrow)
Quadriceps tendon
Striated
Vastus lateralis
Vastus medialis
Intermedius
Rectus femoris
Patellar tendon
Magic angle
Figure 4-17-15
Diagram of lateral stabilizers (B = Biceps

femoris tendon; F = Fibular collateral ligament;
I = Iliotibial tract).
[Figure 4-17-16]
Figure 4-17-16
Extensor Tendons: Injuries
Tears
Trauma
Degeneration
Renal disease, steroids
RA, SLE
Partial vs. complete
Extensor mechanism (quadriceps

and patellar tendons)
869
Patellar Tendinitis [Figure 4-17-17]
Figure 4-17-17
Jumpers Knee
Enlarged (proximal)
+ intrasubstance SI
Spectrum
partial --> complete tears
Patellofemoral Joint
Patellar subluxation
Lateral
Hypoplastic intercondylar notch
Patellofemoral Syndrome
Anterior pain
Patellar tilt / subluxation
Impingement of infrapatellar fat
Cystic Structures
Recesses
Bursae
Ganglia
Patellar tendinits with thickening and

high grade partial tearing in its proximal
fibers
Normal Recesses
Suprapatellar bursa
Infrapatellar cleft
Popliteus hiatus
Gastrocnemius/ Semimembranosus
Posterior recesses
Bursae
Figure 4-17-18
[Figure 4-17-18]
Prepatellar
Infrapatellar
superficial/deep
Semimembranosus
Pes anserine
Tibial collateral
LCL-Biceps Femoris
Other Cystic Masses [Figure 4-17-19]
Meniscal cysts
Ganglia
Intraarticular (cruciates)
Extraarticular (infrapatellar fat)
Intraosseous (cruciate insertions)
Vascular masses
A. Prepatellar bursitis.
B. Semimembranosus bursitis.
Figure 4-17-19
Popliteal artery aneurysm (A)

870
Cystic Adventitial Disease
Figure 4-17-20
Cystic degeneration vessel wall

Popliteal artery common
Sudden onset claudication
MRI findings
intramural cysts
along long axis of vessel
extrinsic compression
MR angiography
Synovial Plica
Embryologic remnants
Infrapatellar
Suprapatellar
Medial
Loose Bodies
[Figure 4-17-20]
Intercondylar notch
Bakers cyst
Popliteus sheath
GRE (T2*)
Take Home Points
Bone contusions?
ACL torn?
ACL graft?
MCL?
Lateral ligaments?
Cyst?
Loose body in posterior joint recess (arrow)
Look for pattern

Taut...primary/secondary signs
Taut...roof impingement...cyclops
Deep and superficial fibers
Biceps, LCL, Iliotibial Band
Recess, bursa, ganglion, meniscal cyst
References
1.
Johnson DL, Urban WP, Jr., Caborn DN, Vanarthos WJ, Carlson CS. Articular cartilage changes seen with
magnetic resonance imaging-detected bone bruises associated with acute anterior cruciate ligament rupture. Am J
Sports Med 1998; 26:409-414.
871
MRI of the Wrist

Mark Anderson, MD
Figure 4-18-1
Technique: Positioning
Supine (arm at side)

Prone (arm extended overhead)
Surface Coil
Thickness
13 mm
Matrix
256512
FOV
10 cm
Technique: Pulse Sequences
T1
T2*
STIR
Gd
Anatomic overview
Ligaments, tendons
Marrow, fluid
Cyst/solid, infxn
Synovitis screen
Os styloideum
Anatomy: Coronal
Bones
Intrinsic ligaments.
TFCC
Anatomy: Axial
Tendons
Three levels
Distal radioulnar joint
Pisotriquetral joint
Hamate
Median nerve (Carpal tunnel)
Ulnar nerve (Guyons canal)
Anatomy: Sagittal
Carpal alignment
Pisotriquetral joint
Triangular fibrocartilage
Anatomy / Pathology
Bones
Intrinsic ligaments (SL, LTL)
TFCC
Tendons
Nerves
Masses
Bones: Normal
Signal intensity
Alignment
sagittal alignment
ulnar variance
Os Styloideum [Figure 4-18-1]
Normal variant
Base of 2nd/3rd metacarpals
+/- Pain
Bursitis
Ganglion
Trauma
MRI of the Wrist
872
Occult Osseous Injuries [Figure 4-18-2]
Figure 4-18-2
Contusion
Bone marrow edema
Fracture
Edema + fx line
Occult Fractures [Figure 4-18-3]
Trauma Screening Protocol
Scaphoid Fracture [Figure 4-18-4]
16% not detected initially

Complications
AVN
Nonunion
Scaphoid AVN
Occult fracture of triquetrum
Figure 4-18-3
[Figure 4-18-5]
Normal T1 = Normal
T1 T2 = Necrotic
T1 + T2 = Ischemia vs. traumatic edema
Contrast enhancement?
AVN Lunate
Mid scaphoid fracture

Left: Coronal T1 - Right: Coronal STIR
Kienbocks Disease
Repetitive trauma, fracture, ulna (-) variance
End arteries
Central position
Figure 4-18-4
AVN Lunate: MRI [Figure 4-18-6]
More than 50% of lunate

Abnormal marrow signal
T1 T2 = Diagnostic
T1 + T2 = Earlier stage
Ulnolunate Impaction [Figure 4-18-7
Ulna plus variance

Degenerative changes
especially lunate
TFC tears
Scaphoid fracture
Figure 4-18-5
Figure 4-18-7
Scaphoid fracture with ischemic changes in proximal pole
Figure 4-18-6
Avascular necrosis of the lunate

(Kienbocks disease).
Ulnolunate impaction syndrome

873
MRI of the Wrist
Intrinsic Ligaments [Figure 4-18-8]
Figure 4-18-8
Scapholunate
Volar Trapezoidal
Middle Triangular
Dorsal Band-like
Lunotriquetral
Smaller (2mm)
Other
Distal carpal row
Incomplete
Intrinsic Ligaments
Pitfalls
Intermediate signal
Attach to bone or articular cartilage
Ligament Pathology [Figure 4-18-9]
Absent
Distorted / Elongated
Widened joint
Discontinuous
Fluid signal on T2
Normal scapholunate ligament
Figure 4-18-9
Carpal Stability
Scaphoid <-> Lunate <-> Triquetrum
Scapholunate Dissociation: DISI [Figure 4-18-10]
Tear or stretching of SLL

dorsal fibers
scaphoid palmar flexes
signet ring deformity
lunate dorsiflexes
S-L angle > 600
Scaphoid fracture
DISI deformity
can result from scaphoid fx
Small scapholunate ligament perforation
Figure 4-18-10
Scapholunate Instability: SLAC Wrist [Figure 4-18-11]
Scapho
Lunate
Advanced
Collapse
Trauma, RA, CPPD
Figure 4-18-11
DISI deformity
SLAC wrist
MRI of the Wrist
874
Lunotriquetral Instability [Figure 4-18-12]
Figure 4-18-12
LTL tear
Associated with TFC tears
VISI deformity
Difficult diagnosis
Triangular Fibrocartilage Complex
Radioulnar ligaments
Meniscus homologue
UCL and ulnocarpal ligaments
ECU tendon sheath
TFC: Normal Anatomy [Figure 4-18-13]
Fibrocartilage
Bow tie
Ulnar styloid dist radius
Attaches to radial cartilage
Central portion / periphery
Peripheral 20% vascularized
VISI deformity
Figure 4-18-13
TFC: Pathology [Figure 4-18-14]
Tear / Perforation
95% accuracy
Partial vs. full thickness
Radial / ulnar
Central / peripheral
Associated injuries
LTL, ECU sheath
Radioulnar Ligaments
[Figure 4-18-15]
Volar / Dorsal margins of TFC

Flat margins - Attach directly to bone
Injury DRUJ instability
Figure 4-18-15
Normal triangular fibrocartilage (TFC)
Figure 4-18-14
Normal volar and dorsal radioulnar ligaments (arrows), and normal TFC
(open arrow).
Extensor Carpi Ulnaris Sheath [Figure 4-18-16]
Ulnar-sided support
Injury leads to
subluxation, tenosynovitis, tears
Figure 4-18-16
Small perforation of the TFC
Subluxed extensor carpi ulnaris tendon (arrow)

875
MRI of the Wrist
Tendons
Figure 4-18-17
Axial plane
Flexors
Carpal tunnel
Extensors
dorsal compartments
Extensor retinaculum
Extensor Compartments
1st
Abd. pollicis longus

Ext. pollicis brevis
2nd
Ext. carpi radialis longus/brevis
Listers Tubercle
3rd
Ext. pollicis longus
4th
Ext. digitorum
Ext. indicis
5th
Ext. digiti minimi
6th
Ext. carpi ulnaris
A. Artifactual intermediate signal in the flexor pollicis longus

tendon on gradient echo image.
B. Tendon appears normal on fat-saturated T2-weighted image
Figure 4-18-18
Tendon Pathology
Tenosynovitis
Surrounding fluid, +/- enlargement
Stenosing (loculated, septations)
Partial tear
Enlarged / thinned / focal signal
Complete tear
Magic Angle Phenomenon [Figure 4-18-17]
Artifactual signal within tendon

~ 55 to main magnetic field
Short TE images
Disappears on long TE images
DeQuervains tenosynovitis
Tendon Pathology [Figures 4-18-18 and 4-18-19]
Figure 4-18-19
Extensor Carpi Ulnaris

6th dorsal compartment
DeQuervains Syndrome
1st extensor compartment
Tenosynovitis
DDX:
Scaphoid fracture
1st CMC arthritis
Flexor carpi radialis tenosynovitis
Flexor tendons
Tenosynovitis
Carpal tunnel syndrome
Severe tenosynovitis of the flexor tendons
Carpal Tunnel [Figure 4-18-20]
Figure 4-18-20
Floor carpal bones

Roof flexor retinaculum
Contents
Flexor tendons
Median nerve
Normal carpal tunnel (long arrow = flexor

retinaculum; short arrow = median nerve)
MRI of the Wrist
876
Median Nerve [Figure 4-18-21]
Figure 4-18-21
Volar / radial position in carpal tunnel

Stable to decreasing size
May appear flattened at hamate
Carpal Tunnel Syndrome
Compressive neuropathy
Pain, paresthesias
Thumb, index, long, radial 1/2 ring
Worse at night
DX: clinical exam, nerve conduction
Normal median nerve within the carpal tunnel
Figure 4-18-22
Carpal Tunnel Syndrome: MRI
Swelling (pisiform)
Flattening / angulation (hamate)
Increased signal intensity T2
Bowing of flexor retinaculum
Carpal Tunnel: Post-op
Volar displacement of tendons/nerve

Free edges of retinaculum
Retinaculum not seen
CT: Post-op Complications
Incomplete retinacular release

Proximal swelling of median nerve
Scarring around nerve
Mass lesion in carpal tunnel
Median nerve neuroma
Normal ulnar tunnel (Guyons canal)
Figure 4-18-23
Guyons Canal [Figure 4-18-22]
Ulnar Tunnel
Ulnar nerve, artery, vein
Boundaries
Floor flexor retinaculum
Roof fascia
Lat. to pisiform and hook of hamate
Ulnar Tunnel Syndrome [Figure 4-18-23]
Ganglion cyst or other mass

Fracture (hook of hamate)
Repetitive trauma
Masses: Anomalous Muscles [Figure 4-18-24]
Accessory palmaris longus

volar
superficial to flexor tendons
Ext. digitorum manus brevis
dorsal
near extensor indicis tendon
Isointense to muscle
on all sequences
Lipoma compressing the structures within the ulnar tunnel
Figure 4-18-24
Accessory palmaris longus muscle (M)

877
MRI of the Wrist
Take Home Points
High resolution imaging!

SLL and LTL?...Coronal thin section images
TFCC?...TFC, radioulnar ligaments, ECU tendon
Tendons?...Flexor and extensor axial images
Masses?...Ganglia 70% dorsal @ SLL
20 % volar @ distal radius
Nerves?...Median carpal tunnel
Ulnar Guyons canal
MRI of the Wrist
878
MRI of the Ankle and Foot

Mark Anderson, MD
Technique
Figure 4-19-1
Surface Coil
One ankle/foot only
T1, T2, Fat Sat
Gd?
Cyst vs. Solid
Infection
Synovitis screening
Ankle/Foot: Imaging Planes
Ankle
Axial
Coronal
Sagittal
Foot
Long Axis
Short Axis
Sagittal
Bones
Fracture of the anterior process of the

calcaneus
Marrow Edema
Activity related
Contusion/occult fracture
Osteonecrosis
Osteomyelitis
Tumor
Figure 4-19-2
Bones: Acute Trauma [Figure 4-19-1]
Contusion, bone bruise

Marrow edema
Hemorrhage
Trabecular fx
Fracture
Osteochondral Lesion [Figure 4-19-2]
Terminology
Osteochondral fracture
Transchondral fracture
Osteochondritis dissecans (OCD)
Ankle
Acute trauma
Talar dome
Mid 1/3 lateral
(inversion, dorsiflexion, LCL)
Posteromedial
(inversion, plantarflexion)
Osteochondral lesion, medial talar dome
Figure 4-19-3
Osteochondral Lesion [Figure 4-19-3]
Talar Dome
Mid 1/3 lateral (inversion, dorsiflexion, LCL)
Posteromedial (inversion, plantarflexion)
Staging
0
Normal cartilage
1
Abnl SI but intact
2
Fissuring not to bone
3
Flap or exposed bone
4
Loose fragment
5
Displaced fragment
Mintz DN, et al. Arthroscopy 2003;19:353-9
Osteochondral lesion, medial talar dome with

overlying cartilage loss
879
Bones: Os Trigonum [Figure 4-19-4]
Ununited tubercle
Os Trigonum Syndrome
Post. Pain
Plantar flexion (ballet)
MRI
Marrow edema
FHL tenosynovitis (stenosing)
Figure 4-19-4
Bones: Access. Navicular [Figure 4-19-5]
Type I distal PT tendon

Type II close proximity to bone
Cornuate navicular
Pain syndrome
Type II and Cornuate
MRI: marrow edema
Tarsal Coalition [Figure 4-19-6]
Os trigonum syndrome
Figure 4-19-5
2nd 3rd Decade

Vague hindfoot pain
Calcaneonavicular
Talocalcaneal
2 signs: talar beak, C sign, etc.
Cartilaginous, Fibrous, Osseous
Bones: Hallux Sesamoids
Flexor hallucis brevis tendons

Stress reaction/fracture (medial)
Osteonecrosis (lateral)
DJD (subchondral changes)
Ligaments
Accessory navicular
Syndesmotic
Lateral
Medial
Spring
Lisfranc
Sinus Tarsi
Plantar Fascia
Figure 4-19-6
Ligaments: Syndesmotic [Figure 4-19-7]
Interosseous ligament
Anterior tibiofibular
Posterior tibiofibular
Talus = rectangular
Calcaneonavicular coalition
Figure 4-19-7
Anterior and posterior tibiofibular ligaments

880
Ligaments: Lateral [Figure 4-19-8]
Figure 4-19-8
Fibular collateral lig complex

Anterior talofibular
Calcaneofibular
Posterior talofibular
Talus = elongated
Ligaments: Medial [Figure 4-19-9]
Deltoid
Tibial collateral lig complex
Deep (tibiotalar)
Superficial
Tibionavicular
Tibiocalcaneal (strongest)
Posterior tibiotalar
Ligaments: Injuries [Figure 4-19-10]
Normal anterior talofibular ligament
Interruption
Laxity
Thickening/irregularity
Edema (acute)
Non-visualization
Figure 4-19-9
Figure 4-19-10
Deep fibers of the deltoid ligament
Torn anterior tibiofibular ligament

(syndesmotic injury)
Ligaments: Chronic Injury [Figure 4-19-11]
Figure 4-19-11
Anterolat Impingement Syndrome

ATAF ligament injury
Persistent pain
Scar tissue in lateral gutter
MRI
Intermediate SI tissue
T1 and T2WI
Ligaments: Spring / Lis Franc
Spring ligament
plantar calcaneonavicular
medial and plantar bands
Lisfranc ligament
medial cuneiform
base of 2nd metatarsal
Scar tissue in anterolateral gutter (arrow)

secondary to chronic anterior talofibular
ligament injury
881
Sinus Tarsi [Figure 4-19-12]
Figure 4-19-12
Cone-shaped space
Wide lateral tarsal canal medial
Fat, nerves, vessels, ligaments
Inferior extensor retinaculum
Cervical ligament
Talocalcaneal interosseous lig
Sinus Tarsi Syndrome
Lateral pain
Sense of hindfoot instability
70% Prior trauma
30% Inflammatory arthritis
PTT tear/dysfunction
Normal sinus tarsi
Figure 4-19-13
Sinus Tarsi Syndrome
MRI Findings
Replacement of normal fat
- SI T1
+ or - SI T2
Plantar Fascia [Figure 4-19-13]
Calcaneus toes
Two bands
Medial
Lateral
Plantar Fasciitis
Inflammation
Mechanical (pes cavus, etc.)
Degenerative (age related)
Systemic disease (RA, seronegative)
DDx:
Calcaneal stress fx
Tendinitis
Heel pad trauma/inflammation
Normal plantar fascia
Figure 4-19-14
Plantar Fasciitis [Figure 4-19-14]
MRI
Thickened fascia (> 4 mm)
+ SI
Fascia and perifascial tissues
Calcaneus
Plantar Fibromatosis [Figure 4-19-15]
Fibrous proliferation
Fibroblasts and collagen
MRI
T1 / - SI
T2 / low to intermediate SI
Variable enhancement
Severe plantar fasciitis with partial tearing

at its origin (arrow).
Figure 4-19-15
Tendons
Change orientation
Pulleys
Osseous or soft tissue
Magic angle effect
Small, enhancing plantar fibroma (arrow) on
post-contrast, fat-saturated T1-weighted image
882
Tendon Pathology [Figure 4-19-16]
Figure 4-19-16
Achilles Tendon [Figure 4-19-17]
Gastrocnemius/Soleus
No tendon sheath (paratenon)
Bursae
Retrocalcaneal
Tendo Achilles (acquired)
Flat/concave ventral margin
Achilles Tendon: Pathology

[Figures 4-19-18 and 4-19-19]
Insertional Tendinitis
Haglunds Syndrome
Bursitis
Thickened tendon
Pump bump
Non-Insertional
Overuse
30-50 y.o.-weekend warrior
Systemic disease
RA, SLE
Local/systemic steroids
Peritenonitis
Chronic Tendinitis
Partial / Complete Tear
MR images and schematic diagrams of the spectrum of tendon pathology
Figure 4-19-17
Figure 4-19-18
Normal Achilles tendon
Figure 4-19-19
Haglunds syndrome (insertional Achilles

tendinopathy and partial tearing;
retrocalcaneal bursitis)
Chronic, non-insertional Achilles tendinopathy

and partial tearing
883
Plantaris Tendon
Figure 4-19-20
Origin near lateral Gastrocnemius

Long tendon
Between med head Gastroc/Soleus
Medial margin of Achilles
Pitfalls
Partial tear of Achilles
Residual fibers of Achilles
Medial Tendons [Figure 4-19-20]
Post Tibial
Flex Digitorum
Artery, vein, nerve
Flex Hallucis
Tom
Dick
and
Harry
Posterior Tibial Tendon
Oval 2X size of FDL

Insertion sites
Medial navicular
Cuneiforms
Bases of Metatarsals 14
Normal medial flexor tendons (T = posterior

tibial; D = flexor digitorum; H = flexor hallucis;
A = neurovascular structures in tarsal tunnel).
Figure 4-19-21
PTT: Pathology
Tenosynovitis, Tears
Factors
Degenerative (middle aged
women)
RA
Abnormal stresses
Loss of arch
PTT Pathology: MRI

[Figure 4-19-21]
Tenosynovitis...Fluid
Partial Tear...Thick, thin, split
Complete Tear...Disruption
Spectrum of posterior tibial tendon pathology

(A = tenosynovitis; B = partial tear; C = complete tear).
PTT Pathology: MRI
Secondary Signs
Pes planus
Spur/edema post medial malleolus
Also look for:
Deltoid ligament
Spring ligament
Sinus Tarsi
Figure 4-19-22
Lateral Tendons [Figure 4-19-22]
Peroneus Longus and Brevis

Posterior to lateral malleolus
Retrofibular groove
Peroneus Brevis
Anterior or medial
Common Sheath
Normal peroneus tendons

(L = peroneus longus; B = peroneus brevis).
884
Peroneus Tendons [Figure 4-19-23]
Figure 4-19-23
Tenosynovitis
Subluxation/Dislocation
Lateral margin of fibula
Retinacular injury or small avulsion fx
Acute or chronic
Entrapment (calcaneal fracture)
Partial/Complete Tear
Peroneus Brevis Split Syndrome [Figure 4-19-24]
Longitudinal tear (lateral malleolus)

May be asymptomatic
MRI
C-shaped
Two tendons
Adjacent fluid/edema
Tarsal Tunnel Syndrome [Figure 4-19-25]
Fibro-osseous tunnel
PT, FDL, FHL tendons
Tibial nerve, artery, vein
Pain, paresthesias sole of foot
Etiologies:
Tumor, ganglion cyst, dilated veins, post-traumatic fibrosis
Lateral dislocation of the peroneus tendons

(arrow)
Figure 4-19-24
Figure 4-19-25
Split peroneus brevis tendon (arrow = intact

peroneus longus tendon)
Ganglion cyst (G) displacing the neurovascular

bundle (arrow) within the tarsal tunnel
Mortons Neuroma [Figure 4-19-26]
Plantar digital nerve

Perineural fibrosis
3rd (2nd) web space
MRI
- SI T1 - SI T2
Figure 4-19-26
Enhancing Mortons neuroma (arrow)
885
Masses: Accessory Muscles [Figure 4-19-27]
Take Home Points
Figure 4-19-27
Isointense to Muscle on MRI

Accessory Soleus
Ventral to Achilles tendon
Peroneus Quartus
Adjacent to Peroneus Brevis
Bone Marrow Edema?...
Ligaments?...
elongated talus
Tendons?...
brevis split
Sinus tarsi?...
Tarsal tunnel...
Mortons neuroma,
plantar fibroma?...
Differential
Syndesmotic rectangular talus
Lateral / Medial Collateral
Magic Angle; PTT 20 signs; P.
Normal fat on T1W images
Space-occupying mass
Low SI on T2W images Give
Gadolinium!
Accessory soleus muscle
References
1.
Mintz DN, Tashjian GS, Connell DA, Deland JT, O'Malley M, Potter HG. Osteochondral lesions of the talus: a new
magnetic resonance grading system with arthroscopic correlation. Arthroscopy 2003; 19:353-359.
886
Osseous Lesions
Unknown Histogenesis
Figure 4-20-1
Unknown Histogenesis
Ewing sarcoma
Eosinophicic granuloma
Hand-Schller-Christian disease
Letterer-Siwe disease
Learning Objectives
Recognize the spectrum of imaging appearances of

these specific osseous lesions
Identify differentiating features
Ewing Sarcoma
Highly malignant primary bone sarcoma

Ewing provided first comprehensive description in
1921, designating it diffuse endothelioma of bone
Later (1924) termed endothelial myeloma of bone,
and Ewing Tumor by Codman
Origin controversial but likely derived from primitive
mesenchyme
Sheets of monotonous malignant "round cells with indistinct

cytoplasmic margins. Areas of necrosis and hemorrhage are
frequent
Ewing Sarcoma: Incidence & Distribution
About 5% of all biopsied tumors

Usually major long bones, femur most common (25%), then humerus (8%)
Long bones involved most commonly
In flat bones, most common pelvis (20%) followed by ribs (11%)
Rare in hands, sternum, T-spine
Ewing Sarcoma: Clinical Presentation
Seventy-five percent 1025 years

Peak incidence 10 to 15 years
Range 5 months to 83 years
Slight male predominence (1.5:1)
Pain & swelling most common symptoms
Constitutional signs to include local heat, fever, anemia, leukocytosis, etc.
Chromosomal trans in 90%; t(11;22) most common, others t(21;22), t(7;22)
Predilection for Caucasions (95%)
Usually solitary and nonfamilial; 10% are reported to be multiple at
presentation
Exceedingly rare familial cases (siblings), case reports in patients with
retinoblastoma
Ewing Sarcoma: Pathologic Features [Figure 4-20-1]
Characterized by sheets of monotonous malignant round cells

Indistinct cytoplasmic borders
Frequent areas of necrosis and hemorrhage
Virtually all PAS positive (glycogen)
Ewing family includes Ewing sarcoma and primitive neuroectodemal tumor
[PNET]
887
Osseous Lesions: Unknown Histogenesis
Radiologic Features: Intergroup Ewing Sarcoma

Study
Figure 4-20-2
[Figures 4-20-2 to 4-20-10]
Distribution: diaphysis 35%, metadiaphysis 59%,

metaphysis 5%, epiphysis <1%
Lesions medullary, symmetric or eccentric
Soft tissue mass in about 90%
Reactive bone 40%, but tumor produces no cartilage or
bone
Cortical thickening 20%
Periosteal reaction due to irritation or edema or tumor
permeation 85%
Onion skin appearance due to cyclic pattern of
periosteal irritation 55%
Perpendicular striations due to rapid continuous lifting of
periosteum 30%
Pathologic fracture in 10%15%; soft tissue calcification 10%
Figure 4-20-3
Figure 4-20-4
MR imaging. Typical features. MR shows large heterogeneous

circumferential soft tissue mass. Soft tissue changes seen to
better advantage on MR. Coronal T1 (Left) and T2 (Right).
Note diaphyseal location, complex periosteal reaction and

cortical thickening
Figure 4-20-6
Figure 4-20-5
Ewing sarcoma with pathological fracture. Radiograph (left) and

bone scan (right)
Hair-on-end periosteal reaction
Ewings sarcoma. Radiograph. Note metadiaphyseal

location and absence of identifiable matrix
888
Figure 4-20-7
Figure 4-20-8
Ewing sarcoma. Flat bone. MR

T2 (left) and T1 (right).
Ewing sarcoma. Flat bone. Radiograph
Figure 4-20-10
Figure 4-20-9
Ewing sarcoma. Flat bone (rib). Large soft tissue

mass obscure osseous origin
Ewing sarcoma. Note permeative osteolysis with

evidence of associated mass
Treatment & Prognosis: Ewing Sarcoma [Figures 4-20-11 and 4-20-12]
Ablative surgery, chemotherapy and radiation therapy

About 30% present with metastases
Mets typically to lungs (85%), bones (69%), pleura (46%), CNS (12%)
The 5-year survival rate for patients w/o mets at presentation: 5570%
Figure 4-20-11
Figure 4-20-12
Ewing sarcoma. Post treatment change

Ewing sarcoma. Local recurrence.

889
Langerhans Cell Histiocytosis (LCH)
Eosinophilic granuloma
Hand-Schuller-Christian disease
Letterer-Siwe disease
LCH: History
1940: Jaffe & Lichtenstein eosinophilic granuloma

1941: Farber, Green & Farber EG could be solitary or multiple
1953: Lichtenstein proposed the name histiocytosis X for the inflammatory
histiocytoses
LCH: Phases
Solitary or multiple lesions localized to bone: Eosinophilic granuloma (> 5 y)

Chronic disseminated histiocytosis: Hand-Schuller-Christian disease (15 y)
Acute or subacute disseminated histiocytosis: Letterer-Siwe disease (<1 y)
Supports the concept that this is a disorder of immune regulation
LCH: Phases
LCH, localized to bone: limited to a single or a few bones

LCH, chronic disseminated: multifocal bone lesions and es involvement of
lymph nodes, skin and abdominal viscera
LCH, acute or subacute disseminated: disseminated multisystem involvement
LCH: More Recently
Classification challenged as vague with overlapping clinical syndromes

Classification includes benign and malignant LCH
More recently classified as localized or multifocal
Single bone lesion or single organ system
Multifocal
Figure 4-20-13
Eosinophilic Granuloma: Incidence &

Distribution
About 1% of all biopsied tumors

Solitary EG is about twice as common as
multifocal EG
About 70% involve flat bones, most commonly
skull (25%), pelvis (20%)
In long bones, femur then humerus
Hands and feet rare in solitary disease
Eosinophilic Granuloma: Clinical

Presentation
Histiocytosis. Note histiocytes with reniform shape and clefts.

Scattered eosinophils are seen
About 90% are 515 years (average 1012)

Male:female about 2:1
More than 95% of patients are white
Most patients present with pain/tenderness
Fever may be present and presentation may
suggest osteomyelitis
Figure 4-20-14
Eosinophilic Granuloma:
Pathologic Features [Figures 4-20-13 and 4-20-14]
Characterized by a collection of histiocytes

Histiocytes are either oval, lobulated or reniform,
w/ clefts or indentations
Eosinophils may be seen singly, in sheets,
clusters or not at all
Birbeck bodies on EM
Histiocytosis. EM. Note Birbeck bodies.

890
Eosinophilic Granuloma: Radiologic Features
Usually permeative destruction in early phase with periosteal

reaction
More sharply defined with time, although lesion may still
enlarge
May have a rind of sclerosis
There may be an associated soft tissue mass in 5%10% of
patients
Figure 4-20-15
Eosinophilic Granuloma: Radiologic Features

[Figures 4-20-15 to 4-20-25]
Skull: beveled edge, button sequestrum

Flat bone: hole within a hole
Long bone distribution: diaphysis (58%), metadiaphysis (18%),
metaphysis (28%), epiphysis (2%)
Spine: vertebra plana
Mandible/maxilla: floating teeth
Figure 4-20-17
Flat bone. Hole within a hole
Figure 4-20-20
Indolent radiographic appearance. Note

epiphyseal lesion of proximal femoral lesion
Figure 4-20-23
MRI. Spine. Single lesion. T1 and T2

Skull. Beveled edge
Figure 4-20-16
Skull. Button sequestrum.
Figure 4-20-18
Flat bone (a) and long bone (b). Hole

within a hole
Figure 4-20-21
Figure 4-20-19
Flat bone (rib). Hole within a hole
Figure 4-20-22
Spine. Almost vertebral plana

Spine. Vertebral plana.
Figure 4-20-24
Floating teeth
891
Figure 4-20-25
Clavicular lesion. Radiograph (a) and

macrosection (b)
Eosinophilic Granuloma: Prognosis & Treatment
Benign course
Simple curettage or intralesional prednisone
Large lesions and vertebral lesions may be treated with low dose RTX
(3001000 rad)
May regress spontaneously
Hand-Schuller-Christian: LCH Chronic Disseminated
Initially described by Hand (1893), then by Schuller (1916); Christian (1920)

Classic triad: destructive skeletal lesions, exophthalmos and diabetes insipidus
Histologically identical to lesions of EG
About 10% of patients with unifocal EG will develop multifocal and
extraskeletal disease
Hand-Schuller-Christian Disease:
LCH Chronic Disseminated
Patients are young, usually less than 5 years

Classic triad in 10%15%, <50% have DI, exophthalmos about 25%
Any bone may be involved, 90% have cranial involvement, 7% hand or foot
lesion(s)
Hepatosplenomegaly and adenopathy
Anemia, fever, neurologic complaints
Fatal in about 15%, morbidity may be high
Letterer-Siwe: LCH Acute Disseminated
Initial reported by Letterer in 1924 (one case) and Siwe in 1933 (7 cases)
Usually develops within the first year of life
Disease disseminated and bone lesions small
Symptoms may be severe
Fatal in about 95% of those who develop disease before 1 year of age
Summary
Review the imaging appearances of Ewing sarcoma and the family of lesions
know as Langerhans cell histiocytosis
Demonstrate how the radiologic images reflect the underlying pathophyiology
and appropriate differentiating features
References
1.
2.
3.
4.
Davis et al. Radiographic features of eosinophilic granuloma of bone. AJR 1989;153:1021

Shapeero et al. Ewing sarcoma. Radiology 1994;191:825
Stull et al. Langerhans cell histiocytosis of bone. RadioGraphics 1992;12:801
Wilkins et al. Ewing's sarcoma of bone. Cancer 1986;58:2551
892
Soft Tissue Lipomatous Tumors

Learning Objectives
Recognize the spectrum of common lipomatous soft tissue masses

Identify the radiologic appearance of the common fatty masses
Identify imaging limitations and pitfalls
Outline:
Fundamental definitions
Incidence of soft tissue tumors
Overview
Common lipomatous tumors
Liposarcoma
Mimics
Cases
Definitions
Soft tissue is the nonepithelial extraskeletal tissue, excluding the RES, glia and
supporting tissue of parenchymal organs
It is derived primarily from mesenchyme, and by convention is comprised of
skeletal muscle, fat, fibrous tissue and the serving vessels and nerves.
Incidence: New Cancers by site 2000
Breast
Lung
Colon/rectum
CNS
Soft Tissue
Bone
184,200
164,000
130,200
16,500
8,100
2,500
CA Cancer J Clin 2000;50:12
Incidence: Variations
It is estimated that the relative frequency of benign to malignant tumors is

100:1
US overall annual incidence: 1.4 per 100K
Age specific incidence 80 years: 8.0 per 100k
Classification
World Health Organization subdivides benign lipomatous tumors into 9 groups

For imaging purposes, it is more useful to use the classification proposed by
Weiss and Goldblum
Weiss SW, Goldblum JR. Enzinger and Weisss Soft Tissue Tumors, 4th ed. St.
Louis; 2001
Classification: Weiss and Goldblum
Lipoma
Superficial
Deep
Multiple
Variants of lipoma
Lipoblastoma
Spindle cell lipoma
Pleomorphic lipoma
Angiolipoma
Chondroid lipoma
893
Figure 4-21-1
Lipomatous tumors
Intramuscular lipoma
Intermuscular lipoma
Lipomatosis nerve
Lipoma tendon sheath
Lipoma joint
Infiltrating lipomas
Lipomatosis
Symmetric Lipomatosis
Adiposis Dolorosa
Hibernoma
Lipoma: Clinical
Tumor of mature fat

Incidence ?
Presents 4060 yrs, uncommon <20
M ~ F, recent reports male predominence
Superficial and deep (deep: chest wall, retroperitoneum, deep
tissues hands and feet)
Deep seated tumors rare, < 1% lipomas
Typically asymptomatic, local pain or tenderness unusual
More common in obese, tumor fat not available for metabolism
Usually small, 80% < 5cm; 1% > 10 cm
Clinical diagnosis 85% accurate
Usually solitary, 5%15% multiple
50%-80% transloc chromosome 12 q13-15
Figure 4-21-2
MR. Subcutaneous lipoma
Lipoma: Radiology [Figures 4-21-1 to 4-21-8]
CT. Subcutaneous lipoma
Radiographs may demonstrate a fat density mass

Fatty nature well demonstrated on CT/MR
CT tissue attenuation -65 to -120HU
Visual comparison more reliable than #
Signal intensity equals SQ fat on MR
No enhancement with contrast (CT/MR)
May contain other mesenchymal elements
The most commom is fibrous tissue
Termed fibrolipoma when significant fibrous tissue present
May be associated with cortical thickening
Occasional chondroid and/or osseous metaplasia ; when long
standing-termed benign mesenchymoma
Figure 4-21-3
CT. Subcutaneous lipoma right shoulder.

Imaging contralateral side may be useful in
identifying subcutaneous lesions
Figure 4-21-4
T1
894
T2
MR. Fibrolipoma
Figure 4-21-5
Figure 4-21-6
Deep lipoma. Retroperitoneum
MR: Unencapsulated lipoma
Figure 4-21-7
Lipoma with metaplastic bone formation (benign

mesenchymoma). Radiograph (left) and macrosection (right)
Figure 4-21-8
Lipoma with metaplastic bone formation

(benign mesenchymoma).
Radiograph (left), CT (middle) and MR (right)
Intramuscular Lipoma
Lipoma arising in skeletal muscle

Most common member of subgroup of lipomatous tumors (fatty tumors arising
in intimate association with non-adipose tissue)
Other entities in this category include: intermuscular lipoma, lipoma of tendon
sheath, and lipomatosis of nerve
895
Figure 4-21-9
Figure 4-21-10
Intermuscular lipoma. Note infiltrating margin.
Intramuscular Lipoma: Clinical

[Figures 4-21-9 to 4-21-11]
Most common 4th 7th decades

Men more commonly affected
Most frequent large muscles extremities (thigh,
shoulder, upper arm)
Typically asymptomatic
Often incidental finding
Figure 4-21-11
In general, the concept that lipomas give

rise to liposarcomas is not
accepted...Based on consultation material
reviewed at the AFIP over several decades,
we never encountered a clear-cut example
of malignant transformation of lipoma,
although a few possible cases have been
reported in the literature.
Weiss and Goldblum
Enzinger and Weisss Soft Tissue Tumors, 4th ed. 2001
Lipoblastoma [Figure 4-21-12]
Relatively rare cellular immature lipoma

Originally described as embryonic lipoma
Occurs almost exclusively in infants, usually
presents by 3 yrs of age, occasionally at birth
Usually in superficial soft tissues or subcutis of the
extremities
Males affected 23X more commonly
Two-thirds to three-quarters are discrete
When diffuse, termed lipoblastomatosis
Radiologically may be indistinguishable from a
liposarcoma
Liposarcoma is exceedingly rare in children &
most pediatric fatty masses are lipoblastoma
Figure 4-21-12
Lipoblastoma in 18 month old. This appearance in an adult

suggests liposarcoma
896
Lipomatosis [Figures 4-21-13 to 4-21-15]
Figure 4-21-13
Diffuse overgrowth mature adipose tissue

Rare, but mild cases may go undiagnosed
Usually present before age 2
Considered congenital
Bone hypertrophy frequent association
Nerve not affected, not confined to extremity
Liposarcoma
Malignant mesenchymal tumor

Second most common soft tissue tumor after MFH
Approximately 16%18% all sarcomas
Presents ages 4060 years
Exceedingly rare in children
Usually extremities or retroperitoneum
Extremity lesions present 510 yrs earlier
Lipomatosis. Clinical photo. MR different patient
Classification: World Health Organisation

(WHO) (From low to high)
Figure 4-21-14
Well differentiated
Myxoid
Pleomorphic
Dedifferentiated
Classification:
World Health Organisation (WHO)
Well differentiated ---> Dedifferentiated

Myxoid ---> Round cell *
Pleomorphic
* Round cell liposarcoma was previously a distinct
subtype, now considered the hypervascular variant
of myxoid liposarcoma
Liposarcoma:
Well-Differentiated [Figure 4-21-16]
Predominantly fatty mass, usually more than 75% fat

Irregularly thickened or nodular septa
Presence of nodular/globular areas
A small number of lipomas will have a similar
imaging appearance
Lipomatosis trunk
Figure 4-21-15
Figure 4-21-16
A
Mild lipomatosis right lower extremity
Well-differentiated
liposarcoma. Typical features.
Radiograph (a), CT (b),
MR T1 (c) and T2 (d).
897
Liposarcoma: Atypical Lipoma
Figure 4-21-17
[Figure 4-21-17]
Lesion histologically indistinguishable from welldifferentiated liposarcoma

Used for lesions in which wide surgical margin is
possible, such as those in subcutaneous tissue
Liposarcoma: Dedifferentiated
[Figures 4-21-18]
Bimorphic lesion with:

WD liposarcoma
Juxtaposed high grade sarcoma (MFH)
Most common dedifferentiated sarcoma
May be a time-related phenomenon
Imaging typically shows a well differentiated fatty
mass
Fatty mass associated with a focal dominant
nonadipose component
Well-differentiated liposarcoma. Typical features
Figure 4-21-18
Liposarcoma: Higher Grade Lesions

[Figure 4-21-19]
Only 50%-80% of the myxoid or pleomorphic types

show fat on imaging studies
Fat usually minor component (<25%)
Hypervascular myxoid (round cell) and pleomorphic
types are typically more heterogeneous
Liposarcoma: Myxoid Lesions [Figure 4-21-20]
Myxoid and round cell lesions are now accepted as

ends of a common spectrum
About 20% of myxoid lesions will have a cyst-like
appearance
Figure 4-21-19
Dedifferentiated liposarcoma, well-differentiated component
Figure 4-21-20
Myxoid liposarcoma, typical imaging features. T1 (upper left),

T2 (upper right), CT (lower left) and gross (lower right)
Myxoid liposarcoma, cyst-like appearance. Radiograph (upper

left), MR T1 (upper right), T2 (lower left), bone scan (lower right)
898
Liposarcoma: Distribution
Type
Well-differentiated
Myxoid
Pleomorphic
Dedifferentiated
%
54
28
7
10
Retro
54
10
5
32
Extrem
54
34
8
4
Liposarcoma: Mimics
Hemorrhage
Malignancies engulfing portion fat
Muscle atrophy with fat replacement
Myxoid tumors: intramuscular myxoma, ES myxoid chondrosarcoma, myxoid
MFH
Neural tumors
Summary
Fatty tumors are common

There is a wide spectrum of lipomatous tumors
Imaging of fatty tumors is frequently characteristic
References
1.
2.
3.
4.
Christopher et al. WHO Classification of tumors. Lyon, France: IARC Press; 2002
Kransdorf et al. Fat-containing masses of the extremities. RadioGraphics 1991;11:81
Peterson et al. Malignant fatty tumors. Skeletal Radiol 2003;32:493
Weiss & Goldblum. Enzinger and Weiss's Soft Tissue Tumors, 4th ed. St. Louis: CV Mosby; 2001
899
Metabolic Bone Disease

Figure 4-22-1
Part I
Rickets and osteomalacia

Scurvy
Learning Objectives
Identify the pathophysiologic alterations that occur

in rickets and osteomalacia and scurvy
Recognize the spectrum of radiological features of
these diseases
Systemic diseases which effect the skeleton

diffusely and are the result of a metabolic
disorders
Biochemistry of vitamin D
Rickets
Figure 4-22-2
Abnormal mineralization and development of the

growth plate
Osteomalacia
Inadequate or delayed mineralization of mature

cortical or spongy bone
Pitt MJ. Rad Clin No Amer:1991;29:97
Osteoporosis
Insufficient quantity of normal bone
Osteopenia
Increased radiolucency of bone
OsteoPorosis
Normal enchondral bone formation
Figure 4-22-3
Paucity of bone
OsteoMalacia
Malformed bone
Vitamin D: Prohormone
D2 Synthetic
D3 Natural
Normal bone formation (left); rachitic bone formation (right)
Vitamin D: Biochemistry
[Figure 4-22-1]
Figure 4-22-4
Vitamin D: 1, 25 Dihydroxyvitamin D
[Figures 4-22-2 to 4-22-4]
Most active form of vitamin D

Calcium/phosphorus homeostasis
Maintenance bone mineralization
Rickets: Radiographic Features
Nonspecific features
Growth plate abnormalities
Skeletal deformities
Osteoid seams
900
Rickets: Nonspecific Features
Figure 4-22-5
Osteopenia
Growth retardation
Rickets: Growth Plate Abnormalities

[Figures 4-22-5 to 4-22-9]
Axial widening
Metaphyseal lucency
Metaphyseal cupping
Figure 4-22-6
Radiographic changes of rickets
Figure 4-22-7
Healing rickets with metaphyseal lucent bands (a) and

macrosection (b)
Dietary rickets with treatment; presentation (a), one

month (b), two months (c), and four months (d)
Rickets: Skeletal Deformities

[Figures 4-22-8 to 4-22-11]
Craniotabes
Rachitic rosary
Bowing of long bones
Scoliosis
Basilar invagination
Triradiate pelvis
Figure 4-22-9
Figure 4-22-8
Craniotabes
Overgrowth wrist cartilage (a) with corresponding clinical photo (b)

901
Figure 4-22-10
Figure 4-22-11
Skeletal deformities
Basilar invagination (a); triradiate pelvis (b)
Osteomalacia: Classic Radiographic Features

[Figures 4-22-12 to 4-22-14]
Osteopenia
Coarse trabecular pattern with unclear margins
Loosers zones
Features can be seen in rickets
Figure 4-22-12
Figure 4-22-13
Coarse trabecular pattern with pseudofracture;

radiograph (a) and macrosection (b)
Figure 4-22-14
Looser zone with (a) and without (b) fracture
Vitamin D [Figures 4-22-15 and 4-23 16]
Vitamin D-deficient
GI malabsorption
Neonatal
25-OH Vitamin D
Liver disease
Anticonvulsant therapy
Looser zones
902
Figure 4-22-15
Figure 4-22-16
Rachitic disease with fracture distal left femur
Rachitic disease from nec
Figure 4-22-17
Renal Related
[Figures 4-22-17 to 4-22-19]
1, 25 Dihydroxyvit D
Renal osteodystrophy
Vitamin D dep rickets
Tumor related
Renal Tubular Disorders
X-linked hypophosphatasia
Familial vitamin D res rickets
Fanconi syndromes
Tumor related
Ifosfamide
Renal osteodystrophy (a) and Fanconi syndrome (b)
Figure 4-22-18
Figure 4-22-19
Rickets due to ifosfamide therapy

Oncogenic osteomalacia
903
No Abnormality [Figure 4-22-20]
Figure 4-22-20
Axial osteomalacia
Hypophospatasia
Metaphyseal chondrodysplasia
Scurvy: The Stinking Disease [Figure 4-22-21]
the whole army was infected by a shocking

disorderthose affected had sore complaint in
the mouth that rotted the gums and caused a most
stinking breath. Very few escaped deaththe
surest sign of its being fatal was bleeding at the
nosebarbers were forced to cut away very large
pieces of flesh from the gums, to enable their
patients to eatit was pitiful to hear the cries and
groans on those on whom this operation was
performed.
De Joinville , 7th Crusade (12491254)
Scurvy
Metaphyseal chondrodysplasia
Figure 4-22-21
Sir James Lind, Ships Surgeon, conducted the

first documented controlled study in 1747 and
proved oranges and lemons were effective
treatment for scurvy
Scurvy: Pathophysiology
Deficiency of dietary vitamin C

Decrease cellular activity
Decreased collagen and osteoid production
Scurvy: Radiographic Features

[Figure 4-22-21 and 4-22-22]
Dense metaphyseal bands

Ring epiphysis
Lucent metaphyseal bands
Metaphyseal beaks
Periostitis
Subpepiphyseal infractions
Henry VIII was thought to have scurvy due to his illtemperament and horrid breath
Figure 4-22-22
Figure 4-22-23
Frankels line and Trummerfelds zone
Scurvy with treatment; presentation (left), one month (middle),

six months (right)
904
Summary
The radiographic features of metabolic bone disease are frequently

characteristic
These changes accurately reflect the underlying pathophysiology
References
1.
2.
3.
4.
5.
Holick. Vitamin D deficiency: what a pain it is. Mayo Clin Proc 2003;78:1457
Leggett et al. Scurvy. NEJM 2001;345:1818
Narchi et al. Symptomatic rickets in adolescence. Arch Dis Chil 2001;84:501
Pitt. Rickets and osteomalacia. In: Resnick. Diagnosis of bone and joint disorders, 4th ed. Philadelphia, W.B.
Saunders Company, 2002:1901
Sundaram et al. Oncogenic osteomalacia. Skeletal Radiol 2000; 29:117
905
Osteonecrosis and Related Conditions

Learning Objectives
Identify the spectrum of radiological features of osteonecrosis

Recognize various associated conditions
Identify differentiating features
Outline
Definitions
Pathophysiology of osteonecrosis
Infarct geometry
Radiologic-pathologic correlation
Associations
Complications
Related conditions
Definitions
Osteonecrosis ischemic death of cellular components of bone and marrow

Aseptic necrosis equivalent to ischemic necrosis and avascular necrosis
Bone infarct osteonecrosis involving the metaphysis or diaphysis
Osteochondrosis variety of conditions in which there is increased bone
density
Figure 4-23-1
Pathophysiology: Osteonecrosis
Cellular changes from ischemic injury

Interruption of intracellular enzymes
Cessation intracellular metabolic activity
Cell death
Cellular sensitivity to anoxia
Hematopoietic elements (6 hrs 12 hrs)
Bone cells (12 hrs 48 hrs)
Marrow fat cells (48 hrs 5 days)
Infarct Geometry: Zones [Figure 4-23-1]
Central zone of cell death

Ischemic injury
Active hyperemia
Normal tissue
Location
Infarct geometry
Osteonecrosis is most common in the epiphysis

Ischemic necrosis or bone infarct occur almost exclusively in areas
of predominantly fatty marrow
Figure 4-23-2
Osteonecrosis: Radiologic-Pathologic
Correlation
[Figures 4-23-2 and 4-23-3]
Phase I:
Phase II:
Phase III:
Phase IV:
Phase V:
Cellular death initial response

Cell modulation
Emergence reactive interface
Remodeling reactive interface
Crescent sign & collapse
Bilateral femoral head osteonecrosis

906
Associations
Trauma
Hemoglobinopathy
Steroids
Alcoholism
Dysbaric disorders
Gaucher disease
Pregnancy
Irradiation
Pancreatitis
Figure 4-23-3
Osteonecrosis: Causes
Thrombophilia (increased tendency to develop

thrombosis)
Hypofibrinolysis (reduced ability to lyse thrombi)
Found in 76% of patients with osteonecrosis*
*Glueck et al. Osteonecrosis. AAOS 1997
Osteonecrosis: Causes [Figure 4-23-4]
Radiograph and specimen radiograph showing osteonecrosis

with collapse and crescent sign
Increased size fat cell compresses sinusoid

vascular bed impedes blood flow
Figure 4-23-4
Corresponding gross and macro section showing osteonecrosis

with collapse and crescent sign
Figure 4-23-5
Radiograph showing typical serpentine margin

of infarct
Aseptic
Alcoholism
Sickle cell anemia

Exogenous sterosis
Pancreatitis
Radiograph showing flattening with collapse and crescent sign
Trauma
Idiopathic
Caisson disease (dysbaric)
Figure 4-23-6
Imaging Features: Radiographs

[Figures 4-23-5 and 4-23-6]
Patchy lucent/sclerotic areas

Serpentine sclerosis
Arc-like subchondral lucencies
Articular collapse
Preservation of joint space
Surrounding osteopenia
Osteonecrosis of proximal pole of scaphoid
with surrounding osteopenia
907
Imaging Features: Scintigraphy
Figure 4-23-7
[Figures 4-23-7]
Decreased or absent uptake initially

Increased uptake with repair & revascularisation
Imaging Features: CT [Figures 4-23-8 and 4-23-9]
Variable findings with age of lesion

Alterations in osseous architecture
Useful to evaluate the integrity of the articular
surface
Bilateral osteonecrosis with increased tracer accumulation on

right, left is normal
Figure 4-23-8
Figure 4-23-9
Bilateral osteonecrosis with collapse on left
CT showing reactive interface bilaterally
Figure 4-23-10
Imaging Features: MRI

[Figures 4-23-10 and 4-23-11]
Ring or band pattern

Double line sign
Joint effusion
Marrow edema
Articular collapse
Osteonecrosis and infarcts with double line sign
Figure 4-23-11
Osteonecrosis with edema pattern
908
Transient Osteoporosis [Figures 4-23-12 to 4-23-14]
Described originally in 3rd trimester

Typically young and middle-aged adults
Progressive hip pain, symptoms regress in 2-6
months
Edema pattern on MR, osteoporosis on
radiographs
Figure 4-23-12
Figure 4-23-13
Transient osteoporosis with edema pattern and no

osteonecrosis
Figure 4-23-14
Note regional osteoporosis of right hip
Radiographic Staging [Figure 4-23-15]
Stage
0
1
2
3
4
5
Findings
Clinically suspected, imaging normal
Clinical findings, abnormal scintigram
Osteopenia, cysts, bone sclerosis
Crescent sign without collapse
Flattening with normal joint space
Joint narrowing with abnormal
acetabulum
Note regional osteoporosis of left hip
Figure 4-23-15
Complications: Osteonecrosis
[Figures 4-23-16]
Cartilaginous abnormalities
Intra-articular loose bodies
Cyst formation
Figure 4-23-16
Bilateral osteonecrosis
Note screw treads extending through

infarct with associated high grade
sarcoma
Infarct with malignant transformation
909
Spontaneous Osteonecrosis
Figure 4-23-17
[Figures 4-23-17 and 4-23-18]
Middle-aged to elderly
Abrupt onset pain, swelling, < rom
Weight bearing surface
Medial femoral condyle
? traumatic, ? vascular, ? meniscal tear
Osteochondritis Dissecans
[Figures 4-23-19 to 4-23-21]
Fragmentation and possible separation of articular

surface
Typically childhood to adolescent
Variable symptoms
Non-weight bearing surface
Classic: lateral medial femoral condyle
Probably traumatic in origin
Spontaneous osteonecrosis medial femoral condyle.

Presentation (a) and at 5 months (b)
Figure 4-23-18
Figure 4-23-19
Spontaneous osteonecrosis medial femoral condyle. T1 (left)

and T2 (right)
Osteochondritis dissecans, classic location
Figure 4-23-20
Figure 4-23-21
MR-Osteochondritis dissecans, classic location
Osteochondritis dissecans, classic location, Radiograph (left)

and MR (right)
910
Osteochondroses
Varied disorders characterized by:

Predilection for children
Involvement of epiphysis or apophysis
Radiographs showing fragmentation, collapse, sclerosis and reossification
Osteonecrosis is not a feature in many, and is secondary (to trauma) in others
Some are normal variations
Osteochondroses: Characterized by Osteonecrosis
Lunate: Kienbck
2nd metatarsal: Frieburg
Femoral head: Legg-Calv-Perthes
Tarsal navicular: Kehler
Capitulum: Panner
Figure 4-23-22
Kienbcks Disease [Figures 4-23-22]
Most common in young adults (2040 yrs)

Trauma hx +/-, pain, swelling, <rom, 75% have
ulna minus variance
Fracture & osteonecrosis histologically
Radiographs normal initially
->increased density, altered shape, collapse and
fragmentation
Osteochondroses: Without Osteonecrosis
Spine: Scheuermann
Tibial tubercle: Osgood-Schlatter
Tibial epiphysis: Blount
Patella: Sinding-Larsen-Johansson
Kienbocks disease
Figure 4-23-23
Scheuermann Disease
[Figures 4-23-23]
Described in adolescent farm workers

Common, seen in about 4%8% population
Presents second decade, M=F
Radiographs show end plate irregularity, narrowed
disc spaces, and Schmorls nodes involving three
or more vertebrae
Osteochondroses: Variations in Normal

Ossification
Calcaneous: Sever
Ischiopubic synchondrosis: Van Neck
Scheurmann disease
Summary
Morphologic changes in osteonecrosis are relatively characteristic, although

they will vary with location.
There are a variety of predisposing conditions, as well as those patients in
which no cause is found.
The term osteochondrosis is used for a variety of conditions, many of which
show no evidence of osteonecrosis
References
1.
2.
3.
4.
Sweet et al. Osteonecrosis: pathogenesis. In: Resnick D, ed. Diagnosis of bone and joint disorders, 4th ed.
Philadelphia: WB Saunders, 2002
Iida et al. Correlation between bone marrow edema and collapse of the femoral head in steroid-induced
osteonecrosis. AJR 2000;174:735
Vande Berg et al. MR imaging of avascular necrosis and transient marrow edema of the femoral head.
RadioGraphics 1993;13:501
Glueck et al. Thromophilia, hypofibrinolysis, and osteonecrosis. Clin Orthop 1997;334:43
911
Approach to the Inflammatory

Arthropathies
Donald J. Flemming, MD
Figure 4-24-1
Inflammatory Arthropathies
Rheumatoid Arthritis
Spondyloarthropathies
Ankylosing spondylitis
Enteropathic arthritis
Crohn, Ulcerative Colitis, Whipple
Psoriatic arthritis
Reiter Disease
Juvenile Chronic Arthritis
Radiographic Assessment
Soft Tissue Swelling

Soft Tissue Calcification
Mineralization
Joint Space Change
Erosion
Bone Production
Subluxation
Distribution
AP radiograph with non specific fusiform soft tissue swelling

surrounding the proximal interphalangeal joint of the ring finger.
Clinical photograph in a different patient with rheumatoid
arthritis and synovitis and fusiform soft tissue swelling involving
the proximal interphalangeal of the index and middle fingers
Soft Tissue Swelling [Figure 4-24-1]
Figure 4-24-2
Symmetrical around joint (fusiform)

Diffuse (Sausage digit)
Lumpy, bumpy
Sausage Digit [Figures 4-24-2
and 4-24-3]
Mineralization
Normal
Juxta-articular
Diffuse
AP radiograph of patient with psoriatic arthropathy and diffuse

swelling of the second toe producing a sausage appearance of
the digit. Clinical photograph in a different patient with psoriatic
arthropathy and sausage enlargement of the third and fourth
toes
Juxtaarticular Osteoporosis [Figure 4-24-4]
Figure 4-24-3
Figure 4-24-4
MR of sausage digit with intermediate signal of tenosynovitis

surrounding the flexor tendon sheath of second digit. Tendon
sheath of first digit is normal. MIP projections following
intravenous contrast administration show diffuse enhancement
of second, third and fourth toes in patient with psoriatic arthritis
and sausage digits
27 year old man with reactive arthritis and erosive disease in

the right first metatarsal phalangeal joint. Loss of subchondral
bone is seen in the second, third, fourth and fifth metatarsal
phalangeal joints indicating hyperemia associated with
inflammation
Approach to the Inflammatory Arthropathies
912
Joint Space Change [Figures 4-24-5 and 4-24-6]
Figure 4-24-5
Widening
Normal
Uniform narrowing
Asymmetrical narrowing
Ankylosis
Figure 4-24-6
The interphalangeal joints should all be

similar in dimension as should the
metacarpal phalangeal joints when
compared to the neighboring
articulations. A horizontal scan pattern
is useful to detect subtle joint space
narrowing. Note the loss of joint space
in the distal interphalangeal joint of the
ring finger in this patient with posttraumatic osteoarthritis
Joint space narrowing is important for differential diagnosis.

Both patients have erosions involving the wrist presenting as
lucencies in the carpal bones.
Patient A has rheumatoid arthritis with diffuse narrowing of all of
the carpal articulations.
Patient B has gout with maintenance of joint space despite
extensive erosive disease
Figure 4-24-7
Erosions [Figures 4-24-7 and 4-24-8]
Aggressive
Marginal
Central
Nonaggressive
Early erosions
Thin cartilage
Absent cartilage
Figure 4-24-8
Photomicrograph of axially sectioned fifth

metatarsal head showing destruction of
subchondral bone that would present as an
erosion on radiography
Typical diarthrodial joint anatomy. In early disease, the articular

cartilage (light blue) prevents synovial inflammation (red) from
damaging subchondral bone (white). Erosions are seen
earliest where cartilage is thinnest or where cartilage is absent.
Minimal or no cartilage is present at the margins of a typical
synovial joint adjacent to the attachment of fibrous capsule
913
Bone Production [Figure 4-24-9]
Figure 4-24-9
Reparative Response
Whiskering/ brush stroke erosions
Overhanging edge of cortex
Subchondral bone
Osteophytes
Enthesopathy
Periostitis
Ankylosis
Most common in females 24:1

Most common in the fourth and fifth decades
Incidence 0.2-0.4/1,000 in females
Prevalence 0.5% 1.0%
Probably heterogeneous disorder
Patient A has psoriatic arthritis with erosions and bone

production in the metatarsal phalangeal and interphalangeal
joints. Bone formation is present amongst the erosive changes
producing a whiskering type appearance and the digits are
dense secondary to osteitis. Patient B has rheumatoid arthritis
with erosive disease in the metatarsal phalangeal joints but no
bone production
Genetic influence HLA-DR4 (DRB1 allele)

Pregnancy increased risk postpartum
Infectious agents ?
Chlamydia
RA Presentations
Gradual onset, polyarthritis typical

Mono or pauciarticular unusual
Abrupt, acute polyarthritis unusual
Systemic disease
Felty Syndrome
RA
Splenomegaly
Leukopenia
Figure 4-24-10
RA Diagnostic Criteria
Morning stiffness -Three or more joints involved

Arthritis of hand joints
Symmetric arthritis
Rheumatoid nodules
Rheumatoid factor 90% patients
Positive CCP increases specificity
Radiographic changes
Four criteria to have diagnosis
Classic manifestations of rheumatoid arthritis. Erosions and

joint space narrowing are present in the proximal
interphalangeal, metacarpal phalangeal and wrist joints in a
bilateral and symmetric distribution
Rheumatoid Arthritis: Radiographic

Manifestations
Figure 4-24-11
Fusiform soft tissue swelling

Diffuse or juxta-articular osteoporosis
Uniform joint space narrowing
Aggressive marginal erosions
No bone production
Synovial/subchondral cysts
Bilateral symmetrical distribution
PA view (A) of the wrist is shows subtle joint space narrowing in

the wrist in this patient with rheumatoid arthritis. The Norgaard
view (B) reveals erosive disease in the pisotriquetral joint the is
impossible to appreciate in the PA projection
RA Hand and Wrist [Figures 4-24-10 and 4-24-11]
100% of patients
MCP, PIP joint space loss/ erosions
Pancarpal joint space loss/ erosions
ulnar styloid/ pisotriquetral early
Ulnar drift carpus and digits
Swan-Neck, Boutonniere deformities
Ankylosis rare limited to carpus
914
Ball-catchers View (Norgaard View)
Figure 4-24-12
[Figure 4-24-11]
Rheumatoid Arthritis MR Findings

[Figure 4-24-12]
Synovial hypertrophy
fat saturated fast spin echo T2 weighted images
gadolinium
Erosions
low signal T1W
low to high signal on T2W
surrounding edema
Hyaline cartilage loss
RA- Feet [Figure 4-24-13 ]
Coronal T1 weighted MR of the wrist shows numerous erosions

depicted by intermediate signal replacing fat in subchondral
bone that are difficult to appreciate on the PA radiograph (B)
80%90% of patients
May precede hand dz 10%20%
Forefoot MTP disease predominates
Midfoot talocalcaneonavicular joint
May see osseous ankylosis
Hindfoot retrocalcaneal bursa
Figure 4-24-13
RA Knee and Hip [Figure 4-24-14]
Knee 80%
Pancompartmental joint space loss
Minimal erosions
Hips 50%
Axial migration
Acetabular protrusio
Medial deviation beyond ilioischical line
3mm in males; 6mm in females
RA Shoulder and Elbow
Shoulder 60% of patients

Erosion in humeral head tend to be lateral
Rotator cuff tear common
Bilateral AC joint erosive disease
Elbow ~ 34% of patients
Classic manifestations of rheumatoid arthritis of the feet.

Erosions and joint space narrowing are seen in the metatarsal
phalangeal joints. The erosions are more readily seen on the
medial aspect of the first through fifth metatarsal heads and the
lateral aspect of the fifth metatarsal head
Figure 4-24-14
RA- Cervical Spine [Figures 4-24-15]
60% 80 % of patients
Atlantoaxial subluxation
Odontoid process erosion
MRI best defines extent of pannus
Apophyseal joint dz
Erosion of joints of Luschka
Spinous process erosions
Figure 4-24-15
Rheumatoid arthritis involving the knee. Note tricompartmental

loss of joint space without erosions or osteophyte formation
Rheumatoid arthritis of the cervical spine with instability at C1C2. Widening of the atlantoaxial joint is seen only in flexion in
this patient
915
Spondyloarthropathies
Family of inflammatory arthritides of synovium and entheses

Axial and asymmetric peripheral arthritis
Genetic predisposition HLA B27
Infectious etiology
Spondyloarthropathy Criteria
Inflammatory spine pain or synovitis

and one or more of following
Positive family hx
Psoriasis/ IBD
Urethritis/cervicitis/diarrhea within 1 month
Buttock pain
Enthesopathy
Sacroiliitis
HLA B27
Normal population -USA ~08%

Ankylosing spondylitis >90%
Reiter Disease 63%75%
Psoriasis not increased without arthritis
with peripheral arthritis 20%
with axial arthritis 50%
IBD with axial arthritis 50%
Figure 4-24-16
Psoriatic Arthritis
Peak ages 2040 years

M:F 1:1
Spine and DIP M>F
Symmetric polyarthritis F>M
Arthritis in 5%8% of patients with psoriasis
Skin dz before arthritis in 75%
Arthritis before skin dz in 10%
Psoriatic Arthritis: Radiographic

Manifestations

Maintenance of mineralization
Dramatic joint space loss
Bone proliferation
Marginal erosions predominate
Pencil-in-cup erosions
Bilateral asymmetric dz
Psoriatic arthropathy of the hands involving the interphalangeal

joints of both hands in a bilateral but asymmetric pattern
Figure 4-24-17
Psoriatic Arthritis:
Radiographic Manifestations
[Figures 4-24-16 and 4-24-17]
Hand/Feet
Distribution
IP joints asymmetric
Ray distribution
RA distribution
Acroosteolysis
Ankylosis ~ 15%
Calcaneal erosion plantar bone proliferation
Wrist pancarpal
Typical central erosion in patient with erosive osteoarthritis

compared to marginal erosions seen in patient with psoriatic
arthritis
916
Erosive Osteoarthritis
Figure 4-24-18
Asymmetrical soft tissues around joint

Normal mineralization
Nonuniform loss of joint space
Central sea gull erosions
Osteophytes
Subchondral sclerosis
Distribution symmetrical
Psoriatic Arthritis:
SI Joints 30%50%
Bilateral asymmetrical (symmetrical)
Erosion (iliac > sacral) and repair
Spine 17%
Large, bulky, lateral bone outgrowths
Unilateral or bilateral, asymmetrical
Infrequent apophyseal involvement in lumbar
spine
Reactive Arthritis: (Reiter Disease)
Young adults
M:F 501:1
Annual incidence 3040/100,000
Frequently associated with infection
Urethritis/cervicitis
Diarrhea Shigella, Salmonella, Campylobacter
Typical fluffy inflammatory plantar calcaneal enthesophyte that

parallels the undersurface of the calcaneus in patient with
reactive arthritis. The bone is dense and an erosion is present
in the posterior superior aspect of the calcaneus
Figure 4-24-19
Reiter Disease/Reactive Arthritis:

Diffuse soft tissue swelling

Early juxta-articular osteoporosis
Late normal mineralization
Uniform joint space loss
Aggressive marginal erosions
Bone production
Bilateral asymmetrical distribution
Feet, ankles, knees and SI joints
25 year old man with reactive arthritis. Erosions and subluxation

are seen in the metatarsal phalangeal joints in a bilateral but
asymmetric pattern. Subtle bone formation is present along the
medial aspect of the navicular and the medial cuneiform of the
left foot as a manifestation of the asymmetric nature of this
disease
Reiter Disease/Reactive Arthritis:

[Figures 4-24-18 to 4-24-20]
Figure 4-24-20
Feet 40%55%
IPs and MTPs
Erosions with repair
Periostitis along diaphyses
Calcaneus 25%50%
May be sole sight of disease
Plantar and posterior erosions
Enthesophytes
Ankle 30%50%
Joint space loss and periostitis
Knee
Effusion
Joint space loss and periostitis
SI joints
Bilateral asymmetric
Erosions and repair
Bone formation at the posteromedial aspect of

the distal tibia in a patient with psoriatic arthritis
917
Reiter vs. Psoriatic
Figure 4-24-21
Juxtaarticular osteoporosis
Periostitis without joint findings
Less ankylosis of IP joints
Tendency to involve MTP vs. IP joints
Lower extremity involvement predominates
Ankylosing Spondylitis
Peak age of onset 1535 years

M:F 35:1
Incidence ~ 6.6/100,000
Strong association with HLA B27
Rare in blacks
Predilection for axial involvement
AS-Radiographic Manifestations
Typical presentation of ankylosing spondylitis with erosions and

sclerosis involving the inferior aspect of the SI joints in a
bilateral and symmetric pattern
[Figures 4-24-21 and 4-24-22]
Sacroiliac disease
Bilateral symmetric same as enteropathic
Erosions predominate iliac vs. sacrum
Sclerosis
Ankylosis
Other pelvic dz
Pubic symphysis 16%23 % erosion and
ankylosis
Enthesitis ilium and ischium
Figure 4-24-22
Sacroiliitis: Differential Diagnosis
Ankylosing Spondylitis
Enteropathic Arthropathy
Psoriasis
Reactive Arthritis
Hyperparathyroidism
Osteiitis Condensans
Infection
Ferguson view of the pelvis with bilateral symmetric sacroillitis

in patient with ankylosing spondylitis
AS-Radiographic Manifestations
[Figures 4-24-23 to 4-24-25]
Spine Disease ascends from lumbar to cervical

Discovertebral destruction
Romanus and Andersson lesion
Shiny corner sign
Squaring of vertebral body
Syndesmophyte
Bamboo spine
Trolley track and Dagger signs
Atlantoaxial disease
Figure 4-24-23
Density confined to the anterior superior and inferior end plates

of the lumbar spine resulting in the classic shiny corner
presentation of ankylosing spondylitis. Note the lack of anterior
concavity of the vertebral bodies that contributes to its
squared appearance
918
Figure 4-24-24
Figure 4-24-25
Lateral radiograph of the cervical spine in patient with

ankylosing spondylitis shows thin posterior and anterior
syndesmophytes and fusion of the facet joints. Lateral
radiograph of the thoracic spine also shows thin posterior and
anterior syndesmophytes
The normal lumbar vertebral body is concave anteriorally. The

41 year old patient with ankylosing spondylitis shows bone
formation at the anterior aspect of the vertebral bodies resulting
is a squared appearance
Figure 4-24-26
DISH: Diffuse Idiopathic Skeletal

Hyperostosis [Figure 4-24-26]
Common disease 12% of elderly population

Flowing bulky paravertebral ossification
Four contiguous vertebral bodies
Thoracic>lumbar>cervical
Enthesophytes particularly pelvis
Absence of erosions/ joint abnormality
AS-Extraskeletal Manifestations
Uveitis
Ascending aortitis/ aortic valve disease
Cardiac conduction abnormalities
Interstitial lung dz - upper lobes
Juvenile Chronic Arthritis
JRA (seronegative) 70%

Still Disease, pauci/monarticular, polyarticular
Juvenile-onset adult type RA 10%
Juvenile-onset ankylosing spondylitis
Psoriatic arthritis
Enteropathic arthritis
Reactive/ Reiter arthritis
Bulky paravertebral ossification in patients with DISH is usually

easily distinguished from the thin anterior syndesmophytes of
ankylosing spondylitis that typically have no horizontal
component
Still Disease: Systemic Disease (Classic)
M:F = 1:1
Age usually less than 5 years
Acute febrile illness
Rash
Generalized adenopathy/hepatosplenomegaly
Pericarditis
Mild joint findings arthralgias/mild arthritis
919
JRA-Still Disease: Pauci or Monoarticular
Females more commonly than males

Large joint disease
Knees, ankles, elbows, and wrists
Figure 4-24-27
JRA: Polyarticular
M:F = 1:1
Symmetric arthritis
Hands MCP, PIP
Wrists
Knees/Ankles
Feet intertarsal, MTT, MTP, IPs
Cervical spine
Juvenile-onset adult type RA

(Seropositive JRA)
Classic manifestation of mono or pauciarticular JRA. The left

knee is enlarged and shows advanced bone age in comparison
to asymptomatic right knee. Note the lack of erosions and joint
space narrowing
F>M
>10 years of age at onset
Polyarticular
Subcutaneous nodules
Vasculitis
Figure 4-24-28
JRA: Radiographic Manifestations

[Figures 4-24-27 to 4-24-31]

Osteoporosis
Joint space loss
Not prominent in monoarticular
May be rapid in sero(+) JRA
Ankylosis hands, wrists, cervical spine
Bone erosion not prominent finding
Periostitis
Phalanges, metacarpals, metatarsals
Ballooned epiphyses
Accelerated skeletal growth
Premature fusion of physes
17 year old man with polyarticular JRA and left hip pain. The
femoral heads are enlarged resulting in ballooned
appearance of the epiphyses
Osteoarthritis
Most common arthropathy

~ 80% of patients over 75 years
Second only to CHD as cause of work disability for
men > 50 years of age
Primary no underlying abnormality
Secondary -preexisting metabolic, anatomic,
traumatic, or inflammatory condition
Figure 4-24-29
Osteoarthritis: Definition
American College of Rheumatology

a heterogeneous group of conditions that lead to
joint symptoms and signs which are associated with
defective integrity of articular cartilage, in addition to
related changes in the underlying bone at the joint
margins.
Coronal SPGR image of the left hip in patient with JRA showing
marked irregularity in the articular cartilage
920
Figure 4-24-30
Figure 4-24-31
Polyarticular JRA of the hands. Generalized osteoporosis and

joint space narrowing is present with striking lack of erosions
Adult patient with JRA as a child. Note ballooned appearance of

the metacarpal heads right greater than left. Wrist involvement
was also aymmetric in this patient. Minimal erosions are seen
Osteoarthritis: Clinical
Increasing prevalence with age over 40

Pain, stiffness, and loss of range of motion
Symptoms may regress or be cyclic
Risk Factors
Heredity AD trait with Heberden nodes
Obesity risk factor for knee and possibly hand
Not risk factor for hip
Hypermobility increases risk
Occupation increased risk in heavy manual labor
No increased risk from recreational sports
Diabetes increases risk
OA in one joint increases risk for other joints
Osteoporosis protective effect in hip OA
Cigarette smoking protective effect
Osteoarthritis and Pain
Most common and important complaint

Source
raised intraosseous pressure
synovitis/bursitis/tenosynovitis
periosteal elevation
muscular imbalance
Less common in very old or young
Psychosocial factors
Radiographic predictors
Osteophytes in knee good predictor
Joint space narrowing in hip predictor
Good in first CMC joint
Poor in hand IP joints
Osteoarthritis: Radiographic Manifestations
Normal mineralization
Nonuniform joint space loss
Absence of erosions
Subchondral new bone formation
Osteophyte formation
Subchondral cysts
Subluxations
921
Osteoarthritis: Subchondral Cysts [Figure 4-24-32]
Not true cysts

Intrusion
Defect in cartilage leads herniation of joint fluid into bone
Cyst size based on joint pressure
Contusion
Repeated insult to subchondral bone leads to resorption
Figure 4-24-32
Osteoarthritis: Osteophytes [Figures 4-24-33 to 4-24-35]
Tend to occur at the margins of joints

Produce enlargement of joint
attempt to stabilize joint
Can be central button osteophyte
May not be dramatic in osteoporotic women
Figure 4-24-33
Typical subchondral lucencies in specimen

radiograph of osteoarthritic femoral head
Osteophytes of the interphalangeal joints of the hands are

usually best appreciated on lateral radiographs
Figure 4-24-34
Figure 4-24-35
Osteoarthritis of the hip with superior lateral joint space

narrowing accompanied by subchondral sclerosis, subchondral
cyst formation and osteophyte production
Large osteophytes projecting from the articular

surfaces of the
medial and lateral femoral condyles
922
Osteoarthritis: Subchondral Sclerosis
Figure 4-24-36
Also known as eburnation

Stimulated by loss of hyaline cartilage
Combination of new bone on existing trabeculae
from microfracture and repair
Osteoarthritis: Radiographic Manifestations

[Figures 4-24-36 and 4-24-37]
Hands
DIP joints Heberden nodes
PIP joints Bouchard nodes
Wrist
First metacarpal-carpal joint
Figure 4-24-37
Patient A shows Heberdon nodes from osteophytes and soft
tissue swelling at the distal interphalangeal joints.
Bouchard nodes are seen at the proximal interphalangeal joints
of patient B
Figure 4-24-38
Hooked osteophytes are seen involving the second and third

metacarpal heads. These may be seen in hemochromatosis,
CPPD arthropathy or osteoarthritis
Osteoarthritis - Knee:
[Figures 4-24-38 and 4-24-39]
May require weight-bearing views

Medial compartment 75%
Patellofemoral joint 48%
Lateral compartment 26%
Pancompartmental
Think deposition dz or prior inflammatory
arthropathy
Non weight bearing AP of the knee (A) shows osteoarthritis in

the medial compartment with subchondral sclerosis and
osteophyte formation but the joint space appears maintained.
AP weight bearing view of the knee (B) shows the expected
loss of joint space in the medial aspect of the knee
Figure 4-24-39
Osteoarthritis - Hip: Radiographic

Manifestations
Superolateral migration
60%
M>F
Medial migration
25%
F>M
Axial migration
Think deposition dz or prior inflammatory dz
56 year old woman with acromegaly. Manifestations of

osteoarthritis are seen but the joint spaces are widened rather
than narrowed
923
Hip Joint Space Narrowing
Osteoarthritis - Foot: Radiographic Manifestations
Occurs along lines of weight bearing

First MTP joint
Hallux rigidus
Hallux valgus
First MTT joint
Talonavicular joint
Dorsal talar beak (coalition vs. DJD)
References
1.
2.
Brower A: Arthritis in Black and White, 2nd ed. Philadelphia, Pa: WB Saunders; 1997: 252.
Resnick D ed. Diagnosis of bone and joint disorders, 4th Ed. Philadelphia: W.B.Saunders, 2002:
924
MRI of the Rotator Cuff

Donald J. Flemming, MD
MRI of Rotator Cuff Tears
Utopia
CSS FT
ESS FT
CCS PT
ESS PT
Sensitivity
89%100%
56%
78%
0%
71%
Specificity
88%97%
73%
83%
68%
71%
CCS-Clinical Community; ESS-Experienced Specialist Arthroscopy 1997;

13:710719
MRI of the Rotator Cuff: Goals
Review anatomy/positioning
Review MR appearance of tears
Discuss problem tears
Discuss clinical mimics of rotator cuff tear
Discuss the radiologic report
Shoulder Pain
Rotator Cuff Disease

Impingement
Arthritis
Adhesive Capsulitis
Cervical Spine
Referred Pain
Instability
Fracture
Osteonecrosis
Nerve Entrapment Syndromes
Bursitis
Figure 4-25-1
Shoulder Imaging
AP radiograph of the shoulder shows superior narrowing of the

humeral acromial space indicative of a large rotator cuff tear.
Coronal oblique T2 weighted in the same patient confirms a
large tear in the supraspinatus tendon
Radiographs
Arthrography
CT Arthrography
Ultrasound
MRI
Radiography [Figure 4-25-1]
Humeroacromial space <7mm

Massive tear
CT and Ultrasound [Figure 4-25-2]
Figure 4-25-2
MRI of the Shoulder
Rotator cuff
Glenoid labrum
Capsule
Biceps tendon
Bone marrow
Acromial shape
AC joint
Sub-deltoid bursa
Supraspinatus notch
Coracohumeral lig
Humeral head shape
Coronally reconstructed CT image following a direct arthrogram

shows a small full thickness rotator cuff tear in the
supraspinatus tendon. Coronally acquired ultrasound image in
the same patient demonstrates the full thickness tear as a focal
hypoechoic defect in the hyperechoic tendon
925
Figure 4-25-3
Coil-dedicated shoulder
Slice thickness 34 mm
Matrix 256x192 or 256x256
FOV 16 cm
Patient position
External rotation vs. neutral
ABER
Contrast Indirect or Direct
Pulse Sequences
Spin-echo
Fast spin-echo (fat-sat)
Sensitive for cuff tear
STIR
Gradient echo
3D volume
Axial gradient echo image (A) with typical planscan for coronal
oblique images drawn perpendicular to the glenoid. Axial
gradient echo image through the supraspinatus at the superior
aspect of the shoulder in internal rotation (B) shows the plane
of acquisition will cross the tendon obliquely. Axial gradient
echo image through the supraspinatus at the superior aspect of
the shoulder in external rotation (C) shows the plane of
acquisition will parallel the tendon
Imaging Planes
Axial
Assess subscapularis, biceps tendon
Coronal obliques
Parallel to supraspinatus tendon
Assess all tendons
Sagittal oblique (FSE T2)
900 to coronals
Assess all tendons
Figure 4-25-4
Plane of Scan [Figure 4-25-3]

Rotator Cuff
Dynamic stabilizer
Complex coordination
Five layers histologically
Components
SITS muscles
Rotator cuff interval
Coracohumeral ligament
Long head biceps tendon
Coronal oblique T1 weighted image through the infraspinatus with

corresponding gross anatomy
Figure 4-25-5
Coronal Anatomy [Figures 4-25-4 to 4-25-6]

Sagittal Anatomy [Figure 4-25-7
Axial Anatomy
Figure 4-25-6
[Figure 4-25-8]
Coronal oblique T1 weighted image through the supraspinatus with

Coronal oblique T1 weighted image through the subscapularis with

926
Figure 4-25-7
Sagittal oblique T2 weighted image at the level of the glenohumeral joint shows
the normal rotator cuff muscle anatomy
Normal Anatomy
http://rad.usuhs.mil/rad/anatomy/shoulder/intro.html
Figure 4-25-8
Rotator Cuff Tear
Impingement
Overuse
Aging
Chronic inflammatory disease
Acute trauma
Instability
Pathogenesis RCT: Neer
Stage I (<25 y/o)

Edema/ hemorrhage
Stage II (25-40 y/o)
Fibrosis/ thickening
Stage III (>40 y/o)
Partial/ Complete Tear
Axial gradient echo image shows the normal subscapularis tendon anteriorally.
The long head biceps tendon is normally situated in the bicipital groove
Figure 4-25-9
Impingement Syndrome
Clinical - not radiologic diagnosis

Pain with abduction and external rotation
Pain with elevation and internal rotation
(Neer impingement sign)
Mechanical causes
Acromial shape, position
AC joint osteophyte
Thick coracoacromial ligament
Instability
Impingement [Figure 4-25-9]

Acromial Variation
Shape
Type I
Type II
Type III
Lateral Downsloping
Anterior Downsloping
Os acromiale
Acromial Variation
Increase in number increases risk of tear

Type I - flat
Type II curved
Type III hooked
Assess on sagittal images
With abduction, flexion and internal rotation,

the rotator cuff may impinge on the
coracoacromial arch
927
Os Acromiale [Figures 4-25-10]
Figure 4-25-10
Increased risk of Cuff Tear

Best seen on axial images
Injury to Syndesmosis
Rotator Cuff Tear Types
Full thickness
Communication between joint space and SA
bursa
Partial thickness
Partial undersurface
Partial Bursal surface
Intrasubstance
Rim rent
Myotendinous
Rotator Cuff Tear
Coronal oblique T1 weighted image posterior to the

acromioclavicular joint (A) shows the syndesmosis of the
accessory ossicle. Coronal oblique T1 weighted image at the
acromioclavicular joint (B) shows the AC joint and
supraspinatus tendon. The os acromiale is best seen on the
axial image (C ) at the level of the AC joint
Primary
Increased signal in tendon
Interruption of tendon
Secondary
Retraction of musculotendinous junction
Obliteration of subacromial bursal fat plane
Fluid in subacromial bursa
Atrophy of muscles
Fluid in muscle belly
Figure 4-25-11
Increased Signal within Tendon:

Short TE images
Magic angle
Connective tissue between fascicles
Tendon overlap (internal rotation)
Degeneration
Tear
Partial volume
Injection
Signal within Tendon: Long TE images
Axial gradient echo image at the superior aspect of the humeral

head (A) shows the anterior aspect of infraspinatus tendon
overlapping (lateral) the posterior aspect of the supraspinatus
tendon (medial). Coronal T1 weighted image at the level of
infra and supraspinatus tendon overlap shows normal high
signal within the junction of the two tendons
Mild degeneration Low Magic angle

Severe degeneration Intermediate Partial Tear
Tear High
Tendon Overlap [Figure 4-25-11]

Partial Tears
Figure 4-25-12
Twice as common as full thickness

Intrasubstance most common
Bursal Surface least common
Poor response to conservative Rx
Increased detection
Contrast
ABER
Significant if >50% of tendon thickness
Coronal T2 weighted image through the supraspinatus tendon

(left) shows a deep partial undersurface tear. Coronal T2
weighted image through the supraspinatus tendon of a different
patient (right) shows fluid signal interrupting the articular
surface of the supraspinatus tendon but the bursal surface is
intact indicating an undersurface tear. Both patients have SLAP
tears of the superior labrum
Partial Undersurface [Figure 4-25-12]
928
Intrasubstance vs Partial US:

Value of ABER [Figure 4-25-13]
Figure 4-25-13
Rim Rent Tear [Figure 4-25-14]
Seen in young patients

Usually anterior
Intrasubstance vs. partial undersurface
Subscapularis Tears [Figure 4-25-15]
Abnormal lift-off test on PE

Uncommon 2% of all tears
LHBT dislocation-49%
Look for on axial and anterior coronals
Sagittals provide more clues
Devastating to surgeon if missed
Easy to miss on arthroscopy
T1 fat saturated image of the shoulder following indirect

arthrography in the ABER position (left) shows the
undersurface of the infraspinatus tendon is intact. The
conventional coronal oblique T1 weighted image through
infraspinatus tendon was suggested an undersurface tear
Figure 4-25-14
Long Head of Biceps Tendon
Abnormality frequently associated with RCT

Medial Dislocation
Abnormal bicipital groove
Chronic impingement
Usually extra-articular
Intra-articular with/without subscapularis tendon injury
Associated with degeneration of tendon
Long Head of Biceps Tendon
Tendonitis
Increased signal in tendon
Thickening of tendon
Rupture of tendon
Intracapsular
Extracapsular
Ovoid/ heart shaped partial tear
Coronal oblique fat sat T2 weighted image

shows horizontally oriented increased signal in
the insertional portion of the posterior
supraspinatus tendon consistent with a tear but
it is difficult to determine whether the tear is an
undersurface or intrasubstance defect
Figure 4-25-15
Subscapularis Insertion [Figure 4-25-16]
Figure 4-25-16
Sagittal T2 weighted image at the level of the lesser tuberosity
shows a focus of high signal in the subscapularis tendon
representing a partial undersurface tear. The tear is also seen
on the axial T1 weighted gradient echo image following indirect
arthrography (right) but is easier to see on the sagittal image
Axial gradient echo image through the subscapularis tendon

(A) that appears to only insert on the lesser tuberosity on this
image. However, photo of a gross specimen and a
photomicrograph through the insertion of the subscapularis
show that the tendon inserts on both the lesser and greater
tuberosity. The transverse ligament covering the bicipital
groove is not actually a ligament but represents the portion of
the subscapularis tendon that inserts on the greater tuberosity
[Courtesy Tim Sanders, M.D.]
929
Rotator Cuff Atrophy
Figure 4-25-17
Rotator cuff tear

Acute brachial neuritis
Nerve entrapment syndromes
Suprascapular nerve entrapment
Quadrilateral space syndrome
Suprascapular Nerve Entrapment
Suprascapular Notch
Supraspinatus/ Infraspinatus innervation
Spinoglenoid Notch
Infraspinatus innervation
Atrophy of SSM and ISM
Look for mass in region of suprascapular notch
Spinoglenoid Notch Entrapment [Figure 4-25-17]
Infraspinatus atrophy
Quadrilateral Space Syndrome [Figure 4-25-18]
Axillary N. Compression
Fibrous band
Pain, paresthesia
Atrophy of deltoid and/or teres minor
Weightlifters
Figure 4-25-18
Coronal oblique fat saturated T2 weighted

image shows a paralabral cyst extending from a
posterior superior labral tear into the
spinoglenoid notch. A clinical photo (B) in the
same patient shows marked atrophy of the right
infraspinatus muscle belly as indicated by the
loss of soft tissue inferior to the scapular spine.
Sagittal T2 image (C) medial to the
spinoglenoid notch shows atrophy and
denervation edema in the infraspinatus muscle
belly
The quadrilateral space is bounded by teres
minor, teres major, long head triceps and the
humerus
Clinical Mimics of Rotator Cuff Tear
Calcific tendonitis
Adhesive capsulitis
Subacromial bursitis
Calcific Tendonitis
Rotator cuff most common site

Primary or secondary disorder?
HADD in tendon
Concretion low T1 and T2
Variable surrounding edema
May erode cortex/ invade marrow
930
Adhesive Capsulitis [Figure 4-25-19]
Figure 4-25-19
Clinical mimic of cuff tear

Capsule thickened
Abnormal enhancement IV gad
Pectoralis Major Tear
Weight-lifters
Sternal and clavicular heads
Sternal head superior on humerus
Clavicular head inferior
Sternal head tear most common
Use torso coil and coronal obliques
Myotendinous vs. tendon
Myotendinous Tear of Pectoralis [Figure 4-25-20]

Figure 4-25-20
Axial T1 weighted gradient echo image (A)

shows marked enhancement in the anterior
inferior capsule following IV contrast
administration indicating adhesive capsulitis in
the atraumatic setting
Coronal oblique T1 (left) and fat sat T2 (right) weighted images

through the pectoralis major muscle show hemorrhage
associated with a myotendinous injury. The distal tendon is
intact
Radiologic Report
Acromion-os acromiale
Tendon normal, tendinosis, tear
Size and location of tear
Massive>5cm
Partial thickness tear
> or < 50% thickness of tendon
Retraction/Muscle atrophy
References
1.
2.
Zlatkin MB, Needell SD, Hoffman C. MRI of the Shoulder, 2nd Edition. Lippincott
Williams & Wilkins, Philadelphia, PA. 2003.
Steinbach LS, Peterfy CG, Tirman PFJ, Feller JF eds. Shoulder Magnetic Resonance
Imaging. Lippincott Williams & Wilkins, Philadelphia, PA. September 1998
931
MR Arthrography of Glenohumeral
Instability
Timothy G. Sanders, MD
Glenohumeral Joint
Intrinsically Unstable joint

Shallow glenoid fossa
Large articular surface of the humeral head
Static Stabilizers
Joint capsule
Glenohumeral Ligaments
Glenoid labrum
Dynamic Stabilizers
Rotator cuff
Long Head of the Biceps Tendon
Figure 4-26-1
Classification
TUBS
Traumatic
Unidirectional
Bankart
Surgery
AMBRI
Atraumatic
Multidirectional
Bilateral
Rehabilitation
Inferior Capsular Shift
Superior glenohumeral ligament
Figure 4-26-2
Multidirectional Instability
AMBRI Patient
Causes of Multidirectional Instability
Hypermobility or Laxity
Stretching or Overuse of Support Structures
MR Imaging not usually Required
MR Findings Nonspecific
MR Useful if Direction Unknown to Rule Out Conventional
Causes
Anterior Stabilizers
Labrum
Glenohumeral Ligaments
Capsule
Subscapularis Muscle
Most Important Anterior Stabilizer: Inferior Glenohumeral
Labroligamentous complex
Anteroinferior labrum
Anterior Band of the Inferior Glenohumeral Ligament
Middle glenohumeral ligament
Figure 4-26-3
Normal Labrum
Anterior and Posterior Labrum best seen in the Axial Plane

LABRUM:
Dark on all Pulse Sequences
May be triangular, rounded, or blunted
Normal Superior Labrum
Seen Best in the Coronal Plane

Superior Labrum:
Dark on all pulse sequences
Triangular
Extends off of Superior Glenoid
MR Arthrography of Glenohumeral Instability
Inferior glenohumeral ligament
932
Superior Glenohumeral Ligament [Figure 4-26-1]
Prevents inferior subluxation with arm in 0 abduction

Courses from superior glenoid tubercle to lesser tuberosity
Parallels Coracoid process
Figure 4-26-4
Middle Glenohumeral Ligament [Figure 4-26-2]
Prevents external rotation of humeral head when

arm is between 45 and 60 of abduction
Originates at superior glenoid tubercle
Courses obliquely superficial to the anterior labrum
Blends with the deep fibers of subscapularis
Most variable of the glenohumeral ligaments
Inferior Glenohumeral Ligament [Figure 4-26-3]
Most important GHL

Prevents anterior subluxation with arm in full
abduction and external rotation
Extends from anterior inferior labrum to humeral neck
Lax with arm in neutral position
Redundant when the arm is in neutral position
Left: Scan plane for the ABER view

Right: Normal ABER view
Figure 4-26-5
Scout Position and Scan Plane for ABER [Figure 4-26-4]
Scan plane along the long axis of the humeral shaft

Coronal scout with arm in ABER position
Stretches anterior band of IGHL
Normal Anatomic Variants
1. Cartilage Undermining
Articular cartilage hyaline- intermediate signal intensity
Labrum- fibrocartilage- low signal intensity
Smooth, tapering
Does not Extend Completely Beneath Labrum
2. Sublabral Foramen (Hole)
Occurs only in the anterosuperior quadrant
Complete detachment of the labrum from the glenoid
3. Sublabral Recess [Figure 4-26-5]
Smooth, tapering
Extends toward the glenoid
No signal extends into the black triangle of the superior labrum
Can mimic a SLAP tear
Buford complex [Figure 4-26-6]
1.5 % of patients
Can mimic anterior labral tear
Thick cord-like MGHL
Absent or diminutive anterior-superior labrum
Sublabral recess
Figure 4-26-6
Anterior Instability
95% of all dislocations

Mechanism
Fall on outstretched arm
Abduction and external rotation
Bufford complex
933
Bankart Lesions [Figure 4-26-7]
Figure 4-26-7
The most common injury following anterior

dislocation
First-time dislocators under 35 y.o.
Anterior labro-ligamentous avulsion with disruption of
the medial scapular periosteum
Osseous Bankart Lesion [Figure 4-26-8]
Fracture of inferior glenoid

Disruption of the cortex of the anteroinferior glenoid
Hill-Sachs Lesion
Bankart lesion
Results from impaction of humeral head against

anterior-inferior glenoid
Associated with Bankart lesion
Normally: Top 3 images round
Hill-Sachs: flattening or concavity
Acute: + Edema
Figure 4-26-8
Double Axillary Pouch Sign [Figure 4-26-9]
Double axillary pouch: small collection of contrast in

inferior labrum seen on coronal images
Perthes Lesion
Bankart variation (non-displaced)

Labro-ligamentous disruption
Medial scapular periosteum intact
May resynovialize in place
Best detected on ABER view
Osseous Bankart
Figure 4-26-9
Nondisplaced Tear Anteroinferior Labrum Perthes Lesion [Figure 4-26-10]

ALPSA Lesion
Anterior labroligamentous periosteal sleeve avulsion

Intact medial periosteal periosteum
Medialized Bankart lesion
Surgical repair technique differs from Bankart
Double axillary pouch
Medialized Bankart Lesion [Figure 4-26-11]
Figure 4-26-10
ALPSA- Anterior labroligamentous periosteal sleeve

avulsion
Chronic Medialized Bankart Lesion
Labrum scars down medially

Scar tissue mounds up covering medialized labrum
and resynovializes
Treatment: complete Bankart and reconstruction
Perthes Lesion
Figure 4-26-11
ALPSA lesion
934
Axillary Nerve Neuropraxy [Figure 4-26-12]
Figure 4-26-12
Axillary nerve can be stretched at time of anterior dislocation

resulting in denervation atrophy: Deltoid and Infraspinatus
muscles
Denervation atropy:
Acute: edema (high signal on T2)
Chronic: fatty (high signal on T1)
First Time Dislocation Over Age 35
Clinical presentation can be confusing

Tear supraspinatus tendon
Fracture greater tuberosity
Avulse subscapularis and anterior capsule from the humerus
MRI can play pivotal role in directing patient therapy
Tear of the Supraspinatus Tendon
First time dislocation over age 35:

Bankart lesion uncommon
Rotator cuff tendon becomes the weak link
Denervation atrophy of Deltoid muscle
First Time Dislocation Over Age 35
Avulsion fracture of the greater tuberosity
Figure 4-26-13
Greater Tuberosity Fracture
Avulsion of the greater tuberosity is often occult

radiographically
Can mimic RCT
Treated conservatively
MRI can accurately distinguish
Avulsion of Subscapularis
Subscapularis muscle can avulse off of lesser

tuberosity
Associated with dislocation of the biceps tendon
Seen best in axial plane
Humeral Avulsion of the Glenohumeral Ligament (HAGL)

Lesion
Disruption of Subscapularis
Disruption of subscapularis at musculotendinous junction

Requires surgical repair
Figure 4-26-14
Hagl Lesion
Humeral avulsion of the glenohumeral ligament

Results from dislocation
No age predilection
MR findings: contrast extravasation from joint
capsule/ avulsion of subscapularis
Hagl Lesion [Figure 4-26-13]
Inferior GHL can disrupt anywhere along course

Humeral attachment/ mid substance
Difficult to detect with scope
Cause of failed repairs
Can present on MRI as avulsion of subscapularis muscle
Reverse Bankart and Hill Sachs Lesion
Posterior Instability
2% - 4% of all traumatic dislocations

20% - 25% of shoulder instability cases in active duty military population
Adduction with internal rotation
Seizure, electrocution, weight lifting, swimming, lineman blocking
Reverse Hill Sachs, Bankart
Posterior Instability [Figure 4-26-14]
Repetitive microtrauma: non-displaced posterior labral tear

Reverse Bankart
Reverse Hill Sachs
935
Glad Lesion [Figure 4-26-15]
Glenolabral articular disruption

Forced adduction injury (humeral head impacts the glenoid fossa)
Clinically a stable lesion
Partial tear anteroinferior labrum / articular cartilage injury
Figure 4-26-15
Glad Lesion [Figure 4-26-15]
Non displaced tear anteroinferior labrum

Best seen on ABER
Articular Cartilage Injury
Best seen on axial or coronal
Posterior Superior Glenoid Impingement
Also known as Internal Impingement

Undersurface tearing of posterior rotator cuff (posterior SST or
IST)
Impingement between posterior labrum and greater
tuberosity
Throwing athletes- posterior shoulder pain
Associated with anterior instability

[Figure 4-26-16]
Undersurface of posterior rotator cuff impinged

between the greater tuberosity and the
posterosuperior labrum
Seen best on ABER view
Glenolabral Articular Disruption (GLAD) Lesion
MR Findings:
Undersurface tear of posterior rotator cuff
Degenerative changes of posterosuperior labrum
Cystic change in greater tuberosity
Internal impingement seen on ABER view
Figure 4-26-16
Glenohumeral Internal Rotation Deficit (GIRD)
[Figure 4-26-17]
Scarring and thickening of the posterior capsule and has recently
been described as a source of potential pain in throwing athletes
MR imaging demonstrates thickening of the posterior capsule
SLAP Tears
The superior labrum, anterior-to-posterior lesion, can include

biceps tendon
Mechanism
Fall on outstretched arm
Repetitive overhead activity (throwing, swimming)
Symptoms: pain with overhead activity, catching, popping
sensation
Important factors to observe
Figure 4-26-17
Abnormal signal in superior labrum

Extent of lesion
Posterior labrum
Anteroinferior quadrant
Biceps involvement
Type of SLAP tear
Glenohumeral Internal Rotation Deficit (GIRD)

936
SLAP Lesion
Type I [Figure 4-26-18]

Fraying and degeneration; but labrum firmly
attached
Type II [Figure 4-26-19]
Fraying of labrum and superior labrum avulsed
from glenoid
Signal extends into the triangle of the superior
labrum
Type III [Figure 4-26-20]
Bucket-handle tear of the superior labrum; biceps
tendon remains intact
On MR imaging: fragment seen displaced into
superior joint space
Type 4 [Figure 4-26-21]
Bucket-handle tear of superior labrum involves
biceps anchor
Biceps involvement best seen on axial or sagittal
images
Figure 4-26-18
SLAP Type I: Fraying of the undersurface of labrum
Figure 4-26-19
Figure 4-26-20
SLAP Type II: Avulsion of labrum from glenoid
Figure 4-26-21
SLAP Type III: Bucket-handle tear of the superior labrum
Pitfalls: SLAP Tears
1. Sublabral recess
Smooth, tapering
No signal in superior labrum
SLAP tear: any signal extending into black
triangle
2. Sublabral recess: axial images
Smooth linear collection of contrast
SLAP on axial images: irregular contrast
collection
Sublabral recess: coronal images
No displacement of superior labrum
Type 2 SLAP tear
Labrum pulled away from glenoid
SLAP Type IV: Bucket-handle tear with involvement of biceps

anchor
Paralabral Cyst
High association with labral tears and GH joint

instability
Superior labral cyst: SLAP tears
Posterior labral cyst: posterior labral tears
Labral tear may resynovialize
Can result in shoulder pain and adjacent nerve
entrapment
DDX: Intramuscular cysts of rotator cuff associated
with PT tear of the cuff tendon
Figure 4-26-22
Paralabral Cysts [Figure 4-26-22]
SLAP tear with superior paralabral cyst

Suprascapular notch
Suprascapular nerve entrapment
Denervation edema: supraspinatus and infraspinatus
muscles
937
Left: SLAP tear with paralabral cyst

Right: Denervation edema within supraspinatus and
infraspinatus muscles
Paralabral Cysts
Figure 4-26-23
Posterior paralabral cysts

Extend into spinoglenoid notch
Entrapment of suprascapular nerve
Persistent shoulder pain for 3 years
Small anterior labral tear with small adjacent
paralabral cyst
Paralabral cysts
Small inferior labral cyst

Teres Minor normal
3 years later; persistent pain
Paralabral cyst larger
Axillary nerve entrapment
Atrophy Teres Minor
Left: Direct arthroscopic repair of Bankart lesion. Suture

anchors at the 3, 4, and 5 oclock positions
Right: Sagittal MR image shows location of suture anchors
Labral Repair: Surgical Approach
Direct repair of labral and capsular lesions

Indirect repairs
Staple capsulorapphy (Du Toit & Roux)
Subscapularis manipulation to tighten anterior capsule (Putti
Platt/ Magnuson Stack)
Movement of the coracoid process (Bristow procedure)
Figure 4-26-24
Direct Repairs
Arthroscopic/ open (deltopectoral interval)

Suture anchors 3-,4-,5-, oclock position
Capsulorapphy (open/ arthroscopic)
Staple redundant capsule
Done in conjunction with direct repair
High failure rate if done as isolated procedure
Osseous Bankart
Recurrent displace Bankart lesion
MR Findings of Bankart Repair
Figure 4-26-25
[Figure 4-26-23]
Suture anchor artifact from repair may

obscure visualization
MR Findings of Failed Bankart

Repair
[Figure 4-26-24]
Recurrent displaced anterior labrum
Failed Bankart Repair [Figure 4-26-25]
Missed HAGL lesion

In one series up to 30% of failed
repairs
Left: Missed HAGL lesion. IGHL avulsed from humeral neck

Center: Missed HAGL lesion. IGHL avulsed from humeral neck
Right: HAGL lesion
Recurrent SLAP Following Repair [Figure 4-26-26]
Displaced fragment anterosuperior labrum

Osteochondral defect anterosuperior glenoid
Figure 4-26-26
5 Months Following SLAP Repair: Recurrent Pain
Fraying and irregularity of superior labrum; no displaced fragment

Partial thickness articular surface tear rotator cuff
Recurrent SLAP tear with a displaced fragment
938
Multidirectional Instability [Figure 4-26-27]
Figure 4-26-27
Treated first with rehabilitation

Surgery
Inferior capsular shift/plication
Decrease volume of GHJ anteriorly, inferiorly, posteriorly
MR capsular thickening
Hardware Complication [Figure 4-26-28]
Proud suture anchor
Synovitis - Prior SA Decompression and Rotator Cuff

Debridement: Recurrent Pain
Synovitis: 4 mm adhesive capsulitis

Normal postop capsule:
2-4 mm after surgical procedure
Thickened and nodular capsule
Postop Infection [Figure 4-26-29]
Infectious versus reactive synovitis difficult to differentiate with

imaging
Thickened enhancing capsule; effusion/ joint destruction/
cartilage loss/ cysts, erosions
. Normal postoperative MR appearance

following inferior capsular shift. Thickened
capsule
Acute Chondrolysis of the Glenohumeral Joint [Figure 4-26-30]
Rapid onset chondrolysis refers to a condition in which widespread

chondrocyte death occurs within a joint over a relatively short period of time
Devastating complication reported following
arthroscopy and reconstruction of the GHJ in young
individuals
Rapid onset pain
Marked loss of motion
Treatment supportive; eventually total joint
arthroplasty
Figure 4-26-28
Chondrolysis Shoulder:
Proposed Etiologies
Damage from use of thermal probe for capsular

shrinkage
Marcaine pump
Left: MR appearance of a proud suture anchor
Unknown infectious agent
Right: CT appearance of a proud suture anchor
Bioabsorbable material
Mechanical trauma at time of arthroscopy
Chemical trauma to the chondrocytes
Event during arthroscopy that triggers an immune response and subsequent
Figure 4-26-29
migration of inflammatory cells into the GH joint
Figure 4-26-30
Postoperative infection
Acute Chondrolysis of the Glenohumeral Joint following
Shoulder Arthroscopy
939
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
Bankart ASB: Recurrent or habitual dislocation of the shoulder-joint. Br J Surg 26: 23-29, 1938
Beltran J, Rosenberg ZS, Chandnani VP, et al: Glenohumeral instability: Evaluation with MR arthrography.
Radiographics 17: 657-673, 1997
Chandnani VP, Gagliardi JA, Murnane TG, et al: Glenohumeral ligaments and shoulder capsular mechanism:
Evaluation with MR arthrography. Rad 196: 27-32, 1995
Cvitanic O, Tirman PFJ, Feller JF, et al: Using abduction and external rotation of the shoulder to increase the
sensitivity of MR arthrography in revealing tears of the anterior glenoid labrum. AJR 169 837-844, 1997
Kaplan PA, Bryans KC, Davick JP, et al: MR imaging of the normal shoulder: Variants and pitfalls. Rad 184: 519524, 1992
Linker CS, Helms CA, Fritz RC: Quadrilateral space syndrome: Findings at MR imaging. Rad 188: 675-676, 1993
Neviaser RJ, Neviaser TJ, Neviaser JS: Concurrent rupture of the rotator cuff and anterior dislocation of the
shoulder in the older patient. JBJS 70-A: 1308-1311, 1988
Neviaser TJ: The anterior labroligamentous periosteal sleeve avulsion lesion: A cause of anterior instability of the
shoulder. Arthroscopy 9: 17-21, 1993
Neviaser TJ: The GLAD lesion: Another cause of anterior shoulder pain. Arthroscopy 9: 22-23, 1993
Palmer WE, Brown JH, Rosenthal DI: Labral-Ligamentous complex of the shoulder: Evaluation with MR
arthrography. Rad 190: 645-651, 1994
Petersilge CA, Witte DH, Sewell BO, et al: Normal regional anatomy of the shoulder. MRI Clin North Am 5: 667681, 1997
Sanders TG, Tirman PFJ, Linares R: The Glenolabral articular disruption lesion: MR arthrography with
arthroscopic correlation. AJR 172: 171-175, 1999
Schweitzer ME: MR arthrography of the labral-ligamentous complex of the shoulder. Rad 190: 641-643, 1994
Synder SJ, Karzel RP, Pizzo WD, et al: SLAP lesions of the shoulder. Arthroscopy 6: 274-279, 1990
Tirman PFJ, Bost FW, Garvin GJ, et al: Posterosuperior glenoid impingement of the shoulder: Findings at MR
arthrography and MR arthrography with arthroscopic correlation. Rad 193: 431-436, 1994
Tirman PFJ, Feller JF, Jansen DL, et al: Association of glenoid labral cysts with labral tears and glenohumeral
instability: Radiographic findings and clinical significance. Rad 190: 653-658, 1994
Tirman PFJ, Feller JF, Palmer WE, et al: The Buford complexA variation of normal shoulder anatomy: MR
arthrographic imaging features. AJR 166: 869-873, 1996
Tirman PFJ, Steinbach LS, Feller, FJ: Humeral avulsion of the anterior shoulder stabilizing structures after anterior
shoulder dislocation: demonstration by MRI and MR arthrography. Skeletal Radiol 25: 743-748, 1996
Wolf EM, Cheng JC, Dickson K: Humeral avulsion of glenohumeral ligaments as a cause of anterior shoulder
instability. Arthroscopy 11: 600-607, 1995
940
Imaging of Upper Extremity Trauma

Timothy G. Sanders, MD
Figure 4-27-1
Anatomic locations
Shoulder girdle, humerus, elbow, forearm, wrist
and hand
Structures involved
Bones, joints, articular cartilage, tendons,
ligaments
Mechanism of injury
Acute trauma, sports related, repetitive stress
injury
Sterno-clavicular Joint
Dislocation most common injury

Anterior more common than posterior
Grade II separation of AC Joint
Best evaluated with limited CT scan
Normal distal clavicle extends above manubrium- use symmetry as guide
Plain film- 40 cephalic angulation
Clavicle Fractures
Mechanism: indirect trauma- fall on outer prominence of shoulder

Most common site of injury is middle third
Healing may result in deformity (extensive callous)
Distal third fracture must evaluate coracoacromial ligament integrity
Figure 4-27-2
Acromio-clavicular Joint Injuries
Mechanism: fall on outer prominence of shoulder

AC joint: weak capsule and inherently unstable
Grade I injury
mild strain of AC joint
Ligaments intact; point tenderness over AC joint
X-rays normal
Treatment conservative- recovery is spontaneous
Grade II injury
moderate strain [Figure 4-27-1]
Disrupted AC ligament
CC ligaments intact
X-ray- widening of AC joint; slight uplifting of distal clavicle
Treatment conservative- recovery is spontaneous
Grade III injury
severe [Figure 4-27-2]
Ruptured AC and CC ligaments
Complete AC separation with increased distance between
coracoid and clavicle
Grade III separation of the left AC joint
Figure 4-27-3
Surgical Repair of AC Joint Separation
Internal fixation: 8-10 weeks

Until CC ligaments heal
Osteolysis of Distal Clavicle [Figure 4-27-3]
Post-traumatic osteolysis
Complication of trauma (occurs within 2 months of injury, self
limiting)
Repetitive stress (wt. lifters)
X-ray: loss of normal cortical line- distal clavicle
DDX: rheumatoid arthritis, infection, hyperparathyroid
Post-traumatic osteolysis of distal clavicle
941
Scapular Fractures
Mechanism: direct trauma to scapula (MVA)

Uncommon fractures
Frequently missed on X-ray
Intra-articular fracture important clinically
Fracture of scapular body- source of considerable pain
Fracture of the neck of the scapula
Non-articular fractures: clinically insignificant; musculature holds fragments in
place; conservative treatment
Scapular Fractures
CT- imaging modality of choice for evaluating scapula
Scapular Fractures
Acromion process fractures

Direct trauma
Restore active range of motion; if severely comminuted- excise fragments
Scapular Fractures: Acromion
Os Acromiale: unfused acromial ossification center
Scapular Fractures: Coracoid
Coracoid fracture: may occur in conjunction with type III AC separation

Treatment conservative
Figure 4-27-4
Stress Fracture of Coracoid Process
Trap shooters shoulder
Imaging of Glenohumeral Joint
Standard AP view is oblique to the GH joint

Excellent osseous detail (homogeneous distribution of soft
tissues)
Glenohumeral True AP View (Grashey view)
Beam tilted 45 laterally
Evaluate GH joint, subluxation, loss of articular cartilage
Less value for fractures of surrounding structures
Axillary view
Evaluate for subluxation/ dislocation; fractures of ant/ post
glenoid
West Point View: variant of the Axillary View
Improves visualization of the anterior glenoid (Bankart lesion)
Scapular Y View
Image along plane of scapula; 60 relative to the AP view
Easily acquired in setting of acute trauma
Evaluate for ant/ post dislocation
Poor evaluation of the osseous structures
Anterior Dislocation [Figure 4-27-4]
AP view of the shoulder demonstrates anterior

dislocation of the humeral head
Figure 4-27-5
Mechanism: fall on outstretched arm

X-ray: humeral head displaced anterior and medial
Associated lesions
Under 35 y.o.
Hill-Sachs defect; Bankart lesion or variant
Over 35 y.o.
1. Avulsion fracture greater tuberosity
2. RCT
3. Subscapularis tear
Anterior Dislocation
Axillary view
Scapular Y view
Lesions Associated with Anterior Dislocation

[Figure 4-27-5]
Occurs secondary to impaction of humeral head against inferior

glenoid rim
942
Hill-Sachs deformity
Lesions Associated with Anterior Dislocation
Mechanism: results from impaction of humeral head against inferior glenoid

rim
Osseous Bankart lesion
Fibrous Bankart lesion best evaluated with MR
Figure 4-27-6
Trauma [Figure 4-27-6]
24 y.o. female: persistent painful shoulder after skiing accident

Radiographically occult humeral head fractures best evaluated
with MR imaging
Posterior Instability
2% - 4% of all traumatic dislocations

20% - 25% of shoulder instability cases in active duty military
population
Adduction with internal rotation
Seizure, electrocution, weight lifting, swimming, lineman blocking
Reverse Hill-Sachs, Bankart
Posterior Dislocation [Figure 4-27-7]
Dislocates straight posterior

Sometimes difficult to detect on AP view: 50% missed
Locked in internal rotation; most reliable sign
Very obvious on axillary view
MR appearance of a radiographically occult

fracture of the greater tuberosity
Trough Sign
Vertical line of dense cortical bone paralleling the medial cortex of the humerus
Results from impaction fracture of the posterior
medial aspect of humeral head
Reverse Hills Sachs Fracture
Figure 4-27-7
Positive Rim Sign
Widening of the joint is termed the positive rim sign

Normally the space between the anterior glenoid rim
and medial humeral head is <6mm
If posterior dislocation suspected- get axillary view
Calcific Tendonitis
HADD: periarticular calcifications: shoulder most

common
Results from chronic repetitive micro-trauma
Easily detected on x-ray; can be subtle on MR
imaging
Left: AP view of the shoulder demonstrating posterior

dislocation of the humeral head
Right: Axillary view demonstrating posterior dislocation of the
humeral head
Humeral Head Fractures [Figure 4-27-8]
Neers Four-segment Classification

Humeral head
Humeral shaft
Greater tuberosity
Lesser tuberosity
Fragment: >1 cm displacement or >45 angulation; considered
significantly displaced
No fragment >1 cm or 45- considered a non-displaced fracture
1 fragment displaced = 2 part fracture; 2 fragments displaced = 3
part fracture; etc
Figure 4-27-8
Humeral Shaft Fractures
Mechanism:
Indirect twisting force: spiral fx
Direct force: transverse fx
Usually involve mid-shaft
Shaft fractures heal easily
Rarely require internal fixation
Ball-and-socket joint tolerates some degree of angular and
rotational malalignment
Neers Four-segment Classification
943
Humeral Shaft Fractures [Figure 4-27-9]
Figure 4-27-9
Common location for pathologic fractures

Minor trauma
Children:
Unicameral bone cyst (fallen fragment)
Fibrous dysplasia
Adults:
Metastatic lesion
Elbow Fractures: Adult
Radial head: most common fracture of upper limb in young adults

Mechanism: fall on outstretched arm
Frequently occult; oblique views; joint effusion
Treatment: non-displaced- conservative; comminuted- resection
Elbow Fractures: Child [Figure 4-27-10]
Supracondylar fracture: Most common elbow fracture in childhood

Risk: brachial nerve injury
Mechanism: Fall on outstretched arm
Posterior fat pad sign
Anterior sail sign
In setting of trauma indicates occult elbow fracture
Pathologic fracture through a unicameral bone
Anterior humeral line
cyst
Intersect the middle 1/3 of capitellum on lateral view
Subtle supracondylar fracture results in posteriorly displaced capitellum
Figure 4-27-10
Elbow Dislocations
Mechanism: fall on outstretched arm- common in child and adult

Posterior or posterolateral
Fractures (minor): coronoid/ radial head: child- medial epicondyle
Complications of Dislocation
Brachial artery or nerve damage

Post-traumatic ossification: stiffness- intra and periarticular
adhesions: forms in subperiosteal and capsular hematoma
Posterior Dislocations in Child
Associated with medial epicondyle avulsion in 50% of cases

Up to 30% become entrapped in the joint following reduction
Treatment: open reduction for >1 cm displacement of medial
epicondyle
Radiocapitellar Line [Figure 4-27-11]
Abnormal anterior humeral line
Line drawn along long axis of radius should intersect capitellum in any
projection
Night Stick Fracture
Mechanism: direct force to forearm (usually middle 1/3)

Isolated fracture of the mid-shaft of the ulna: Usually no displacement or
angulation
Must exclude associated dislocations
Figure 4-27-11
Normal radiocapitellar line

944
Isolated Fractures Ulna: Monteggia [Figure 4-27-12]
Ulnar fracture (usually proximal 1/3); radial head dislocation

Mechanism: fall on outstretched arm (forced pronation of forearm)
Treatment:
Open adult/closed child
Restrict pronation/ supination
Figure 4-27-12
Isolated Fractures Radius: Galeazzi
Radius fracture/ distal radioulnar joint dislocation

High incidence non-union, delayed union with closed reduction
Tx: ORIF- still a tendency to dislocate after ORIF
Monteggia fracture
Essex-Lopresti [Figure 4-27-13]
Comminuted radial head fracture/ DRUJ dislocation or instability

Interosseous ligament:
Intact: radial head resection
Disrupted: radial head prosthesis
Fall on Outstretched Hand
Figure 4-27-13
Childhood: distal radial buckle fracture

Young adults: scaphoid fracture
Older adults (>40): Colles fracture
Colles Fracture
Most common fracture of distal radius in patient over 40 y.o.

FOOSH injury with wrist in dorsiflexion
Distal fragment displaced dorsally
Colles Fracture Classification
A- Extra-articular fx radius
B- (A) + ulnar styloid fx
C- Intra-articular fx distal radius
D- (C) + ulnar styloid fx
E- Comminuted fxs of radiocarpal and radioulnar joints
F- (E) + ulnar styloid fx
Smith Fracture (Reverse Colles)
Comminuted fracture of the radial head
Volar angulation of distal fragment

Younger patient
High energy trauma with wrist in volar flexion
Barton Fracture (Reverse)
Longitudinal shear injury of the distal radius

Barton: Dorsal rim - Reverse Barton: Volar rim
Transverse load with shearing forces
Often requires internal fixation: unstable fracture
Radial Styloid Hutchinsons/ Chauffeurs Fracture
Intra-articular fracture of the radial styloid
Distal Radial Fracture in Childhood
Transverse metaphyseal fracture

Most common forearm fracture ages 4-10 y.o.
May be complete or incomplete
Torus/ Buckle fracture
Evaluation of Suspected Scaphoid Fracture
Snuff Box Tenderness

1. Additional radiographic views
Oblique view
Scaphoid view
2. Cross-sectional imaging
MRI
CT
Bone scan
945
Top: MR imaging demonstrating an

intact interosseous membrane of the
forearm
Bottom: MR imaging demonstrating
disruption of the interosseous
membrane of the forearm
Scaphoid fracture: Complication rate is high

[Figures 4-27-14 and 4-27-15]
Figure 4-27-14
AVN
Delayed/ nonunion
Osteoarthritis
AVN complication of scaphoid fracture
Recurrent blood supply
Risk factors: delayed diagnosis, displacement,
proximal fracture site
Figure 4-27-15
Left: Avascular necrosis of the proximal pole of the scaphoid
Right: Nonunion of a scaphoid fracture with secondary
osteoarthritis
Figure 4-27-16
Recurrent blood supply to the scaphoid bone
Scapholunate Ligament Disruption [Figure 4-27-16]
Normal scapholunate distance = 2mm

Gap accentuated with clenched fist view
Scapholunate Ligament
Wrist MR arthrography can increase detection rate of subtle

interosseous ligament disruption
Increased scapholunate distance indicating

disruption of the scapholunate ligament
Carpal Dislocations [Figure 4-27-17]
Dislocations
Lunate: lunate dislocates in volar direction
Perilunate: capitate dislocated dorsally/ lunate remains normal
Figure 4-27-17
Left: AP view of wrist demonstrates lunate dislocation

Center: Lateral view of wrist demonstrate lunate dislocation
Right: Perilunate dislocation: posterior capitate dislocation, lunate remains in normal
position relative to the radius
946
Triquetrum Fracture [Figure 4-27-18]
Shearing forces, dorsal avulsion fracture seen only on the lateral

view
Point tender over dorsal aspect of wrist
Mechanism: fall on out-stretched hand
Immobilize in plaster for 3 weeks
Associated with lunate, perilunate dislocation
Figure 4-27-18
Hamate Fracture [Figure 4-27-19]
Point tenderness over hook of hamate

Direct trauma to volar aspect of wrist; surgical resection of hook
Fracture of First MC- Bennett Fracture [Figure 4-27-20]
Oblique intra-articular fracture/subluxation of base of 1st MC

The large fragment subluxes; smaller fragment maintains position
Closed treatment: persistent subluxation/ traumatic arthritis
Usually treat with ORIF
Bennett Bad
Triquetrum fracture
Bennett Fracture
Deforming forces
Abductor pollicis longus pulls the distal fragment in a
proximal/dorsal direction
Adductor pollicis muscle stabilizes the volar ulnar lip of the
articular surface in its normal position
Thus results in distraction of the 2 fracture fragments
Figure 4-27-19
Fracture of First MC- Rolando Fracture
Comminuted Bennett fracture

Dorsal subluxation and a separate dorsal fragment
Usually treated closed as pinning does not work well on
comminuted fragments
Worse prognosis than the Bennett fracture
Rolando Ruined
Skiers Thumb Injury (Gamekeepers thumb)
Disrupt Ulnar Collateral Ligament

May have small avulsion fracture
Pin or ORIF
Hook of Hamate Fracture
Figure 4-27-20
Stress Views [Figure 4-27-21]
Stener Lesion: MR imaging

Interposition of adductor pollicis apponeurosis between torn
UCL and base of proximal phalanx
Surgical lesion
Figure 4-27-21
Bennett Fracture
Disrupted ulnar collateral ligament
947
Mallet Finger (Baseball Finger) [Figure 4-27-22]
Figure 4-27-22
Sudden resisted flexion of DIP joint

Finger jammed or distal tip hit with a ball
Avulsion of extensor digitorum tendon
ORIF and splint in full extension 6 weeks
Volar Plate Avulsion
Hyperextension avulsion injury

MCP or PIP joint
Avulsion of Flexor Digitorum: Jersey Finger
[Figure 4-27-23]
Forced hyperextension
Avulsion of flexor digitorum profundus
Osseous fragment displaced proximally
Isolated Tendon and Pulley Injuries [Figure 4-27
Figure 4-27-23
24]
Rupture of pulley system occurs with forced

extension
Bowstringing of flexor tendon
Common injury in rock climbers
Mallet finger: avulsion of

extensor digitorum tendon
Phalangeal Dislocations: Coachs Finger
[Figure 4-27-25]
Usually dorsal; often associated with volar plate injury

Simple: closed reduction;
Complex: ST entrapment: open reduction
Boxers Fracture
4th or 5th MC
Dorsal angulation
Treatment open >35 angulation
Distal Tuft Fracture
Jersey Finger: Avulsion
injury of the flexor digitorum

Fracture of distal tuft of phalanx
profundus
Mechanism: crushing injury
Ignore the fracture and treat soft tissue injury
Watch for complication of osteomyelitis with open fracture/ nail
bed injury
Figure 4-27-24
Figure 4-27-25
Coachs Finger: Interphalangeal dislocation
References
1.
2.
Manaster, BJ. Handbook of Skeletal Radiology, second edition. St Louis,

MO: Mosby, 1997: 171-225
Rogers, LF. Radiology of Skeletal Trauma, third edition. Philadelphia, PA,
Churchill Livingstone, 2002, 593-929
Rock Climbers Finger: Flexor pulley disruption
948
Crystal Deposition Diseases and

Neuropathic Osteoarthropathy
Crystal Deposition Diseases
Gouty arthritis
Monosodium urate
CPPD crystal deposition disease
Calcium pyrophosphate dihydrate
HA crystal deposition disease
Calcium hydroxyapatite
Related Disorders
Hemochromatosis
Iron deposition
Alkaptonuria
Homogentisic acid
Gout
Monosodium urate crystals

Intra-articular, periarticular
Acute inflammatory response
Chronic granulomatous reaction
Gout: Primary (Idiopathic)
Male: Female = 20 : 1
4050 years of age
(Familial history)
Gout: Secondary
Hereditary diseases
Myeloproliferative diseases
Endocrine disorders
Drug therapy
Gout: Clinical Stages
Asymptomatic hyperuricemia
Acute gouty arthritis
Interval phase
Recurrent arthritis
Chronic tophaceous gout
Gouty Arthritis
Polyarticular
Asymmetric
1st MTP joint (90%)
Tarsometatarsal
Carpometacarpal
Olecranon bursitis
949
Crystal Deposition Diseases and Neuropathic Osteoarthropathy
Gouty Arthritis [Figures 4-28-1 to 4-28-3]
Figure 4-28-1

Tophi (calcification rare)
Well-defined erosions
Overhanging edge
Preserved joint space
Extra-articular erosions
Figure 4-28-2
Gouty arthritis. Radiograph of the distal

interphalangeal joint shows characteristic welldefined erosion of bone with overhanging edge
of new bone (arrow). Note also preservation of
joint space
Gouty arthritis. A. Pathological specimen shows tophaceous

material (star) and sharp interface with adjacent bone (arrow).
B. Clinical radiograph shows large well-defined erosions of the
first metatarsal head with overhanging edge of new bone
(arrow). Note multiple other erosions including the base of the
first metatarsal
Figure 4-28-3
CPPD Crystal Deposition Disease: Terminology
Pseudogout
Chondrocalcinosis
Pyrophosphate arthropathy
CPPD Crystal Deposition Disease
Usually idiopathic
Occasionally hereditary
Over 50 years of age
Incidence 5% to 34%
CPPD Crystal Deposition Disease:

Related Disorders
Primary hyperparathyroidism
Hemochromatosis
Gouty arthritis. There are multiple well-defined

erosions, particularly at the bases of the
metacarpals at the common carpometacarpal
joint

Sites of Calcification
Fibrocartilage
Hyaline cartilage
Synovial membrane
Joint capsule
Ligaments
Tendons
Bursae
950
Fibrocartilage Calcification
Knee menisci
Symphysis pubis
Figure 4-28-4
Pyrophosphate Arthropathy
Joint space narrowing

Bone sclerosis
Osteophyte formation
Subchondral cysts
Differentiation of Pyrophosphate
Arthropathy from Degenerative Joint
Disease
Unusual articular distribution

Unusual intra-articular distribution
Variable osteophyte formation
Prominent subchondral cysts
Progressive destruction
Calcification
CPPD crystal deposition disease. A. Frontal radiograph of the

knee shows chondrocalcinosis including meniscal fibrocartilage
calcification (white arrows) and hyaline cartilage calcification
(gray arrow).
B. Lateral radiograph shows complete loss of patellofemoral
joint space
CPPD Crystal Deposition Disease: Knee

[Figure 4-28-4]
Meniscal calcification
Hyaline cartilage calcification
Patellofemoral arthropathy
Subchondral cysts
Osseous bodies

Wrist and Hand [Figures 4-28-5 and 4-28-6]
Figure 4-28-5
Triangular fibrocartilage calcification

Radiocarpal arthropathy
Stepladder configuration (SLAC)
Metacarpophalangeal arthropathy
Absence of erosions
Figure 4-28-6
CPPD crystal deposition disease. Frontal radiograph of the

wrist shows joint space narrowing between the radius and
scaphoid and between the lunate and capitate (scapholunate
advanced collapse [SLAC]). There is extensive
chondrocalcinosis including the triangular fibrocartilage (arrow)
as well as a large subchondral cyst in the radius (star)
CPPD crystal deposition disease. Frontal radiograph shows

narrowing of the second and third metacarpophalangeal joints
with sclerosis, osteophytes, and subchondral cysts. Note
hyaline cartilage calcification (green arrow) and probable
synovial and capsular calcification (yellow arrows)
951
Hemochromatosis
Primary
Increased iron absorption
Secondary
Increased iron intake
Multiple blood transfusions
Hemochromatosis: Clinical Findings
Onset between 40 and 60 years

Men more often than women
Bronze pigmentation
Cirrhosis
Diabetes mellitus
Cardiac failure
Arthropathy
Figure 4-28-7
Hemochromatosis:
Pathologic/Radiologic Findings
[Figure 4-28-7]
Iron in synovioblasts
CPPD crystal deposition
Osteoporosis
Symphysis pubis calcification
Hyaline cartilage calcification
Uniform MCP joint involvement
Hook-like osteophytes
Hemochromatosis. Frontal radiograph shows narrowing of the

second through fifth metacarpophalangeal joints as well as
multiple large hook-like osteophytes (arrows)
HA Crystal Deposition Disease
Primary
Secondary
Periarticular
Intra-articular
Primary Periarticular HA Crystal Deposition Disease:

(Calcific Tendinitis)
Middle-aged
Monoarticular
(Asymptomatic)
Localized pain
Tenderness to palpation
Restriction of motion
(Fever and malaise)

(Calcific Tendinitis) [Figure 4-28-8]

Poorly defined calcification
Sharply defined calcification
Resorption of calcification
Figure 4-28-8
Intra-Articular Hydroxyapatite Crystal

Deposition Disease: Milwaukee Shoulder
Elderly women
Shoulder pain
Decreased mobility
HA crystal shedding
Activated collagenase
Neutral protease
Tissue destruction
Calcific tendinitis. A. Radiograph of the shoulder in external

rotation shows poorly defined calcification corresponding to the
supraspinatus tendon (arrow).
B. Radiograph of the shoulder of a different patient in internal
rotation shows sharply defined calcification corresponding to
the infraspinatus or teres minor tendon (arrow)
952
Milwaukee Shoulder: Radiologic Findings
Amorphous calcification
Glenohumeral joint narrowing
Subchondral sclerosis
Bone destruction
Rotator cuff disruption
Acromiohumeral abutment
Alkaptonuria: Clinical Findings
Homogentisic acid oxidase deficiency

Onset between 20 and 30 years
Bluish-black pigmentation (ochronosis)
Dark colored urine
Cardiovascular
Genitourinary
Upper respiratory
Articular
Alkaptonuria: Pathologic Findings
Connective tissue pigmentation

Fibrocartilage and hyaline cartilage
Fibrillation, fragmentation
Granulation tissue
Osseous proliferation
Alkaptonuria: Radiologic Findings
Disc calcification
Annulus fibrosus
Diffuse
Multilevel disc narrowing
Vacuum phenomena
Osseous sclerosis
Alkaptonuria: Radiologic Findings [Figure 4-28-9]
Knees, hips, shoulders

Symmetric cartilage loss
Osseous sclerosis
Collapse and fragmentation
Intra-articular bodies
Figure 4-28-9
Neuropathic Osteoarthropathy
Charcot joint
Tabetic arthropathy
Neurotrophic joint
Neuropathic arthropathy
Neuroarthropathy
Neuroarthropathy: Etiologies
Diabetes mellitus
Alcoholism
Syringomyelia
Syphilis
Leprosy
Meningomyelocele
Congenital insensitivity to pain
Steroid administration (intra-articular)
Alkaptonuria. A. Lateral radiograph of the lumbar spine shows

uniform loss of disc height and associated bone sclerosis.
There is disc calcification bridging all levels anteriorly except
for L4-L5, which shows large osteophytes and a vacuum
phenomenon.
B. Frontal radiograph of the knee shows uniform joint space
loss and bone sclerosis
953
Neuroarthropathy: Pathogenesis
Figure 4-28-10
French theory
German theory
Neurotraumatic
Neurovascular
Neuroarthropathy: French Theory
Mitchell (1831)
Charcot (1868)
Damage to CNS trophic centers
Altered bone and joint nutrition
Osseous and articular atrophy
Neuroarthropathy: German Theory
Volkmann, Virchow
Insensitivity to pain
Recurrent trauma
Neuroarthropathy: Neurotraumatic Theory
Eloesser (1917)
Posterior sensory nerve section
Continued weightbearing
Joint destruction
Chemical analysis: no atrophy
Neuroarthropathy: Neurovascular Theory
Neuropathic osteoarthropathy. Frontal

radiograph of the shoulder shows almost
complete resorption of the humeral head
except for some osseous debris medially
(arrow). Note the extremely sharp margin of the
remaining portion of the humeral shaft
Figure 4-28-12
Neurally initiated vascular reflex

Increased bone blood flow
Osteoclastic bone resorption
Fracture and repair
Neuroarthropathy: Clinical/Pathologic Findings
Swollen, deformed joint

Usually painless
Detritic synovitis
Rapid progression
Neuroarthropathy: Radiologic Findings

[Figures 4-28-10 to 4-28-12]
Destruction (atrophy)
Dislocation
Disorganization
Debris
Detritus
Density (sclerosis)
Distension (effusion)
Figure 4-28-11
Neuropathic osteoarthropathy. Frontal
radiograph of the foot shows characteristic
destruction, disorganization, and debris around
the tarsometatarsal joints
Neuropathic osteoarthropathy. A. Lateral radiograph of the foot

shows extensive destruction of the bones of the midfoot with
dislocation and disorganization. Note extensive vascular
calcification from diabetes.
B. Followup lateral radiograph less than one month later shows
further bone destruction with almost complete disappearance
of the talus and the anterior portion of the calcaneus
954
MRI of the Elbow

Mark E. Schweitzer, MD; William B. Morrison, MD
Figure 4-29-1
Anatomy
Osseous-radius
ulna
humerus
Ligamentous-medial collateral
lateral collateral
Musculotendinous
Posterior: triceps
Anterior: biceps, brachialis
Medial: flexor-pronator
Lateral: common extensor
Neurovascular
Articular Anatomy
Capitellum-hemispherical-articulates with radius

Trochlea-spool 300 degree arch-articulates with ulna
Coronoid fossa-ant/sup to trochlea, small-articulates with
coronoid
Olecranon fossa 180 degrees-art. with semilunar notch
Lesser sigmoid notch- radial side of proximal ulna/PRUJ
Biomechanics
Three articulations:
Ulnar-tochlear
Radiocapitellar
Proximal radioulnar
0-140 degrees flex/ext
75 pronation
80 supination
Effusion and synovitis.

Note complex fluid in the joint
distending the anterior and posterior
fat pads (arrows). The fat pads are
intracapsular but extrasynovial
Effusion [Figure 4-29-1]
Fat pads are intracapsular and extrasynovial

DDx:
Fracture
Internal Derangement (e.g., ligament, cartilage)
Arthritis (e.g., RA, OA, septic)
Figure 4-29-2
Cartilage Loss
Difficult to see directly (cartilage thin)

Subchondral marrow edema best sign
Phytes-posterior/medial/coronoid
Confirm cart loss / cause impingement
Associated with effusion and bodies
OCD [Figure 4-29-2]
Capitellum- 3rd most common site in body (after

ankle, knee)
Repetitive microtrauma during valgus (assoc with
MCL)
Symptoms = pain, locking
Sequelae- bodies/OA
Staging-fluid/cyst under fragment=loose (unstable)
955
Unstable OCD of the capitellum. Note contrast extending

under osteochondral fragment (arrow) on this MR arthrogram
MRI of the Elbow
Pitfalls: NL variation
Trochlear sulcus
Posterior capitellar pseudodefect
DDx: location; no underlying edema
Figure 4-29-3
Intraarticular Bodies [Figure 4-29-3]
Often adherent to synovium

Intraarticular, not loose
Usually begin as cartilage defects and grow
Often from OCDs)
Variable signal characteristics
Use GRE (TE>7), tend to bloom
In the recesses, usually olecranon / coronoid
MR imaging: arthrography, or effusion
Synovial Folds
Embryologic remnant
Several locations
Posterior/lateral catches
Medial (meniscoid) most common
May mimic bodies clinically and on MR
Synovial Folds
Lateral plica syndrome: Posterolateral catching/locking
Posterior Impingement
Part of tennis elbow spectrum

Osteophytes- often related to chronic MCL overload, hyperextension (e.g.,
pitchers)
Bodies in olecranon fossa
Non-union of old triceps avulsion
Nerve Impingement
Intraarticular body (arrow) surrounded by

contrast in the olecranon recess in a patient
with posterior impingement
[Figure 4-29-4]
Potential locations of nerve impingement

Median/radial nerve
Proximal arcade of Struthers (avian spur)
Impinged by fascicle of lacertus fibrosus
Median nerve branch through pronator teres muscle (pronator
syndrome)
Supinator syndrome, radial tunnel syndrome, interosseous
syndrome
Look for muscle edema distally
Ulnar nerve [Figure 4-29-4]
Cubital tunnel
Focal edema/STS/sublux/mass
Look above and below
Associated with epicondylitis, MCL injury
Figure 4-29-4
Epicondylitis
Medial: golfers elbow (common flexor origin)

Lateral: tennis elbow (common extensor origin)
Spectrum from degeneration to partial to completetear
Increased T1 signal
Increased STIR, T2 signal
Linear vs. diffuse
Histologically- angioblastic changes/ fibrillar collagen degeneration
MRI of the Elbow
956
Enlargement of the ulnar nerve (arrow) with

surrounding soft tissue inflammation in a
patient with
cubital tunnel syndrome
Lateral Epicondylitis [Figure 4-29-5]
Figure 4-29-5
Repetitive overload of extensors

Tennis elbow
35-70 years old
Pain/tenderness focally, may radiate proximally
Usually extensor carpi radialis
Biceps: Anatomy
Long head: superior glenoid

Short head: coracoid
Two heads merge distal to the bicipital groove
Insertion onto radial tuberosity at elbow
Intimate with brachialis
Proximal synovial sheath
Distally paratenon, bicipitoradialis bursa and lacertus fibrosus
Biceps Pathophysiology
Degeneration
Primary (overuse injury)
Or direct frictional effect
Mechanical-pronation leads to impingement between radius and
ulna
Hypovascular-critical zone
distally like rotator cuff
Biceps Tendinosis
Lateral epicondylitis (tennis elbow) with

focal fluid signal (arrow) at the common
extensor tendon origin indicating a partial tear
Figure 4-29-6
Common, but rare to image

Imaging/ clinically overlap with partial tear, bursitis
Very distal at insertion
Biceps-Partial Tears
Attritional
Pain
No pop, usually no ecchymosis
More marrow edema and bursitis
Surgery not usually needed unless large
Biceps Injury: Distal vs Proximal [Figure 4-29-6]
Both: muscle belly retraction popeye arm

Fluid dissects around muscle belly
Both: sudden snap, arm hematoma
Distal young/ proximal older
Distal sports/ proximal chronic impingement, RCT
Proximal: surgery uncommon (two heads), except for repair of
cuff, resection of spur
Distal: surgery common
Associated marrow edema
Associated bursitis
Complete distal biceps tendon tear.

Note end of retracted tendon (arrow) with
muscle bunched up proximally resulting in a
popeye arm
Bicipital Radialis Bursitis
Distal biceps lacks a sheath

Apparent fluid around is bursal
Close to insertion
Ddx; vessel
Assoc with biceps tears (esp partial), RA, mechanical maybe 1st
957
MRI of the Elbow
Triceps Injuries
Fairly uncommon
Spectrum tendonitis (posterior tennis elbow/ posterior impingement), snapping,
to tear
Risk factors: steroids, SLE, CRF, RA, gout
Within 23 cm of insertion, usually at
Associated olecranon bursitis
Associated soft tissue edema
Look for avulsion fx
Figure 4-29-7
Olecranon Bursitis [Figure 4-29-7]
Anatomic bursa
Normally no fluid visible
Bursitis: Fluid, loss of subQ fat adjacent to olecranon
Causes
Trauma
RA
Gout
Infection
Muscle Disorders
Tears
DOMS
Neuropathy-Parsonage Turner Syndrome edema-like muscle
signal
Infection
Osseous Injury [Figure 4-29-8]
Effusion on Xray: presumed fx

F/U X-ray vs MR
Bone marrow edema after trauma: Bone bruise vs. fracture
T1: focal low signal (linear) = fracture
No line, ill-defined edema = bruise
Olecranon bursitis
Figure 4-29-8
Avulsion
Chronic avulsive stress

Tendinopathy
Usu subtle edema at enthesis
Avulsion fx
Ligamentous or tendinous
Thin, longitudinally oriented edema at cortical margin
Osteochondral Impaction
Analogous bruises from ACL tears

Transient disloc/sublux
Often both sides of joint
Fracture Complications
OA
Bodies
Capsular fibrosis
Non union / malunion
Associated ligament injury/instability (e.g., Essex Lopresti)
AVN
Pain
Limited ROM
Instability
MRI of the Elbow
958
Occult radial neck fracture
AVN
Figure 4-29-9
Older: typical risk factors for AVN

Younger = Panners disease
Capitellum
Boys; 4-10 years
Decreased vasc to growing epiphysis
Usu. spontaneously resolves
If >10, higher risk of complications
Ligament Disorders
Medial
Lateral
Medial Collateral Ligament
Three segments
Anterior bundle
Most important soft tissue static constraint to valgus
stability
Posterior bundle
Transverse bundle
MCL
[Figures 4-29-9 and 4-29-10]
Anatomy
Anterior/posterior/transverse bands
Strongest is anterior
Pathophysiology
Overhead throwing /valgus overuse, weakens/incompletely
heals, reinjures
Partial tears T sign vs. complete tears
Old tears show thickening +/- bowing
Association with epicondylitis
Medial collateral ligament tear (arrow)

and osteochondral impaction injury
(arrowhead)
Figure 4-29-10
Lateral Ligaments
Components
Lateral collateral ligament proper (LCL) (Radial collateral
ligament)
Annular ligament
Lateral ulnar collateral ligament (LUCL)
Annular Ligament
Fibro-osseous ring that encircles and stabilizes the radial head

Attaches on the anterior and posterior edges of the lesser sigmoid
notch
Anterior portion taught in supination and posterior portion taught
in pronation
Radial Collateral Ligament
Medial collateral ligament tear (arrow) and

lateral ulnar collateral ligament tear
(arrowhead) in a
patient with recent elbow dislocation
Extends from the lateral epicondyle and attaches to the annular ligament
Immediately deep to the common extensors
About half to a third size of MCL
Maintains humeroradial apposition in the presence of varus stress
Lateral Ulnar Collateral Ligament
With annular ligament (PRUJ) and radial collateral ligament (radial head)
makes up lateral lig complex
Sweeps posteriorly past the radial neck and inserts on the ulna
Stabilizer for rotational and varus stress
959
MRI of the Elbow
Posterolateral Rotatory Instability
Instability of the elbow manifested by painful clicking of the elbow in extension

Radial head moves posteriorly in relation to the capitellum
Essential lesion - tear of the LUCL
Lateral pivot shift test
Supination with axial and valgus stress
Lateral pivot shift test - supination with axial and valgus stress
LUCL INJURIES
Caused by a fall on an outstretched hand

Iatrogenic injury during release or repair of lateral epicondylitis
Epitrochlear Lymph Node
Mimics a mass
SubQ
Inflammatory-like signal
Cat scratch fever
Assoc fasciitis
MR Arthrography Indications
Bodies
MCL injuries
OCDs
Subtle cartilage loss
Elbow MRA
Ligament tear
Extracapsular leakage of contrast
Medial or lateral collateral ligament tear
IA bodies
Anterior, posterior recesses
OCD
Same dx as knee
Esp capitellum
References
1. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist,
and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-379.
2. Bordalo-Rodrigues M, Rosenberg ZS. MR imaging of entrapment neuropathies at the elbow. Magn Reson Imaging
Clin N Am. 2004 May;12(2):247-263.
3. Potter HG, Ho ST, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskelet Radiol. 2004
Mar;8(1):5-16
4. Chung CB, Kim HJ. Sports injuries of the elbow. Magn Reson Imaging Clin N Am. 2003 May;11(2):239-53.
5. Steinbach LS, Palmer WE, Schweitzer ME. Special focus session. MR arthrography. Radiographics. 2002 SepOct;22(5):1223-1246.
6. Zou KH, Carrino JA. Comparison of accuracy and interreader agreement in side-by side versus independent evaluations
of MR imaging of the medial collateral ligament of the elbow. Acad Radiol. 2002 May9(5):520-5.
7. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist,
and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-79
8. Bordalo-Rodriguez M, Rosenberg ZS. MR Imaging of entrapment neuropathies at the elbow. Magn Reson Imaging
Clin N. Am. 2004 May; 12(2):247-63.
9. Chung CB, Chew FS, Steinbach L. MR imaging of tendon abnormalities of the elbow. Magn Reson Imaging Clin
N. Am. 2004 May;12(2):233-45.
10. Kaplan LJ, Potter HG. MR imaging of ligament injuries to the elbow. Magn Reson Imaging Clin N. Am. 2004
May;12(2):221-32, v-vi.
11. Fowler KA, Chung CB. Normal MR imaging anatomy of the elbow. Magn Reson Imaging Clin N. Am. 2004
May;12(2):191-206, v.
12. Potter HG, Ho St, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskeletal Radiol. 2004
Mar;8(1):5-16.
13. Savnik A, Jensen B, Norregaard J, Egund N, Danneskiold-Samsoe B, Bliddal H. Magnetic resonance imaging in the
evaluation of treatment response of lateral epicondylitis of the elbow. Eur Radiol. 2004 June;14(6):964-9. Epub
2003 Dec 11.
MRI of the Elbow
960
Skeletal Metastases, Myeloma, Lymphoma

Michael E. Mulligan, MD
Figure 4-30-1
Incidence of Metastases [Figure 4-30-1]
30% of all patients with Cancer

Skeleton 3rd most common site
More than 80% due to PTBLK
#1-Breast, #2-Prostate, #3-Lung
Spine lesion Breast 75%
Femur lesion Breast 50%
Skull lesion MM, B, L
Hands/Feet Lung
P=prostate T=thyroid B=breast L=lung K=kidney
Mechanism of Spread to Bones

Hematogenous/Contiguous
Marrow vessels unusual, rich sinusoidal system with large

endothelial gaps
Batsons plexus has direct connection to IVC/SVC with no valves
Arterial mechanism for distal mets?
Batson OV. Ann Surg 1940;112:138
Figure 4-30-2
Bone Metastases
48 year old man with renal cell

carcinoma metastases mimicking
multiple myeloma
Different T1, T2 signal

Different Gadoliniumenhancement
The holy grail DWI
Radiology 1998; 207:305-7
Acute Vertebral Collapse - Osteoporosis or Malignancy
[Figure 4-30-2]
Symptoms and Signs

Pain, most common symptom, but only in 2/3 patients
Pathologic fracture
Common sign, esp bad in spine, femur
Pathologic Fractures [Figure 4-30-3]
510% of all patients with mets

50% or more cortex gone - 2/3 will develop pathologic fracture
61 year old woman with breast
Less than 50% cortex gone - 1/5 will develop pathologic fracture
cancer, focal depression of L2
Any lesion in femoral neck
superior endplate is indicative of
Avulsion of lesser trochanter
metastatic disease
Any lesion prox femur >2.5cm
Figure 4-30-3
Mirels score - site, size, l/b, pain
3.0 cm lesion with persistent pain after XRT
Mirels, H. Clin Ortho Rel Res 1989; 249: 256-64
55 year old man with lung

cancer and cortical cookie
bite metastasis
961
Metastases, Myeloma, Lymphoma
Bone Metastases: Systemic Features [Figure 4-30-4]
Figure 4-30-4
Hypercalcemia
Hypertropic Osteoarthropathy, triad of
Periosteal reaction
Clubbing
Pain
Hypertrophic Osteoarthropathy
Classic Triad
Joint swelling, 30%40% patients
5% patients with lung cancer
Cause? may be paraneoplastic, due to a growth hormone releasing factor
Bone Metastases: Radiologic Features
Pure lysis
Lysis with blowout (renal, thyroid)
Mixed lytic/blastic (breast, lung, GI)
Pure blastic (prostate, carcinoid, medulloblastoma)
Osteoclast Activating Factor (A Cytokine)
Stimulates clasts to synthesize collagenase

Produced by normal activated leukocytes
Dependent on prostaglandin E
Prostaglandin inhibitors can reduce/obliterate osteolysis
JBJS 68A:310, FEB 1986
Bone Metastases: Radiologic Features
Ivory vertebra
Pathologic fx
Periosteal rx prostate, lung neuroblastoma, GI tumors
Soft tissue mass (lung)
Missing pedicle
Intracortical lung cancer
Ivory Vertebra(ae):
Differential Diagnosis [Figure 4-30-5]
#1 Pagets
#2 Hodgkins
#3 Metastasis
Figure 4-30-5
50%
30%
20%
2 year old Irish setter

with lung cancer.
Foreleg bones show
classic periosteal
changes of H.O
Figure 4-30-6
Breast Carcinoma
The most common source of

bone mets in women
Spine #1 site
65% lytic, 25% mixed,
10% blastic
Prostate [Figure 4-30-6]
The most common source of

metastases in men
More than 1/3 of patients
75% blastic, 15% mixed, 10%
lytic
Humoral factor stimulates
blasts
59 year old woman with multiple ivory

vertebrae secondary to breast cancer
metastases
962
76 year old man with florid

periosteal reaction around distal
fibula metastasis
Lung Cancer
15% of patients have mets to bone

80% lytic, 15% mixed, 5% blastic
Small cell 20% blastic
Renal Cell Carcinoma [Figure 4-30-7]
25%30% of patients have mets to bone

90% Lytic
Figure 4-30-7
71 year old man with blowout type metastasis from

renal cell cancer. Note active hyperemia on
angiogram
Figure 4-30-8
Thyroid Cancer
8% of patients have metastases

Lytic
Neuroblastoma [Figure 4-30-8]
1st choice any patient under 10 years old

Can mimic primary malignancy
Usually multiple, often symmetric
Histology confused with Ewings
Periosteal rx aggressive
Workup of Patient with Metastases
1 History / physical
2 Lab studies
Direct workup based on 1, 2
Primary not found in up to 60% patients
3 year old boy with

neuroblastoma. Metastasis in
proximal radius shows
permeative appearance similar
to Ewings sarcoma
963
Solitary Focus Bone Scan [Figure 4-30-9]
Figure 4-30-9
Seen in 2%15% of cancer patients

% due to metastatic disease varies by site of involvement
10% solitary rib lesions
50%60% in other locations (spine)
10% malignant even if there is DJD in the area
Rib J Nucl Med 1985;26:11401143

All sites Radiology 1976;121:663667
Malignant Round Cell Tumors of Bone
Myeloma
Lymphoma
Ewings sarcoma
Neuroblastoma
Rhabdomyosarcoma
PNET
Myeloma Types
MGUS monoclonal gammopathy of undetermined significance

(1% of all SPEPs)
Asymptomatic myeloma (no bone lesions)
Symptomatic multiple myeloma
(classic, generalized, osteosclerotic [POEMS], multiple myeloma
with osteosclerosis, leukemic)
Non-Secretory myeloma 3% of all cases
Solitary plasmacytoma in bone 3%-5% of cases
Extramedullary plasmacytoma 5%
Myeloma: Pathologic Features
Plasma cells proliferate in erythropoietic areas

Grossly dark red, tan; soft
Histology sheets of malignant plasma cells obliterate the
marrow
Special studies markers for light chains
Amyloid 10%
Osteoclast stimulating factor
65 year old man with solitary rib

abnormality on whole body bone
scan, proven to be esophageal
cancer metastasis
Osteoclast-Stimulating Factor
A cytokine (lymphotoxin alpha)

Similar to O-AF
Produced by myeloma cells, T cells
Interferon is a cytokine antagonist
Bisphosphonates used to counter osteoclastic resorption
Cancer 1997; 80:1557-63
Classic Multiple Myeloma: Clinical Features
Signs and Symptoms: pain, bleeding diathesis, infection, renal insufficiency

Lab findings: monoclonal spikes (IgG, IgA), B-J proteinuria, anemia,
hypercalcemia, elevated alkaline phosphatase
Imaging W/U: X-rays, CT/MRI, Nucs, PET, PET/CT
MM has highly malignant course
B-J=Bence-Jones MM=multiple myeloma
964
Durie/Salmon PLUS Staging* [Figure 4-30-10]
Radiologists role is to help determine the true tumor burden throughout the
skeleton
Stage IA:
normal skeletal survey or single lesion
Stage IB:
< 5 focal lesions or mild diffuse disease
Stage IIA/B:
5-20 focal lesions or moderately diffuse
Stage IIIA/B:
>20 focal lesions or severe diffuse disease
Subclasses A&B
(A = nl renal function, B = abnl)
Stage is generally predictive of survival
IA median survival = 60 months
IIIB median survival = 15 months
Figure 4-30-10
*Durie et al. Myeloma management guidelines: a

consensus report.
The Hematology Journal 2003; 4: 379-398
Myeloma: Imaging Features [Figure 4-30-11]
80% of new MM patients abnormal skeletal survey

Multiple myeloma - punched out lesions
endosteal scalloping
Solitary plasmacytoma: bubbly, any margin +/- soft
tissue mass
Generalized form- just osteopenia
Sclerosing - < 3%, assoc with POEMS syndrome
Spine MR 3 patterns; mild, moderate, severe
Sclerotic Myeloma:
Multiple myeloma with sclerosis or POEMS syndrome

Polyneuropathy
Organomegaly
Endocrinopathy
Monoclonal gammopathy
Skin changes
From left to right: mild, moderate, and severe.

The three types of myelomatous spinal
involvement for the Durie/Salmon PLUS staging
system (T1-weighted images)
[Figure 4-30-12]
Figure 4-30-11
NEJM 1992;327:19191923
Figure 4-30-12
56 year old man with myeloma.

Humeral radiograph shows typical endosteal
scalloping and macrosection shows plasma cells
filling the marrow space with osteoclasts along the
endosteal surface
Blastic or sclerotic lesions that are

usually painless are typical in the
POEMS syndrome
965
Myeloma: Differential Diagnosis [Figure 4-30-13]
Metastatic disease
B cell malignancy
ALL, NHL, CLL, Waldenstroms
Figure 4-30-13
Figure 4-30-14
Plasmacytoma
Differential Diagnosis [Figure 4-30-14]
Metastasis: thyroid, renal

Primary: Fibrosarcoma, MFH
Primary Lymphocytic Lymphoma of

Bone [Figure 4-30-15]
Figure 4-30-15
56 year old man with myeloma,.
skull radiograph shows typical
punched out lytic lesions
71 year old woman
with aggressive
looking solitary
plasmacytoma
mimicking blowout
type metastasis
70 year old man with PLB, skull radiograph
shows multiple lytic lesions, some with central
sequestra (arrow)
Primary Lymphoma: Path Features
Gross pinkish-grey, fish-flesh

Histology similar to nodal lymphoma round cells of various sizes (Ewings
monotonous)
Reticulin stain meshwork of fibers around each cell
Primary Lymphoma: Clinical Features
Figure 4-30-16
Non-Hodgkins (94%), Hodgkins (6%)

Rare, @ 3% malignant tumors
Any age, but rare under 10 years
Stage like soft tissue lymphoma
Solitary and multifocal (skull, femur, tibia)
Osteoclast-stimulating factor
PLB 237 AFIP CASES
151 M, 86 F, ratio 1.8:1

Average age 42 years (range: 2 to 88 years)
Long bones n=162 (71%)
Flat bones n=78 (22%)
Other sites (including spine, small bones) n=17
Mulligan, et al. AJR 1999; 173: 1691-1697
Typical Features PLB [Figure 4-30-16]
Location: Metadiaphyseal n=120 (54%)

Pattern: Permeative n=130 (55%)
Cortical involvement: n=148 (62%)
Periosteal reaction: interrupted or solid single layer n=57 (65.5%)
Soft tissue mass: n=113 (48%)
Mulligan, et al. AJR 1999; 173: 1691-1697

966
57 year old woman with

PLB, tibia lesion shows
all of the most common
radiographic features
Variations PLB [Figure 4-30-17]
Figure 4-30-17
Locations
Epiphysis n=11 (5%)
Diaphysis n=45 (19%)
Intracortical n=16 (7%)
Patterns
Normal x-ray n=12 (5%)
Geographic n=26 (11%)
Blow Out n=2 (< 1%)
Blastic n=4 (2%)
Radionuclide, CT and MRI Findings
Radionuclides n=56, markedly increased uptake

n=36 (64%)
CT n=45, MRI n=20
Cortical holes by CT or MR
Large n=20 (31%), small n=45 (69%)
Soft tissue mass
Seen by CT n=36 (80%)
Seen by MR n=20 (100%)
Variations PLB
Markedly abnormal bone scan and MR exam in 57

year old man with thigh pain and normal xrays.
This type of extensive marrow replacement pattern
with normal xrays is highly suggestive of round
blue cell tumors like PLB and Ewings sarcoma
[Figure 4-30-18]
Periosteal reaction
Multiple layers n=26 (10.2%)
Sunburst n=4 (1.6%)
Pathologic fracture n=52 (22%)
Sequestra n=37 (15.6%)
Crossing joint n=12 (5%)
Figure 4-30-18
AJR 1999; 173: 1691-1697
Primary Lymphoma: Differential Diagnosis
Metastatic lymphoma
Ewings sarcoma
Neuroblastoma / PNET
Rhabdomyosarcoma
Osteomyelitis
Eosinophilic granuloma
Summary PLB
Usually has an aggressive appearance

CT or MRI showing large soft tissue mass without large
cortical holes is typical
Wide range of appearances
Normal x-rays
Geographic lesions
Blow out lesions
Blastic lesions
Large lytic lesions with soft tissue

mass and sequestra should put PLB
high up in the differential diagnosis
References
1.
2.
3.
4.
5.
6.
7.
Durie et al. Myeloma management guidelines: a consensus report. The Hematology Journal 2003; 4: 379-398
Mirels H. Metastatic disease in long bones. Clin Orthop Relat Res 1989;249:256-264
Mulligan M et al. Skeletal Metastatic Disease. In: Pope et al, Imaging of the Musculoskeletal System.
Philadelphia: Elsevier, 2006
Mulligan M. Imaging techniques used in the diagnosis, staging, and follow-up of patients with myeloma. Acta
Radiologica 2005;46:716-724
Mulligan M, McRae G, Murphey M. Imaging features of primary lymphoma of bone. AJR 1999; 173: 1691-1697
Roodman GD. Mechanisms of bone metastasis. N Engl J Med 2004;350:1655-1664
Weber K et al. An approach to the management of the patient with metastatic bone disease. Instr Course Lect
2004;53:663-676
967
Imaging of Hematologic Disease
Figure 4-31-1
Thomas Lee Pope, Jr, MD, FACR

Objectives
To identify some of the common hematologic disorders

To describe the clinical and epidemiological aspects of these
entities
To demonstrate the most significant MSK imaging findings
Hematologic Disease
Hereditary anemias
Sickle cell anemia
Thalassemia
Rare anemias: Fanconis, thrombocytopenia with absent
radii syndrome (TAR)
Coagulation disorders
Hemophilia
Myelofibrosis
Characteristics of the Hereditary Anemias
Aberrations and/or abnormalities of RBC shape

Molecularly distinctive
Autosomal dominant
Electrophoresis
The Five Ins
In sufficient ossification
In farction
In fection
In failure (anemia)
In volution (spleen)
Hand Foot Syndrome: Notice the

diffuse involvement with regions of
osteolysis and periosteal reaction
Figure 4-31-2
Sickle Cell Disease
One of the most common inherited blood disorders (> 100,000

born with the disease worldwide per year)
One of the most prevalent genetic disorders in the US (>
80,000 African Americans)
Hemoglobin S gene (carrier state)
Autosomal dominant
Carried by 8% of African Americans or ~ 2 million US
Blacks
Hemoglobin SS disease (Sickle cell anemia)
Autosomal recessive
1 birth in 400 in African Americans
Occurs in 0.3%-1.3% of NA Blacks or ~ 50, 000 in the US
Economic Impact of SC Disease
75,000 hospitalizations yearly

Average of $6, 300 per hospitalization
$475 million in health care costs alone
Does not include lost wages, productivity, etc
Likely > $1 billion yearly cost
Acute (upper image) and chronic (lower image)

Salmonella osteomyelitis
968
Characteristics of sickled and normal RBCs

NORMAL
Disc-shaped
Soft and compressible
Easily flows through vessels
Life span of > 120 days
SICKLE
Sickle-shaped
Hard (tough and not malleable)
Sticks in blood vessels
Life span of < 20 days
Major Pathology-Vascular occlusion
Figure 4-31-3
[Figure
4-31-1]
Hand-Foot syndrome
Dactylitis
Infarction (any site)
Infection
Marrow hyperplasia
Hand foot syndrome (Dactylitis)
Up to 50% of sickle cell anemia children

2 months to 6 years
Pain, low grade fever, diffuse non-pitting edema of
the extremities
Vaso-occlusion with osteonecrosis
Infection is major DDx
Distinction: Clinically
Infection
Courtesy of Dr. Hilary Umans New York, NY
Figure 4-31-4
[Figure 4-31-2]
50X less common than infarction

Salmonella much more frequent pathogen in SCA
patients
Infecting organisms:
Salmonella 70%
Staph aureus 10%
Shigella sonnei, E coli, Arizona hinshawii and
Serratia
Proposed mechanisms:
Vascular insufficiency
Decreased phagocytosis-low O
Decreased splenic function
Multiple hospitalizations
MR features of acute Salmonella osteomyelitis
Classic features of avascular necrosis with areas

of osteolysis and osteosclerosis with preservation
of articular space
Figure 4-31-5
Figure 4-31-6
Radiographic and anatomic gross specimen

correlation of the rim sign
MR imaging of intramedullary infarcts
969
Bone within Bone Appearance
Figure 4-31-7
S Sickle cell disease

T Thoratrast
O Osteopetrosis
P Paget disease
Heavy metal
Hypervitaminosis D
http://chorus.mcw.edu
H-Shaped Vertebral Bodies [Figure 4-31-8]
Another manifestation of ischemia and infarction

>10 years of age
Incidence:
43% of SS
36% of Sickle/Thalassemia
25% of SC
Thalassemia
Bone within a bone apprearance in sickle cell anemia
1925 - Cooley and Lee

Synonyms: Cooleys anemia, mediterranean anemia, leptocytosis
Impaired alpha or beta chain Hgb production
Homozygous beta thalassemia (800-1000 US persons - NE
corridor between Boston and NY)
Heterozygous trait (2.5% of Italian Americans, 7%-10% of Greek
Americans)
Figure 4-31-8
Types of Thalassemia
Alpha
Least severe:
Silent carrier = loss of 1 alpha globulin gene - often
incidental finding
Most severe:
Hydops fetalis = loss of 4 alpha globulin genes - die in
utero
Beta:
Spectrum
Minor = slight anemia
Major = life-threatening anemia requiring transfusions
Risk of Fe++ overload
H shaped vertebral bodies of sickle
cell disease
Figure 4-31-10
Figure 4-31-9
Classic Hair on end appearance of

Thalassemia
Marrow expansion in Thalassemia with widening

of the medullary canal and thinning of the cortices
970
Imaging Features of Thalassemia
Figure 4-31-11
[Figures 4-31-9 to 4-31-12]
Diffuse marrow expansion

Skull - (hair on end) appearance
Face - (rodent-like facies)
Long bones Erlenmeyer flask deformity
Extramedullary hematopoeisis
Rare minor features: Growth disturbances, fractures, crystal
deposition
Gauchers Disease
Ashkenazic Jews of Eastern European descent

Defect of beta glucosidase
Accumulation of glycosyl ceramide in the RE cells of BM,
spleen, and liver
Hepatosplenomegaly, yellow skin, scleral pigmentation, acid and
alkaline phosphatase elevation
Imaging features
AVN of the hip and femoral head

Osteoporosis
Marrow expansion with cortical thinning
Erlenmeyer flask deformity
Lytic lesions and sometimes periostitis
Erlenmeyer flask deformity
Differential Diagnosis: Erlenmeyer Flask Deformity
Osteoporosis
Chronic anemia (Sickle cell disease)
Gaucher disease
Niemann- Pick (enzyme deficiency)
Fibrous dysplasia
Metaphyseal dysplasia (Pyles disease)
Figure 4-31-12
Extramedullary Hematopoiesis
[Figures 4-31-13 and 4-31-14]
Blood production in fetal regions

Liver, spleen, adrenal, thymus, heart, lung,
nodes, renal pelvis, GI tract, dura mater (almost
anywhere !)
Major causes:
Hematologic disease (SS and
thalassemia)
Myelofibrosis
Leukemia
Hodgkins
Hyperparathyroidism
Rickets
Carcinomatosis
Figure 4-31-14
Erlenmyer flask deformity in Gauchers disease.

Crumpled tissue paper cytoplasm on histology
Figure 4-31-13
Extramedullary hematopoesis with hepatosplenomegaly and

posterior mediastinal masses
Extramedullary hematopoesis with MR correlation

971
Fanconis Anemia [Figure 4-31-15]
Figure 4-31-15
Onset after first decade

Severe anemia, pancytopenia, brown
pigmentation
Death 23 years after appearance
Anomalies:
Short stature, microcephaly, delayed
ossification
Hip dysplasia, renal anomalies
Radius absent (50%)
Thumb always absent
Thrombocytopenia with Absent Radii

(TAR) [Figure 4-31-16]
Fanconis anemia:
Congenital hypomegakaryocytic thrombocytopenia

Apparent at birth or shortly thereafter
Anomalies:
Bilateral radial aplasia always present
Thumb is present (differentiation from Fanconis)
If kids survive for the first two years, the anemia often
spontaneously resolves
Figure 4-31-16
Hemophilia
Oldest known bleeding disorder

First noted in offspring of Queen Victoria of England
Mutation in Queen Elizabeths X chromosomes
Group of X-linked recessive disorders
Gene carried by women and expressed in men
All races affected
20, 000 hemophiliacs in US
400 new cases/year
Severity related to lack of clotting factor
70% have < 1% of normal clotting factor
TAR with absent radii syndrome: Note that the

thumb is present
Figure 4-31-17
Major Types of Hemophilia
Hemophilia A
85% of all cases
Factor VIII (antihemophiliac factor-AHF)
deficiency
70% have < 1% of normal amounts of AHF
Hemophilia B (Christmas disease)
15% of all cases
Factor IX (Plasma thromboplastin componentPTC) deficiency
Joint Disease
Figure 4-31-18
Acute hemarthroses: tense, swollen, red and tender

joints, pain, LOM, fever, increase in WBC
Stages of joint disease:
Stage I: STS
Stage II: Osteoporosis
Stage III: Osseous lesions
Stage IV: Cartilage destruction
Stage V: Joint disorganization
Knee [Figures 4-31-17 and 4-31-18]
Acute hemorrhagic effusions in two patients with

hemophilia
Marked articular space narrowing and cartilage

destruction with massive subchondral cyst
formation
Dense effusions
Juxtaarticular osteoporosis
Subchondral irregularity
Epiphyseal overgrowth
Squaring of inferior pole of patella (20%-30%)
972
Elbow [Figure 4-31-19]
Figure 4-31-19
Low SI synovial proliferation [Figure 4-31-20]

Differential Diagnosis of Hemophilia
Juvenile chronic arthritis

Single joint tough to differentiate
Distribution may be helpful:
JCA hands/feet/big joints
Hemophilia - knee, ankle, elbow
PVNS, infection, especially TB (monoarticular)
NM diseases: CP,
muscular dystrophy,
Figure 4-31-20
polio
Hemophiliac involvement of two elbows in two
different patients
Figure 4-31-21
Low signal intensity of synovial proliferation in

hemophilia.
Medial talar tilt quite characteristic of hemophilia
Kerr R: Imaging of MSK complications of hemophilia. Sem in MSK
Figure 4-31-22
Other Less Common Imaging Findings
Ectopic ST ossification (periarticular pelvis, thigh,

paraspinal, knee)
Hemophiliac pseudotumor
Osteonecrosis (epiphyseal fragmentation)
Fractures
Chondrocalcinosis
Pseudotumor [Figures 4-31-22 to 4-31-26]
2% of patients
Femur, pelvis, tibia, hands and feet
Locations:
Soft tissue, intraosseous, and subperiosteal
ST
Hard palpable subcutaneous masses
Intraosseous and subperiosteal
Lytic, expansile, destructive, aggressive process
Soft tissue pseudotumor of hemophilia
Park JS, Ryu KN: AJR 2004;
Figure 4-31-23
Subperiosteal pseudotumor of hemophilia

Park JS, Ryu KN: AJR 2004; 183:55-61
973
Figure 4-31-24
Figure 4-31-25
Intraosseous pseudotumors of hemophilia

Park JS, Ryu KN: AJR 2004; 183:55-61
Differential Diagnosis for Hemophiliac

Pseudotumor
Malignancy
Osteosarcoma
Chondrosarcoma
Ewing tumor
Metastases
Infection
Intraosseous pseudotumor of the ilium in a

hemophiliac
Kerr R: Imaging of MSK complications of hemophilia in
MSK Radiology 7:2, 2003
Figure 4-31-26
Myelofibrosis
Affects progenitor (stem) cells of the bone marrow

Primary (idiopathic) and secondary forms
Major manifestations
Fibrotic or sclerotic bone marrow
Other designations:
Idiopathic myelofibrosis
Myeloid metaplasia
Agnogenic myeloid metaplasia
Primary (Idiopathic) Myelofibrosis
Bone marrow replaced by fibrosis

Unknown cause
> 50 year old, incidence - 2/100,000
Findings:
BM fibrosis with hepatosplenomegaly
Anemia
Increased nucleated RBCs
Leukocytosis or leukopenia
Abnormal WBCs
Intraosseous blowout lesions of hemophilia
Figure 4-31-27
Primary (Idiopathic) Myelofibrosis

[Figure 4-31-27]
Unknown cause
> 50 year old, incidence - 2/100,000
Findings:
BM fibrosis with hepatosplenomegaly
Anemia
Increased nucleated RBCs
Leukocytosis or leukopenia
Abnormal WBCs
Diffusely dense bones characteristic of
Diagnosis - BM aspiration
myelofibrosis
Rx: Transfusions, chemo, Interferon, splenectomy,
radiation
50%-80% of patients have elevated serum or urinary uric acid levels
Secondary gout occurs in 5-20% of patients
974
Secondary Myelofibrosis
Malignant disease
Leukemias, Polycythemia vera, MM, Hodgkins disease, NHL, cancer
Chronic infection
Tuberculosis, osteomyelitis
Toxins
X- or gamma radiation, benzene exposure
Imaging Findings [Figure 4-31-15]
Generalized osteosclerosis (most common)

Cortical thickening
Osteopenia
Rarely periostitis
Review
Sickle Cell
Vaso-occlusion
Hand-Foot syndrome
AVN and medullary bone infarcts
H-shaped (Lincoln log) vertebral bodies
Bone within a bone appearance
Salmonella infection
Thalassemia
Hair on end
Pseudohemangiomatous appearance
Erlenmeyer flask deformity (differential-Gauchers)
Rodent facies
Hemophilia
Wide intercondylar notch
Erosions
Medial slope of distal tibia at ankle
Pseudotumor
Myelofibrosis
Primary and secondary forms
Diffusely dense bones
Hepatosplenomegaly
BM bx to make dx
References
1.
2.
3.
4.
5.
6.
7.
"What is Sickle Cell Disease". Sickle Cell Information Center. December 16, 2003. Copyright 1997. The
Georgia Comprehensive Sickle Cell Center at Grady Health System, The Sickle Cell Foundation of Georgia, Inc.,
Emory University School of Medicine, Department of Pediatrics, Morehouse School of Medicine.
http://www.scinfo.org/sicklept.htm
Funaki B. "Sickle cell anemia: Bone manifestations", "Bone within a bone". Chorus: Collaborative Hypertext of
Radiology. (Kahn CE ed). July 2004. Medical College of Wisconsin. February 1995.
http://chorus.rad.mcw.edu/doc/01060.html
Kahn CE. " Erlenmeyer flask deformity". Chorus: Collaborative Hypertext of Radiology. (Kahn CE ed). May
2004. Medical College of Wisconsin.
< http://chorus.rad.mcw.edu/doc/00648.html>
Kerr R. Imaging of musculoskeletal complications of hemophilia. Semin Musculoskelet Radiol 2003; 7:127-136.
Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics 2001; 21:971-994.
Park JS, Ryu KN. Hemophilic pseudotumor involving the musculoskeletal system: spectrum of radiologic findings.
Wong AL, Sakamoto KM, Johnson EE. Differentiating osteomyelitis from bone infarction in sickle cell disease.
Pediatr Emerg Care 2001; 17:60-63; quiz 64.
975
Generalized Musculoskeletal Disorders

Figure 4-32-1
Learning Objectives
To describe a group of entities not well covered in

the rest of the course
To outline the imaging features of these diseases
To introduce the listener to these entities so that
he/she can study about them further
Outline of Diseases
Osteoporosis (and its sequelae)

Osteogenesis imperfecta
Neurofibromatosis
Collagen vascular-like diseases
SLE
Scleroderma
Polymyositis/dermatomyositis
Disuse osteoporosis in a 76 yo with left sided CVA
Terminology
Osteopenia paucity of bone

Osteoporosis
Decreased bone mineral density
Normal in quality
Decreased in quantity
30%-50% of cancellous bone must be gone to recognize
Types of Osteopenia
Localized
Regional or segmental
Generalized or diffuse
Localized Osteopenia/Osteoporosis
Focal areas of bone loss

Infection
Arthritides
Figure 4-32-2
Regional
Osteopenia/Osteoporosis
Segmental area of
decreased BMD
Disuse (immobilization)
Chronic regional pain
syndrome (CRPS)
(RSD (Reflex
sympathetic dystrophy)
[Figure 4-32-2]
Transient osteoporosis
(bone marrow edema)
Regional migratory
osteoporosis
Chronic regional pain

syndrome (Reflex
sympathetic dystrophy)
Note the increased
radionuclide accumulation
on the early and late views
of the bone scan
976
Disuse/Immobilization Osteoporosis
Major cause
Immobilization for traumatic injury
Motor paralysis
Inflammatory lesions of bones and
joints
Changes take 7-10 days (maximal at 23 months)
Patterns: uniform, spotty, bands, cortical
lamination or scalloping
May appear very aggressive!!!
Figure 4-32-3
[Figure 4-32-1]
Chronic Regional Pain SyndromeReflex Sympathetic Dystrophy

[Figure 4-32-1]
Elderly
Trivial trauma
Pain, swelling, temperature changes
Transient Regional Osteoporosis

[Figure 4-32-3]
Transient regional osteoporosis (bone marrow edema) of the hip:

Note the osteopenia of the right hip and the proximal diffuse
increased radionuclide accumulation on the bone scan
Note the marrow replacement in the proximal right femur on T1
and the increased signal intensity (edema) on the T2 weighted
images
General term
Conditions sharing features of:
Rapidly developing, self-limited, reversible osteoporosis
Absence of clear cut inciting events
Major types:
Transient osteoporosis (bone marrow edema) of the hip
Regional migratory osteoporosis
Figure 4-32-4
Rapidly developing, self-limiting and reversible

Knee, ankle, foot and hip
Joint nearest involved likely to be next involved
Transient Regional Osteoporosis

(bone marrow edema) of the hip
1st -women in the third trimester of pregnancy

Middle aged males
LE > UE
Osteoporosis and BM edema
Differential diagnosis: AVN, infection
Generally spontaneously resolves in 9-12 months
Pathology of osteoporosis (rib specimens)
Generalized Osteopenia/Osteoporosis
Diffuse decreased BMD

Senile osteoporosis
Medications (Steroids, heparin)
Systemic diseases (Deficiency states)
Scurvy
Malnutrition
Calcium deficiency
Senile Osteoporosis [Figure 4-32-4]
Pommer-1985 increased porosity

Most commonly encountered metabolic disease
Reduction in bone quantity, normal in quality
F>M, 4:1, (equal incidence) > 80 yo
Most pain source: compression fxs and kyphosis
PE kyphosis, shortened stature, and spinal rigidity
977
Epidemiological data
Surgeon General Report, October, 2004

Half population in US > 50 yo with low bone mass and risk for
fracture
1.5 million/year osteoporosis-related fx
34 million with hip osteopenia
Caucasian females > 50 yo 40% chance of fx in lifetime (13% for
males)
Hip fracture:
Risk of mortality within 3 months is 4X greater than normal
20% of fx victims die or wind up in nursing home within year
after event
Annual cost of treating osteoporosis: $18 BILLION
Figure 4-32-5
Pathology of osteoporosis [Figure 4-32-4]

Osteoporosis life cycle - Fracture index [Figure 4-32-5]
Osteoporosis Measurement [Figure 4-32-6]
Dual energy X-ray absorptiometry (DEXA)

Conventional X-ray (radiogrammetry)
Single photon absorptiometry (SPA)
Dual photon absorptiometry (DPA)
Neutron activation analysis
Quantitative CT (QCT)
Figure 4-32-6
Lumbar
Hip
Distal Radius
Dual Energy X-ray Absorptiometry (DEXA)
Relative tissue attenuation from dual energy X-ray source

Easy to perform
Most reproducible technique with the least coefficient of variation (COV)
Detects changes of 13%
Expressed in gm/cm2
Primary indication: Estrogen deficiency to determine therapy
BMD Terms
BMD measured in gm/cm2

T-score: Patients BMD compared to normative data (Normal = 25 yo women)
Z-score: Patients BMD compared to her aged-matched controls
World health organization uses T scores to classify a patients bone mineral
status
978
WHO Classification of BMD
STANDARD: Mean BMD of 25 yo women

NORMAL: BMD from the mean to 1 standard deviation below the mean (mean
to -1SD)
OSTEOPENIA: T-score from 1 to 2.5 SD below mean (-1SD and -2.5 SD))
OSTEOPOROSIS: T-score below 2.5 SD below mean (> -2.5 SD)
Osteoporosis also established by presence of a non-traumatic vertebral
compression fracture
Figure 4-32-7
Important implications
Fracture risk doubles with each drop of 0.1 below the

mean of the T-score
Risk of fracture also doubles for each decade the
patient is > 50 yo
Goal is to eventually be able to calculate an
absolute fracture risk- more holistic measurement
method
Patients respond better to you have a 70% of
Photomicrograph of senile vertebral osteoporosis
developing a fracture than they do to your T-score
is whatever
Figure 4-32-8
Senile Osteoporosis: Imaging Features
Increased radiolucency on X-ray (osteopenia)

Cortical thinning
Altered trabecular patterns
Senile Osteoporosis: Complications
Acute fractures
Spine (L>T>C)
Distal radius (Colles)
Proximal femur
Humerus (neck)
Ankle (malleoli)
Insufficiency fractures
Covered in osseous stress injury talk
Lateral radiographs of three patients with codfish

vertebral bodies
Spinal effects of osteoporosis
Decreased bone density

Accentuation of primary trabeculae
Cortical thinning
Changes in vertebral shape
Biconcave CODFISH (fish) shape
Endplate deformities (Schmorls nodes, cortical
irregularities)
Wedged vertebrae
Vertebrae plana (pancake/silver dollar)
Senile vertebral osteoporosis

Codfish vertebral bodies
Figure 4-32-9
[Figure 4-32-7]
[Figure 4-32-8]
Femoral neck fracture locations
[Figure 4-32-9]
A = subcapital
B = neck
C = basicervical
D = intertrochanteric
E = subtrochanteric
979
Garden classification [Figure 4-32-10]
< II = Percutaneous pinning

> II = THA (AVN risk)
Figure 4-32-10
Bohndorf, Imhoff, Pope: Musculoskeletal Imaging: A Multimodality Approach

George Thieme Verlag, 2001
Figure 4-32-11
Garden Type I [Figure 4-32-11]

Intertrochanteric Fractures
Extracapsular
Periosteum present
Low incidence of nonunion or AVN (~1%)
Distinction from basicervical often difficult (no clinical
concern)
Most comminuted, 15% severely
GT/LT may be displaced by gluteus or iliopsoas
May have other non-suspected injuries in pelvis
Osteogenesis Imperfecta (OI)
[Figure 4-32-12]
Skeletal, skin, sclera and dentin abnormality

1/30K affected
1/50K severely
20-50K in US
85% AD
Major types:
Congenita
Tarda
Garden I type of femoral neck fracture treated with

Knowles pins
Figure 4-32-12
OI vs Child Abuse
Metaphyseal corner fxs uncommon in OI

Sternal, rib, scapular, skull and bucket handle fxs
common
Fxs continue to occur in protective custody
Other non-MSK findings not present:
Retinal hemorrhage
Visceral intramural hematomas
Intracranial bleeding
Pancreatitis
Splenic trauma
OI-congenital type
OI-tarda
Congenital type of Osteogenesis Imperfecta
Figure 4-32-13
[Figure 4-32-12]
[Figures 4-32-13 and 4-32-14]
Tarda form of Osteogenesis Imperfecta: Note

healing fractures with exuberrant callus formation
980
Figure 4-32-14
Figure 4-32-15
Note multiple fractures, intramedullary rods and

dynamic hip screw and Harrington rods for
scoliosis
Tarda form of Osteogenesis Imperfecta: Note

exuberrant callus formation and intramedullary rod
placement for fractures
Neurofibromatosis (NF)
Described first by Tiresius (1773) and Smith (1849)

Named for von Recklinghausen - noticed association of neural and cutaneous
elements in 1882!!
Defects of all three cell layers = phakomatosis
1/3000 births
Estimated ~100,000 in US
One of humanitys most common genetic disorders
Mutation rate is 1/10,000 gametes/generation
Greater than that for ALL OTHER COMMON GENETIC DISORDERS
AD with variable gene expression (FH in 60%)
Equal incidence in male and female and Caucasian and non-Caucasian
Neurofibromatosis (NF)
Two distinct clinical forms:

NF-1 (vonRecklinghausens) - Caf-au-lait spots, neurofibromas, skeletal
deformities
NF-2 - Acoustic neuromas
Can lead to disfigurement, blindness, deafness, dermal/brain/spinal tumors,
loss of limbs, malignancies, learning disabilities
WAS NOT Elephant Mans DiseaseJohn Merrick had Proteus Syndrome
(cell growth disturbance with hemihypertrophy and macrodactyly)
Imaging features [Figures 4-32-16 to 4-32-20 overleaf]
Spinal changes:
Dural ectasia
Vertebral scalloping
Foraminal enlargement
Pedicle erosion
Mesodermal dysplastic changes:
Scoliosis
- Typical
- Dysplastic, sharply angulated, < 6 segments of lower T spine
(pathognomonic of NF)
Pencilling and spindling of the transverse processes
Long bones (due to neurofibromas or mesodermal dysplastic changes)
Pencilling
Bone erosions
Pseudarthrosis (characteristically of the tibia)
Associated with nonossifying fibromas
Ribs
Scalloped and irregular (twisted ribbons)
Erosions of inferior rib surfaces
981
Figure 4-32-16
Figure 4-32-17
Neurofibromatosis: Note accentuated scoliosis

Neurofibromatosis: Note kyphosis, vertebral
anomalies and widened neural foramen
Figure 4-32-19
Figure 4-32-18
Neurofibromatosis: Note mesodermal dysplastic

changes in the pelvis of two different individuals
Figure 4-32-20
Neurofibromatosis: Note posterior vertebral

scalloping and dural ectasia
Neurofibromatosis with the

characteristic pseudoarthrosis of the
tibia
982
Meningoceles [Figures 4-32-21 and 4-32-22]
About 2/3 of patients with NF

70%-80% of all meningoceles in NF patients
Most common presentation: Asymptomatic post
mediastinal mass
Protrusion of dura and arachnoid through IV foramen
and posterior rib cage into the extrapleural thoracic
cavity - nonca++
Presence of ca++ excludes meningocele
Figure 4-32-21
Systemic Lupus Erythematosis (SLE)
Lupus -Latin for wolf malar erythema looked like

the bite of a wolf
Generalized connective tissue disorder
F>M, second to fourth decade, rare over the age of
45
Higher incidence in AA and Hispanics
Fever, anorexia, weight loss, polyarthralgias, skin
rash
Chronic disease with acute episodes
Variable prognosis
Intrathoracic meningocele: Note the posterior

mediastinal mass on the right
Figure 4-32-22
Three Categories of SLE
Discoid-skin rash only, 20% of patients with SLE

Systemic-chronic, inflammatory, multisystem
disorder of the immune system
Drug-induced-Chlorpromazine, hydralazine,
isoniazid, methyldopa, procainamide
CT and MR imaging of intrathoracic meningocele
Musculoskeletal Imaging Findings
Figure 4-32-23
[Figure 4-32-23]
Major: deforming nonerosive arthropathy

Minor:
Osteonecrosis
Insufficiency fractures
ST calcification
Acroosteolysis
Tendon weakening and rupture
Subchondral cysts
Myositis
Polyarthritis
Osteomyelitis and septic arthritis
Symmetric Polyarthritis
Articular symptoms and signs common-75%-90% of

patients
Non-deforming nonerosive arthropathy of SLE
Frequently bilateral and symmetric
(differential diagnosis is post-Streptococcal
5%-40% with disease
(Jacouds) arthropathy
Hands > knees > wrists > shoulders
ST swelling, periarticular osteopenia
Reversible and little functional effect
!!Cartilage and osseous destruction rare without underlying osteonecrosis!!
Scleroderma
Unknown cause
F:M (4:1)
3rd to 5th decade
< 20 cases/million/year
Variable prognosis
Up to 65% MSK involvement at presentation
Death: Lung, heart and renal involvement
983
CREST Syndrome
Figure 4-32-24
First described by Winterbauer in 1964 as CRST

Velayos added esophageal involvement to make
CREST in 1979
50,000 to 100,000 in US
7 times more common in females
Calcinosis
Raynauds phenomenon
Esophageal abnormalities
Sclerodactyly
Telangiectasia
Calcinosis [Figure 4-32-24]
Abnormal calcium deposition in ST without calcium

metabolism abnormality
Fingers, forearms, and extensor surfaces of elbows and knees
Calcinosis of Scleroderma
Figure 4-32-25
Raynauds phenomenon [Figure 4-32-24]
Often the first symptom of scleroderma

Ischemia of fingers, toes and ears
Numbness, tingling and burning pain
Attacks precipitated by cold, vibration and emotional
stimuli
Acroosteolysis
[Figure 4-32-25]
Acroosteolysis of scleroderma
Gastrointestinal (esophageal) involvement
Fibrosis and atrophy of the smooth muscle

Hypermobility, dysphasia, reflux esophagitis and strictures
Sclerodactyly
Replacement of the normal connective tissue with dense collagen bundles

Skin = thin, appears smooth and is tightly bound
Fingers narrow and taper distally
Telangiectasia
Permanent dilatation of capillaries and venules

Face, lips, tongue and fingers
Idiopathic inflammatory myopathies
Dermatomyositis and Polymyositis

2:1 female to male ratio
5 cases/million/year (incidence increasing)
Dermatomyositis
Complement-mediated (terminal attack complex) vascular inflammation
Polymyositis
Direct cytotoxic effect of CD8+ lymphocytes on muscle
Idiopathic inflammatory myopathies
Dermatomyositis
Men > 40 yo
Skin rash and muscle weakness
Primary malignancies:
Lung, prostate, female pelvic organs, breast or GI tract
Precedes detection of tumor months to years
Polymyositis:
Primary malignancies: Lung, NHL
984
Imaging Findings [Figures 4-32-26 to 4-32-29]
ST abnormalities
ST thickening and edema
Soft and periarticular calcification (IM > SQ)
Articular abnormalities
Radial subluxation or dislocation of IP of thumb
(floppy thumb) = quite characteristic
Erosions of multiple sites in hands
Flexion deformities (MCP)
Swan neck deformity
Figure 4-32-26
Polymyositis with soft tissue calcification
Figure 4-32-28
Figure 4-32-27
Floppy thumb of
dermatomyositis/polymyositis
Dermatomyositis with extensive soft
tissue calcification
Figure 4-32-29
Swan neck deformity
MC flexion, PIP hyperextension and flexion at the DIP

Most common inrheumatoid arthritis
Other imaging Findings
RBS
Increased accumulation at sites of calcification (Technetium
and gallium)
MR Imaging
Muscle atrophy
Fatty replacement
Decreased SI correlating with activity of disease
Increased SI on T2WI and STIR
Remember!
PM, DM, Scleroderma, SLE, mixed CVD and overlap syndromes

may all look alike
ST calcification
Articular and osseous abnormalities
Dermatomyositis with increased signal

intensity in the right gluteal region
985
Summary
Osteoporosis
Most common metabolic disease
Insufficiency fractures may mimic mets/myeloma
Diagnosis of exclusion in young patient with osteopenia out of proportion
to age
Easily fractured and exuberant callous formation
Neurofibromatosis
Pencilling, pseudarthrosis,, posterior scalloping, thoracic meningocele
Systemic lupus erythematosis
Ulnar deviation without erosions (differential is Jacouds (poststreptococcal arthritis)
Scleroderma
CREST, acroosteolysis
Inflammatory muscle disease
Dermatomyositis and polymyositis
Nonspecific findings (look like scleroderma)
Must look for malignancy in these patients
References
1.
Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York,
NY, Thieme Medical Publishers, 2001
986
Osseous Musculoskeletal Stress Injuries

Outline
Biomechanical considerations
Historical perspective
Epidemiology and clinical manifestations
Anatomic approach with examples
Stress injury look alikes
Avulsive and muscular tug injuries
Unusual activities causing stress injury
Recommended work-up
Bone Fatigue
1922 - Muller
Isolated a segment from the radius of a dog
Created a fatigue fracture of the ulna
1949/50 - Rutishauser/Majno
First description of the histologic aspects of fatigue fractures
Muller W. Bruns Beitr. klin. Chir. 127:251-290, 1922

R/M. Schweiz. med. Wschr. 79: 281-88, 1949. 6:333-42, 1950.
Historical Perspective
1855 - Breithaupt - Prussian military surgeon

Clinical features of painful feet on long marches
1887 - Pauzet - army doc
Exostoses on PE from bone proliferation soldiers feet
1897 - Stechow - Prussian guard
First imaging in 36 cases of MT stress fractures
1905 Blecher
First femoral neck stress fracture
1921 Deutschlander - German physician
Comprehensive study of stress lesions in > 50 yo
1936 - Asal German
First large series of 590 stress fractures in German troops
Types of MSK stress injury
Soft tissue injury

Bruises
Muscle tears
Musculotendinous injury
Collagen injury
Tendon and ligament strain/tears
Cartilage injury
Chondral injury (hyaline and fibrocartilage)
Osseous abnormalities
Stress reaction (sclerosis/lucency/periosteal reaction)
Stress fracture
Stress injuries
Most common in lower extremities

Dissipation of ground reaction forces (GRF) (running, walking, marching,
jumping)
Bone exposed to stress (ie load) and strain (ie deformation) with weightbearing
Factors influencing bone response:
Bone geometry and bone density
Jumping and landing GRF up to 12 X body weight
987
How Do Stress Injuries Develop?
Wolffs law
Bone remodels in DIRECT reponse to the forces applied to it
Normally a happy marriage/relationship between osteoblasts
and osteoclasts
Increased stresses cause increased osteoclastic activity with
transient weakening
Transient weakening predisposes to microdamage
Coalescence of microdamage stress reaction or injury
Cascade
Fissures
Microfractures
Osteoblastic response (periosteal reaction or cancellous
clouding)
Coalescence
Fatigue reaction/injury
Figure 4-33-1
Epidemiology
20% of all injuries seen in sports medicine clinics

Between 4.7% and 15.6% of injuries in runners
20-25% of stress injuries in tibia, fibula or MT
Females: 49% with very irregular menstruation, 39% with
irregular menstruation
Study of 320 athletes with stress injury:
69% runners
8% fitness class participants
5% racket sports
4% basketball players
Track athletes have highest incidence
Clinical Features of Stress Injury
Pain associated with activity

Pain resolves without activity
Pain in characteristic location associated with activity
PE: ST swelling, point tenderness
Grade 4 Stress injury of the tibia,

pathological fracture of the fibula in
patient with RA on steroids
Condensation of cancellous bone
Perpendicular to the long axis
Risk Factors for Stress Injury
Intrinsic
Low BMD
Lower limb misalignment
Muscle fatigue
Weakness/strength imbalance
Pathologic bone
Menstrual/hormonal irregularities
Genetic predisposition
Extrinsic
Excess volume/intensity of training
Change in training surface (density or topography)
Worn out training shoes
Inadequate nutrition
Cigarette smoking
988
Activities associated with Stress Injury
Lower extremity
Running
Marching
Soccer (pelvis)
Basketball
Skating (fibula)
Jumping (pelvis)
Swimming (tibia, MT)
Ballet (pelvis, spine)
Upper extremity
Baseball:
Throwing-Humerus, scapula, olecranon, first rib
Batting-Ribs
Catching-Patella, tibia
Javelin throwing-ulna
Basketball
Volleyball
Activities associated with Stress Injury
Rowing, kayaking-Second through tenth ribs

Running with hand held weights-Scapula
Coughing-ribs
Trapshooting-Coracoid process
Major categories of Stress Injury
Fatigue fracture
Abnormal muscular stress of torque
Bone of normal elastic resistance
Insufficiency fracture
Normal or physiologic activity
Bone deficient in mineral or elastic resistance
*****Imaging findings are similar
Risk factors - Insufficiency fractures
Osteoporosis
Metabolic disease
Hyperparathyroidism
Osteomalacia/Rickets
Cushings disease
Paget disease
Diabetes mellitus
MR Grading System Stress injuries
0 Normal study
1 Subtle periosteal edema (IR, FS T2-W images)
2 Periosteal edema and increased marrow SI on FS T2-W images
3 More extensive edema (T1-W and T2-W)
4 Discrete fracture line visible on MR or on radiography
[Figure 4-33-1]
Fredericson M, Bergman AG et al: Am J Sports Med 1995;23:472-481
Anatomic approach
Lower extremity: Foot (MT, navicular, calcaneus), tibia, femur

Pelvis (Insufficiency and adolescent)
Upper extremity
Unusual causes of stress injury
Mimics of stress injury
Recommended workup
989
Metatarsal stress fractures [Figures 4-33-2 to 4-33-5]
Second most common stress fracture site behind the

tibia
First described in military recruits
Flat flexible feet = MT stress fractures
Cavus feet = tibial stress fractures
Distance runners and ballet dancers
In order: 2, 3, 1, 4, 5
Figure 4-33-2
Navicular stress fracture [Figures 4-33-6 and 4-33-7]
0.7%-2.4% of all stress fxs

Activities: Track and field (59%), Australian football
(19%), Basketball (10%)
First description: 1958 in greyhounds
1970 - humans
Most common in runners
Increasing dorsal midfoot pain radiating down medial
arch
Rx: Non-weight bearing cast (86% success rate)
Figure 4-33-3
Second MT fatigue fracture
Figure 4-33-4
Right 2nd MT fatigue fracture and 1 month f/u in

42 yo male
Figure 4-33-5
MR imaging of third MT fatigue fracture, soccer

player
Sesamoid necrosis in ballet dancer

990
Figure 4-33-6
Figure 4-33-7
Classic navicular fatigue fracture

[Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]
Figure 4-33-8
Classic fatigue fracture of calcaneus
Microangiopathic studies cadaveric

feetnavicular supplied by both
ant and post tibial arteries, enter at
small waist of cortical bone and
branch out to supply the medial
and lateral 1/3 Central 1/3,
under greatest stress with relative
avascularity
[Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]
Tibia stress fracture
Figure 4-33-9
Three major types

Medial tibial plateau
Tibial diaphysis
Anterior mid tibia
Medial tibial plateau [Figure 4-33-9]
Less frequent than tibial diaphysis

Often misdiagnosed as pes anserinus bursitis or
tendoninitis
Less critical stress injury
Treatment
Rest for 4-6 weeks
Then return to full activity
Medial tibial fatigue fracture
Figure 4-33-10
Medial tibial stress syndrome

Shin splints[Figure 4-33-10]
MR features of medial tibial stress

syndrome (shin splints)
991
Figure 4-33-11
Figure 4-33-12
Aggressive periosteal reaction in the tibia in

fatigue fracture
Diaphyseal fatigue fracture with histologic
correlation from the AFIP fascicles
Figure 4-33-13
Tibial diaphyseal Fatigue Fracture[Figures 4-33-11 and 4-33-12]
Posteromedial border of tibia

Tensile forces produced along anterior convex side, compressive
forces along posterior concave margin
465 injuries causing exertional leg pain 75% in posteromedial
tibial diaphysis
Difficult to tell stress injury from shin splints (medial tibial stress
syndrome)
Anterior mid-tibia [Figure 4-33-13]
Most common in jumping and leaping athletes

Focal cortical thickening and sclerosis
Dreaded black line
Propensity to nonunion
Risk of complete displaces fracture
Require more aggressive treatment
Diaphyseal fatigue fracture with the

dreaded black line
Figure 4-33-14
Longitudinal Tibial Stress Fracture

[Figure 4-33-14]
Devas 1960
Patients MAY not give h/o increased activity
Saifuddin (Clin Rad 1994):
Two cases
Stress fx located superomedial to the nutrient
foramen of the tibia
Foramen weakens bone at this site?
? insufficiency fracture
Longitudinal stress fractures of the tibia
Longitudinal fatigue fracture
Craig et al, Henry Ford Hospital, Detroit,

Skeletal Radiology, 2003
Six cases
All female (age range - 15-69 yo)
Diagnosis made by finding cleft on multiple axial
images
5/6 patients had:
Edema starting at level of the entrance of the
nutritent vessel into the medullary cavity
Vertical fx identified below this level on the
anteromedial tibial cortex
Figure 4-33-15
Compression type of femoral neck fatigue fracture
992
Femoral neck stress injury [Figures 4-33-15 and 4-33-16]
Any athlete (jogger/runner) with hip, thigh or groin

pain
Two types
Tension type:
Superior cortex
Older osteoporotic patients
Compression type:
Younger athletic patients
Treatment: 2-3 months non-weight bearing with
gradual return
Pelvic Stress Injury [Figures 4-33-17 to 4-33-21]
Running increases risk of stress lesions in sacrum

and ischial rami
Sacral fractures more common in young women
(sxs mimic sacroiilitis)
Fatigue:
Anteroinferior sacral wing unilaterally
Insufficiency:
Elderly women, irradiated women
Often bilateral (Honda sign)
Figure 4-33-16
Fatigue fracture which progressed to complete

femoral neck fracture
Figure 4-33-17
Figure 4-33-19
Sacral insufficiency fracture
Figure 4-33-18
Bilateral sacral insufficiency

fractures (the Honda sign)
Figure 4-33-20
CT of previous figure on left showing
the classic CT findings of right sacral
insufficiency fracture
Figure 4-33-21
Symphysis pubis stress reaction in soccer player

Bilateral sacral and symphyseal insufficiency
fractures in elderly female S/P external beam
radiation for cervical cancer. Note the bilateral
nature of the sacral fractures
993
Adolescent Stress Lesions [Figure 4-33-22]
Muscular tug (avulsive) lesions (Classic lesion:

Cortical desmoid)
Avulsion injury and sequelae
Tendons, ligaments stronger than bone in
adolescents
May mimic primary soft tissue neoplasm in acute
setting
May mimic primary bone neoplasm after healing has
occurred
Figure 4-33-22
Recommended Workup - Suspected Stress

Injury
Correlate clinical situation with imaging
Think stress injury in the correct setting (you may
be the only clinician who does so!!)
Initial study is the conventional radiograph
Young patient-CT or MR imaging
Elderly patient
Typical site-CT or MR
Unusual site-RBS as screen, then CT or MR
16 yo with bilateral healed avulsions whose

diagnosis was made at age 42!
References
1.
2.
3.
4.
5.
Chamay A. Mechanical and morphological aspects of experimental overload and fatigue in bone. J Biomech 1970;
3:263-270.
Craig JG, Widman D, van Holsbeeck M. Longitudinal stress fracture: patterns of edema and the importance of the
nutrient foramen. Skeletal Radiol 2003; 32:22-27.
Mller W. Bruns Beitr. klin. Chir. 127:251-290, 1922
Rutishauser E, Majno G. [Lesions of normal and pathological bones due to overstrain.]. Bull Schweiz Akad Med
Wiss 1950; 6:333-342.
Tschantz P, Rutishauser E. [The mechanical overloading of living bone: initial plastic deformations and adaptation
hypertrophy]. Ann Anat Pathol (Paris) 1967; 12:223-248.
994
Pelvis and Lower Extremity Trauma:

An introduction
Figure 4-34-1
Outline
Caveats
Major highlights
Not enough time to cover in depth
You must read more on your own to
supplement this lecture
Pelvic trauma
Acetabular trauma
Lower extremity trauma
Femur
Knee
Ankle
Foot
Talus
Calcaneus
Classic fxs
Radiographic anatomy [Figure 4-34-1]

Major mechanisms of pelvic injury
[Figure 4-34-2]
Pelvic Radiographic Anatomy, ip = iliopubic line, ii =

ilioischial line, SIJ = sacroiliac joints
Most Popular Classifications Pelvic Ring Fractures
Figure 4-34-2
Tile
Pelvic stability
Young-Burgess
Degree of injury
Major divisions
Ring sparing
AP compression
Lateral compression
Vertical shear
Complex
Major mechanisms of pelvic injury (Tony Wilson,

Seattle Washington)
Young-Burgess vs Tile Classifications

FRACTURE TYPE
YOUNG-BURGESS
TILE
Ring sparing
Not included
Type A
Anterior compression
AP compression
Types I-III
B1
(B1, 1.1-1.3)
Lateral compression
Lateral compression
Types I-III
B2
(B2, B2.1-2.2)
Vertical shear
Vertical shear
C
(C1-3)
995
Pelvis-Lower Extremities Trauma
Young-Burgess Classification Pelvic Ring Fractures

Lateral compression (Most common)
Types I and II
Anteroposterior (AP) compression
Types I, II and III
Vertical shear
Figure 4-34-3
Lateral compression
Most common mechanism of pelvic injury

Lateral blow to the side of the pelvis
Three types depending on severity
KEYS TO THIS INJURY:
Horizontal fxs of pubic ramus/rami
Crush (buckling) fx of sacrum
Lateral compression Type I injury-STABLE

(Note the disruption of the sacral foraminal
(arcuate) lines-arrows)
Figure 4-34-4
Lateral compression - Young-Burgess

classification [Figures 4-34-3 to 4-34-5]
I Ipsilateral sacral compression fx (stable)

II- I + associated iliac wing fx
Rotationally unstable
Vertically stable
III I + II with contralateral open book appearance
(windswept pelvis)
Windswept pelvis - Lateral compressionipsilateral - AP compressioncontralateral [Figure 4-34-6]
Lateral compression Type I injury
Severe anterior force

Internal rotation of ipsilateral hemipelvis with external rotation of
contralateral hemipelvis
Rolled over look
Figure 4-34-5
Figure 4-34-6
Windswept pelvis
Lateral compression Type I injury
AP Compression
Blows to front of pelvis

MVA
Three types depending on which ligaments involved
Increases volume of pelvis
Major risk = hemorrhage
Often brain/abdominal injuries
Vertical fx inf pubic rami (one or both sides)
> 50% post acetabular wall
< 10% sacral fx
996
AP Compression - Young-Burgess classification

[Figures 4-34-7 to 4-34-9]
Type I = SP disrupted (all ligs intact)

Type II
SP diastasis < 2.5 cm
Torn ligaments:
SP, SS, ST and ventral (anterior SI)
Type III
SP diastasis > 2.5 cm
Torn ligaments:
SP, ST, SS and both ventral (anterior) and
dorsal (posterior) SI
Figure 4-34-7
AP compression Type II injury
Figure 4-34-8
Figure 4-34-9
AP Compression injury with vertical fracture of the

sacrum
AP compression injury (Note marked

widening of the symphysis pubis)
AP Compression, Type II
Disruption of sacrospinous, sacrotuberous and ventral (anterior) SIJ

ligaments
Diastasis of SP > 2.5 cm
Diastasis of both SIJ anteriorly
Open book appearance
Rotationally unstable
Vertically and posteriorly stable
Figure 4-34-10
AP Compression, Type III
Type I and II
Disruption of all SIJ ligaments
Complete separation of iliac wing from sacrum
Complete pelvic instability
Rotationally, vertically and posteriorly unstable
Vertical Shear
Fall from height or head and back trauma

Least common
Disruption of SP or SIJ
Cephalad or caudad displacement of pelvis (best seen on
OUTLET film)
Rotationally, vertically and posteriorly UNSTABLE
Vertical Shear [Figures 4-34-10 and 4-34-11]
Disruption of SP, ST, SS, and ant/post SIJ ligaments

Characteristics
Vertical pubic rami fractures
SIJ disruption +/- adjacent fractures
Hemipelvis vertically (cranially) displaced
997
Vertical shear injury Note vertical

migration of left hemipelvis
Clues
Horizontally oriented pubic fracture

Think lateral compression, look closely at sacral arcuate lines
Vertically oriented pubic fracture
With AP displacement, think AP compression
With vertical displacement, think vertical shear
Posterior wall acetabular fx
Think AP compression
Central acetabular fxs
Think lateral compression
Figure 4-34-11
Complications Pelvic Ring Disruption [Figure 4-34-12]
Mortality 5%-50% (reflect severity)

AP compression 26%
Vertical shear 25%
Complex 17%
Lateral compression 13%
Head injury and hemorrhage (internal iliac branches or superior
gluteal artery near sciatic notch)
Acetabular Injury
Significant trauma (MVA, falls)

Associated pelvic ring fractures
Pattern of acetabular injury depends on:
1. Position of femoral head at time of traumatic event
FH externally rotated = anterior column
FH adducted = acetabular roof
FH abducted = forces transmitted inferiorly
2. Direction of force
Anterior force = posterior wall and column
Lateral force = medial acetabular wall (transverse type)
Therapy depends on proper classification
Acetabular Columns Letournel and Judet
Anterior
Iliac wing to anterior acetabulum
Incorporates superior pubic ramus
Posterior
Sciatic notch to posterior acetabulum to ischium
Soft tissue findings of vertical shear

injury
Figure 4-34-12
Inverted Y column principle

Radiographic Evaluation Acetabulum
[Figure 4-34-13]
AP pelvis
Judet views
45 degree oblique views
Right
RPO = Iliac oblique
RAO = Obturator oblique
Left
LPO = Iliac oblique
LAO = Obturator oblique
Soft tissue complications of pelvic ring fractures
Figure 4-34-13
Acetabular fractures
Letournel and Judet classification, 1993

Ten different patterns
Five elementary (run in single plane)
Five associated (combination of elementary)
Difficult to remember
Most common:
Posterior wall
Transverse with posterior wall
Both column (most common type)
Add T-shaped and transverse = 90%
Pelvic CT anatomy (see Harris et al: AJR

2004;182:1363-75)
998
Letournel and Judet, 1993
Figure 4-34-14
Elementary (simple) fractures

Posterior wall
Posterior column
Anterior wall
Anterior column
Transverse
Complex (associated) fractures
T-shaped
Posterior wall posterior column
Transverse posterior wall
Anterior with posterior hemitransverse
Both columns
CT patterns of acetabular fractures

Hunter, RCNA 1997
Axial CT image through roof of acetabulum

Column Fracture
Transverse Fracture
Wall Fracture
Normal
Wall Fracture [Figures 4-34-14 and 4-34-15]
Weight-bearing columns of acetabulum not disrupted

Posterior wall most common
Major complications:
Hip joint instability
Osteonecrosis
Transverse Fractures [Figures 4-34-16 and 4-34-17]
Medial and lateral components

Fx line anterior to posterior
Separates walls from columns
CT = sagittal plane
Figure 4-34-15
Posterior wall acetabular fracture
Figure 4-34-16
CT of posterior wall fracture
(Same patient )
T-type transverse fracture

999
Column Fracture
Figure 4-34-17
Craniocaudad (coronal) direction

Front and back halves
Ant/post only or both
Associated with other fxs (post column, post wall)
Conceptualize: Grasp ASIS could move acetabulum
freely
Obturator fx = column type or T-shaped fracture
Questions To Ask Yourself
Obturator ring fx?

T-shaped or column fx
Ilioischial line disrupted?
Posterior column or transverse fx patterns
Iliopectineal line disrupted?
Anterior column or transverse-type fxs
Is iliac wing above acetabulum fractured?
Fracture of anterior column
Is the posterior wall fractured?
Isolated or combo with post column or transverse
fxs
Is spur sign present?
Almost assuredly both column fracture
CT of T-type transverse fracture

with Sagittal (lower left)
and Coronal (lower right) reconstruction
Hip Trauma
Dislocations
Hip fxs
Common injury in multi-trauma
Common in the elderly
Osteoporosis and cerebrovascular disease
Prone to falls
Hip Dislocations
5% of all dislocations
High energy trauma (MVA, MCA, etc.)
~90+% posterior
Commonly associated with femoral shaft, patella and post acetabular fxs
Clinically
Limb shortening, internally rotated and adducted
10-15% transient sciatic nerve palsy (direct impingement)
Hip Fractures
Intracapsular
Subcapital
Mid cervical
Basicervical
Extracapsular
Intertrochanteric
Subtrochanteric
Femoral neck fxs 3-6X > women
Intertroch fx = frequency
Subtrochanteric Fractures
Fracture line extends between LT and point 5 cm distally

Direct trauma
Older patient, less force required
High incidence of malunion or nonunion
? secondary to greater proportion of cortical bone to trabecular bone in this
region
Rx: Intramedullary rod
1000
Knee Injury
Soft tissue signs

ST swelling, lipohemarthrosis
Fractures
*Supracondylar
*Condylar
*Tibial plateau
Impaction (lateral femoral condylar notch)
Tibial avulsion fractures
Segond fracture
Patellar fractures and dislocation
Tibial Plateau Fractures
Valgus stress, 85% involve lateral tibial plateau

Fat-fluid level
Schatzker classification (6 types)
Depression = cartilage thickness (3mm)
Meniscal injury ~ 50%
Rx: Lateral buttress plate and screw
Schatzker Classification
I=split fx (younger)
II=split + depression of LTP (older)
III=depression - splitting
IV=MTP +/- depression
V=split fx through MTP and LTP
VI=dissociation of TP from underlying diaphysis
Tibial Plafond (pilon) Fracture
High energy axial loading (talus on tibial plafond)

Ankle and distal tibial metaphyseal fx, intraarticular
20%-25% open
Associated injuries:
Compartment syndrome, vertebral compression fractures
Often require ORIF
Post-traumatic arthritis common
Maisonneuve fracture
External rotation of ankle

Fibular fx
Serious injury
Requires ORIF with screws
Removed 8-12 weeks after injury
Ankle Fractures
Common injuries
Soft tissue changes (STS, effusion)
Classification schemes
Lauge-Hansen
Difficult to remember
Not very reproducible
Danis-Weber (AO)
Easy to remember
Reproducible
Danis-Weber Classification
Type A: Horizontal avulsion fx below mortise, stable, Rx: Closed reduction

and casting (without displaced MM fx)
Type B: Spiral fibular fx level of mortise, external rotation, stable or unstable,
Rx: Closed reduction unless fragments displaced
Type C: Above mortise, disruption of lig attachment of tibia/fibula distal to fx,
unstable, Rx: ORIF
1001
Ankle Fractures
Key is re-establishing tibiotalar joint congruence

Mortise view important
1-2 mm displacement of talus in mortise dramatically
changes contact area and pressure
40% decrease in contact area with 1mm lateral talar
shift
Talar Neck Fractures [Figure
Figure 4-34-18
4-34-18]
3%-5 % of foot fractures

Dorsally directed force on braced foot (aviators
astragulus WWI pilots), now most commonly MVA
Main blood supply of talar body enters neck through
sinus tarsi and proceeds retrograde to supply body
Neck fxs compromise vascularity
Hawkins sign (no AVN)
Talar Neck Fractures - Hawkins

classification [Figure 4-34-19]
Hawkins II talar neck fracture
Figure 4-34-19
Figure 4-34-20
Risk of AVN: I = 10%; II = 40%; III = 90%; IV = 100%

Hawkins classification of talar neck fractures
(Bohndorf K, Imhoff H, Pope T: Synopsis of MSK Imaging: A
Multimodality Approach, Thieme)
Lateral Process of Talus fx [Figure
Calcaneal Fractures [Figure
4-34-20]
Snowboarders fx
Eversion
Lat process caught between LM and calcaneus
May be caused by inversion and dorsiflexion
4-34-21]
Most frequently fractured tarsal bone (60% of all fxs)

2% of all fxs in adults
5% - 9% bilateral
10% LS compression fxs
Peroneal tendon entrapment or compartment
Intraarticular 70%, extra-articular 30%
Most common EA = calcaneal body fx
Anterior process = 15%
Difficult to treat if displaced
Figure 4-34-21
Lateral process of talus

fracture (Snowboarders
fracture)
Coronal reconstruction
of CT of calcaneal
fracture
CT of calcaneal fracture
1002
Classification systems Calcaneal Fractures
Figure 4-34-22
Bohler (1931)
Essex-Lopresti (1952)
Intraarticular vs extra-articular
Types: Tongue and Joint depression
Rowe (1963)
Saunders CT classification (1992)
Others: Hanover, Rowe, Palmer, Souer and Remy
Sanders Classification
I = Non-displaced
Non-operative
II = 2 parts (split)
ORIF
III = 3 parts (split and depression
ORIF
IV = Comminuted
Defies open reduction
Measures height of PF
A = most cephalic point of tuberosity to posterior
border of subtalar joint
B = posterior border of subtalar joint to anterior
process
Normal: 20 - 40
Anatomy of the tarsal joints (Bohndorf K, Imhoff H,
Pope T: Synopsis of MSK Imaging: A
Multimodality Approach, Thieme)
Foot Injuries
5th MT
Avulsion (pseudo-Jones or tennis fracture)
Jones
Stress fx (fatigue or insufficiency)
LisFranc
Figure 4-34-23
Jones Fracture
Transverse fx
2-3 cm distally
Displaces on weight bearing
35%-50% persistent non-union
Google Search: Sponsored links (Jones Fracture lawsuits-Recover medical

expenses-Find attorneys and help nationwide-personal-injury-lawyer.com)
Lisfranc [Figures
4-34-22 and 4-34-23]
Napoleonic surgeon
Developed quicker technique of forefoot amputation for gangrene
Faster wiithout having to cut bone
Injury in foot never described by him
Commonly misdiagnosed
Summary
Divergent (left) and Homolateral

(right) types of LisFranc injury
Reviewed major pelvic, acetabular and lower extremity traumatic

lesions
Meant as an introduction
Supplement with reading and study
Xerox major classifications of fractures
Have readily available in MSK reading area
Consult classifications frequently
Supplement clinical experience with personal reading EVERY DAY
References
1.
2.
3.
4.
Bohler L: Diagnosis, pathology, and treatment of fractures of the os calcis. J Bone Joint Surg 13:75-89, 1931.
Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York,
NY, Thieme Medical Publishers, 2001
Borrill J, Funk L, Deakin S. Orthoteers: The guiding light in orthopaedic education. 2006.British Orthopaedic
Association.. <http://www.orthoteers.org/>
eMedicine (James WD, Adler J, Lutsep HL, Lorenzo CT, Lin EC, Ho SSW, Roy H, Gellman H, Meyers AD eds)
1003
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
1996-2005 eMedicine.com, Inc . WedMD. <http://www.emedicine.com/>

Essex-Lopresti P. The mechanism, reduction technique, and results in fractures of the os calcis. Br J Surg 1952;
39:395-419.
GE: Healthcare reimagined. Copyright General Electric Company 1997-2006. GE Medical Systems. 2006.
<http://www.amershamhealth-us.com/>
Harris JH, Jr., Lee JS, Coupe KJ, Trotscher T. Acetabular fractures revisited: part 1, redefinition of the Letournel
anterior column. AJR Am J Roentgenol 2004; 182:1363-1366.
Hunter JC, Brandser EA, Tran KA. Pelvic and acetabular trauma. Radiol Clin North Am 1997; 35:559-590.
Letournel E, Judet R. Fractures of the acetabulum, 2nd ed. Heidelberg, Germany: Springer-Verlag,1993
MacLeod M, Powell JN. Evaluation of pelvic fractures. Clinical and radiologic. Orthop Clin North Am 1997;
28:299-319.
Palmer I. The mechanism and treatment of fractures of the calcaneus: open reduction with the use of cancellous
grafts. J Bone Joint surg 1948;30-A(1):2-8
Perry DC, DeLong W. Acetabular fractures. Orthop Clin North Am 1997; 28:405-417.
Rowe CR, Sakellarides HT, Freeman PA, et al. Fractures of the os calcis: long term follow-up study of 146
patients. JAMA 1963;184:920-923
Sanders, R., Hansen, S.T. & McReynolds, I.S.: Fractures of the calcaneus, in Jahss, M. (Ed.): Disorders of the foot
and ankle, Philadelphia, W.B. Saunders, 1991. p. 2326-2354.
Souer, R. & Remy, R.: Fractures of the calcaneus with displacement of the thalamic portion. J Bone Joint Surg [Br]
57: 413-421, 1975.
Wheeless' Textbook of Orthopaedics. Copyright 1996-2005 Data Trace Publishing Company. Duke University
Medical Center's Division of Orthopaedic Surgery. Data Trace Internet Publishing Company. 2006.
1004
Musculoskeletal Seminar I
Mark D. Murphey, MD
UNKNOWN CASE #1: HISTORY
15 year old male with longstanding hindfoot pain
UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS

LESIONS WITH SEQUESTRA-LIKE APPEARANCE
Osteomyelitis
Metastasis
Fibrosarcoma/ Malignant Fibrous Histiocytoma (MFH)
Lymphoma
Osteoblastoma

CORTICAL LUCENCY/CENTRAL CALCIFICATION

Osteoid osteoma
Brodie abscess
UNKNOWN CASE #1: FINDINGS
Diffuse sclerosis of calcaneus

CT-solid periosteal reaction causing sclerosis on radiographs
Subchondral low density lesion with central calcification
Diffuse edema/focal lesion related to posterior subtalar joint with
joint effusion
T1
UNKNOWN CASE #1: OSTEOID OSTEOMA
1025 years, M>F (3:1)

Night pain relieved by ASA
Lytic nidus (<1.5-2.0 cm); central calcification
Intracortical-extensive periosteal reaction
Intramedullary-often subtle little sclerosis
Lymphofollicular synovitis
CT/MRI important for surgical guidance
T2
UNKNOWN CASE #1: OSTEOID OSTEOMA

TREATMENT OPTIONS
Surgical excision
Percutaneous removal
Percutaneous ablation
Medical
GRE
1005
27 year old female with 5 years chronic left hip pain
UNKNOWN CASE #2: OSSEOUS LESIONS

BOTH SIDES OF JOINT
Arthritis
Infection
PVNS
Synovial chondromatosis
Amyloidosis
UNKNOWN CASE #2: PVNS
Young males-3rd to 4th decade

Synovial proliferation with hemosiderin deposition
Extrinsic erosions common in hip
Joint space normal; limited osteopenia
Can appear like OA in hip
Joint fluid-nodular thickening at arthrography
MRI often characteristic low intensity, fluid foci
RX -synovectomy-adjuvant radionuclide therapy
Proton Density
T2
5 year old male with mild midfoot pain
Symptomatic side
UNKNOWN CASE #3: OSTEOCHONDROSES
Fragmentation
Sclerosis
Collapse
AVN, trauma, normal
UNKNOWN CASE #3: KOHLER DISEASE
Described 1908
M>F; 46:1; 37 years old
Often asymptomatic
Unilateral 7580%
AVN ?
Treatment-immobilization
Asymptomatic side
1006
13 year old female 1 year of pain now severe and worsening
UNKNOWN CASE #4: BRODIE ABSCESS
Subacute osteomyelitis
Medullary or cortical lucency surrounding sclerosis
Channel-like lesion may extend to or across growth plate
Staph aureus
MRI or CT to evaluate soft tissue extension
T2
30 year old female with progressive clubbing of fingers
UNKNOWN CASE #5: ACROOSTEOLYSIS

BANDLIKE: DIFFERENTIAL DIAGNOSIS
Hyperparathyroidism
Polyvinyl chloride
Hajdu-Cheney syndrome
Post-traumatic
UNKNOWN CASE #5:

WORMIAN BONES: DIFFERENTIAL DIAGNOSIS
Normal
Cleidocranial dysplasia
Cretinism
Hypophosphatasia
Pyknodysostosis
1007
UNKNOWN CASE #5: HAJDU-CHENEY SYNDROME
Autosomal dominant; described 1948

Bathrocephaly, wormian bones, open sutures
Acroosteolysis
Poor dentition
Osteoporosis
4 year old male with left hip pain

EPIPHYSEAL LESIONS
Chondroblastoma
Giant cell tumor (GCT)
Subchondral cyst/Intraosseous ganglion
Infection
Clear cell chondrosarcoma
Lytic lesion epiphysis and metaphysis

Small amount surrounding sclerosis
MRI and CT no joint fluid or calcification
UNKNOWN CASE #6: EOSINOPHILIC GRANULOMA

(LCH)
515 years; M:F-2:1

95% of patients Caucasian
Solitary 67%
Flat bones involved-70%
Lytic hole within hole appearance
Diaphysis (58%), metadiaphysis (18%), metaphysis (28%),
epiphysis (2%)
1008
Musculoskeletal Seminar II
Mark D. Murphey, MD
62 year old male with polyarticular joint pain
Asymmetric erosive arthritis hands and feet: MCP, and IP joints

New bone formation
Limited osteopenia
UNKNOWN CASE #1: SERONEGATIVE

SPONDYLOARTHROPATHY CHARACTERISTICS
Asymmetry
Bone Production
Less juxtaarticular osteopenia
Distribution
UNKNOWN CASE #1: PSORIATIC ARTHRITIS

POSSIBLE PRESENTATIONS
DIP and PIP joints

Arthritis mutilans
Oligoarthritis or ray distribution
Rheumatoid like (rare)
Sacroiliitis/spondylitis
1009
42 year old female with right low back pain
Unilateral destruction on both sides right sacroiliac joint

Thickening of iliacus muscle
Focal sclerotic fragments in joint
UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS SACROILIITIS
Unilateral-Infection, RA, Gout, Psoriatic, Reiter

Bilateral asymmetric-RA, Gout, Psoriatic, Reiter
Bilateral symmetric-AS, Enteropathic, Psoriatic, Reiter, RA
1 year old female with hand and foot pain
Soft tissue swelling about several fingers

Periosteal reaction along several rays: phalanx hand,
metacarpal and metatarsal
UNKNOWN CASE #3:

DIFFERENTIAL DIAGNOSIS DACTYLITIS
Infection
Pyogenic
Unusual organism-TB
Sickle cell anemia
Thermal injury
UNKNOWN CASE #3: SICKLE CELL ANEMIA
Musculoskeletal changes
Osteomyelitis (salmonella)
H-type vertebrae
Osteopenia
Diffuse sclerosis
1010
11 year old male with ankle pain after previous fracture
5 months after initial fracture
UNKNOWN CASE #4: SALTER-HARRIS FRACTURE
25%33% growth sequelae

Only 10% important
Follow for 2 years-X-rays
Look for bowing/shortening
Initial Salter-Harris IV fracture without good reduction

Subsequent lateral bowing of fibula and tibia
Epiphyseal plate irregular
CT/conventional tomography-osseous bar bridging plate
38 year old male with calf pain and mass, no history of trauma
T2
T1
1011
Nonspecific enhancing inflammation and edema in calf

with more focal mass medially
Biopsy-soft tissue osteosarcoma
3 weeks later early calcification on CT predominantly peripheral
4 weeks later thick rind of calcification peripherally
UNKNOWN CASE #5: MYOSITIS OSSIFICANS

(HETEROTOPIC BONE FORMATION)
No history of trauma 25%

Soft tissue mass
Subsequent calcification
Zonal phenomenon X-ray and path
Follow-up for maturation
14 year old female with enlarging right foot mass

CALCIFIED SOFT TISSUE MASS
Myositis ossificans
Gout, collagen vascular disease
Hyperparathyroidism, tumoral calcinosis
Hemangioma
Soft tissue chondro/osteosarcoma
Synovial sarcoma
Soft tissue mass plantar aspect mid to forefoot

Faint calcification-CT and mag views;
smooth erosion of 2nd and 3rd metatarsals
Large soft tissue mass on MRI mildly heterogeneous and
hyperintense T2W
UNKNOWN CASE #6: SYNOVIAL SARCOMA
2040 years old

68% lower extremity particularly knee
Most begin periarticular (< 10% intraarticular)
Biphasic epithelioid and spindle cell element on histo (also
monophasic )
Radiographs soft tissue mass, joint effusion (1020%),
calcification (30%), erosion or destruction adjacent bone
Metastases-lungs and lymph nodes
1012
Musculoskeletal Seminar III

Mark D. Murphey, MD
63 year old woman with vague calvarial pain

LYTIC SKULL LESIONS

Metastases
Myeloma
Paget disease
Brown tumor
Focal skull lytic lesions: frontal and parieto-occipital

Bone scan multifocal area increased activity
Radiographs multiple lesions trabecular
thickening
PAGET DISEASE
Common 3% of people over 40 years

Lytic, blastic or mixed phases
Most frequent to involve: spine, skull, pelvis
Trabecular thickening bone enlargement
PAGET DISEASE: COMPLICATIONS
Osseous deformity
Fractures
Neurologic symptoms
Arthropaty
Neoplasm
1013
14 year old boy with thigh pain
T1
T2
Cortical scalloping femur

Hair-on-end periosteal reaction
Broad based soft tissue mass
No medullary involvement
UNKNOWN CASE #2: PERIOSTEAL OSTEOSARCOMA
Most chondroblastic
85% diaphysis femur/tibia
Same age group as conventional osteosarcoma
Better prognosis
55 year old man with hip pain
T1
UNKNOWN CASE #3:

FINDINGS
Osteopenia of left hip

MRI decreased intensity left
proximal femur T1W, and
diffuse increased signal T2W
No focal defects on MRI; effusion
Enhances with gado; hot on bone scan
Returns to normal in several months
T2
1014
UNKNOWN CASE #3: TRANSIENT OSTEOPOROSIS HIP /

BONE MARROW EDEMA SYNDROME
Middle aged males

Spontaneous pain; worsened by weight-bearing
Symptoms regress 26 months
Migratory form may recur at nearby joint
Cause unknown-bone marrow edema
Relationship to AVN ?
82 year old man with slowly enlarging mass in the thigh
T2
T1

CALCIFIED SOFT TISSUE MASS
Myositis ossificans
Aneurysm
Lipoma/liposarcoma
Soft tissue osteosarcoma/chondrosarcoma
Synovial sarcoma
Large mass thigh with mineralization calcification/ossification

MRI/CT:
Fat component
Hemorrhagic component
Myxoid component
UNKNOWN CASE #4: MYXOID LIPOSARCOMA
Myxoid variety most common liposarcoma (4050%)

Intermediate grade
See lipomatous components with CT/MRI (4050%)
(We believe 90%-95% by MR)
Mineralization not rare in liposarcoma
1015
T1
GD
40 year old female with arthralgias
Interphalangeal joint subluxations

No erosions
Osteopenia

SUBLUXATIONS/NO EROSIONS
Systemic Lupus Erythematosis (SLE)

Mixed Connective Tissue Disease (MCTD)
Juvenile chronic arthritis
Ehlers-Danlos
Jaccoud arthropathy
UNKNOWN CASE #5: SYSTEMIC LUPUS ERYTHEMATOSIS
Musculoskeletal changes
Deforming nonerosive arthropathy
Tendon rupture
Joint and bone infection
Acrosclerosis
1016
37 year old female with progressive ankle deformity

What is the underlying disease?
What process involves the ankle subsequently?
How can the processes be correlated?
DIFFERENTIAL DIAGNOSIS BENIGN POLYOSTOTIC LESIONS

Enchondromatosis
Fibrous dysplasia
Hereditary multiple exostoses
Paget disease
Neurofibromatosis (Type 1)
Angiomatous lesions
UNKNOWN CASE #6: NEUROPATHIC JOINT
Cause pain sensation vs. neurovascular

Destruction, debris, density increase, disorganization
Diabetes, syphilis, cord-damage-syrinx
Lytic expansile benign appearing polyostotic lesions

Fibula, femur, metatarsal
Subsequently ankle-fragmentation, debris, destruction,
increased density
UNKNOWN CASE #6: FIBROUS DYSPLASIA
Endocrine Abnormalities
Sexual precocity
Cushings
Acromegaly
5 years after initial images
Hyperthyroidism
Diabetes mellitus (hypothalamic dysfunction)
Diagnosis case #6-fibrous dysplasia (polyostotic) with neuropathic ankle due to diabetes mellitus
1017
Musculoskeletal Seminar IV
Mark D. Murphey, MD
Several patients with various wrist subluxation patterns: Match with pattern
Lunate
Perilunate
Barton fracture/subluxation
1018
UNKNOWN CASE #1: WRIST SUBLUXATIONS
Perilunate 75%, usually with transscaphoid fracture rest of carpus-dorsal

Lunate 25% lunate rotated volar, capitate remains aligned to radius
UNKNOWN CASE #1: WRIST SUBLUXATIONS
Barton fracture of dorsal rim of radius with dislocation of carpus

Reverse Barton fracture of volar rim of radius with dislocation of carpus
31 year old female with underlying systemic disorder
Dysplastic changes thoracolumbar junction

Short segment scoliosis
Posterior vertebral body scalloping
1019

POSTERIOR VERTEBRAL SCALLOPING
Normal variant L45

Neurofibromatosis (Type I)
Tumor/increased intraspinal pressure
Achondroplasia
Acromegaly
Ehlers-Danlos, Marfan , Osteogenesis Imperfecta
Mucopolysaccharidosis

DYSPLASTIC THORACOLUMBAR JUNCTION
Neurofibromatosis (Type 1)
Cretinism
Idiopathic
Achondroplasia
UNKNOWN CASE #2: NEUROFIBROMATOSIS I

MUSCULOSKELETAL MANIFESTATIONS
Cranium-enlarged empty orbit, left lambdoid suture defect

Spine scoliosis, posterior vertebral scalloping, lateral meningocoeles
Pseudoarthrosis (tibia), bowing, fractures
Ribbon ribs
Neurofibroma 5% malignant degeneration
Localized gigantism
Multiple nonossifying fibromas
13 year old boy with bilateral hip pain
T1

IRREGULAR EPIPHYSES (MULTIPLE)
Normal variant
Hypothyroidism
Epiphyseal dysplasia
Trevor disease
Mucopolysaccharidosis
Multiple irregular epiphyses

Bilateral femora
Right humerus
Delayed skeletal maturation
Changes of slipped capital femoral epiphysis (SCFE)
1020
T2
UNKNOWN CASE #3: CAUSES OF SCFE
Idiopathic
Rickets-renal
Trauma, obesity
Hypothyroidism, hypoparathyroidism
Radiation
UNKNOWN CASE #3:

MUSCULOSKELETAL CHANGES IN HYPOTHYROIDISM
Delayed skeletal maturation, Wormian bones

Epiphyseal dysgenesis with osteoarthritis
Thoracolumbar junction gibbus
SCFE; ligamentous laxity
Osteoporosis; soft tissue calcification
Soft tissue edema, carpal tunnel syndrome
UNKNOWN CASE #3: EPIPHYSEAL DYSGENESIS
Ossification from multiple sites

Femoral, humeral centers and talus
Not due to vascular insufficiency
May disappear with treatment
May lead to premature osteoarthritis (OA)
45 year old man with wrist pain
UNKNOWN CASE #4:

DIFFERENTIAL DIAGNOSIS CHONDROCALCINOSIS
CPPD deposition/arthropathy
Hemochromatosis
Hyperparathyroidism
All others poor association
1021
Osteoarthritic changes unusual locations radiocarpal and MCP

joints (2nd through 5th)
Chondrocalcinosis TFFC, no scapholunate separation
Hook-like osteophytes metacarpal heads
UNKNOWN CASE #4: HEMOCHROMATOSIS MUSCULOSKELETAL CHANGES
Osteoporosis
Chondrocalcinosis (2060%)
Arthropathy (2450%) looks like osteoarthritis
Differences from CPPD arthropathy-involvement of 4th and 5th MCP
joints; hook-like osteophytes metacarpal heads; less scapholunate
separation; pericapitate narrowing
39 year old woman with low back pain
2 weeks prior to previous radiographs
1022
UNKNOWN CASE #5: DIFFERENTIAL DIAGNOSIS NARROW DISK SPACE
Degenerative disk disease (DDD); herniated disk (trauma)

Inflammatory arthritis
Scheuermann disease
Osteomyelitis
Neoplasm (very rare)
Amyloid chronic renal failure (CRF)
T1
T2
Rapid disk space narrowing L23 over two week interval

Subtle endplate destruction L23 level
T1W-MR: marrow replacement L23 with disk involvement
T2W-MR: increased marrow intensity L23 with disk involvement
UNKNOWN CASE #5: INFECTIOUS SPONDYLODISCITIS
Usually starts in anterior subchondral bone then spreads rapidly to disk

Bacterial vs. unusual cause (TB)
Drug abusers predisposed
65 year old man with right pain
1023

CHONDROID LESION
Benign
Enchondroma, bone infarct, chondroblastoma, chondromyxoid fibroma
(CMF), osteoblastoma
Malignant
Chondrosarcoma - intramedullary, juxtacortical, clear cell, mesenchymal,
myxoid, dedifferentiated, extraskeletal
T2
T1
Lytic lesion proximal femur some areas of surrounding sclerosis

Cortical permeation inferomedial on conventional tomography
Matrix on CT and tomography-chondroid
Soft tissue mass best seen on MRI
RADIOLOGIC DIFFERENTIATION OF
CHONDROSARCOMATOUS LESIONS
Aggressive chondroid lesion with soft tissue mass

High grade conventional chondrosarcoma
Mesenchymal chondrosarcoma
Large fluid component bone or soft tissue
Myxoid chondrosarcoma
Change in appearance or foci of more aggressive nature
Diagnosis: Dedifferentiated chondrosarcoma
1024
Musculoskeletal Seminar V
Mark D. Murphey, MD
60 year-old man with 6 months of knee pain

Biopsied and diagnosed as myeloma.
Is this a tenable diagnosis?
What is the correct diagnosis and why was the initial pathology incorrect?
T1
T2 Fat Sat
T1 GD
T2 FAT SAT
UNKNOWN CASE # 1: FINDINGS
Radiographs Geographic 1A lesion with channel/tract like component

inferiorly (subtle)
MRI Marrow replacement T1W
Rim enhancement (fluid filled mass)
Homogeneous very high signal T2W
Surrounding edema
Tract like component inferiorly
Differential diagnosis UBC, ABC (no expansion) intraosseous hematoma,
ablated lesion, Brodie abscess
1025
UNKNOWN CASE #1: SUBACUTE OSTEOMYELITIS BRODIE

ABSCESS
Described in 1832 - chronic/subacute

Walled-off with central fluid, often sterile (staph- only cultured in 50% of cases)
Children (M>F), metaphysis, tibia
Intramedullary; channel-like lucencies
May cross growth plate or be cortical
Periosteal reaction/sequestra may be seen
UNKNOWN CASE #1: BRODIE ABSCESS
Biopsied at margin in reactive tissue

Led to erroneous diagnosis
Myeloma (untreated) not a tenable diagnosis
asthis is a solid lesion
Importance of radiologic/pathologic correlation
59 year old veteran involved in mild MVA (first film) with progressive pain
(second set of films 2 weeks later and MRI)
First Radiograph
Second radiographs 3 weeks prior to

first radiograph
T2
T1
1026
Relatively rapid destruction of shoulder

Fragments in joint
Sharp surgical margin
MRI replacement of humeral head with high intensity on T2W
History of drained syrinx 40 years ago and cervical spine MRI shows severe
myelomalacia
UNKNOWN CASE # 2: NEUROPATHIC SHOULDER-SYRINX
Cause pain sensation vs. neurovascular

Radiologic- destruction, debris, density increase, disorganization
Diabetes, syphilis, cord damage - syrinx
UNKNOWN CASE # 3: HISTORY
15 year-old boy with hip pain
T1
STIR
Marrow replacement right femoral neck T1W

medial transcervical region
High signal on STIR
surrounding edema periosteum/ST
horizontal low signal linear band medially
Subsequent near total resolution
STRESS FRACTURE: FEMUR
Medial femoral neck - fatigue type

heal with symptomatic treatment (3 to 12 months)
crescentic MR abnormality above lesser trochanter
Lateral femoral neck - insufficiency type
possible cause most subcapital fractures
DO NOT HEAL: COMPLETE/DISPLACED
Garden staging (< Grade 2 percutaneous pins) (> Grade 2 THA due to
development of AVN)
Usually horizontal/oblique rarely longitudinal
thigh splints (stress reaction)
1027
23 year-old man with knee pain, masses and lesions on radiographs

Diagnosis of bone lesions
Underlying condition
T1
T1
T2
T2 WITH FAT SAT
1028
NEUROFIBROMATOSIS 1: SKELETAL MANIFESTATIONS
Mesodermal dysplasia
Kyphoscoliosis
Facial, orbital, lambdoid suture (left) defects
Multiple nonossifying fibromas
Meningocele
Posterior vertebral scalloping
Rib deformity (ribbon ribs)
Congenital pseudarthrosis (tibia)
Focal hypertrophy (gigantism)
Localized neurofibroma - most common
least characteristic, often deep/multiple
superficial lesions (fibroma molluscum)
Plexiform neurofibroma - pathognomonic
early childhood
precedes cutaneous neurofibromas
53 year-old man with lateral knee mass and pain
T1
STIR
STIR
1029
PROTON DENSITY
STIR
Tibiofibular/lateral tibiofemoral joint osteoarthritis

High fluid content multilocular mass laterally
multilocular / surrounding edema
appears to arise from tibiofibular joint
components in bone (tibia and femur) and soft tissue
D/DX -- Ganglion/Synovial cyst, myxoid tumor
1030
GANGLION/SYNOVIAL CYST
Etiology unknown- neoplasm, trauma, inflammatory

Young adults-most common mass hand/wrist
Pain-may affect adjacent nerves
Location
ST: Hand, foot, knee, hip, shoulder
Intraosseous: medial malleolus, wrist, knee
BOTH
Thick walled unilocular/multilocular
high protein content affects CT/T1W MR
walls/septa may enhance
CT/MRI/Sono - cystic mass
may rupture cause surrounding edema
47 year-old woman with mid to low back pain

Most likely diagnosis?
Two other possible diagnoses?
T1
T2
T2
STIR
1031
Multifocal bone scan areas increased radionuclide activity

spine, SC joints, SI joints
CT-multifocal sclerosis
anterior/posterior paralleling endplates
erosions/bone production costovertebral joints
cause of hot bone scan
MRI - multifocal areas marrow abnormality
low T1W, high T2W/STIR
anterior/posterior paralleling endplates
no soft tissue mass
Radiographs- subtle sclerosis, sacroiliitis
D/DX - Metastases, myeloma, lymphoma
1032
ANKYLOSING SPONDYLITIS: CLINICAL CHARACTERISTICS
Peak age of onset 15-35 years

M:F 3-5:1
Incidence 6.6/100,000
HLA-B27 > 90%
Rare in blacks
Predilection axial involvement
ANKYLOSING SPONDYLITIS: DISTRIBUTION
Osseous ankylosis
Ligament/Tendon ossification
Spine/SI joints
symmetric
Pelvis - symphysis, ischium, iliac, hips
Peripheral changes unusual early (10% - 50%)
asymmetric
ANKYLOSING SPONDYLITIS: SPINE CHANGES
Osteitis -shining corners

Squared vertebral bodies
Syndesmophytes
Bamboo spine
Calcified disc, fused facets/ligaments
Pseudarthrosis/fractures
Atlantoaxial disease/Cauda equina
Other diagnoses- Reiter/Psoriatic, SAPHO
1033
Radiologic Pathology 2006-2007 - Volume 2 - Index

25-OH Vitamin D 902
Abscess 796
Soft Tissue Infection 827
Subperiosteal 823
Access. Navicular 880
Accessory Muscles Masses (Ankle and Foot) 886
Accessory Soleus 886
Acetabulae protrusio 816
Acetabular
Columns Letournel and Judet 998
Injury 998
Trauma 995
Acetabulum 998
Achilles Tendon 883
Achondroplasia 1020
ACL 867
Post-operative 868
Tear 866, 867
Acromegaly 773
Acromial Variation 927
Acromio-clavicular Joint Injuries 941
Acromion 942
Acroosteolysis 983, 1007, 1008
Actinomyces 833
Actinomycosis 829, 833
Active infection 825
Acute Osteomyelitis 821
Adamantinoma 721, 771, 776
Adductor pollicis 947
Adenomatoid odontogenic tumor 855
Adhesive Capsulitis 931
Adolescent Stress Lesions 994
Adult Palmar Fibromatosis 779
Adult Plantar Fibromatosis 780
Aggressive Malignant Osteoblastoma 750
Aggressive infantile Fibromatosis 778
Aggressive Osteoblastoma 750
Alcoholism 907, 953
Alkaline phosphatase 812
Alkaptonuria 949, 953
Allograft 707
ALPSA Lesion 934
Aluminum Toxicity 716
Ameloblastic fibroma 852
Ameloblastic fibro-odontoma 857
Ameloblastoma 852, 854
Amyloid 795
Amyloidosis 716, 1006
B2 microglobulin 716
Carpal tunnel syndrome 716
Destructive spondyloarthropathy 716
Discovertebral erosions 717
Anatomy of the tarsal joints 1003
Aneurysm 804
Aneurysmal Bone Cyst 749, 784, 787
Angioblastoma 776
Angiomatoid 780
Angiomatosis 805, 808
Angiomatous lesions 1017

Angiosarcoma 721, 805, 810
Ankle 879, 995
Fractures 1001
Ankylosing Spondylitis 912, 916, 918, 1033
Ankylosis 914
Anterior Dislocation (Glenohumeral) 942
Anterior Drawer 867
Anterior Instability (Glenohumeral) 933
Anterior talofibular 881
Anterior tibiofibular ligament 880
Anterolisthesis 840
Anteroposterior (AP) compression (Pelvic Trauma) 996
Arthritis 795, 1006
Juvenile Chronic 919
Psoriatic 916
Septic 825
Tuberculous 832
Articular cartilage 859
Aseptic necrosis 906
Aspergillosis 835
Aspirin/nonsteroidals (Osteoma) 746
Atlantoaxial subluxation 915
Atlas Fractures 843
Atypical Mycobacterium 833
Autograft Healing 706
Avascular Necrosis 719, 959, 1010, 1020
Aviators astragulus 1002
Avulsion 865
Avulsive cortical injury 777
Axial Compression Injury 846
Axial Osteomalacia 904
Axillary Nerve Neuropraxy 935
Axillary View 942
Bacillary angiomatosis 837
Ballooned epiphyses 920
Bamboo spine 918
Banana fracture 816
Bankart Lesion 932
Bankart Repair 938
Barton Fracture (Reverse) 945
Baseball Finger 948
Basilar invagination 816, 901
Bathrocephaly 1008
Batsons plexus 961
Benign Bone Tumors: Age Distribution by Decade 723
Benign Fibrosis Histiocytoma 773
Benign Polyostotic Lesions 1017
Bennett Fracture 947
Biceps Injury 957
Partial Tears 957
Tendinosis 957
Biceps Femoris 869
Biceps Tendon 929
Bicipital Radialis Bursitis 957
Bilateral Facet Dislocation 842
Birbeck bodies (Eosinophilic Granuloma) 890
Blade of grass 813
I1
Blastic Disease 814

Blastic Lesions 967
Blastic Phase 812
Blastomycosis 835
Blount 911
Blow out lesions 967
BMD 978
BMD (WHO Classification) 979
Bohler 1003
Bone Autograft 706, 707, 710
Bone bruise 865, 879, 958
Bone enlargement 814
Bone Graft Complications 708
Fracture 709
Joint Instability 709
Nonunion and Pseudarthrosis 708
Resorption 709
Bone Graft Substitutes 710
Bone Infarct 769, 906, 1024
Osteonecrosis 769
Bone Island 743
Bone Metastases 961
Bone Production 914
Bone scan 744
Bone Tumors 720
Cartilage 720
Histiocytic 721
Marrow 720
Notochord 721
Osteoid 720
Unknown Origin 721
Vascular 721
Bone Tumors (Incidence) 722
Important Factors in the Diagnosis of 723
Primary Benign 722
Primary Malignant 722
Bone within Bone 970
Botryoid odontogenic cyst 852
Bouchard nodes 923
Bowing of long bones 901
Boxers Fracture 948
Brachial artery 944
Brachial nerve injury 944
Breast Carcinoma 962
Bristow procedure 938
Brodie abscess 748, 824, 1007, 1026
Brown tumor 711, 853, 1013
Brucella 829
Brucellosis 829
Bucket handle 861, 862
Bumpy (Soft Tissue Swelling) 912
Bursae (Knee) 870
Bursitis (Bicipital Radialis) 957
Bursitis (septic) 826
Burst Fracture 846
Button osteophyte 922
Cafe-au-lait spots 773, 981
Calcaneal Fractures 1002
Calcaneofibular 881
Calcaneus 995
Calcific
Myelitis 792
Tendinitis 952
Tendonitis 930
Tendonitis (Glenohumeral) 943
Calcified falx cerebri 853
Calcified Soft Tissue Mass 1012, 1015
Calcinosis 984
Calcitonin 818
Calcium deficiency 977
Calcium hydroxyapatite 718, 792, 949
Cancellous (Osteoid Osteoma) 746
Candidiasis 835
Candle flame 813
Cap thickness 758
Capillary hemangioma 809
Capitate (Dislocation) 946
Capitellum 955
Capsulorapphy 938
Carcinomatosis 971
Carpal Dislocations 946
Carpal Stability 874
Carpal Tunnel 872, 876
Syndrome 877
Cartilage metaplasia 799
Cartilage nodules 799
Cartilaginous Lesions 757
Chondroblastoma 757
Chondromyxoid fibroma 757
Chondrosarcoma 757
Enchondroma 757
Juxtacortical chondroma 757
Osteochondroma 757
Caseating necrosis 830
Cavernous hemangiomas 761
Cavernous spaces 806
Cellulitis 826
Cementoblastoma 854
Central giant cell granuloma 852
Cervical Burst Fracture 846
Cervical Spine Trauma 839
Cervical spondylosis 848
Charcot joint 953
Chondroblastoma 720, 1008, 1024
Codman Tumor 763
Chondroblasts 764
Chondrocalcinosis 950, 1021
Chondroid 738
Chondroid Lesion 1024
Chondroid Matrix (Enchondroma) 761
Chondroid Matrix (Intramedullary Chondrosarcoma) 765
Chondromyxoid Fibroma 720, 1024
Chondrosarcoma 762, 764, 1024
Dedifferentiated 769
Extraskeletal 768
Mesenchymal 768
Myxoid 768
Chondrosarcomatous Lesions 1024
Chordoma 721, 786
Christmas disease 972
Chronic ACL Tear 867
Chronic Granulomatous Disease of Childhood 827
I2
Chronic hematoma 796

Chronic infection 818
Chronic Osteomyelitis 825, 852
Chronic Recurrent Multifocal Osteomyelitis (CRMO) 827
Chronic regional pain syndrome 976
Chronic renal failure 793
Chronic sclerosing osteomyelitis 856
Chronic symmetric plasma cell osteomyelitis 827
Clavicle
Clay Shoveler Fracture 843
Clear Cell Chondrocytes 767
Clear Cell Chondrosarcoma 763, 764, 767, 1008
Clear Cell Sarcoma 792, 803
Cleidocranial dysplasia 1007
Clutton joints 834
Coachs Finger 948
Coccidioidomycosis 835
Codman Tumor 763
Collagen Vascular Diseases 793, 907, 1012
Collagen vascular-like diseases 976
Collateral ligament 869
Collateral ligaments 859
Colles fracture 945
Column Fracture (Pelvis) 1000
Complete pelvic instability 997
Compression Fracture 841
Congenital insensitivity to pain 953
Congenital Syphilis 834
Contiguous spread 820
Contusion 865
Cooleys anemia 970
Coracoid 942
Coronoid fossa 955
Cortical
Osteoid Osteoma 746
Desmoid 777
Fibrous Dysplasia 775
Involvement 738, 739
Lucency/Central Calcification 1005
Resorption 712
Thickening (Chondrosarcoma) 765
Cotton wool 814
CPPD 718, 949
Arthropathy 792
Deposition/arthropathy 1021
Cranial Sclerosis 744
Craniotabes 901
CREST Syndrome 984
Cretinism 1007, 1020
Crohn's disease 912
Cruciate ligaments 859
Cryptococcosis 836
Crystal deposition (Thalassemia) 971
Crystal Deposition Disease 718, 949
Cushing syndrome 773
Cyst (Paralabral) 938
Cystic hygroma 808
Cystic Masses (Knee) 870
Cysticercosis 837
Dactylitis 830, 834, 969, 1010
Dagger sign 918
Danis-Weber Classification 1001
Decubitus ulcers 825

Dedifferentiated Chondrosarcoma 764, 769
Deep Endosteal Scalloping 765
Deforming nonerosive arthropathy 983
Degenerative Joint Disease 951
Dental Anatomy 849
Dentigerous cyst 852
Dentition 849
Deoxyhemoglobin 848
DeQuervains Syndrome 876
Dermatofibrosarcoma Protuberans (DFSP) 771, 781, 782, 802
Dermatofibrosis lenticularis disseminata 744
Dermatomyositis 976
DeSmet 860
Desmoid 746
Extraabdominal 779
Desmoplastic fibroma 771, 777
DEXA 978
Diabetes 825
Insipidus 892
Mellitus 773, 825, 953
Diffuse sclerosis 1010
Diphoshonates 818
Direct implantation 820
Discitis 828
Discography 828
Discoid 860
DISI deformity 874
Distal radial buckle fracture 945
Distal radioulnar joint 872
Distal Tuft Fracture 948
Disuse/Immobilization Osteoporosis 977
Double Axillary Pouch Sign 934
Double line sign 908
Doughnut sign 785
Drug Abusers 827
Du Toit & Roux 938
Dual Energy X-ray Absorptiometry (DEXA) 978
Dupuytren Exostosis 759
Durie/Salmon PLUS Staging (Myeloma) 965
Dysbaric disorders 907
Dysplasia Epiphysealis Hemimelica: Trevor Disease 759
Dysplastic Thoracolumbar Junction 1020
Dysprosium 165 795
Early focal cemento-osseous dysplasia 850
Echinococcus 837
ECU tendon sheath 875
Ehlers-Danlos 1016
Elbow 955
Dislocations and Fractures 944
Embolization 748, 808
Enchondroma 720, 760, 769, 1024
Enchondroma vs. Chondrosarcoma 769
Enchondromatosis 1017
Endocrinopathies 773
Endodontic procedures 854
Endosteal scalloping 770
Enostosis 720, 743
Enteropathic arthritis 912
Enthesopathy 914
Enucleation 852
I3
Eosinophilic Granuloma 721, 887, 890, 967

LCH 1008
Epicondylitis (Elbow) 956
Epidermoid Carcinoma 826, 834
Epidermoid Inclusion Cyst 784, 790
Epidural hematoma 848
Epiphyseal Lesions 1008
Dysgenesis 1021
Dysplasia 1020
Epiphysis 767
Epithelial nests 776
Epitrochlear Lymph Node 960
Erlenmeyer flask deformity 971
Erosions 913
Erosive Osteoarthritis 917
Erupting teeth 854
Essex-Lopresti 945, 1003
Ewing Sarcoma 721, 887, 964, 967
Ewing Sarcoma (Intergroup Study) 888
Exophthalmos 892
Exostoses 856
Exostosis (Subungual - Dupuytren Exostosis) 759
Extensor Carpi Ulnaris Sheath 875
Extensor tendons 859
Extent of Musculoskeletal Neoplasm 738
Extra-abdominal Desmoid Fibromatosis 778
Extra-articular erosions (Gouty Arthritis) 950
Extramedullary Hematopoiesis 971
Extraskeletal Chondrosarcoma 764, 768
Facet 840
Fallen fragment sign 787
Familial vitamin D res rickets 903
Fanconi syndromes 903, 968
Fanconis Anemia 972
Felon 825
Felty Syndrome 914
Femur 995
Fibrocartilage Calcification 951
Fibroma molluscum 1029
Fibromas 746
Fibromatosis 771, 778
Fibromatosis: Types 778
Fibrosarcoma 771, 780, 781, 802
Fibrosarcoma/MFH 748
Fibrosis Histiocytoma 773
Fibrous cortical defect 771
Fibrous dysplasia 771, 773, 856, 944, 1017
Fibrous Histiocytoma (Malignant) 721
Fibrous medullary defect 771
Fibroxanthoma (Nonossifying fibroma) 771
Fibular (lateral) 869
Fibular collateral lig complex 881
Fibular collateral ligament 867
Filariasis 837
Finger (Trauma) 948
First MC (Fracture) 947
Flexion Teardrop Fracture 843
Flexor Digitorum (Avulsion) 948
Flipped (meniscal tear) 862
Florid cemento-osseous dysplasia 856
Fluid - fluid level 764, 785, 802
Fluorosis 818
Focal cemento-osseous dysplasia 855, 857

Focal scerosing osteomyelitis 854
Foot 879, 995
Injuries 1003
Fracture (First MC) 947
Fractures 816
Fractures (Pathologic) 961
Frieburg 911
Full thickness (Rotator Cuff Tear) 928
Galeazzi 945
Ganglia 870
Ganglia (Knee) 870
Ganglion 792
Ganglion/Synovial Cyst 1031
Ganglion/synovial cyst/bursa 796
Gardner Syndrome 852, 856
Gardner Syndrome (Osteoma) 746
Gastrocnemius 869
Gastrocnemius/ Semimembranosus 870
Gaucher disease 818, 907, 971
GCT 769
Geodes 790
Geographic Contusion (Knee) 866
Geographic Pattern (Bone Tumors) 725
Geographic 1A: Differential Diagnosis 725
Geographic IB: Differential Diagnosis 726
Geographic IC: Differential Diagnosis 726
Giant Bone Island 743
Giant Cell (Reparative) Granuloma 780
Giant Cell Tumor 721, 784, 1008
Giant Cell Tumor Tendon Sheath (GCT-TS) 794
Giant Cells 784
Gigantism 1029
Glad Lesion 936
Glenohumeral 932
Instability 932
Joint 932
Ligaments 932
Glenohumeral Internal Rotation Deficit (GIRD) 936
Glenohumeral Labroligamentous complex 932
Glenohumeral ligament (avulsion) 935
Glenoid labrum 932
Glomus 721
Glomus Tumor 805, 808
Gnathic Osteosarcoma 754
Golfers elbow 956
Goltz syndrome 744, 784
Gorham 809
Gorlin syndrome 853
Gout 718, 817, 949, 1010, 1012
Gouty arthritis 949, 950
Gouty tophus 804
Gracilis 869
Granulomatous Disease of Childhood 827
Grashey view 942
Greater Tuberosity Fracture 935
Ground glass 774
Group B strep 822
Guinea worm (dracunculosis) 837
Gumma 834
Guyons canal 872, 877
HA Crystal Deposition Disease 952
I4
Hagl Lesion 935

Hajdu-Cheney Syndrome 1008
Hallux 924
rigidus 924
valgus 924
Hamartoma 743
Hamate Fracture 947
Hand-Foot syndrome 969
Hand-Schller-Christian disease 887, 892
Hangee Fracture 845
Hangman Fracture 845
Hanover 1003
Hawkins classification of talar neck fractures 1002
Hawkins sign 1002
Heberden nodes 923
Hemangioendothelioma (HE) 721, 805, 810
Hemangioma 721, 804, 805, 1012
Arteriovenous 805
AV malformation 852
Capillary 805, 809
Cavernous 805
Venous 805
Hemangiopericytoma (HPC) 721, 768, 805, 810
Hematogenous Osteomyelitis: Adult 824
Hematogenous Osteomyelitis: Child 822
Hematogenous Osteomyelitis: Infant 822
Hematogenous Vascular Supply 820
Hematologic Disease 968
Hemochromatosis 949, 950, 952, 1021
Hemodialysis elbow 718
Hemoglobinopathy 907
Hemophilia 968, 972
Hemophiliac pseudotumor 973
Hemorrhage 781, 790
Hereditary Hyperphosphatasia: Juvenile Paget Disease 818
Hereditary multiple exostoses (HME) 757, 760, 1017
Heterotopic Bone Formation: Myositis Ossificans 800
High - Grade Surface 754
High output congestive failure 812
Hill-Sachs Lesion 934, 943
Hip
Fractures 1000
Joint instability 999
Trauma 1000
Histoplasmosis 836
HLA B27 916
Hodgkin 966, 971
Hook of hamate 877
Hoop stresses 860
H-Shaped Vertebral Bodies 970
H-type vertebrae 1010
Human/animal bites 825
Humeral Fractures 943
Humphrey 859
Hutchinson triad 834
Hyaline cartilage cap 757, 758
Hyaluronic acid 795
Hydrops Fetalis 808
Hydroxyapatite 715
Hydroxyapatite Crystal Deposition 952
Hydroxyproline 812
Hygroma 808
Hypercementosis 854
Hyperextension 840
Hyperextension Dislocation 844
Hyperextension Injuries 843
Hyperextension: Teardrop Fracture 845
Hyperflexion 840
Hyperflexion Injuries 840
Hyperflexion Sprain 840
Hyperparathyroidism 711, 773, 852, 856, 950, 971, 1007,
1012, 1021
Hyperthyroidism 773
Hypertrophic Osteoarthropathy 962
Hypophosphatasia 904, 1007
Hypothyroidism 1020, 1021
Idiopatic osteosclerosis 854
Ifosfamide 903
Iliotibial band 867
Iliotibial tract 869
Imaging for Staging Musculoskeletal Neoplasm 738
Immature cementoblastoma 850
Immature periapical cemental dysplasia 850
Impingement Syndrome 927
anterolateral 881
Infantile dermal/digital fibromatosis 778
Infantile myofibromatosis 778
Infarction 969
Infection 1006
Infectious Spondylodiscitis 1023
Inferior Glenohumeral Labroligamentous complex 932
Inferior Glenohumeral Ligament 933
Inflammatory 780
Infrapatellar 870
Infrapatellar cleft 870
Insufficiency fractures 979, 983
Interbody Fusion 710
Intermedius 869
Interosseous ligament 880
Interosseous syndrome (Elbow) 956
Interspinous widening 840
Intertrochanteric Fractures 980
Intraarticular Bodies (Elbow) 956
Intra-Articular Hydroxyapatite Crystal Deposition Disease
952
Intracortical fibrous dysplasia 775
Intramedullary (Chondrosarcoma) 764
Intramedullary Extent 738
Intramedullary Hemorrhage 848
Intranuclear inclusions 812
Intraosseous ganglion 784, 790, 1008
Intrasubstance Tear (Rotator Cuff) 929
Invisible Margin (Bone Tumors) 728
Involucrum 820
Irradiation 907
Irregular Epiphyses (multiple) 1020
Isolated Fractures Radius: Galeazzi 945
Isolated Fractures Ulna: Monteggia 945
Isolated Tendon Injuries 948
ITB Friction Syndrome 869
Ivory vertebra 814, 962
Jaccoud arthropathy 1016
Jaffe-Campanacci syndrome 773
I5
Jaws 849
Jefferson Fracture 846
Jersey Finger 948
Joint Arthroplasty (Complications of ) 699
Dislocation / Abnormal Alignment 702
Fractures and Nonunion 702
Heterotopic Bone Formation 699, 703
Loosening and/or infection 699
Radionuclide Evaluation 700
Small Particle Disease 699, 701, 702
Joint involvement 738, 739
Abscess 741
Bursitis 741
Diabetic muscle ischemia 741
Fibromatosis 741
Gadolinium 742
Hematoma 741
intraarticular resection 739
Lymphocele 741
Muscle flap 741
Myositis ossificans 741
Myxoid liposarcoma 741
Post-Operative Imaging (Bone Neoplasm) 741
Radiation necrosis 741
Reactive lymph node 741
Seroma 741
Soft Tissue Mass - Benign 740
Soft Tissue Mass - Malignant 740
Subtraction MRI 742
Synovial cell sarcoma 741
Joint Replacement 699
Jones Fracture 1003
JRA 919
Polyarticular 920
Still Disease: Pauci or Monoarticular 920
Jumpers Knee 870
Juvenile aponeurotic fibroma 778
Juvenile Chronic Arthritis 912, 919, 973, 1016
Juvenile Paget Disease 818
Juvenile-onset adult type RA 920
Juvenile-onset ankylosing spondylitis 919
Juxtaarticular Osteoporosis 912
Juxtacortical Chondroma 720, 762, 767
Juxtacortical Chondrosarcoma 767
Juxtacortical Osteosarcoma 752
Kaplan 860
Kaposi sarcoma 837
Kasabach - Merritt 809
Keloid formation 744
Keratocyst 852
Kienbck 911
Kienbcks Disease 911
Klippel-Trenaunay-Weber 809
Knee 995
Knee Injury 1001
Knee Stabilizers 867
Kehler 911
Kohler Disease 1006
Kyphosis 830, 840
Labral Repair 938
Labrum 932
Lamina dura 850
Laminar Fractures 843

Langerhans Cell Histiocytosis (LCH) 748, 887, 890, 1013,
1008, 1017
Laser therapy 808
Lateral compression (Pelvis Trauma) 996
Lateral Epicondylitis (Elbow) 957
Lateral inferior geniculate artery 863
Lateral Meniscus 859
Lateral Process of Talus Fractures 1002
Lateral Stabilizers 869
Lateral Tendons (Ankle and Foot) 884
Lateral Ulnar Collateral Ligament 959
Lauge-Hansen 1001
LCL-Biceps Femoris 870
Ledderhose disease 780
Legg-Calv-Perthes 911
Leiomyosarcoma 781, 802
Leprosy 829, 833, 953
Lesion matrix 738
Letournel and Judet classification 998
Letterer-Siwe disease 887, 892
Leukemia 971
Ligament and Tendon Involvement 739
ligament(ous) injury 840
Limb Salvage Procedures 737
Lipoblastoma 896
Lipoma 720, 804, 1015
Lipoma Arborescens 798
Lipoma Intramuscular 895
Lipomatosis 897
Liposarcoma 720, 781, 802, 893, 897, 1015
Atypical 898
Dedifferentiated 898
Higher Grade Lesions 898
Myxoid Lesions 898
Pleomorphic Liposarcoma 899
Well-Differentiated 897
Loa loa 837
Location in Bone: Axial (Bone Tumors) 724
Locked facets 842
Long Head of Biceps Tendon 929
Long Head of the Biceps Tendon 932
Longitudinal Tibial Stress Fracture 992
Loose Bodies 871
Looser zones (Osteomalacia) 715
Loosers zones 902
Lower Extremity Trauma 995
Low-Grade Chondroid Lesion 769
Low-Grade chondrosarcoma 769
Lumpy 912
Lunate 946
Lung Cancer 963
Lunotriquetral Instability 875
Lyme disease 834, 835
Lymphangioma 721, 805
Lymphoma 720, 748, 961, 964
Lymphoma of Bone (Primary Lymphocytic) 966
Lytic Patterns (Bone Tumors) 728
lytic phase 812
Lytic Phase 813
Lytic Skull Lesions 1013
Macho-Macho 852
I6
Madura Foot 837

Maffucci syndrome 760, 761, 809
Magic Angle Phenomenon 876
Magnuson Stack 938
Malignant Bone Tumors: Age Distribution by Decade 723
Malignant Fibrous Histiocytoma (MFH) 721, 771, 780, 781
Malignant melanoma of soft parts 803
Malignant myositis 801
Malignant transformation (Multiple Enchondromatosis) 761
Mallet Finger 948
Malnutrition 977
Marrow Edema (Osteoid Osteoma) 747
Marrow hyperplasia 969
Massive Osteolysis of Gorham 809
Mastocytosis 818
Matrix Formation 723
Matrix Formation (Bone Tumors) 729
Mazabraud syndrome 773
McCune Albright syndrome 773
Medial Collateral Ligament (MCL) 867
Injuries 868
Medial Collateral Ligament (Elbow) 959
Medial Meniscus 859
Medial migration (Osteoarthritis: Hip) 923
Medial Tendons (Ankle and Foot) 884
Median nerve 872, 877
Impingement 956
Melorheostosis 744
Meningoceles 983
Meningomyelocele 953
Meniscal Cyst 798, 870
Meniscal Flap 862
Meniscal tears 798, 858, 861
Menisci (Calcification) 951
Menisci (Post-surgical) 863
Meniscofemoral ligaments 863
Meniscus homologue 875
Mesenchymal cells 768
Mesenchymal Chondrosarcoma 764, 768
Metabolic Bone Disease 900
Metachronous Osteosarcoma 750
Metaphyseal chondrodysplasia 904
Metastases 748, 768, 1013
Skeletal 961
Metastatic lymphoma 967
Methemoglobin 848
MFH (Malignant Fibrous Histiocytoma) 802
MFH/ fibrosarcoma 769
Middle Glenohumeral Ligament 933
Milk - Alkali syndrome 793
Milwaukee Shoulder 952
Mithramycin 818
Mixed Connective Tissue Disease (MCTD) 1016
Mixed/Blastic Disease 814
Monostotic 773, 812
Monteggia 945
Mortons Neuroma 885
Motheaten (Bone Tumors) - Differential Diagnosis 727
Mucoepidermoid carcinoma 852
Mucopolysaccharidosis 1020
Mucormycosis 836
Multidirectional Instability (Glenohumeral) 939
Multiple enchondromatosis 760, 761

Multiple Myeloma 964
Multiple myeloma with sclerosis or POEMS syndrome 965
Multiple tori and exostoses 856
Musculoskeletal Infection 820
Musculoskeletal Neoplasm - Extent 738
Musculoskeletal Neoplasm - Staging - Surgical Implications
737
Musculoskeletal Tumors - Staging 734
Histologically Benign 735
Histologically Malignant 736
Mycetoma 837
Mycobacteria 829
Mycobacterium 833
Mycobacterium Leprae 833
Myelitis (Calcic) 792
Myelofibrosis 818, 968, 971, 974
Myelography 840
Myeloma 720, 961, 964, 1013
Myeloma/plasmacytoma 786
Myelomalacia 848
Myeloproliferative diseases 949
Myositis 837, 983
Myositis Ossificans 792, 800, 1012
Myotendinous (Rotator Cuff Tear) 928
Myotendinous Tear of Pectoralis 931
Myxoid 780
Myxoid Chondrosarcoma 764, 768
Myxoma 773, 792
Myxomatous neoplasms 796
Narrow Disk Space 1023
Nasopalatine duct cyst 850
Navicular 880
Neck of the scapula 942
Necrosis - Avascular 719
Neoplasm (Paget Disease) 817
Nerve Impingement (Elbow) 956
Nerve root avulsion 840
Neuroarthropathy 953
Neuroblastoma 963, 964, 967
Neurofibromatosis (NF) 976, 981
Neurofibromatosis (Type 1) 773, 1017, 1020, 1029
Neurogenic tumor 786
Neuropathic 834
Joint 1017
Osteoarthropathy 949, 953
Shoulder-Syrinx 1027
Neurovascular involvement 738, 739
Nevoid basal cell carcinoma syndrome 853
NF (Neurofibromatosis) 981
NF-1 (vonRecklinghausens) 981
NF-2 - Acoustic neuromas 981
Nidus (Osteoid Osteoma) 746
Night Stick Fracture 944
Nocardia 833
Nodular Fasciitis 792, 801
Non-Hodgkin 966
Non-Insertional Achilles Tendon Pathology 883
Nonossifying fibroma (NOF) 771, 981
Nonosteogenic fibroma 771
Nonspecific spindle cell sarcoma 781, 802
ODonoghues Triad 867
I7
Oblique meniscomeniscal ligament 863

Occult fracture 865
OCD 955
Odontogenic cyst 852
Odontogenic keratocyst 852
Odontogenic myxoma 852
Odontoid Fracture 847
Odontoma 855, 857
OI (Osteogenesis Imperfecta) 980
Olecranon bursitis 949, 958
Olecranon fossa 955
Ollier disease 760
Ollier Syndrome 762
Oncogenic osteomalacia 773
Os Acromiale 928, 942
Os odontodeum 847
Os Trigonum 880
Osgood-Schlatter 911
Osler-Weber-Rendu 809
Osseous bowing 812
Osseous deformity 816
Osseous Lesions both Sides of Joint 1006
Osseous Neoplasm 733
Ossicle 860
Ossifying fibroma 775, 855, 857
Osteitis deformans 812
Osteoarthritis 920
Osteoarthritis (secondary) 826
Osteoarthropathy (Hypertrophic) 962
Osteoarthropathy (Neuropathic) 949, 953
Osteoblastic Metastasis 743
Osteoblastoma 720, 743, 748, 763, 855, 857, 1008, 1024
Osteochondral fracture 879
Osteochondral Lesion 879
Osteochondritis 834
Osteochondritis Dissecans 799, 865, 879, 910
Osteochondroma 720, 757
Osteochondroses 911, 1006
Osteochondrosis 906
Osteoclast 962
Osteofibrous dysplasia 771, 775, 776
Osteogenesis Imperfecta (OI) 976, 980, 1007
Osteoid 738
Osteoid Osteoma 720, 743, 746, 748, 763, 1005, 1008
Osteolysis (Post-Traumatic - Clavicle) 941
Osteolysis of Gorham 809
Osteoma 720, 743, 745, 857
Osteomalacia 711, 714, 809, 900
Osteomyelitis 718, 748, 821, 824, 825, 827, 830, 856, 967,
1026
Osteomyelitis (salmonella) 1010
Osteonecrosis 769, 906, 999
Osteonecrosis (Spontaneous) 910
Osteopathia striata 744
Osteopenia 714, 900, 979, 1010
Osteopetrosis 856
Osteophyte (button) 922
Osteophyte formation 921
Osteopoikilosis 744
Osteoporosis 711, 900, 976, 979, 1008
Osteoporosis (transient) 909
Osteoporosis circumscripta 813
Osteosarcoma 743, 750, 751, 769, 857, 1012

Intramedullary 751
Juxtacortical 752
Parosteal 753
Telangiectatic 752
Osteosarcoma (Sclerosing) 754
Osteosarcoma: Low Grade Intramedullary 754
Osteosarcoma: Soft Tissue 755
Extraskeletal 755
Osteosarcoma : Intracortical 756
Osteosarcomatosis 755
Osteosclerosis 711, 714
Overhanging edge 914
Overhanging edge (Gouty Arthritis) 950
Oxalosis 718
Oxyhemoglobin 848
P V N S 793
Paget Disease 812, 1013, 1017
Pagets disease 856
Pain (Congenital Insensitivity) 953
Palmar Fibromatosis 779
Palmer 1003
Pancreatitis 907
Panne 911
Panners disease 959
Paralabral Cyst 937
paramyxovirus (measles) 812
Parathormone (PTH) 711
Paravertebral soft tissue 828
Paronychia 825
Parosteal Osteosarcoma 753
Parrot Beak 862
Parsonage Turner Syndrome 958
Partial thickness (Rotator Cuff Tear) 928
Patellar Dislocation 866
Patellar Retinacula 867, 869
Patellar Tendinitis 870
Patellar tendon 869
Patellofemoral Joint 870
Patellofemoral Syndrome 870
Pathologic Fractures 961
Pattern of Bone Destruction and Lesion Margin 725
Geographic 725
Motheaten 725
Permeative 725
Transition Zone 725
PCL 867, 868
PCL Tear 868
Pectoralis Major Tear 931
Pedicle erosion 981
Pedicle sclerosis 749
Pedicolaminar Fracture-Separation 844
Pelvic Ring Disruption 998
Pelvic Ring Fractures 995
Pelvic Stress Injury 993
Pelvis Trauma 995
Pencilling (Long Bones) 981
perched facets 842
Percutaneous ablation (radiofrequency) 748
Percutaneous removal (Osteoid Osteoma) 748
Embolization 748
Radiofrequency 748
I8
Periapical cemento-osseous dysplasia 854

Periapical cyst 850
Periapical granuloma 850
Periarticular Calcification 715
pericapsular fat planes 826
Periodontal ligament 850
Periosteal Osteosarcoma 753, 1014
Periosteal reaction 723
Periosteal Reaction (Intramedullary Chondrosarcoma) 765
Periosteal reaction (PTH) 711
Periosteal Reaction: Aggressive 731
Codman triangle 731
Hair-On-End 731
Laminated 731
Sunburst 731
Periosteal Reaction: Nonaggressive 730
Buttressing 730
Expansion 730
Septation 730
Solid 730
Periosteal/juxtacortical (Chondrosarcoma) 764
Periostitis 914
Permeative (Bone Tumors) - Differential Diagnosis 727
Peroneus Brevis 884
Peroneus Brevis Split Syndrome 885
Peroneus Longus and Brevis 884
Peroneus Quartus 886
Perthes Lesion 934
Pes anserine 870
Phemister triad 832
Phlebolith 806
Picture frame 814
Pigmented Villonodular Synovitis (PVNS) 793
Pillar Fracture 844
Pisotriquetral joint 872
Plantar Fascia 882
Plantar Fasciitis 882
Plantar Fibromatosis 780, 882
Plantaris Tendon 884
Plasmacytoma 720, 966
PNET 964, 967
POEMS syndrome 964, 965
Polyarthritis 983
Polymyositis 976
Polyostotic 773, 812
Polyostotic Lesions 732
Malignant 732
Neurofibromatosis (type 1) 732
Paget disease 732
Popliteus hiatus 870
Popliteus tendon 859
Positive Rim Sign 943
Posterior Dislocation (Glenohumeral) 943
Posterior Impingement (Elbow) 956
Posterior Instability (Glenohumeral) 935, 943
Posterior recesses 870
Posterior Superior Glenoid Impingement 936
Posterior talofibular 881
Posterior Tibial Tendon 884
Posterior tibiofibular ligament 880
Posterior Vertebral Scalloping 1020
Posterolateral Rotatory Instability (Elbow) 960

Post-traumatic cyst 784, 790
Pregnancy 907
Prepatellar 870
Primary Lymphocytic Lymphoma of Bone 966
Primary Lymphoma 966
(Calcific Tendinitis) 952
Prostaglandin (Osteoma) 746
Prostate Carcinoma 962
Pseudarthrosis (Tibia) 981
Pseudogout 950
Pseudomonas 821
Pseudotumor (Hemophiliac) 973
Pseudoxanthoma Elasticum 792
Psoriasis 916
Psoriatic arthritis 912, 916, 1009
Psoriatic Sacroiliitis 1010
Pubic fracture 998
Pulley Injuries 948
Puncture wounds 825
Putti Platt 938
PVNS 792, 973, 1006
Pyknodysostosis 1007
Pyomyositis 826
Pyrophosphate arthropathy 950, 951
Quadriceps tendon 869
Quadrilateral Space Syndrome 930
RA 869
Rachitic rosary 901
Radial Collateral Ligament 959
Radial Fracture 945
Radial head 944
Radial head dislocation 945
Radial nerve impingement 956
Radial Styloid Hutchinsons/ Chauffers Fracture 945
Radial tunnel syndrome 956
Radiation 757
Radiation - internal synovectomy 795
Radiation Induced Chondrosarcoma 764
Radiocapitellar Line 944
Radiolucent Lesions
Multilocular (Macho-Macho) 852
Periapical 850
Pericoronal 852
Radiopaque and Mixed Lesions
Ground Glass 856
Interradicular 855
Multifocal Confluent 856
Periapical 854
Target Lesion, Dense 857
Radioulnar ligaments 875
Raynauds phenomenon 984
Reactive Arthritis 917
Rectus femoris 869
Recurrent Multifocal Osteomyelitis 827
Reflex sympathetic dystrophy 976
Reiter Disease 912, 916, 917, 1010
Renal disease 869
Renal Insufficiency - Chronic (MSK Manifestations) 711
Renal Osteodystrophy 711, 903
I9
Renal Tubular Disorders 903

Rhabdomyosarcoma 769, 781, 802, 964, 967
Rheumatoid Arthritis 797, 817, 912, 914, 941
Rheumatoid arthritis, JRA 748
Ribbon ribs 1029
Rickets 714, 900, 971
Acetabuli Protrusio 714
Basilar invagination 714
Triradiate pelvis 714
Rim Rent Tear (Rotator Cuff) 929
Ring sequestra 825
Rolando Fracture 947
Romanus and Andersson lesion 918
Rotator cuff 932
Atrophy 930
Tears 915, 925
Types 928
Rowe 1003
Saber shin 834
Sacral Lesions: Differential Diagnosis 786
Sacroiliac disease 918
Sacroiliitis 1010
Sacrospinous ligaments 997
Sacrotuberous ligament 997
Saddle nose 834
Salmonella 821
Salter-Harris Fracture 1011
Sanders Classification 1003
SAPHO 827
Sarcoid 837
Sarcomatous transformation 817
Sartorius 869
Saucerization (Juxtacortical Chondroma) 762
Saunders 1003
Sausage digit 912
Scaphoid fracture 945
Scapholunate Ligament 946
Disruption 946
Scapular Y View 942
Scapular Fractures 942
Schatzker Classification (Tibial Plateau Fractures) 1001
Scheuermann Disease 911
Sclerodactyly 984
Scleroderma 793, 976, 985
Sclerosing osteomyelitis of Garre 825
Sclerosing Osteosarcoma 754
Scoliosis 901, 981
Scurvy 900, 904, 977
Secondary chondromatosis - trauma 799
Secondary Chondrosarcoma 764
Secondary osteoarthritis 826
Segond fracture 807, 1001
Semimembranosus 869, 870
Semitendinosus 869
Senile osteoporosis 977
Septic Arthritis 718, 748, 825
Septic bursitis 826
Septic tenosynovitis 826
sequestra 824, 967
Sequestra-Like Appearance 1005
Sequestrum 820
Seronegative Spondyloarthropathy 1009
Serpentine sclerosis 907

Serpentine vessels 806
Sever 911
Shepherds Crook 774
Shiny corner sign 918
Sickle cell anemia 818, 968, 1010
Simple Bone Cyst 786
Sinding-Larsen-Johansson 911
Sinus lesions 745
Sinus Tarsi Syndrome 882
Sinus tracts 822
Skeletal Metastases 961
Skull: beveled edge, button sequestrum 891
SLAC Wrist 874
SLAP Tears 936
SLE 869, 976
SLE (Systemic Lupus Erythematosis) 983
Slipped Epiphyses 715, 826
Small cell carcinoma 964
Smith Fracture (Reverse Colles) 945
Soft Tissue abscess 826
Soft Tissue Chondroma 800
Soft tissue chondrosarcoma 1012
Soft Tissue Ganglion 795
Soft Tissue Hemangioma 805
Soft Tissue infection 825
Soft Tissue Lipomatous Tumors 893
Soft Tissue Masses Differential Diagnosis 804
Soft Tissue Neoplasm 733
Elastofibroma and fibromatosis 733
Lipomatous lesions 733
Neurogenic tumors 733
PVNS and ganglion 733
Soft Tissue Sarcoma Incidence 781
Solitary Focus Bone Scan 964
Souer and Remy 1003
Sphenoid 745
Spina ventosa 830
Spinal cord edema/hematoma 848
Spine Infections 828
Spinoglenoid Notch Entrapment 930
Spirochetes 834
Spondylitis 918
Spondyloarthropathies 912, 916
Spondylodiscitis 828, 830, 1023
Spondylolisithes 845
Spontaneous healing (Osteoid Osteoma) 748
Spontaneous osteonecrosis 865, 910
Sporotrichosis 836
Squamous cell carcinoma 852
Staph aureus 821, 828
Stener Lesion 947
Sterno-clavicular Joint 941
Steroid administration 953
Steroids 869, 907
Steroids (Osteopenia/Osteoporosis) 977
Stewart-Treve syndrome 810
Still Disease 919
Storioform 780
Stress fracture 748
Femur 1027
I 10
Stress Injuries 987

Subacute osteomyelitis 748, 824, 1007, 1026
Subchondral cyst 784, 790, 921, 1008
Subchondral cyst/intraosseous ganglion 763
Subchondral Resorption 713
Subchondral Sclerosis 923
Sublabral Foramen 933
Subligamentous extension 830
Subligamentous/Subtendinous Resorption 713
Subperiosteal (Osteoid Osteoma) 746
Subperiosteal abscess 822, 823
Subperiosteal Resorption 712
Subscapularis (Avulsion) 935
Subscapularis (Disruption) 935
Subscapularis Muscle 932
Subscapularis Tears 929
Subtrochanteric Fractures 1000
Subungual Exostosis 759
Sunburst 967
Super bone scan 718
Superior Glenohumeral Ligament 933
Superolateral migration (Osteoarthritis - Hip) 923
Supinator syndrome (Elbow) 956
Supracondylar fracture 944
Suprapatellar bursa 870
Suprascapular Nerve Entrapment 930
Supraspinatus Tendon (Tear) 935
Swan-Neck, Boutonniere deformities 914
Symmetric Polyarthritis 983
Symphysis pubis (Calcification) 951
Synchronous Osteosarcoma 750
Syndesmophyte 918
Synovial Chondroma 792
Synovial Chondromatosis 792, 799, 1006
Synovial
Cyst 790, 792
Folds (Elbow) 956
Lipoma 792, 798
Osteochondromatosis 799
Plica 871
Sarcoma 781, 792, 793, 795, 802, 1012
Synovitis (Postoperative - Glenohumeral) 939
Syphilis 834, 953
Syringomyelia 848, 953
Systemic Lupus Erythematosis (SLE) 983, 1016
Tabetic arthropathy 953
Talar Neck Fractures 1002
Talus 995
Tarsal Coalition 880
Tarsal joints 1003
Tarsal Tunnel Syndrome 885
Telangiectasia 984
Telangiectatic Osteosarcoma 751
Tendinitis (Calcific) 952
Tendon Sheath 794
Tennis elbow 956
Tenosynovitis 876
Tenosynovitis (septic) 826
Tetracycline 748
TFCC 872
Thalassemia 968, 970
Thickened trabeculae 815
Thrombocytopenia 809
Thrombocytopenia with Absent Radii (TAR) 968, 972
Thumb Injury 947
Thyroid Cancer 963
Tibial collateral 870
Tibial Plateau Fractures 1001
Tile 995
Tile Classification 995
Tophi 950
Tori 856
Torus/ Buckle fracture 945
Transchondral fracture 879
Transient Osteoporosis 909
Transient Osteoporosis Hip 1015
Transient Regional Osteoporosis 977
Transverse Fractures (Pelvis) 999
Transverse ligament 863
Trap shooters shoulder 942
Trauma 839
Trauma (Dysbaric Disorders) 907
Trauma (Pelvis and Lower Extremity) 995
Trauma (Upper Extremity) 941
Traumatic bone cyst 850
Traumatic Spondylolisithes 845
Trevor Disease 759, 1020
Triangular Fibrocartilage (Calcification) 951
Triangular Fibrocartilage Complex 875
Triceps Injuries 958
Triquetrum Fracture 947
Triradiate pelvis 901
Trochlea 955
Trochlear sulcus 956
Trolley track sign 918
Tropical ulcer 834
Trough Sign 943
T-score 978
Tuberculosis 829, 973
Tuberculous
Arthritis 748, 832
Osteomyelitis 830
Spondylodiscitis 830
Tubulation (Osteochondroma) 758
Tumoral Calcinosis 792, 1012
Turner syndrome 808
UCL and ulnocarpal ligaments 875
Ulcer (tropical) 834
Ulcerative Colitis 912
Ulcers 825
Ulnar
fracture 945
nerve 872
Tunnel Syndrome 877
Unicameral bone cyst (UBC) 784, 944
Unilateral Facet Injury 841
Unusual infection 818
Upper Extremity Trauma 941
Van Neck 911
Vanishing Bone Disease 809
Vastus lateralis 869
Vastus medialis 869
Ventral (anterior) SIJ ligament 997
Vertebra Plana 891
I 11
Vertebral scalloping 981

Vertebroplasty 808
Vertical shear (Pelvic Trauma) 996
VISI deformity 875
Vitamin D: Prohormone 900
Volar Plate Avulsion 948
Voorhoeve Disease (Osteopathia Striata) 744
Wall Fracture (Pelvis) 999
West Point View 942
Whipple 912
Whiskering 914
Widened hip joint 826
Wimberger sign 834
Wormian Bones 1007
Wrisberg 859
Wrist 872
Wrist Subluxations 1019
Xenograft 709
X-linked hypophosphatasia 903
Yaws 834
Young-Burgess 995
Young-Burgess Classification 996
Young-Burgess vs Tile Classifications 995
Yttrium 90 795
Zonal pattern 801
Zonal phenomena 801
Z-score 978
I 12
Radiologic
Pathology
Fifth Edition
VOLUME 3
Neuroradiology and Pediatric
2006
2007
Editors

Mark D. Murphey, MD

Associate Editor
Illustrators
Heike Blum, MFA

Washington DC, USA

Washington, DC
20306-6000
_____________________________________
_____________________________________
987654321
ISBN 1-933477-00-8
Preface
Acknowledgements
Pathology,
iii
Faculty VOLUME 3
Neuroradiology
Pediatric Radiology
Kelly K. Koeller, MD, FACR

Washington, DC
and
Mayo Clinic
Rochester, MN

Washington, DC
Dorothy I. Bulas, MD
Professor of Radiology and Pediatrics

Children's National Medical Center
The George Washington University School of Medicine
and Health Sciences
Washington, DC
Patricia A. Hudgins, MD
Emory University Medical Center
Atlanta, GA
Gael J. Lonergan, MD
Chief of Radiology
Children's Hospital of Austin
Austin, TX
Mary E. (Lee) Jensen, MD
Director of Interventional Neuroradiology

Professor of Radiology and Neurosurgery
University of Virginia Health System
Charlottesville, VA
William E. Shiels II, D.O.
Chairman, Department of Radiology

Children 's Hospital
Columbus, OH
Erin Simon Schwartz, MD

University of Pennsylvania School of Medicine
Pediatric Neuroradiologist
The Children's Hospital of Philadelphia
Philadelphia, PA
Marilyn J. Siegel, MD
Professor of Radiology and Pediatrics
Mallinckrodt Institute of Radiology
Washington University Medical School
St. Louis, MO
and
Washington, DC
James G. Smirniotopoulos, MD
Professor of Radiology, Neurology, and Biomedical

Informatics
Chair, Radiology and Radiological Sciences
Bethesda, MD
Wendy R. K. Smoker, MS, MD, FACR
University of Iowa Medical Center
Iowa City, IA
iv
Table of Contents VOLUME 3

Neuroradiology
Imaging of Demyelinating Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1037

Lymphoma and Uncommon Neuroepithelial Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1045
Cerebral Intraventricular Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1058
Imaging of the Temporal Bone: Anatomy and Congenital Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1068
Imaging of the Temporal Bone: Infectious and Neoplastic Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1076
Imaging of the Orbit: The Globe and Conal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1088
Imaging of the Orbit: Intraconal and Extraconal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1097
Patterns of Location: Infratentorial and Supratentorial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1106

Patterns of Enhancement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1126
The WHO 2000 Brain Tumor Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1137
Non-Astrocytic Gliomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1149
Extraaxial Tumors: Other Non-Glial Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1158
Neoplasms of the Meninges . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1164
Pinealomas and, other Pineal Region Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1175
The Phakomatoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1184
Mary E. Jensen, MD
Subarachnoid Hemorrhage and Intracranial Aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1210

Intracranial Vascular Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1220
Intracranial Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1231
Paranasal Sinuses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1240
Sella and Parasellar Region . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1250
Congenital Spinal Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1260
Wendy R. K. Smoker, MS, MD, FACR
Imaging of the Suprahyoid Neck: Superficial, Parapharyngeal and Carotid Spaces . . . . . . . . . . . . . . . . .1266
Imaging of the Suprahyoid Neck: Masticator and Parotid Spaces . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1274
Imaging of the Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity . . . . . . . . . . . . . . . . . . . .1282
Spine: Degenerative Disease and Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1290
Spinal Tumors, Cysts, and Mimics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1298
Congenital Abnormalities of the Brain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1307
Neuroradiology Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1318

Neuroradiology Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1322
Pediatric Radiology
Childhood Urinary Tract Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1329

Neonatal GI Tract Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1341
Acute GI Disorders of Infants and Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1353
Diseases Affecting The Pediatric Airway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1363
Vascular Rings and Slings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1374
Cystic Renal Disease of Childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1381
Marylin J. Siegel, MD
Pediatric Renal Tumors: Infancy and Young Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1390

Pediatric Adrenal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1402
Pediatric Pelvic Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1414
Bone Marrow Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1425
Congenital Lung Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1435
Lung Diseases in Neonates . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1444
Pediatric Cardiac Imaging Part I: Vascular Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1453
Pediatric Cardiac Imaging Part II: Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1463
Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1472

Forensic Radiology of Child Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1491
Dorothy I. Bulas, MD
Neonatal Brain: Radiologic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1501
William E. Shiels II, DO
Pediatric Liver Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1509

Pediatric Hip Sonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1518
Pediatric Radiology Seminar I: Pulmonary Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1521

Pediatric Radiology Seminar II: Skeletal Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1527
Pediatric Radiology Seminar III: Cystic Fibrosis & Pulmonary Infections of Immunocompromised Child .1535
vi
Neuroradiology
Imaging of Demyelinating Diseases

Imaging Hallmarks
White matter location

May involve basal ganglia
Little or no mass effect
Usually no calcification
May or may not enhance
Figure 5-1-1
Normal Lesions [Figure 5-1-1]
Virchow-Robin spaces
Perivascular space of deep penetrating vessels
Follows CSFsignal intensity
Ependymitis granularis
Frontal horn
Ependymitis granularis [Figure 5-1-2]
Frontal horn capping

Swamp of the brain
Perivascular spaces follow CSF signal
Axons with low myelin content
on all MR sequences
Interstitial CSF backed up
Loss of ependyma
Periventricular hyperintensity: increased in ischemic WM states
No lymphatics in the brain
Demyelinating Disorders
Multiple sclerosis
Vascular
Viral / post-viral demyelination
Toxic / metabolic encephalopathies
Iatrogenic white matter degeneration
Figure 5-1-2
Multiple Sclerosis
Unknown etiology
Viral: measles, Epstein-Barr virus (EBV)
Genetic: chromosome 6, human lymphocyte antigens (HLA)
loci
Autoimmune: associations with Graves, myasthenia gravis,
ulcerative colitis, Crohn's, SLE
Females (60%): especially with optic neuritis
95% cases: 18-50 years old
Cooler climates: northern Europe, North America; southern South
America
Multiple Sclerosis
Relapsing-remitting: 70%
Numbness, dysesthesia, burning sensations
2 clinical attacks from 2 separate lesions
At least 24 hours and at least 30 days apart
Ependymitis granularis
Partial or complete remission for months or years
Progressive: 20%
Primary progressive: slow onset without distinct attacks
Secondary progressive: relapsing-remitting form with progressive disability
Monosymptomatic demyelinating: 10%
McDonald et al, Ann Neurol 2001: 50-121-127
Neuroradiology
1037
Imaging of Demyelinating Disease
Multiple Sclerosis - Clinical
Figure 5-1-3
Uthoff's phenomenon: symptoms worsened with

exposure to heat
Children: very rare especially before puberty
Pregnancy
Decreased risk during 2nd and third trimester ?
...but exacerbation rate of 75% in first 6 months
post-partum
Multiple Sclerosis - Pathology
Microglial infiltration
Myelin disintegration and
Perivascular cuffing of
focal hypercellularity
lymphocytes -->
Predilection for periventricular zone
Active (enhancing) MS plaque compared to

chronic (non-enhancing) MS plaque
Multiple Sclerosis - Imaging
Periventricular distribution: classic

3 or more lesions during event: sensitive
indicator for MS within 10 years
Ovoid lesions often perpendicular to walls of
ventricles (Dawson's fingers)
Little mass effect for size of lesion
Corpus callosum-septum pellucidum interface
lesions: increase specificity and sensitivity of
diagnosis
Figure 5-1-4
Radiology 1991;180:467-474; Radiology 1991;

180:215-221; Neurology 2003; 61:602-611
Multiple Sclerosis - Imaging [Figure 5-1-3]
Active plaques enhance (ring-like in some)

Chronic lesions: do not enhance
Atrophy in chronic MS
McDonald Criteria - MR
Dissemination in space (3 or more)

1 enhancing lesion or 9 T2 hyperintense
lesions
1 or more infratentorial lesion
1 or more juxtacortical lesion
3 or more periventricular lesions
Dissemination in time (at least 1)
MRI more than 3 months after clinial event, enhancing
lesion at different site
No enhancing lesion: new T2 lesion or enhancing lesion on
f/u study at least 3 months later
MRI less than 3 months, new enhancing lesion on f/u study
Figure 5-1-5
Multiple Sclerosis MR [Figure 5-1-4]
Chronic cases: atrophy

Fluid-attenuated inversion recovery (FLAIR): hyperintense
MR Spectroscopy
Decreased N-acetyl
aspartate (NAA) in
chronic plaques
Increased choline,
lipids and lactate
1038
Tumefactive MS plaque. Lack of

mass effect and non-neoplastic
(NAA > choline) MR spectrogram are
important clues to correct diagnosis
Neuroradiology
Tumefactive Multiple Sclerosis [Figure 5-1-5]
Tumor-like but
Lack of mass effect: most important clue for
demyelination
Figure 5-1-6
Multiple Sclerosis Variant Types [Figure 5-1-6]
Concentric sclerosis (Balo's)

Alternating bands of myelination and
demyelination, often in concentric fashion
Acute: (Marburg)
Rapid course
Death in months
Severe axonal loss
Neuromyelitis optica (Devic syndrome)
Both visual and spinal cord signs
Multiple Sclerosis vs. Transverse Myelitis
Balo concentric sclerosis
[Figure 5-1-7]
Spinal cord MS plaques: 7%

Multiple sclerosis: peripheral, usually less than two segments, limited to one
side
Clinical cord syndrome: 60% had brain lesions
Transverse myelitis: usually holocord, commonly
involves gray matter
Figure 5-1-7
Tartaglino et al, Radiology. 1995;195:725-32; Tartaglino

et al, Radiology. 1996;201:661-9
Vascular White Matter Disease
Microangiopathy
Arteriosclerosis / venous collagenosis
Hypoxic-ischemic encephalopathy
Posterior Reversible Encephalopathy Syndrome
(PRES)
Amyloid angiopathy
Vasculitis
Migraine
Transverse myelitis
Edwards, ed, Neuroimaging clinics 1993
Senescent White Matter Changes [Figure 5-1-8]
Microangiopathy, deep white matter ischemia, leukoariosis, etc.

Demyelination, axonal loss, gliosis, ischemic changes
30%-80% "normal" elderly patients
More lesions, more likely to have neuropsychologic and cognitive
problems
? correlation with dementia
Binswanger's: clinical diagnosis, reserved only
for dementia cases
Do not involve corpus callosum
Figure 5-1-8
Arteriosclerosis
Long penetrating end arteries

Few or no collateral vessels
Pons, thalami, basal ganglia, deep white matter
Prevalence increased with age and history of stroke
Venous Collagenosis
Noninflammatory stenosis
Occlusion of subependymal veins
Associated with periventricular white matter changes
Moody et al, Radiology 1995; 194:469-476

Neuroradiology
1039
Senescent white matter changes.

Note subcortical location
Posterior Reversible Encephalopathy Syndrome (PRES)

[Figure 5-1-9]
Figure 5-1-9
Hypertensive events: renal failure, pre/eclampsia,

immunosuppressive drugs
Loss of normal autoregulation: elevated hydrostatic
pressure mediated by venous vasoconstriction?
Posterior cerebral circulation: less sympathetic
innervation, less ability to vasoconstrict
Visual field deficits, headache, somnolence
T2 hyperintensity
Diffusion: usually increased (not restricted)
Perfusion (CBV, CBF): decreased, normal, or
increased
Casey et al, AJNR 2000; 21:1199-1206 Brubaker et al,

AJNR 2005; 26:825-830; Schuuring et al AJNR 2003;
24:2085-2088
Senescent white matter changes.

Note subcortical location
Viral and Postviral Demyelination
Encephalitis
Acute disseminated encephalomyelitis
Subacute sclerosing panencephalitis
Human immunodeficiency virus infection and complications
HIV encephalitis
Progressive multifocal leukoencephalopathy
Acute Disseminated Encephalomyelitis (ADEM)
1-3 weeks post-infection or vaccination

Monophasic: rubeola, vaccinia, varicella, mycoplasma, mumps, rubella
No virus or bacteria isolated on autopsy
Hemorrhagic type (Hurst variant): rapidly progressive onset
Children > adults
Good prognosis overall but 10%-20% significant neurological deficit or death
Diagnosis of exclusion: long-term follow-up needed to rule out MS
Honkaniemi et al, AJRN 2001; 22:1117-1124; Rosman et al, J Child Neurol. 1997;
12:448-54
Figure 5-1-10
ADEM Pathology and Imaging
Autoimmune response -> perivenous

demyelination
CT: normal or nonspecific hypoattenuation
No mass effect
ADEM: MR Findings [Figure 5-1-10]
Asymmetric WM lesions
Varying in size and number
Optic neuritis, myelitis
Kesselring et al, Brain 1990;113:291-302
1040
Neuroradiology
Progressive Multifocal Leukoencephalopathy (PML)
Defective cell-mediated immunity

Marked decrease in prevalence with highly active anti-retroviral therapy
(HAART)
Pre-HAART: 1-7% of AIDS patients; 55-85% cases related to AIDS
584 y/o; peak: 6th decade
JC virus (papovavirus) reactivation
Affects oligodendrocytes: demyelination
Extremely poor prognosis (death in 6 months) if untreated
Figure 5-1-11
Baqi et al, AIDS 1997; 11:1526-7
PML Path and Imaging [Figure 5-1-11]
Predominantly parieto-occipital and frontal

Posterior fossa: 1/3 cases
Subcortical white matter
Typically no mass effect or enhancement
Enhancement indicative of long-term survival?
Characteristic scalloped lateral margin at gray matter-white
matter junction
May show hemorrhage
Whiteman et al, Radiology 1993; 187:233-240; Thurnher et al AJNR

2001; 22:977-984
PML with relative lack of mass effect
and sparing of cortical gray matter
HIV Encephalitis [Figure 5-1-12]
Much less common with anti-retroviral therapy

Deep white matter and gray matter
Psychomotor slowing, mental status changes, memory problems, apathy
Direct or indirect infection of oligodendrocytes
Demyelination and vacuolation
Axonal loss and microglial nodules
Figure 5-1-12
Thurnher et al AJNR 2001; 22:977-984
HIV Encephalitis
Imaging often normal early in course

Diffuse mild cerebral atrophy
Cortical first, then central
Ill-defined patchy areas
Central white matter, basal ganglia, thalamus
Bilaterally symmetric
Usually no necrosis or edema
No enhancement
Thurnher et al AJNR 2001; 22:977-984; Olson et al, Radiology 1988;

169:445-448
HIV encephalitis with characteristic

cortical atrophy
Toxic Demyelination
Alcohol
Ion balance disorders
Osmotic myelinolysis
Extrapontine myelinolysis
Organic toxins (lipophilic solvents)
Carbon monoxide poisoning (interval form)
Drug abuse (poisoned heroin)
Neuroradiology
1041
Alcohol and the Brain [Figure 5-1-13 and 5-1-14]
Figure 5-1-13
Atrophy
Cerebral hemisphere
Superior vermis
Marchiafava-Bignami disease
Corpus callosum demyelination, necrosis
Rare: cortical laminar necrosis
Wernicke encephalopathy
Thiamin deficiency
Ophthalmoplegia, ataxia, confusion
Medial thalamic nuclei
Mamillary bodies: atrophy
Arbelaez et al, AJNR 2003; 24:1955-57; Johkura et al, AJNR 2005;

26:670-3; Donnal et al, AJNR 1990; 11:893-894
Marchiafava-Bignami disease with
corpus callosal lesions. Note atrophy of
cerebral hemisphere and superior
cerebellar vermis
Osmotic Myelinolysis [Figure 5-1-15]
Central pontine myelinolysis

Spastic quadraparesis, pseudobulbar palsy
Incidence?: 0.163.7 % of autopsy cases
Rapid osmotic change
Vascular injury in gray matter - white matter
apposition regions
Demyelination: spares periphery of pons
CT: Hypoattenuated
Figure 5-1-14
Ruzek et al, AJNR 2004; 25:210-213
Osmotic Myelinolysis [Figures 5-1-15 and 5-1-16]
MR:
T1WI: hypointense
T2WI: hyperintense
May return to normal in months to year
Extrapontine (10%): basal ganglia, other sites
Iatrogenic Demyelinating Disorders:

Chemotherapy
Mineralizing microangiopathy
Methotrexate
Periventricular, centrum semiovale
Patients < 5 y/o, meningeal leukemia, high-dose
therapy: greatest risk
Wernicke encephalopathy with

pathognomonic hyperintensity of both medial
thalami
Figure 5-1-16
Davis et al, AJR 1986; 147:587-592; Cajade-Law et al in

Neuroimag Clin, Edwards, ed. 1993;3:361-377
Figure 5-1-15
Osmotic (central pontine) myelinolysis

on CT and T2 MR images
Extrapontine myelinolysis
1042
Neuroradiology
Radiation Injury [Figure 5-1-17]
Acute: no imaging findings

Early delayed: >2 months after therapy
White matter, basal ganglia, cerebral peduncles
Late:
Focal radiation necrosis: > 1 year
Diffuse radiation injury: > 1 year
Geographic pattern: conforms to radiation port
Necrotizing leukoencephalopathy: as early as 3 months posttherapy
Figure 5-1-17
Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404
Radiation Necrosis [Figure 5-1-18]
Tumor-like
Metabolic imaging
Increased activity: high-grade tumors
Normal or decreased activity: radiation necrosis
Less reliable for low-grade tumors
MRS: increased lactate and choline in tumors vs. increased
lactate in necrosis
Diffusion-weighted imaging:
Tumors: usually hypointense
Necrosis: usually hyperintense
Radiation injury to white matter with

typical "geographic" pattern of
involvement
Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404
Demyelination Imaging Hallmarks
Figure 5-1-18
White matter location

May involve basal ganglia
Usually no calcification
Summary
Normal
Virchow-Robin spaces: follow CSF
Ependymitis granularis: frontal horn
Multiple Sclerosis
Periventricular
Clinical diagnosis
Vascular demyelination
Senescent white matter changes
Subcortical, do not involve corpus callosum
Posterior Reversible Encephalopathy Syndrome
(PRES)
Viral / postviral demyelination
ADEM
PML
HIV encephalitis
Toxic / metabolic demyelination
Alcohol
Osmotic myelinolysis: centra pons
Iatrogenic demyelination
Chemotherapy and radiation injury
Radiation necrosis mimicking appearance of a

glioblastoma multiforme
References
1. Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR findings in two patients. AJNR Am J
Neuroradiol 2003; 24:1955-1957.
2. Baqi M, Kucharczyk W, Walmsley SL. Regression of progressive multifocal encephalopathy with highly active
antiretroviral therapy. Aids 1997; 11:1526-1527.
Neuroradiology
1043
3. Brubaker LM, Smith JK, Lee YZ, Lin W, Castillo M. Hemodynamic and permeability changes in posterior
reversible encephalopathy syndrome measured by dynamic susceptibility perfusion-weighted MR imaging. AJNR
Am J Neuroradiol 2005; 26:825-830.
4. Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of fluidattenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J
Neuroradiol 2000; 21:1199-1206.
5. Davis PC, Hoffman JC, Jr., Pearl GS, Braun IF. CT evaluation of effects of cranial radiation therapy in children.
6. Donnal JF, Heinz ER, Burger PC. MR of reversible thalamic lesions in Wernicke syndrome. AJNR Am J
Neuroradiol 1990; 11:893-894; discussion 895-896.
7. Frohman EM, Goodin DS, Calabresi PA, et al. The utility of MRI in suspected MS: report of the Therapeutics and
Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2003; 61:602-611.
8. Gean-Marton AD, Vezina LG, Marton KI, et al. Abnormal corpus callosum: a sensitive and specific indicator of
multiple sclerosis. Radiology 1991; 180:215-221.
9. Honkaniemi J, Dastidar P, Kahara V, Haapasalo H. Delayed MR imaging changes in acute disseminated
encephalomyelitis. AJNR Am J Neuroradiol 2001; 22:1117-1124.
10. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. AJNR Am J Neuroradiol 2005;
26:670-673.
11. Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis. MRI findings and the distinction
from multiple sclerosis. Brain 1990; 113 ( Pt 2):291-302.
12. McDonald WI, Compston A, Edan G, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines
from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001; 50:121-127.
13. Moody DM, Brown WR, Challa VR, Anderson RL. Periventricular venous collagenosis: association with
leukoaraiosis. Radiology 1995; 194:469-476.
14. Nesbit GM, Forbes GS, Scheithauer BW, Okazaki H, Rodriguez M. Multiple sclerosis: histopathologic and MR
and/or CT correlation in 37 cases at biopsy and three cases at autopsy. Radiology 1991; 180:467-474.
15. Olsen WL, Longo FM, Mills CM, Norman D. White matter disease in AIDS: findings at MR imaging. Radiology
1988; 169:445-448.
16. Rosman NP, Gottlieb SM, Bernstein CA. Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic
resonance imaging findings in a child. J Child Neurol 1997; 12:448-454.
17. Rowley HA, Dillon WP: Iatrogenic white matter diseases. Neuroimaging Clin N Am 3:379404, 1993
18. Ruzek KA, Campeau NG, Miller GM. Early diagnosis of central pontine myelinolysis with diffusion-weighted
imaging. AJNR Am J Neuroradiol 2004; 25:210-213.
19. Schuuring J, Wesseling P, Verrips A. Severe tacrolimus leukoencephalopathy after liver transplantation. AJNR Am J
Neuroradiol 2003; 24:2085-2088.
20. Tartaglino LM, Croul SE, Flanders AE, et al. Idiopathic acute transverse myelitis: MR imaging findings. Radiology
1996; 201:661-669.
21. Tartaglino LM, Friedman DP, Flanders AE, Lublin FD, Knobler RL, Liem M. Multiple sclerosis in the spinal cord:
MR appearance and correlation with clinical parameters. Radiology 1995; 195:725-732.
22. Thurnher MM, Post MJ, Rieger A, Kleibl-Popov C, Loewe C, Schindler E. Initial and follow-up MR imaging
findings in AIDS-related progressive multifocal leukoencephalopathy treated with highly active antiretroviral
therapy. AJNR Am J Neuroradiol 2001; 22:977-984.
23. Whiteman ML, Post MJ, Berger JR, Tate LG, Bell MD, Limonte LP. Progressive multifocal leukoencephalopathy in
47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology 1993; 187:233-240.
1044
Neuroradiology
Lymphoma and
Uncommon Neuroepithelial Tumors
CNS Lymphoma
6.6%-15.4% of all primary brain tumors

Only GBM, meningioma, and low-grade astrocytoma are more common
Less than 1% of all body lymphomas
Primary lymphoma much more common than secondary
Miller et al, Cancer 1994; 74:1383-1397; Henry et al, Cancer 1974; 34:1293-1302
Clinical
Wide age range

Peak: 4th to 5th decades
Smaller peak: 1st decade (AIDS)
Nonspecific clinical presentation
Expanding mass lesion
Encephalitis
Stroke
Cranial nerve palsies
Figure 5-2-1
Koeller et al, Radiographics 1997; 17:1497-1526
Immunocompromised Patients
Opportunistic neoplasm
Incidence much higher than in
immunocompetent patients
AIDS-defining diagnosis in HIV+ patients
2% of AIDS patients develop CNS lymphoma
CNS mass lesion in AIDS patient
Toxoplasmosis #1, lymphoma #2
Lymphoma: #1 in pediatric AIDS patient
Rosenblum et al, Ann Neurol 1988: 23:S13-S16;

Koeller et al, Radiographics 1997; 17: 1497-1526
Two masses, both lymphoma, with one located

around the ventricle while the other arises from
the leptomeninges
Gross Pathology [Figure 5-2-1]
Intra-axial nodule
Grayish-pink, homogeneous, circumscribed
Multifocal: 50%
Leptomeningeal
Uveal
Intradural spinal
Figure 5-2-2
Histopathology [Figure 5-2-2]
Small blue cell tumor

Almost always B-cell type
Perivascular space
Perivascular distribution of small blue lymphocytes

(arrow, vessel)
Neuroradiology
1045
Lymphoma & Uncommon Neuroepithelial Tumors
CT / MR Findings [Figures 5-2-3 to 5-2-5]
Figure 5-2-3
NCCT: Hyperattenuated mass

Negative CT does not exclude diagnosis
MR:
T1WI: iso-to-hypointense to gray matter
T2WI: hypointense
Little mass effect for size of lesion
Virtually all enhance
Ring-like: necrosis; common in
immunocompromised hosts
Lee et al, AJR 1986; 147:747-752; Schwaighofer et al,

AJNR 1989; 10:725-729; Dina, Radiology 1991;
179:823-828
Figure 5-2-4
Lymphoma with characteristic

CT hyperdensity and T2 hypointensity
Figure 5-2-5
CNS Lymphoma with true water restricted

diffusion (DWI hyperintensity, left; ADC
hypointensity, right)
General Neuroimaging Features [Figure 5-2-6]
Supratentorial location
Deep gray matter: classic, 33%
Cerebral white matter: 55%
Cerebellar lesions: 10%
Multiple lesions: 11%-47%
More common in immunocompromised
Recurrences: 50% at original site
Lymphoma in AIDS patient with ring-like

enhancement secondary to necrosis
Figure 5-2-6
General Neuroimaging Features
Hugs ependyma or leptomeninges

Butterfly pattern: corpus callosum
Ghost tumor: vanishes with steroid or radiation therapy
Avoid prior to biopsy
Rare: calcification, hemorrhage
Jiddane et al, J Neurosurg 1986; 65:592-599; Vaquero et al, J Neurosurg 1984;

60:174-176
Lymphoma of right
basal ganglia
1046
Neuroradiology
PET / SPECT-Thallium [Figure 5-2-7]
Figure 5-2-7
Hypermetabolic
Non-neoplastic lesions: hypometabolic
High specificity and sensitivity
False positives: interpretation errors,
occasional hypermetabolic abscess
Hoffman et al, J Nucl Med 1993; 34:567575; Villringer et al, J Comput Assist
Tomogr 1995; 19:532-536
Neuroepithelial Tumors
Astrocytic
Pleomorphic xanthoastrocytoma
Toxoplasmosis or lymphoma ?
Oligodendroglial
PET image shows hypermetabolic activity consistent with
Mixed glial
lymphoma
Ependymal
Choroid plexus
Pineal parenchymal
Neuroblastic
Glial of uncertain origin
Neuronal and mixed neuronal-glial
Ganglioglioma / Gangliocytoma
Desmoplastic Infantile Ganglioglioma
Dysplastic cerebellar gangliocytoma
Dysembryoplastic neuroepithelial tumor
Cerebellar Liponeurocytoma
Embryonal
Supratentorial PNET
Atypical teratoid / rhabdoid tumor
Ganglioglioma / Gangliocytoma
About 1% of all brain tumors

Children and young adults
80% <30 years old; peak: 10-30 years of age
Males slightly more common
Most common tumor seen in chronic temporal lobe epilepsy
15%-25% of medically refractory seizures
Cerebral hemisphere predilection
Temporal lobe: most common (38%)
Optic nerves, pituitary and pineal glands, spinal cord, ventricles, cranial
nerve (1 report)
Johnson et al, Pediatr Neurosurg 1997; 27:203-207; Athale et al, Neuroradiology

1999; 16:790-792
Ganglioglioma / Gangliocytoma [Figure 5-2-8]
Good prognosis overall

Ganglioglioma: WHO I or II
Biphasic tumor: neoplastic ganglion cells and glial cells
Malignant degeneration of glial cells (WHO III): 6%
Gangliocytoma
Only mature ganglion cells
WHO grade I
AFIP: really cortical dysplasia?
Figure 5-2-8
Silver et al, Surg Neurol 1991; 35:261-266; Kalyan-Raman and

Olivero, Neurosurgery 1987; 20:428-433
Ganglioglioma with biphasic
appearance on histology. Arrow
points to a ganglion cell
Neuroradiology
1047
Ganglioglioma [Figure 5-2-9]
Figure 5-2-9
Typically peripheral mass

Cystic / solid: 52%
Solid: 43%
Purely cystic: 5%
Little mass effect or surrounding edema
Calcification common
Skull remodeling
Enhancement variable
Zentner et al, AJNR 1994; 57:1497-1502; Dorne et al, AJNR 1986;

7:281-285; Castillo et al, AJNR 1990; 11:109-114
Ganglioglioma [Figures 5-2-10 to 5-2-12]
Typical MR findings
T1WI: iso-to-hypointense
T2WI: hyperintense
Occasional T1 hyperintensity
Leptomeningeal spread: rare
Castillo et al, AJNR 1990; 11:109-114; Tampieri et al, AJNR 1991;

12:749-755; Tien et al, AJR 1992; 159:391-393
Ganglioglioma with typical peripheral

location and calcification
Ganglioglioma
Positron Emission Tomography (PET): heterogeneous metabolic activity

MR Spectroscopy: increased choline-creatine ratio
Provenzale et al, AJR 1999; 172:1103-1107; Kumabe et al,

Neurosurgery 1999; 45:183-187
Figure 5-2-10
Desmoplastic Infantile
Ganglioglioma / Astrocytoma
First described in 1987

Superficial cerebral astrocytoma
Desmoplastic cerebral astrocytoma of
infancy
Rare: 0.6% of brain tumors
16% of all infant brain tumors
Vast majority: less than 1 year (range:
up to 17 years)
Males more common (2:1)
Rapid onset: increasing head circumference
Usually more than one lobe: typically frontal and parietal
Ganglioglioma in temporal lobe
VandenBerg et al, J Neurosurg 1987; 66:58-71; Taratuto et al, Cancer 1984;

54:2505-2512
Figure 5-2-12
Figure 5-2-11
Ganglioglioma in medial portion

of right temporal lobe
Large ganglioglioma
1048
Neuroradiology
Desmoplastic Infantile Ganglioglioma /

Astrocytoma [Figure 5-2-13]
Figure 5-2-13
WHO grade I
Desmoplastic stroma
Neuronal component: ganglioglioma
Neoplastic astrocytes: astrocytoma
Meningocerebral: involves cortex & leptomeninges
Duffner et al, Neurosurgery 1994; 34:583-589
Desmoplastic Infantile Ganglioglioma /

Astrocytoma
[Figure 5-2-14]
Large heterogeneous mass

Cyst-like areas
Superficial soft tissue area
Slightly hyperattenuated
Frequently attached to dura
Intense enhancement
No calcification
Occasional vasogenic edema
Desmoplastic infantile ganglioglioma
Figure 5-2-14
Duffner et al, Neurosurgery 1994; 34:583-589; Martin

et al, AJNR 1991; 12:1195-1197; Tenreiro-Picon et al,
Pediatr Radiol 1995; 25:540-543
Dysplastic Cerebellar Gangliocytoma

(Lhermitte-Duclos Disease)
Original case, 1920 (Lhermitte and Duclos)

36-year-old male with progressive neurologic
deficits
Cerebellar mass: diffuse ganglioneuroma
Dysplastic cerebellar gangliocytoma
Numerous other names proposed
Histogenesis?: hamartoma vs. neoplasm
Hamartoma favored
Lhermitte and Duclos, Bull Assoc Fr Etude Cancer,

1920;9:99-107; Wiestler et al, WHO classification,
2000; 235-237

50%: Cowden disease

Autosomal dominant: susceptibility gene
10q23
Colonic polyps, cutaneous tumors, thyroid
DIG with intensely enhancing soft tissue component
tumors, breast tumors, meningioma, glioma
along meningocerebral interface
MR screening of family members
recommended
Diagnosis established when Lhermitte-Duclos combined with either:
Thyroid cancer
Breast cancer
Macrocephaly
Robinson and Cohen, Neurosurgery 2000; 46:371-383; Kulkantrakorn et al,

Neurology, 1997; 48:725-731; Nelen et al, Nat Genet 1996; 13:114-116
Neuroradiology
1049

Figure 5-2-15
CT findings
Usually hypoattenuated
Isoattenuated: normal
Calcification uncommon
Meltzer et al, Radiology 1995; 194:699-703

(Lhermitte-Duclos Disease) [Figure 5-2-15]
MR findings
Classic appearance
Cerebellar mass
Striated
No edema
No enhancement
Non-classic cases: non-specific appearance
Classic striated cerebellum appearance in

Lhermitte-Duclos disease
Meltzer et al, Radiology 1995; 194:699-703

T1 hypointensity / T2 hyperintensity:
Inner molecular layer, granular cell layer, and loss of central
white matter within folia
Figure 5-2-16
Kulkantrakorn et al, Neurology 1997; 48:725-731

Lipomatous medulloblastoma
15 cases reported by year 2000
Similar to central neurocytoma except:
Cerebellar location
Older age group
Peak age: 5th-6th decades (mean: 51 years old)
Kleihues et al, WHO classification 2000, 110-111
Cerebellum or cerebellopontine angle
WHO grade I or II
Well-differentiated neuronal cells
Focal lipomatous differentiation
Low mitotic activity
Good overall prognosis but few cases
No role for radiation therapy or chemotherapy?
Kleihues et al, WHO classification 2000, 110-111
Cerebellar Liponeurocytoma [Figure 5-2-16]
Cerebellar mass
CT: hypoattenuated with focal areas of fat attenuation
MR: hypointense with scattered focal T1 hyperintensity
Moderate enhancement
Kleihues et al, WHO classification 2000, 110-111; Cacciola et al, Acta

Neurochir 2002; 144:829-833; Alkadhi et al 2001; J Neurosurg
95:324-331
Inherent T1 hyperintensity and mild
enhancement in cerebellar
liponeurocytoma
1050
Neuroradiology
Dysembryoplastic Neuroepithelial Tumor
First described in 1988 (Daumas-Duport et al)

Benign tumor of cortex or deep gray matter
Children and young adults with partial seizures
Peak age: 10-30 years
Males more common
Neurologic deficits not common
Excellent prognosis even with partial resection
Very rare malignant transformation
Figure 5-2-17
Daumas-Duport et al, Neurosurgery 1988;23:545-556;

Daumas-Duport et al, WHO classification, 2000;
Hammond et al, J Neurosurg 2000;92:722-725

[Figure 5-2-17]
WHO Grade I
Simple form vs. complex form: controversial
Multinodular: complex form
Cortical dysplasia: focal
Specific glioneuronal element: columnar pattern
Freely floating neurons
Temporal lobe: 62%
Frontal lobe: 31%
Other sites: caudate nucleus, cerebellum, pons
DNT with floating neuron
Figure 5-2-18
Daumas-Duport et al, Neurosurgery 1988;23:545-556;

Daumas-Duport et al, WHO classification, 2000

[Figure 5-2-18]
CT findings
Hypoattenuated
Calcification: uncommon (~ 5%)
May produce remodel skull
No surrounding edema
MR findings
T1WI: hypointense
T2WI: hyperintense
Occasional soap-bubble appearance
More multinodular than gangliogliomas?
Koeller and Dillon, AJNR 1992;13:1319-1325; Kuroiwa et al,

JCAT, 1994;18:352-356; Ostertun et al, AJNR 1996;17:419430
Pleomorphic Xanthoastrocytoma
Originally described in 1979 (Kepes et al)

12 cases of supratentorial tumors involving the
leptomeninges
Believed to arise from subpial astrocytes of the cortex
Less than 1% of all brain neoplasms
Importance: characteristic imaging appearance, highly
amenable to surgical resection
Kepes et al, Cancer 1979:44:1839-1852

DNT in classic cortical location and showing
exophytic extension with pressure erosion of
inner table of skull
Neuroradiology
1051
Usually adolescents or young adults (average age: 26 years)

Wide age range: 5-82 years
Long history of seizures
Supratentorial: 98%
Temporal lobe: 47%
Multi-lobe: 10%
Rare: thalamus, cerebellum, spinal cord, orbital globe
Giannini et al, Cancer 1999; 85:2033-2045; Pahapill et al, Neurosurgery 1996;

38:822-829
Figure 5-2-19
Pleomorphic Xanthoastrocytoma [Figure 5-2-19]
WHO Grade II:

Pleomorphism
Lipidized neoplastic glial cells: xanthoma-like
Overall good prognosis
81% 5-year survival
70% 10-year survival
Higher recurrence rate
Malignant transformation: 20%
Kepes et al, WHO classification 2000, 52-54; Giannini et al,

Cancer 1999; 85:2033-2045
Classic: large cystic lesion with an enhancing mural

nodule (<50%)
Meningocerebral location
Meningeal infiltration rare
Cortical involvement common
Perivascular space extension
Calcification rare
Usually no surrounding edema
PXA with vacuoles secondary to lipidized

neoplastic glial cells
Figure 5-2-20
Tien et al, AJR 1992:159:1397-1404; Lipper et al, AJNR

1993; 14:1397-1404; Pahapill et al, Neurosurgery 1996;
38:822-829
Pleomorphic Xanthoastrocytoma [Figure 5-2-20]
MR findings
Heterogeneous mass
T1WI: hypo-to-isointense
T2WI: hyper-to-isointense
Soft tissue component usually enhances
intensely
Leptomeningeal enhancement: characteristic
PXA as large heterogeneous mass

and enhancing soft tissue component
Lipper et al, AJNR 1993; 14:1397-1404
Supratentorial Primitive Neuroectodermal Tumor
Cerebral medulloblastoma
1% of all pediatric CNS neuroepithelial tumors
6% of all pediatric PNETs
Age range: 4 weeks to 10 years (mean age: 5.5 years)
Males more common (2:1)
Rorke et al, WHO classification, 2000, 141-144
1052
Neuroradiology
Nonspecific clinical features related to site

Cerebrum: seizures, increased intracranial pressure, motor deficits
Suprasellar region: visual or endocrine problems
Not pineal: pineoblastoma
Poor prognosis, especially in children < 2 years old
34% 5-year survival rate (up to 85% for cerebellar medulloblastoma)
WHO grade IV
Virtually identical to medulloblastoma
Undifferentiated or poorly differentiated neuroepithelial cells
Dense cellularity: small blue cell tumor
Pleomorphism
Rosette formation

[Figure 5-2-21]

CT: iso-to-hyperattenuated
Cysts or necrosis common
Calcification: 50%-70%
Vasogenic edema present but not usually
extensive
T1WI: hypointense to gray matter
T2WI: hypointense predominantly
Figure 5-2-21
Atypical Teratoid / Rhabdoid Tumor
First reported in 1978

Various names
Rhabdomyosarcomatoid variant of Wilms tumor
Embryonal small cell tumor
Rhabdoid tumor
Biologic behavior and some histologic features
similar to malignant rhabdoid tumor of the kidney
Beckwith and Palmer, Cancer 1978;41:1937-1948; Rorke

and Biegel, WHO classification 2000, 145-148; Rorke et
al, J Neurosurg 1996; 85:56-65; Hanna et al, AJNR
1993; 14:107-115
Atypical Teratoid / Rhabdoid Tumor
2.1% of all primary CNS tumors in children

94%: < 5 years old
Rarely presents in adults (4 cases)
Males more common (1.4:1)
Non-specific symptoms (lethargy, failure to thrive)
Head tilt
Cranial nerve palsy: usually VI or VII
Rorke and Biegel, WHO classification 2000, 145-148;

Burger et al, Am J Surg Pathol 1998; 22:1083-1092
Supratentorial PNET with CSF dissemination

Neuroradiology
1053
Atypical Teratoid / Rhabdoid Tumor [Figure 5-2-22]
Figure 5-2-22
WHO grade IV
Soft lobulated mass
Necrosis and hemorrhage common
Rhabdoid cells
Mixed with primitive neuroepithelial, epithelial, and
mesenchymal elements
Not a germ-cell tumor
Poor prognosis: most die within one year
Rorke and Biegel, WHO classification, 2000, 145-148
Posterior fossa: most common location (52%)

Especially cerebellopontine angle
Supratentorial: 39%
Including intraventricular
Pineal: 5%
Spinal: 2%
Multifocal: 2%
90% have monosomy or deletion of

chromosome 22
Figure 5-2-23
Rorke and Biegel, WHO classification, 2000, 145-148
Imaging: often mimics medulloblastoma

CT: hyperattenuated
Cysts and hemorrhage common
Vasogenic edema common
Hanna et al, AJNR 1993:14:109-114; Caldemyer et al, Pediatr

Neurosurg 1994; 21:232-236; Rorke and Biegel, WHO classification,
2000, 145-148

MR findings
T1WI: hypo-to-isointense
T2WI: variable
Soft tissue: iso-to-hypointense
Cyst-like: hyperintense
Heterogeneous enhancement
Subarachnoid seeding: 33% at presentation
Posterior fossa ATRT
Hanna et al, AJNR 1993;14:109-115; Rorke and Biegel, WHO classification, 2000,
145-148
Figure 5-2-24
Posterior fossa ATRT. Hyperattenuation on CT is similar to medulloblastoma but shows foraminal

extension on MR images
1054
Neuroradiology
Summary - Lymphoma
Rapid increase in prevalence

Fourth most common primary cerebral neoplasm
Second most common intracranial mass in immunocompromised patient
Periventricular
CT: hyperattenuation
T2W: hypointense
Peripheral Mass
Ganglioglioma
Variable size with common calcification
Most common cause of chronic temporal lobe epilepsy
Gangliocytoma: lacks glial component
Dysembryoplastic neuroepithelial tumor (DNT)
Almost always involves cortical margin
Uncommon calcification
Temporal lobe: 62%
Soap bubble appearance
Cerebellar Mass
Striated cerebellar mass

Dysembryoplastic cerebellar gangliocytoma (Lhermitte-Duclos)
With fat content
Cerebellar liponeurocytoma
Meningocerebral interface
Desmoplastic infantile ganglioglioma
Large heterogeneous cerebral hemisphere mass
No calcification
Pleomorphic xanthoastrocytoma
Cyst-like mass with enhancing mural nodule
Calcification rare
Older patients: adolescents and young adults
Young child
Supratentorial PNET
Large heterogeneous cerebral hemisphere mass
Cerebral medulloblastoma
Atypical teratoid / rhabdoid tumor
Predilection for posterior fossa
Mimics medulloblastoma
Subarachnoid seeding common
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System. IARC Press Lyon, 2000, page 145-148.
42. Rorke LB, Hart MN, McLendon RE: Supratentorial primitive neuroectodermal tumour (PNET). In: Kleihues P,
Cavenee WK, eds. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of
the Nervous System. Edited by: Kleihues P, Cavenee WK. Lyon: IARC Press; 2000:141-144.
43. Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and
childhood: definition of an entity. J Neurosurg 1996; 85:56-65.
44. Rosenblum ML, Levy RM, Bredesen DE, So YT, Wara W, Ziegler JL. Primary central nervous system lymphomas
in patients with AIDS. Ann Neurol 1988; 23 Suppl:S13-16.
45. Schwaighofer BW, Hesselink JR, Press GA, Wolf RL, Healy ME, Berthoty DP. Primary intracranial CNS
lymphoma: MR manifestations. AJNR Am J Neuroradiol 1989; 10:725-729.
46. Silver JM, Rawlings CE, 3rd, Rossitch E, Jr., Zeidman SM, Friedman AH. Ganglioglioma: a clinical study with
long-term follow-up. Surg Neurol 1991; 35:261-266.
47. Tampieri D, Moumdjian R, Melanson D, Ethier R. Intracerebral gangliogliomas in patients with partial complex
seizures: CT and MR imaging findings. AJNR Am J Neuroradiol 1991; 12:749-755.
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51. Tien RD, Tuori SL, Pulkingham N, Burger PC.Ganglioglioma with leptomeningeal and subarachnoid spread:
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52. VandenBerg SR, May EE, Rubinstein LJ, et al. Desmoplastic supratentorial neuroepithelial tumors of infancy with
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Neuroradiology
1057
Cerebral Intraventricular Neoplasms

Ten Most Common Intraventricular Tumors*
Ependymoma (18%)
Subependymoma (11%)
Central Neurocytoma (10%)
Subependymal Giant Cell
Astrocytoma (6%)
Other Astrocytomas (9%)
Colloid Cyst (4%)
Choroid Plexus Papilloma (24%)

Choroid Plexus Carcinoma (2%)
Meningioma (6%)
Metastasis (2%)
*Based on 397 cases in AFIP Archives
Ependymoma
Arise from ependymal cells of ventricular wall

Also central canal of spinal cord
3%-9% of all neuroepithelial tumors
6%-12% of pediatric brain tumors
30% of all brain tumors in children < 3 years of age
3rd WHO classification, 2000
Ependymoma [Figure 5-3-1]
Broad age range

Posterior fossa: 6 years old (mean)
Supratentorial: 18-24 years old (mean)
Fourth ventricle: 58%
Lateral & third ventricle: 42%
Supratentorial: more commonly extraventricular
Rare: ovaries, soft tissues, mediastinum, sacrococcygeal region
JCAT 1995; 19:518-526; Childs Nerv Syst 1991; 7:177-182;

J Neurosurg 1979; 51:383-391
Figure 5-3-1
Ependymoma
Increased intracranial pressure and hydrocephalus

Adults: 5-year survival rate = 57%, 10-year = 45%
Less favorable prognosis
Children: especially those under 2 years of age
Fourth ventricle location
Recurrence common
Gross total resection curative
Radiation therapy in partial resection cases
3rd WHO classification, 2000; Childs Nerv Syst 1990; 6:375-378;

Neurosurgery 1995; 37:655-667; Neurosurgery 1993; 32:169-175
Ependymoma
May extend into brain
Fourth ventricle: foraminal extension common
Ependymoma extending into

cerebellopontine angle from fourth
ventricle
1058
Neuroradiology
Ependymoma
Figure 5-3-2
WHO grade II
Perivascular pseudorosettes
Rare mitotic figures
Variants
Cellular
Papillary
Clear cell
Tanycytic
Anaplastic
Ependymoma
CT: iso- to hyperattenuated

Calcification: 40%-80%
Enhancing soft tissue component
Non-enhancing cyst-like portion
MR: heterogeneous
Isointense to gray matter on T1WI
Hyperintense to gray matter on T2WI
Post-op residual: decreased survival
Ependymoma with calcification on

non-contrast CT
Radiology 1982; 143:97-101; Br J Radiol 1994; 67:223-243;

JCAT 1995; 19:518-526; Neurosurgery
1991; 28:666-672
Figure 5-3-3
Subependymoma

Arise from subependymal glial layer
Incidence: 0.4 (asymptomatic) - 0.7%
(symptomatic)
Most smaller than 2 cm diameter
Symptomatic: 3-5 cm
Hydrocephalus (80%), focal
neurologic deficit (27%), seizures
(9%), subarachnoid hemorrhage
(4.5%)
J Neurosurg 1945; 2:232-240; Acta

Neurochir 1989; 96:15-25;
AJR 1995; 165:1245-1250; Neurosurgery
1986; 19:594-598
Ependymoma with foraminal extension on MR
Subependymoma
Males more common

Older than 15 years of age: 82%
Fourth ventricle: > 50%
Lateral ventricle: ~ 45%
Well-circumscribed avascular mass
Pedicular attachment to ventricular wall
Neurosurgery 1986; 19:594-598; J Neurosurg 1978: 49:689-696
Subependymoma
Expansive but not infiltrative

WHO grade I
Dense fibrillary matrix
Cysts and nests
Low mitotic activity
Low recurrence rate
10% mixed with ependymoma or other tumor
Neurosurgery 1986; 19:594-598; 3rd WHO classification, 2000

Neuroradiology
1059
Subependymoma
CT: lobulated mass

Iso-to-slight hypoattenuated
Calcification: 32%
Cystic degeneration: 18%
At least some enhancement: 84%
MR: hypointense compared to white matter on T1WI
Hyperintense on T2WI
Figure 5-3-4
Neurosurgery 1986; 19:594-598; AJR 1995; 165:1245-1250;

AJNR 1995; 16:2121-2129; AJNR 1990; 11:83-91; Surg Neurol 1990;
33:329-335
Ependymoma
vs.
Subependymoma
Iso- to hyperattenuated
Iso-to-hypoattenuated
Calcification, cysts more

common
Calcification, cysts
less common
ntense enhancement more

common
More variable enhancement
Extraventricular extension
Rarely extends beyond

ventricular margin
Subependymoma with calcification

in right frontal horn on CT
Figure 5-3-5
Neurosurgery 1986; 19:594-598
Central Neurocytoma

Confusion with intraventricular oligodendroglioma
Central: lateral and third ventricles
Extraventricular central neurocytoma for those located
elsewhere (brain, cerebellum, spinal cord)
0.25%-0.5% of all intracranial tumors
Acta Neuropathol 1982;56:151-156; 3rd WHO classification, 2000;

Brain Pathol 1993; 3:297-306
Central Neurocytoma
Broad age range: 8 days to 67 years

Mean age: 29 years
50%: 20-30 years of age
75%: 20-40 years of age
Short clinical course (mean: 3 months)
Increased intracranial pressure, mental status changes, visual
deficits
3rd WHO classification, 2000; Brain Pathol 1993; 3:297-306
Central Neurocytoma
Arise from septum pellucidum or ventricular wall

Lateral ventricle near foramen of Monro: 50%
Lateral and third ventricles: 15%
Bilateral: 13%
Third ventricle alone: 3%
Surg Neurol 1998: 49:197-204; 3rd WHO classification, 2000
1060
Subependymoma of right lateral

ventricle on axial T1-weighted
pre-contrast and post-contrast
images
Neuroradiology
Central Neurocytoma [Figure 5-3-6]
Figure 5-3-6
WHO grade II
Striking resemblance to oligodendroglioma
Fried egg appearance
Pineocytomatous rosettes
Calcification: 50%
Neuronal differentiation
? Glial differentiation
Central Neurocytoma [Figure 5-3-7]
Well-circumscribed lobulated mass

Broad attachment to septum pellucidum or ventricular wall
CT: hyperattenuated compared to brain
Cyst-like areas: 66%
Calcification: 50%
MR: hyperintense compared to white matter on T1WI and T2WI
Hemorrhage rare
Central neurocytoma with

characteristic "fried egg"
histologic appearance
Neuroradiology 1991; 33:143-148; JCAT 1989: 13:495-497
Figure 5-3-7
Central neurocytoma with bilateral involvement on CT and MR in a

24-year-old woman with headaches and no neurological deficit
Subependymal Giant Cell Astrocytoma
Most common brain neoplasm in tuberous sclerosis (6%-16%)

Neurocutaneous phakomatosis
Autosomal dominant in 20%-50% of cases
Tubers and subependymal nodules: 90%-100%
Can it occur without TS? - controversial
1.4% of all pediatric brain tumors
Most: first or second decades (mean: 11 years)
Figure 5-3-8
Neurosurgery 1991;28:864-868; 3rd WHO classification;

Pediatr Radiol 1992;22:485-489; Pediatr Neurosurg 1994: 20:233-239
Subependymal Giant Cell Astrocytoma [Figure 5-3-8]
Virtually always located near the foramen of Monro

Hydrocephalus, seizures
Higher incidence of cardiac rhabdomyomas
Surgical resection for symptomatic SEGA or with documented growth
Non-responsive to radiation therapy
3rd WHO classification, 2000; Pediatr Radiol 1992; 22:485-489

Subependymal giant cell
astrocytoma with characteristic
location near foramen of Monro
Neuroradiology
1061
WHO grade I
Slow growth with benign biologic behavior and low recurrence rate
Earlier diagnosis associated with longer survival
Probably arise from subependymal nodules
Mixed glioneuronal pattern
Figure 5-3-9
Pediatr Neurosurg 1994: 20:233-239
Subependymal Giant Cell Astrocytoma [Figure 5-3-9]
CT: iso- to slightly hypoattenuated

MR: hypointense compared to white matter on T1WI,

heterogeneously hyperintense on T2WI
Neonates: hyperintense on T1WI, hypointense on T2WI
Intense enhancement on CT and MR
Distinguishes SEGA from most subependymal nodules
Annual surveillance screening post-op and in first-degree
relatives
Pediatr Neurosurg 1994; 20:233-239; AJNR 1999: 20: 907-916;

Pediatr Radiol 1991; 21:432
Other TS manifestations
Cortical tubers
Adenoma sebaceum
Shagreen patch
Retinal hamartoma
Renal angiomyolipoma
Cardiac rhabdomyoma
Rectal polyps
Subependymal giant cell astrocytoma

with calcification on CT
Figure 5-3-10
Colloid Cyst
Most common neuroepithelial cyst: probably arises

from endoderm
Young to middle-aged adults
Positional headache: acute CSF obstruction
Antero-superior third ventricle
Variable composition: mucoid material with old
blood, cholesterol crystals, serous fluid,
paramagnetic ions
Lach et al, J Neurosurg 1993; 78:101-111;

Shaungshotti et al, Arch Pathol Lab Med 1965; 80:214224
Colloid Cyst [Figure 5-3-10]
Variable appearance
Hyperattenuated on CT
Hypo-to-hyperintense on T1WI
Hypo-to-hyperintense on T2WI
May ring enhance
Solid enhancement: not colloid cyst
Waggenspack and Guiunto, AJNR 1989;10:105-110;

Lach et al, J Neurosurg 1993; 78:101-111
1062
Colloid cyst on non-contrast axial CT, axial T2weighted, sagittal T1-weighted, contrast-enhanced
coronal T1-weighted images
Neuroradiology
Choroid Plexus
Neuroepithelial tissue
Cerebrospinal fluid (CSF) production: 450 ml/day (avg.)
Atrium of the lateral ventricle
Foramen of Monro and third ventricle
Fourth ventricle and foramen of Luschka
Absent in cerebral aqueduct
Choroid Plexus Tumors
Lateral ventricle (50%), fourth ventricle (40%), third ventricle (5%)

Multiple: 5%
Lateral ventricle: no gender predilection
Fourth ventricle: males more common
0.4%-0.6% of all intracranial tumors
2%-4% of pediatric brain tumors
10%-20% of brain tumors in less than 1 year of age
50% manifest in first decade
Incidence: 0.3 per million
Figure 5-3-11
J Neurosurg 1988: 69: 843-849
Hydrocephalus
Increased CSF production
CSF flow obstruction
Hemorrhage: CSF absorption
Association with Li-Fraumeni and Aicardi syndromes
Neurosurgery 1989; 25:327-335; J Neurosurg 1952; 9:59-67;

J Neurosurg 1998; 88:521-528; Radiology 1989; 173:81-88
Choroid plexus papilloma with

Pedicular attachment common
histology similar to that of normal
Lateral ventricle: trigone
choroid plexus tissue
Third ventricle: roof
Fourth ventricle: posterior medullary velum
Bobble-head doll syndrome: intermittent ventricular obstruction
CSF Seeding: occurs in both papillomas and carcinomas
Papilloma
vs.
Choroid plexus papilloma

80%
WHO grade I
100% 5-year survival
Children and adults
Lateral ventricle: 1st
decade
Fourth ventricle: first 5
decades
Carcinoma
Choroid plexus carcinoma
20%
WHO grade III
26-50% 5-year survival
Much more common in children
Post-op residual disease: very poor
prognostic factor
Necrosis, parenchymal invasion
Choroid Plexus Papilloma [Figure 5-3-11]
Histology resembles normal choroid plexus

Prominent fronds of fibrovascular connective tissue
WHO grade I
Neuroradiology
1063
Choroid Plexus Carcinoma [Figure 5-3-12]
Figure 5-3-12
Hypercellularity
Nuclear pleomorphism
High nucleus-cytoplasm ratio
Mitotic activity
Brain parenchyma invasion
WHO grade III
Choroid Plexus Papilloma
Well-circumscribed cauliflower-like mass

Prominent lobulated margin
CT: iso-to-hyperattenuated
Calcification: 24%
Lateral ventricle: more common on left side? - Probably not
Foraminal extension characteristic
Choroid plexus carcinoma with

markedly heterogeneous histologic
appearance
3rd WHO classification, 2000; J Neurosurg 1998; 88:521-528; Radiology 1989;

173:81-88
Choroid Plexus Papilloma [Figure 5-3-13]
MR: iso-to-hypointense compared to normal brain parenchyma on T1WI

Flow voids common
Enlarged choroidal artery
Amenable to pre-operative embolization
US: lobulated echogenic mass
Bi-directional flow throughout diastole
3rd WHO classification, 2000; Radiology 1989: 173:81-88
Choroid Plexus
Carcinoma [Figure 5-3-14]
Figure 5-3-13
More heterogeneous
Extraventricular extension
Vasogenic edema
Slightly less hydrocephalus?
3rd WHO classification, 2000;

J Neurosurg 1998; 88:521-528;
Radiology 1989; 173:81-88
Choroid plexus papilloma of left lateral ventricle atrium on MR
Figure 5-3-14
Choroid plexus carcinoma with extension into

adjacent brain parenchyma and spread within
ventricles
1064
Neuroradiology
Intraventricular Meningioma
Most common atrial mass in adults

Usually older than 30 years of age
Mean: 42 years
Lateral ventricle >> third or fourth ventricle
Arise from arachnoidal cap cells within choroid plexus, tela choroidea, or
velum interpositum
0.7% of all meningiomas
Almost all are benign
Predilection for sarcomatous change in children
Figure 5-3-15
Neurosurgery 1987; 20:465-468; AJNR 1995; 16:1378-1381;

Radiology 1984; 153:435-442
CT: well-defined globular mass

Hyperattenuated compared to brain
Calcification: 50%
Neurosurgery 1987: 20:465-468; AJNR 1995; 16:1378-1381;

Surg Neurol 1994: 42:41-45
Intraventricular Meningioma [Figure 5-3-15]
MR: iso-to-hypointense compared to gray matter on T1WI

Iso-to-hyperintense on T2WI
MRS: decreased NAA, creatine
Increased choline
Intraventricular meningioma on
contrast-enhanced axial T1weighted MR image
AJNR 1999; 20:882-885; AJNR 1994; 15:435-444
Choroid Plexus Metastasis
Rare: 0.9-4.6% of all cerebral metastasis

Renal cell carcinoma and lung carcinoma: most common in adults
Children: neuroblastoma, Wilms tumor, retinoblastoma
Others: melanoma, gastric carcinoma, colon carcinoma, lymphoma
Lateral ventricle: most common
Renal cell carcinoma metastasis may mimic meningioma
Figure 5-3-16
South Med J 1998; 91:1159-1162; Neurosurgery 1983; 13:430-434
Choroid Plexus Metastasis
[Figure 5-3-16]
CT: iso- or hyperattenuated

MR: hypointense on T1WI, hyperintense on T2WI
Intense enhancement usually
Br J Radiol 1994; 67:223-243
Summary
Fourth Ventricle
Ependymoma
Subependymoma
Third ventricle
Colloid Cyst
All the others: less common
Lateral Ventricle (anterior half)
Subependymoma
Central Neurocytoma
Ependymoma
Astrocytoma
Neuroradiology
Choroid plexus metastasis

from renal cell carcinoma
1065
Lateral Ventricle (posterior half)

Choroid Plexus Papilloma / Carcinoma
Meningioma
Metastasis
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Neuroradiology
1067
Imaging of the Temporal Bone: Anatomy

and Congenital Lesions
Middle Ear
Epitympanum
Malleus head
Short process of incus
Mesotympanum
Muscles: tensor tympani (V3), stapedius (VII)
Ossicles: rest of malleus and incus, stapes
Ligaments
Nerves: chorda tympani (VII), Jacobsons nerve (IX)
Inner Ear: Cochlea
Anterior to vestibule
Promontory: bony ridge
Modiolus: central axis, cochlear nerve
Apex (cupola)
Basal turn
Cochlear aqueduct: perilymphatic duct
Inner Ear: Vestibule
Posterior to cochlea
Oval window niche: partition from middle ear
Lamina cribrosa: partition from internal auditory canal (IAC)
Vestibular aqueduct: endolymphatic duct
Inner Ear: Semicircular Canals (SCC) [Figures 5-4-1 to 5-4-15]
Lateral (horizontal)
Superior: arcuate eminence
Posterior
Oriented 90 to each other
Rotational acceleration
Figure 5-4-1
Axial CT at superior portion of temporal

bone shows lumen of superior
semicircular canal and upper portion of
mastoid antrum and epitympanum
Figure 5-4-2
Subarcuate artery canal traversing

through hoop of superior semicircular
canal
Temporal Bone Anatomy and Congenital Lesions
1068
Neuroradiology
Figure 5-4-3
Superior portion of vestibule with malleus
(anterior) and incus (posterior) within the
epitympanum
Figure 5-4-4
Superior portion of internal auditory canal
with labyrinthine canal containing the
facial nerve on its way to the geniculate
ganglion and tympanic facial segment
along medial wall of middle ear
Figure 5-4-5
Superior portion of cochlea and internal
auditory canal. Note vestibular aqueduct
arising from posterior margin of temporal
bone
Mid-portion of internal auditory canal and

cochlea. Sinus tympani is located just lateral
to vestibule. Small bony peak lateral to
vestibule is pyramidal eminence. Facial nerve
canal is located posterolateral to pyramidal
eminence. Middle ear shows parallel lines
sign: tensor tympani tendon anteriorly and
incudostapedial junction with stapedial struts
posteriorly. Struts mark the site of the oval
window
Figure 5-4-6
Figure 5-4-7
Inferior portion of cochlea. Bony plate
separating it from the middle ear is the
cochlear promontory. Note jugular bulb in
posterior temporal bone
Figure 5-4-8
Neuroradiology
Basilar turn of cochlea. Internal carotid

artery is seen anterior to the cochlea and
is delimited from the middle ear by a
bony plate. Eustachian tube arises just
lateral to the artery and heads along an
anteromedial pathway towards the
nasopharynx
1069
Coronal view shows internal carotid

artery inferiorly separated from middle
ear by bony plate. Cochlea is located
immediately superior. Geniculate
ganglion is located just superolateral to
cochlea. Middle ear contains ossicles
(malleus anterior to incus) within
epitympanum. Tegmen tympani is bony
plate separating middle ear from brain
Figure 5-4-9
Figure 5-4-10
Moving posteriorly, facial nerve segments
are seen above and lateral to cochlea
Figure 5-4-11
Cochlea is separated by cochlear
promontory from middle ear. Anterior
portion of internal auditory canal is just
coming into view. Scutum is well seen
along superior margin of medial external
auditory canal
Figure 5-4-12
Mid-portion of internal auditory canal with

crista falciformis along its lateral margin.
Vestibule now appears with lateral and
superior semicircular canals. Facial
nerve is located immediately inferior to
lateral semicircular canal and above oval
window
Figure 5-4-13
Posterior margin of vestibule with facial
nerve as small soft tissue just prior to
reaching posterior genu
Figure 5-4-14
Facial nerve at posterior genu
1070
Neuroradiology
Figure 5-4-15
Mastoid segment of facial nerve
extending inferiorly to the stylomastoid
foramen
Outer ear
Middle ear
Inner ear
Vascular
Internal carotid artery
Jugular vein
Cholesteatoma
Encephalocele
Congenital Malformations
External and middle ear (1st and 2nd branchial arch) develop independent of
inner ear (ectodermal)
IAC development separate from inner ear development
Anomalies of all 3 parts are rare
Dysplasias and trisomies 13, 18, 21
Fisher and Curtin, Otolaryngol Clin North Am 1994; 27:511-531
Outer Ear Anomalies
Congenital aural dysplasia

Bilateral: 33%
Genetic disorder association
External auditory canal (EAC) atresia: failure of recanalization
(26th gestational week)
Fibrous vs. bony plate
CT: middle ear dysplasia or cholesteatoma
Figure 5-4-16
Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al,

AJNR 1997; 18:53-65
Middle Ear Anomalies
Temporomandibular joint (TMJ) anomalies

TMJ higher and more posterior than normal
Facial nerve displaced
Vertical portion more anterior than normal
Very important pre-operative finding
Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al,

AJNR 1997; 18:53-65
Inner Ear Anomalies [Figure 5-4-16]
Lateral semicircular canal anomaly

Last semicircular canal to form
Usually short and wide, less commonly narrow
Anomalous shortening and widening

of lateral semicircular canal
Jackler and Luxford, Laryngoscope 1987; 97:2-14
Neuroradiology
1071
Complete Labrynthine Aplasia
Figure 5-4-17
Michels deformity
3rd gestational week
Inner ear absent
Small cystic cavity: single or multiple
Incomplete Partition / Dilatational Defects [Figure 5-4-17]
Mondinis dysplasia (1791): cochlea with 1 and 1/2 turns

Second most common form of congenital deafness (Schiebes
deafness #1)
7th gestational week
Small cochlea with incomplete or absent intrascalar septum
Basilar turn present
Common cavity in place of middle and apical turns
Paparella, Ann Otol Rhinol Laryngol Suppl 1980; 89(2 Pt 3):1-10
Other Cochlear Anomalies [Figure 5-4-18]
Common cavity
Mondini dysplasia
Cochlea and vestibule fused

25% of all cochlear anomalies
Cochlear aplasia
Rest of inner ear normal or malformed

Cochlear Hypoplasia
Figure 5-4-18
Small cochlear bud
Large Endolymphatic Duct and Sac (LEDS)
Large vestibular aqueduct syndrome

Dilated vestibular aqueduct
Most common radiologic finding in early-onset SNHL
> 1.5 mm diameter (lateral SCC)
MR: look at T2W images
Often associated with incomplete partition cochlear anomalies
Progressive sensorineural hearing loss (SNHL)
Etiology: hyperosmolar protein transmission?
Valvassori and Clemis, Laryngoscope 1978; 88:723-728; Mafee,

AJNR 1992;13:805-819; Jackler and De la Cruz, Laryngoscope 1989;
99:1238-1243; Dahlen et al, AJNR 1997; 18:67-75; Davidson et al,
AJNR 1999; 20:1435-1441
Inner Ear Anomalies: Associations
Otocraniofacial
Crouzons, Aperts, etc.
Otocervical
Klippel-Feil, Goldenhars, etc.
Otoskeletal
Osteogenesis imperfecta, osteopetrosis, etc.
Enlarged vestibular aqueduct. Note

size in comparison to lateral
semicircular canal
Romo, Casselman, and Robson in Som and Curtin, Head and Neck Imaging, 4th
ed., Mosby, 2003
1072
Neuroradiology
Aberrant Internal Carotid Artery [Figure 5-4-19]
Figure 5-4-19
90% females
More common on right side
Pulsatile tinnitus, conductive hearing loss (HL), otalgia
Enhancing mass in hypotympanum (inferior tympanic canaliculus)
Sinnreich et al, Otolaryngol Head Neck Surg 1984; 92:194-206;

Thiers et al, AJNR 2000; 21:1551-1554
Absent Internal Carotid Artery
May be incidental discovery

High association with intracranial aneurysms
Nearly 30% present with subarachnoid hemorrhage
Aberrant internal carotid artery with

soft tissue attenuation within middle
ear and absent bony margin (Case
courtesy of Wendy Smoker, MD,
FACR)
Keen, Clin Proc 1946; 4:588-594; Martinez-Granero et al, Rev Neurol

1997; 25:1207-1209
Persistent Stapedial Artery
Figure 5-4-20
Rare: most seen at surgery

Precursor for middle meningeal artery
Small canal from carotid canal
Crosses cochlear promontory
Widened facial canal
Absence of foramen spinosum
Thiers et al, AJNR 2000; 21:1551-1554
High Jugular Bulb (Megabulb) [Figure 5-4-20]
Various definitions described

Most common vascular anomaly of petrous temporal bone
3%7% incidence
More common on right side
75% jugular vein larger on right
Usually poorly pneumatized mastoids
No bony dehiscence
Importance: surgical impact
Overton and Ritter, Laryngoscope 1973; 83: 1986-1991; Caldemyer et

al, RadioGraphics 1997; 17:1123-1139
Other Jugular Vein Anomalies
Dehiscent jugular bulb

Direct communication with middle ear
Lateral: pulsatile tinnitus, conductive hearing loss
Medial: Menieres disease
Jugular diverticulum
Above, medial, posterior to petrous pyramid
More common on left-side and in females
High jugular bulb with dehiscence

along internal auditory canal
Figure 5-4-21
Couloigner et al, Eur Arch Otorhinolaryngol 1999; 256:224-229;

Pappas et al, Otolaryngol Head Neck Surg, 1993; 109:847-852
Pial siderosis with thin bands of T2

hypointensity secondary to
subarachnoid hemorrhage
Neuroradiology
1073
Pulsatile Tinnitus Lesions
Figure 5-4-22
[Figures 5-4-21 and 5-4-22]
Congenital
Aberrant internal carotid artery
Dehiscent jugular bulb
Tumor
Paraganglioma
Hemangioma
Vascular
Arteriovenous malformation / fistula
Aneurysm
Pial siderosis: VIII n. especially prone
Congenital Cholesteatoma (Epidermoid)
Abnormal flow voids secondary to arteriovenous

fistula
[Figures 5-4-23 and 5-4-24]
Child with conductive HL

Aberrant epithelial rests
Epitympanum, incudostapedial joint > petrous
apex
Globular mass +/ bone destruction
Follows fluid signal intensity
May have peripheral enhancement
Figure 5-4-23
Figure 5-4-24
Peron and Schuknecht, Arch Otolaryngol 1975:

101:498-505; Gao et al, AJNR 1992; 13:863-872
Congenital Dehiscence of Tegmen

Tympani [Figure 5-4-25]
Fusion of petrosquamosal suture by 1 year of

age
Tiny openings: up to 34% of population
Encephalocele, fistula: rare (requires dural
weakening)
Ossicular epidermoid with
Coronal plane best
bone erosion
Gavilan et al, Arch Otolaryngol 1984; 110-206-207;
Gottlieb et al, Arch Otolaryngol 1998; 124:1274-1277
Contrast-enhanced coronal
T1-weighted image shows
rim enhancement of
epidermoid involving right
temporal bone
Figure 5-4-25
Axial T2-weighted image shows focal hyperintensity in region of epitympanum.

Coronal CT images show soft-tissue density in epitympanum. Surgical exploration
confirmed encephalocele
1074
Neuroradiology
References
1.
2.
3.
4.
5.
6.
7.
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10.
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12.
13.
14.
15.
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17.
18.
19.
20.
21.
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Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR. The jugular foramen: a review of anatomy, masses, and
imaging characteristics. Radiographics 1997; 17:1123-1139.
Couloigner V, Grayeli AB, Bouccara D, Julien N, Sterkers O. Surgical treatment of the high jugular bulb in patients
with Meniere's disease and pulsatile tinnitus. Eur Arch Otorhinolaryngol 1999; 256:224-229.
Dahlen RT, Harnsberger HR, Gray SD, et al. Overlapping thin-section fast spin-echo MR of the large vestibular
aqueduct syndrome. AJNR Am J Neuroradiol 1997; 18:67-75.
Davidson HC, Harnsberger HR, Lemmerling MM, et al. MR evaluation of vestibulocochlear anomalies associated
with large endolymphatic duct and sac. AJNR Am J Neuroradiol 1999; 20:1435-1441.
Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation. Epidermoid tumor of the
cerebellopontine angle. AJNR Am J Neuroradiol 1992; 13:863-872.
Gavilan J, Trujillo M, Gavilan C. Spontaneous encephalocele of the middle ear. Arch Otolaryngol 1984; 110:206207.
Gottlieb MB, Blaugrund JE, Niparko JK. Imaging quiz case 1. Tegmental encephalocele. Arch Otolaryngol Head
Neck Surg. 1998 Nov;124(11):1274, 1276-7.
Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope 1989; 99:1238-1242;
discussion 1242-1233.
Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on
embryogenesis. Laryngoscope 1987; 97:2-14.
Keen JA. Absence of both internal carotid arteries. Clin Proc 1945-1946;4:588
Mafee MF, Charletta D, Kumar A, Belmont H. Large vestibular aqueduct and congenital sensorineural hearing
loss. AJNR Am J Neuroradiol 1992; 13:805-819.
Martinez-Granero MA, Martinez-Bermejo A, Arcas J, et al. [Unilateral agenesis of the internal carotid artery in
childhood: description of a case]. Rev Neurol 1997; 25:1207-1209.
Mayer TE, Brueckmann H, Siegert R, Witt A, Weerda H. High-resolution CT of the temporal bone in dysplasia of
the auricle and external auditory canal. AJNR Am J Neuroradiol 1997; 18:53-65.
Overton SB, Ritter FN. A high placed jugular bulb in the middle ear: a clinical and temporal bone study.
Laryngoscope 1973; 83:1986-1991.
Paparella MM. Mondini's deafness. A review of histopathology. Ann Otol Rhinol Laryngol Suppl 1980; 89:1-10.
Pappas DG, Jr., Hoffman RA, Cohen NL, Holliday RA, Pappas DG, Sr. Petrous jugular malposition (diverticulum).
Otolaryngol Head Neck Surg 1993; 109:847-852.
Peron DL, Schuknecht HF. Congenital cholesteatomata with other anomalies. Arch Otolaryngol 1975; 101:498505.
Robson CD, Robertson RL, Barnes PD. Imaging of pediatric temporal bone abnormalities. Neuroimaging Clin N
Am 1999; 9:133-155.
Romo LV, Casselman JW, Robson CD. Temporal Bone: Congenital Anomalies. In: Som P.M., Curtin H.D. (eds)
Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1275-1360.
Sinnreich AI, Parisier SC, Cohen NL, Berreby M. Arterial malformations of the middle ear. Otolaryngol Head
Neck Surg 1984; 92:194-206.
Smith ME, Fisher C, Weiss SW. Pleomorphic hyalinizing angiectatic tumor of soft parts. A low-grade neoplasm
resembling neurilemoma. Am J Surg Pathol 1996; 20:21-29.
Thiers FA, Sakai O, Poe DS, Curtin HD. Persistent stapedial artery: CT findings. AJNR Am J Neuroradiol 2000;
21:1551-1554.
Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope 1978; 88:723-728.
Neuroradiology
1075
Imaging of the Temporal Bone: Infectious

and Neoplastic Lesions
Figure 5-5-1
Conductive Hearing Loss
Ossicular motion impeded

Cholesteatoma
Hemangioma
Glomus tympanicum
Trauma: disruption
Congenital
Otosclerosis
Inflammatory Disease
Mechanism
Eustachian tube dysfunction
Decreased intratympanic pressure
Children: otitis media
Adults: nasopharyngeal carcinoma
Thin-section CT: soft tissue and fluid look alike
Hounsfield units not helpful
Nemzek and Schwartz in Som and Curtin,

Head and Neck Imaging, 4th ed, 2003, Mosby
Acquired Cholesteatoma
[Figures 5-5-1 to 5-5-5]
Exfoliated keratin within sac of stratified squamous epithelium

98% of middle ear cholesteatomas
Probably from retraction pocket in pars flaccida
Prussaks space: ossicles displaced medially
Bone destruction
Scutum and ossicles:coronal plane best
Pars tensa: lateral semicircular canal, axial plane best
Artist rendition of retraction pocket at

superior tympanic annulus caused by
negative intratympanic pressure
Figure 5-5-2
Buckingham and Valvassori, Otolaryngol Clin North Am 1973; 6:363
Figure 5-5-3
Retraction pocket fills with epithelial

debris from external auditory canal,
creating a cholesteatoma
Gross photograph of an acquired cholesteatoma,
a sac of keratin lined with squamous epithelium
Temporal Bone Infectious and Neoplastic Lesions
1076
Neuroradiology
Acquired Cholesteatoma
May not be able to distinguish from simple debris

early in course
MR: T1 & T2 prolongation
Does not enhance (granulation tissue does)
Treatment: excision or exteriorization
Open cavity (canal wall down) mastoidectomy
Radical: stapes left
Modified radical (Bondy): all ossicles left
Phelps and Lloyd, Radiology 1986; 37:359-364;

ODonoghue et al, Clin Otolaryngol 1987; 12:89; Ishii et
al, JCAT 1991; 15:934-937; Nemzek and Schwartz in
Som and Curtin, Head and Neck Imaging, 4th ed.,
Mosby, 2003
Acquired Cholesteatoma: Complications
Figure 5-5-4
Coronal CT image of acquired cholesteatoma with

erosion of the scutum and ossicles
[Figure 5-5-6]
Labyrinthine fistula: lateral SCC most common

Labyrinthitis
Facial nerve canal
Tegmen tympani: intracranial
Figure 5-5-5
Sigmoid sinus erosion / thrombosis

Automastoidectomy: into EAC
MR recommended
Silver et al, Radiology 1987; 164:47; Schwartz,

Radiology 1984; 153:443-447; Nemzek and Schwartz in
Som and Curtin,
Head and Neck Imaging, 4th ed, Mosby, 2003
Middle ear soft tissue without bone erosion (not

cholesteatoma!). Left: granulation tissue. Right:
middle ear fluid
Figure 5-5-6
Cholesteatoma with erosion of

mastoid bone adjacent to sigmoid
dural sinus
Neuroradiology
1077
Mastoiditis
Figure 5-5-7
Osteomyelitis: patchy opacification

Loss of mastoid septations
Demineralization
Coalescent: single cystic cavity
Complications
Bezold abscess: zygomatic root, EAC
Gradenigo syndrome: petrous apicitis
VI palsy, V neuralgia, chronic otitis
Sigmoid sinus thrombosis
Meningitis, epidural abscess
Castillo et al, AJR 1998; 17:1491-1495; Mafee et al, Radiology 1985;

155:391
Necrotizing External Otitis
Osteomyelitis: bone destruction

Diabetics: Pseudomonas
AIDS: Aspergillus
Cartilage portion: fissures of Santorini
Spreads rapidly into adjacent spaces
Parotid, facial nerve, intracranial
Goal: determine extent of disease by CT and MR
In-111 WBC study: post-therapy
Coalescent mastoiditis. Only a

single cavity remains within the
mastoid bone as a result of
osteomyelitis
Slattery and Brackmann, Otolaryngol Clin North Am 1996; 29:795-806; Ress et al,
Laryngoscope 1997; 107:456-460; Grandis et al, Radiology 1995: 196:499-504
External Ear Masses
Figure 5-5-8
[Figure 5-5-9]
Exostosis: chronic cold water exposure

Usually broad-based and bilateral
Bony portion of EAC
Not an osteoma
Usually unilateral, pedunculated,
and lateral to EAC
DiBartolomeo, Ann Otolaryngol 1979; 88(suppl 61):2-20;

Turetsky et al, AJNR 1990; 11:1217-1218
External Ear Masses
Keratosis obturans
< 40 years old
Sinusitis, bronchiectasis
Hearing loss
Smooth external auditory canal (EAC) widening
Entire EAC often filled
EAC cholesteatoma: 0.5% of all cholesteatomas
Otorrhea
Focal erosions
Mastoiditis with posterior fossa epidural abscess

(Case courtesy of Vanessa Albernaz, MD)
Figure 5-5-9
Piepergerdes et al, Laryngoscope 1980; 90:383-391
Bilateral exostoses
1078
Neuroradiology
External Ear Neoplasms [Figure 5-5-10]
Figure 5-5-10
Skin cancers
Most common malignant ear tumor
Basal cell carcinoma
Melanoma
Ceruminoma
Parotid tumors
Metastasis
Schuknecht, Pathology of the Ear, Harvard, 1974; Maya et al in Som

and Curtin, Head Neck Imaging, 4th ed, Mosby, 2003
Cerebellopontine Angle Masses: The AMEN

Acoustic schwannoma (6091%)

Meningioma (3%-7%)
Epidermoid (2%-6%)
Nonacoustic schwannoma (1%-5%): V, VII
Others
Ependymoma, medulloblastoma, pilocytic astrocytoma
Paraganglioma
Arachnoid cyst
Lipoma, dermoid, teratoma
External auditory canal erosion

secondary to neoplasm
Brackmann and Bartels, Otolaryngol Head Neck Surg 1980; 88:555-559;

Valavanis et al, Clinical Imaging of the Cerebellopontine Angle, Springer-Verlag,
1980; Gonzalez-Revilla, Johns Hopkins Hosp Bull 1948(83):187-189
Figure 5-5-11
Acoustic Schwannoma [Figure 5-5-11]
8%-10% of intracranial tumors

60%-90% of CPA tumors
Most: 30-70 years old
Neurofibromatosis type 2 (NF2): children, bilateral
in 96%
Schwann cell tumors, multiple meningiomas,
gliomas
First-degree relative counseling +/- imaging
screening
Kasantikul et al, J Neurosurg 1980; 52:28-35; Martuza

and Eldridge, N Engl J Med 1988; 318:684-688; Kishore
and OReilly, Clin Otolaryngol 2000; 25:561-565
Canalicular vestibular schwannoma with smooth

remodeling of the canal wall and loss of crista
falciformis
Acoustic (Vestibular) Schwannoma [Figure 5-5-12]
Sensorineural hearing loss, vertigo, tinnitus

Speech discrimination impaired: telephone use
Arise from vestibular division CN VIII usually
Direct pressure on cochlear division
Benign neoplasm
Slow growth (0.2 cm per year)
Well circumscribed globular mass
Histology: Antoni A and B fibers [Figure 5-12-8]
Figure 5-5-12
Komatsuzaki and Tsunoda, J Laryngol Otol 2001;

115:376-379; NIH Consensus Development Conference,
Arch Neurol 1994; 51:201-207 ; Lanser et al, Otolaryngol
Clin North Am 1992; 25:499-520
Antoni A (left) and Antoni B (right) cell populations

of a schwannoma
Neuroradiology
1079
Vestibular Schwannoma: Imaging
Figure 5-5-13
[Figures 5-5-13 to 5-5-16]
IAC widening with mushroom expansion

Giant: usually no IAC involvement
CT: usually isodense to cerebellum
Calcification and hemorrhage rare
T1WI: iso- to hypointense
T2WI: hyperintense
Intense enhancement: into porus acousticus and no
dural tail
Maya et al in Som and Curtin, Head and Neck Imaging,

4th ed, Mosby, 2003; Moller et al, Neuroradiology 1978;
17:25-30; Tali et al, AJNR 1993; 14:1241-1247;
Schmalbrock et al, AJNR 1999; 20:1207-1213
Vestibular schwannoma with classic mushroom

morphology on pre-contrast and post-contrast
axial T1-weighted images
Figure 5-5-14
Figure 5-5-15
Coronal T2-weighted FSE image of

right-sided vestibular schwannoma
Figure 5-5-16
Focal enhancement of deep portion of
internal auditory canal secondary to
arteriovenous malformation (not
vestibular schwannoma)
Cystic degeneration of large vestibular

schwannoma
1080
Neuroradiology
Vestibular Schwannoma: Therapy
Figure 5-5-17
Surgical resection
Larger masses
Translabyrinthine: protect facial nerve
Smaller masses
Retrosigmoid: suboccipital approach
Middle cranial fossa
Stereotactic radiosurgery (gamma knife) up to 4 cm
Poor surgical risk patients: serial MR
Jackler and Pitts, Otolaryngol Clin North Am 1992; 25:361-387;

House and Shelton, Otolaryngol Clin North Am 1992; 25:347-359;
Fucci et al, Am J Otol 1999; 20:497-508; Nakamura et al, AJNR 2000;
21:1540-1546
Meningioma
[Figures 5-5-17 and 5-5-18]
Usually eccentric to porus acousticus

IAC involvement uncommon (16%)
Frequently trans-spatial
Broad dural base: hemispherical
Obtuse bone-tumor angle
Dural tail: 52%-72%
Hyperostosis: highly characteristic
NCCT:usually hyperdense (calcification: 25%)
T1WI: isointense to gray matter
T2WI: variable
Tentorial meningioma with extension

into cerebellopontine angle
Figure 5-5-18
House and OConner, Handbook of Neurotological

Diagnosis, Marcel-Dekker, 1987; Valavanis et al,
Neuroradiology 1981; 22:111-121; Moller et al;
Neuroradiology 1978; 17:25-30
Epidermoid
[Figure 5-5-19]
Soft, pearly tumor

Irregular margins: cauliflower
Follows CSF density and signal
Usually no enhancement
Diffusion-weighted imaging: hyperintense to CSF
Differential Diagnosis: arachnoid cyst, cysticercosis,
atypical dermoid, lipoma
Berger and Wilson. J Neurosurg 1985; 62:214-219;

Gao et al, AJNR 1992; 13:863-872;
Tampieri et al, AJNR 1989; 10:351-356;
Tsuruda et al, AJR 1990; 155:1059-1065
Facial Nerve Palsy
Cerebellopontine angle meningioma with

numerous flow voids and fluid-fluid level. Note
extension through foramina
Figure 5-5-19
MR: imaging study of choice

Bells palsy: > 50%, nerve not enlarged
Idiopathic, by definition (probably HSV)
Imaging usually not performed
Tumors: 6%, enlarged nerve
Geniculate ganglion
Schwannoma
Hemangioma
Epidermoid
Parotid tumor spread
Tien et al, AJNR 1990; 11:735-741; Daniels et al,

Radiology 1989; 17:807-809
Epidermoid of middle cranial fossa with extension
into posterior fossa
Neuroradiology
1081
Temporal Bone Paragangliomas
Glomus jugulare: jugular foramen

Jacobsons (IX) and Arnolds (X) nerve
Glomus tympanicum: cochlear promontory
Most common middle ear tumor
Most common etiology of retrotympanic vascular mass
Vagal paraganglioma: jugular ganglion
Females 5:1; peak age: 40-60 years old
Figure 5-5-20
Rao et al, RadioGraphics 1999; 19:1605-1632
Jugulotympanic Paraganglioma [Figure 5-5-20]
Neuroendocrine tumor
Paraganglia: chemoreceptor function
Functioning: 1%-3%, catecholamine secretion
Early symptoms
Conductive HL, pulsatile tinnitus
Slow growth but locally invasive
Mortality rate: 15%
Metastasis very rare
Path: chief cells (Zellballen), sustentacular cells
Zellballen histologic appearance of
paraganglioma
Figure 5-5-21
Jugulotympanic Paraganglioma: Imaging

[Figures 5-5-21 to 5-5-23]
CT: irregular margins, moth-eaten erosion

Glomus jugulare: may extend down carotid sheath
MR: Salt and pepper appearance
Salt: hyperintense foci (slow flow, hemorrhage)
Pepper: serpentine flow voids
Intense enhancement
Angiography: ascending pharyngeal artery
Radiologists goal: define extent
Figure 5-5-22
Glomus tympanicum (paraganglioma)

(Case courtesy of William Kelly, MD)
Glomus jugulotympanicum (paraganglioma)

1082
Neuroradiology
Figure 5-5-23
Salt-and-pepper appearance of glomus jugulare on MR images
Jugular Foramen Masses
Paraganglioma: 90%
Schwannoma: 9%
Meningioma: <1%
Malignant neoplasms: <1%
Carcinoma
Sarcoma
Mets
Figure 5-5-24
Papillary Endolymphatic Sac Tumor [Figure 5-5-24]
Papillary
endolymphatic sac
tumor in different
patients
Ipsilateral hearing loss, facial nerve palsy, vestibular

dysfunction
Females more common
von Hippel-Lindau association
Adenomatous tumor
Bone destruction, intratumoral bone spicules
T1WI: hyperintense
Heffner, Cancer 1989; 64:2292-2302; Lo, AJNR 1993;14:13221323; Palmer et al, Otolaryngol Head Neck Surg 1989; 100:6468; Mukherji et al, Radiology 1997; 202:801-808
Differential Diagnosis Petrous Apex
Cholesterol granuloma (cyst): most common

Epidermoid
Follows cerebrospinal fluid (CSF) signal
Solid mass: resection
Chondrosarcoma
Mucocele
Carotid artery aneurysm
Meningocele
Curtin and Som, Otolaryngol Clin North Am 1995; 28:473-496
Cholesterol Granuloma (Cyst)
Retention cyst: obstruction in petrous apex

Chocolate cyst, Blue-domed cyst: within mastoidectomy cavity
Young, middle-aged adults
Hearing Loss, tinnitus, cranial nerve palsies
Hemorrhage and foreign-body reaction: cholesterol crystals and blood
(brownish fluid)
Lo et al, Radiology 1984;153:705-711; Graham et al, Laryngoscope 1985;

95:1401-1406; Latack et al, AJNR 1985; 6:409-413; Griffin et al, AJNR 1988;
8:825-829; Greenberg et al, AJNR 1988; 9:1205-1214
Neuroradiology
1083
Cholesterol Granuloma (Cyst)
CT
Isodense to brain
Expansile, especially posterior
Sharp smooth margins
MR: hemorrhage
Figure 5-5-25
[Figure 5-5-25]
Lo et al, Radiology 1984;153:705-711;

Latack et al, AJNR 1985; 6:409-413;
Chang et al, Laryngoscope 1998; 108:599-604;
Muckle et al, Am J Otol 1998: 19:219-225;
Palacios and Valvassori, Ear Nose Throat J 1999;
78:234
Chondrosarcoma
Cholesterol granuloma
[Figure 5-5-26]
Most common primary neoplasm of petrous apex

Off the midline: sutures
Petrosphenoidal
Petro-occipital
Locally invasive
Bone destruction: no sclerosis
T1 and T2 prolongation
Intense but heterogeneous enhancement
Grossman and Davis, Radiology 1981; 141:403-408; Meyers et al, Radiology

1992; 184:103-108; Bourgouin et al, JCAT 1992; 16:268-273
Temporal Bone Fracture

Frequency
Axis
Blow
Middle ear injury
Inner ear injury
Facial paralysis
Tegmen tympani
disruption
Longitudinal
80%
Long
Temporoparietal
Likely
Rare
1020%, usually
incomplete &
delayed
Common
Transverse
20%
Short
Occipital
Rare
Common
4050%, usually
acute & complete
Less common
Schwartz and Curtin in Som and Curtin,

Head and Neck Imaging, 4th ed., Mosby, 2003
Figure 5-5-26
Chondrosarcoma
1084
Neuroradiology
Ossicular Derangement
[Figure 5-5-27]
Need 1 mm CT images or thinner

Incus: most vulnerable
Subluxation from malleus
Dislocation
Incudostapedial disruption
Most common cause of post-traumatic conductive HL
Normal: <1 mm between lenticular process of incus & stapes head
Figure 5-5-27
Lourenco et al, Am J Otol 1995; 16:387-392; Swartz et al; Radiology 1989;

171:309-317
Otosclerosis
Primary endochondral bone within otic capsule replaced by

spongy vascular bone (otospongiosis)
Slowly progressive
Presents 1030 years old with tinnitus
Hearing loss later
Females more common (70%)
Bilateral 80% (usually asynchronous)
Valvassori, Otolaryngol Clin North Am, 1973; 6:379-389;

Reudi, Arch Otolaryngol 1963; 78:469-477
Otosclerosis
Fenestral type (80%): CHL

Begins at anterior oval window
Cochlear type (20%): SNHL
Almost always with fenestral type
Double Ring sign
Demineralized areas: active disease
Chronic disease: may appear normal
MR: punctate enhancement
Ossicular derangement
Mafee et al, Radiology 1985; 156:703-708; Swartz et al, Radiology 1985; 155:147150; Sakai et al, Am J Otolaryngol 2000; 21:116-118
Summary
Challenging complex anatomy

Facial nerve course: critical for pre-operative evaluation
Bony plate between hypotympanum and ICA canal
Tegmen tympani
Cholesteatoma: bone erosion
Cerebellopontine Angle: AMEN
Vestibular schwannoma: most common
Meningioma
Epidermoid
Non-acoustic schwannoma
Jugular foramen
Paraganglioma
Schwannoma
Petrous apex
Cholesterol granuloma vs. epidermoid
Chondrosarcoma, Chordoma, Metastasis
Otosclerosis
Radiologists Goals
Define extent of lesion
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Neuroradiology
1087
Imaging of the Orbit:

The Globe and Conal Lesions
The Bony Orbit
Bones
Frontal
Maxilla
Sphenoid
Zygoma
Ethmoid
Lacrimal
Palatine
Figure 5-6-1
[Figure 5-6-1]
The Bony Orbit
Superior orbital fissure

Middle cranial fossa
Cranial nerves III, IV, VI, V1
Superior and inferior ophthalmic veins

Inferior orbital fissure
Pterygopalatine (V2) & infratemporal fossae
Anterior Orbit
Figure 5-6-2
Orbital septal system

Anterior: well-developped (preseptal space)
Arises from periosteum of anterior bony orbit
Attaches to tarsal plates of eyelids
Posterior: incomplete
Lacrimal gland
Lacrimal sac and nasolacrimal duct
Globe
The bony orbit
[Figure 5-6-4]
Anterior chamber: aqueous humor

Iris and ciliary body
Posterior chamber: aqueous humor
Lens apparatus
Vitreous body: gel-like (collagen fibrils)
Most: free water
Lacrimal glands in superolateral

portion of the bony orbit
Figure 5-6-3
Figure 5-6-4
Nasolacrimal ducts in inferomedial

portions of bony orbit
Normal globe with anterior and

posterior chambers located anterior
to the lens and the vitreous body
constituting most of the globe
Imaging of the Orbit: The Globe and Conal Lesions
1088
Neuroradiology
Globe
[Figure 5-6-5]
Retina
Neural sensory inner layer (photoreceptors)
Retinal pigmented epithelium (RPE) outer layer
Ora serrata
Uvea: choroid (vascular); Bruchs membrane
Iris
Ciliary body
Sclera: fibrous layer; cornea anteriorly
Tenons capsule (bulbar fascia)
Normal: only one layer seen
Figure 5-6-5
Retrobulbar (Postseptal) Space

[Figures 5-6-6 to 5-6-8]
Fat with fibrous septa

Extraocular muscles (EOM) (The Cone)
Rectus: medial, lateral, superior, inferior
Annulus of Zinn: optic canal
Intermuscular septa (incomplete posteriorly)
Oblique: superior (trochlear), inferior
Levator palpebrae superioris
Optic nerve: glial-lined
Vessels
Figure 5-6-6
Close-up view of the posterior globe

layers. Retina is innermost, followed
by choroid and sclera. The macula
is located lateral to the optic disk
Figure 5-6-7
Extraocular muscles. The superior oblique

muscle travels through the trochlea, a bony
strut near the superomedial margin of the
bony orbit
Coronal view of the six extraocular

muscles and levator palpebrae
superioris
Figure 5-6-8
Papilledema with increased fluid

surrounding optic nerves bilaterally
caused by a supratentorial
oligodendroglioma
Neuroradiology
1089
Cranial Nerves III, IV, VI
Figure 5-6-9
Motor control of EOMs

Cranial Nerve III: all EOMs except
Lateral rectus: Cranial Nerve VI
Superior oblique: Cranial Nerve IV
LR6SO4
Sensory control: V1 primarily, V2 (infraorbital region)
Senile Macular Degeneration
Most common cause of legal blindness in the elderly

Hyalinization of macula, thickening of Bruchs membrane
Pigment epithelium detachment
Serous subretinal space fluid
Hemorrhage: fibrous scar, macular loss
Computed tomography (CT): mimics uveal melanoma
Magnetic resonance (MR): variable
Mafee, in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby,
2003
Posterior Hyaloid Detachment
Separation of the hyaloid base (posterior hyaloid membrane)

from retinal sensory epithelium
Usually adults with myopia
Liquefaction of vitreous
Children: persistent hyperplastic primary vitreous
Association: macular degeneration
Intravitreal and curvilinear layer
Not connected to optic disk
Retinal detachment, with

characteristic V-shape created by
anchor points at optic disk and ora
serrata
Mafee in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003
Retinal Detachment (RD)
[Figure 5-6-9]
Sensory retina separates from RPE

Rhegmatogenous RD: tear in retina
RPE: can heal (laser therapy)
Ultrasonography (US) superior to MR or CT for detection
Causes: mass, fibroproliferative disease, toxocara, choroidal
lesions
V-shape: apex at optic disk
Figure 5-6-10
Mafee and Peyman, Radiol Clin North Am 1987;25:487-507
Choroidal Detachment
[Figure 5-6-10]
Hemorrhagic: contusion
Serous: ocular hypotony (choroidal inflammation, trauma,
glaucoma therapy)
U-shaped
Anchor points: short posterior ciliary artery, vortex veins
No connection with optic disk
Mafee and Peyman, Radiol Clin North Am 1987;25:487-507
Leukocoria
Retinoblastoma
Persistent hyperplastic primary vitreous (PHPV)
Retinopathy of prematurity (ROP)
Congenital cataract
Coats disease
Toxocariasis
Total retinal detachment
Choroidal detachment, with typical Ushape created by anchor points at

ciliary body and vessels away from
the optic disk
Mafee, in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003
1088
1090
Neuroradiology
Retinoblastoma
Figure 5-6-11
Most common intraocular tumor of childhood

Incidence 1:15,000
Virtually all patients < 6 years-old
80% 3 years old or younger
13 months: average age at presentation
No gender or racial predilection
Retinoblastoma gene: chromosome 13q14
Germinal (inherited) 85% bilateral
Somatic (not inherited) unilateral
Unilateral: 60%-70%
Association: osteosarcoma, other sarcomas
Abramson et al, Ophthalmology 1984; 91:1351-1355; Pendergrass

and Davis, Arch Ophthalmol 1980; 98:1204-1210; Ellsworth, Trans
Am Ophthalmol Soc 1969; 67:462-534; Kaufman et al, Radiol Clin
North Am 1998; 36:1101-1117
Retinoblastoma
Gross photograph of retinoblastoma
[Figure 5-6-11]
Figure 5-6-12
Ophthalmoscopic diagnosis primarly

Small gray-white intraretinal lesions, calcification, seeding
Ultrasonography: 80% accurate
Stage 1: confined to the globe
Stage 2: extraocular extension to orbit or optic nerve
Stage 3: extra-orbital extension
92% 5-year survival for intra-ocular lesions but near 100%
mortality when extends beyond eye
Kodilyne, Am J Ophthalmol 1967; 63:467-481; Abramson et al, Arch

Ophthalmol 1981; 99:1761-1762
Retinoblastoma Pathology
Neuroectodermal origin: primitive embryonal retinal cells

(retinoblasts)
Rosettes: Flexner-Wintersteiner or Homer-Wright type
Highly malignant: necrosis, mitotic figures
Calcification
Kyritsis et al, Nature 1984; 307:471-473
Retinoblastoma - Imaging
[Figure 5-6-12]
CT: imaging study of choice

Retinoblastoma with characteristic
Calcification: >90% of cases
calcification on CT
Child < 3 y/o: highly suggestive for diagnosis
Tri- / tetralateral retinoblastoma with pineal and/or suprasellar masses
MR: not as specific as CT
Hyperintense on T1WI and PD
Hypointense on T2WI
May miss lesions as large as 4mm
Better for intracranial extension, extraocular spread
Char, Ophthalmology 1984; 91:1347-1350; Mafee, Radiol Clin North Am 1987;

25:667-681; Mafee et al, Ophthalmology 1989; 96:965-976
Persistent Hyperplastic Primary Vitreous (PHPV) [Figure 5-6-13]
Failure of embryonic hyaloid system (primary vitreous) to regress normally and

form the secondary vitreous by 5th-6th gestational month
Isolated or part of more complex abnormality

Bilateral: Norries, Warburgs
Usually unilateral leukokoria and microphthalmos
Lens opacity, RD, vitreous hemorrhage
Persistent hyaloid (Cloquets) canal
No calcification
Mafee and Goldberg, Radiol Clin North Am 1987; 25:683-692

Neuroradiology
1089
1091
Retinopathy of prematurity
Figure 5-6-13
Coats Disease [Figure 5-6-14]
Juvenile males most common

Usually unilateral (85%-90%)
Peripheral telangiectasias
Leak lipoproteinaceous exudate
Retinal detachment
May mimic retinoblastoma clinically
Coats, R Lond Ophthalmol Hosp Rep 1908; 17:440525; Reese, Am J Ophthalmol 1956; 42:1-8; Edward et
al, Radiol Clin North Am 1988; 36: 1119-1131
Toxocariasis
Chorioretinitis: Toxocara canis (nematode)

Granuloma: eosinophilic abscess
Persistent hyperplastic primary vitreous (PHPV)
CT: homogeneous intravitreal density
with hyaloid canal
Retinal detachment, organized vitreous,
inflammatory exudate
Irregular thickening of uveoscleral coat
MR: subretinal exudate, variably hyperintense on all sequences
Margo et al, Pediatr Ophthalmol Strabismus 1983; 20: 180-184; Wilder, Trans Am
Acad Ophthalmol Otolaryngol 1950; 55:99-104
Figure 5-6-14
Uveal Melanoma
Uvea
Choroid, ciliary body, iris
Derived from mesoderm & neuroectoderm
Most highly vascular part of eyeball
Melanoma: most common neoplasm of choroid
Whites (15:1)
Incidence increases with age
Arises from choroid, elevates and may rupture Bruchs
membrane (mushroom shape)
Yanoff and Fine, Ocular Pathology, Harper and Row, 1975; Mafee,
Radiol Clin North Am 1998; 36:1083-1099
Uveal Melanoma
Gross photograph of Coats Disease
[Figure 5-6-15]
Metastasis: liver > lung > bone > kidney > brain
Diagnosis usually made by ophthalmoscopy or US
CT: elevated, hyperdense, sharply marginated (usually) solid mass
MR
Hyperintense on T1WI and PDW
Hypointense on T2WI
Duffin et al, Arch Ophthalmol 1981; 99:1827-1830; Enochs et al, Radiology 1997;
204:417-423; Mafee in Som
and Curtin, Head and Neck
Imaging, 4th ed., Mosby,
2003
Figure 5-6-15
Uveal melanoma
1090
1092
Neuroradiology
Uveal Metastasis
[Figure 5-6-16]
Usually in the plane of the choroid with little increased thickness

Mottled appearance, diffuse outline
Breast and lung cancer most common
Retina or choroid
Bilateral 1/3 (melanoma rarely bilateral)
Mucinous adenocarcinoma: mimic melanoma
Figure 5-6-16
Mafee, Radiol Clin North Am 1998; 36:1083-1099
Orbital Trauma
[Figures 5-6-17 and 5-6-18]
CT: imaging modality of choice

Fractures
Isolated: orbital rim (Waters view)
Blowout: inferior wall; medial wall: 1/2
Nasoethmoidal (NOE) complex
canthal injury common
Zygomatic complex (ZC)
Lefort types: I, II, III
Orbital apex-optic canal
Hemorrhage
Retrobulbar: most common
Extraconal
Subperiosteal
Sub-Tenon capsule
Subdural (optic nerve sheath)
Optic nerve injury
Eyeball injury: phthisis bulbi
Foreign Body
Bilateral uveal metastases
Figure 5-6-17
Figure 5-6-18
Blow-out fracture
Orbital fracture extending towards

optic canal
Neuroradiology
1091
1093
Conal Lesions
Graves
Pseudotumor
Others
Lymphoproliferative disease
Metastasis: 7%, breast carcinoma, nodular
Arteriovenous fistula or vascular congestion
Acromegaly
Amyloid
Cysticercosis / Trichinosis
Thyroid Orbitopathy (Graves Disease)
Autoimmune disorder: orbital soft tissues, thyroid, extremities

Superior cervical lymph channel: drains both thyroid and orbit
Incidence: 0.5% (U. S.)
Most common orbital disorder
Most common cause of exophthalmos in adults
15%28% of unilateral exophthalmos
80% of bilateral exophthalmos
Figure 5-6-19
Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999;

Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby,
2003
Graves Disease
Most (up to 80%) patients are or will be hyperthyroid

Euthyroid (10%)
Family history: 30%
Range: 1586 years old (peak: 3050)
Females much more common (4:1)
Males, patients > 50y/o: more severe disease
Kendler et al, Arch Ophthalmol 1993; 111:197-201
Graves Disease
Acute phase
Inflammatory reaction: congestion, hypertrophy, fibrosis of
orbital fat / muscles
Mucopolysaccharides accumulate in EOMs
Chronic phase: exophthalmos (34%93%)
Fibrosis
Restrictive myopathy
Diplopia
Graves Disease
Figure 5-6-20
Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999
Graves Disease: Imaging
[Figures 5-6-19 and 5-6-20]
Muscle Enlargement
Inferior rectus
Medial rectus
Superior muscle complex
Lateral rectus
? Related to innervation and fiber size
Tendon spared
Dirty retrobulbar fat: inflammation
Apex: optic nerve compression
Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby,
2003
Graves Disease with sparing of the
tendinous insertions
1092
1094
Neuroradiology
Pseudotumor
Nongranulomatous inflammation
No known cause
Second most common (~5%) orbital disease
(after Graves disease)
Children: 6%16% of all cases, more frequently bilateral
Blodi and Gass, Br J Ophthalmol 1968; 52:79-93; Flanders et al, J Comput Assist
Tomogr 1989; 13:40-47; Weber et al, Radiol Clin North Am 1999; 37:151-168
Figure 5-6-21
Pseudotumor
Acute form
Abrupt onset of pain usually
Lid swelling, redness, ptosis, proptosis
Chronic form
Fixation signs: diplopia, proptosis
Sites
Lacrimal gland (lacrimal adenitis)
Extraocular muscles (myositic form)
Cavernous sinus (Tolosa-Hunt)
Tolosa, J Neurol Neurosurg Psychiatry 1954; 17:300-302; Hunt,

Neurology 1961; 11:56-62
Pseudotumor: EOM
Tendons involved (unlike Graves disease)

Ragged fluffy muscle border
Inward bowing of muscle contour at globe insertion
Dirty retrobulbar fat
May extend intracranially (apical orbital inflammation) or onto
optic nerve (ON) sheath (perineuritis)
Bone destruction rare
Trokel and Hilal, Am J Ophthalmol 1979; 87:503-512;

Flanders et al, J Comput Assist Tomogr 1989; 13:40-47
Pseudotumor: Imaging
[Figures 5-6-21 and 5-6-22]
CT
Nonspecific
MR
Hypointense on T1WI and T2WI
Pseudotumor with tendinous

involvement and rapid response to
steroid therapy on follow-up
Figure 5-6-22
Pseudotumor with characteristic T1

and T2 hypointensity
Neuroradiology
1093
1095
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
24.
25.
26.
27.
28.
29.
30.
31.
32.
Abramson DH, Ellsworth RM, Kitchin FD, Tung G. Second nonocular tumors in retinoblastoma survivors. Are
they radiation-induced? Ophthalmology 1984; 91:1351-1355.
Abramson DH, Ellsworth RM, Tretter P, Javitt J, Kitchin FD. Treatment of bilateral groups I through III
retinoblastoma with bilateral radiation. Arch Ophthalmol 1981; 99:1761-1762.
Blodi FC, Gas JD. Inflammatory pseudotumour of the orbit. Br J Ophthalmol 1968; 52:79-93.
Char DH, Hedges TR, 3rd, Norman D. Retinoblastoma. CT diagnosis. Ophthalmology 1984; 91:1347-1350.
Coats G, Lond R. Forms of retinal diseases with massive exudation. Ophthalmol Hosp Rep 1908; 17:440-525.
Duffin RM, Straatsma BR, Foos RY, Kerman BM. Small malignant melanoma of the choroid with extraocular
extension. Arch Ophthalmol 1981; 99:1827-1830.
Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA. Coats' disease and persistent hyperplastic primary
vitreous. Role of MR imaging and CT. Radiol Clin North Am 1998; 36:1119-1131, x.
Eller AW, Jabbour NM, Hirose T, Schepens CL. Retinopathy of prematurity. The association of a persistent hyaloid
artery. Ophthalmology 1987; 94:444-448.
Ellsworth RM. The practical management of retinoblastoma. Trans Am Ophthalmol Soc 1969; 67:462-534.
Enochs WS, Petherick P, Bogdanova A, Mohr U, Weissleder R. Paramagnetic metal scavenging by melanin: MR
imaging. Radiology 1997; 204:417-423.
Flanders AE, Mafee MF, Rao VM, Choi KH. CT characteristics of orbital pseudotumors and other orbital
inflammatory processes. J Comput Assist Tomogr 1989; 13:40-47.
Hunt WE, Meagher JN, Lefever HE, Zeman W. Painful opthalmoplegia. Its relation to indolent inflammation of the
carvernous sinus. Neurology 1961; 11:56-62.
Jakobiec FA, Tso MO, Zimmerman LE, Danis P. Retinoblastoma and intracranial malignancy. Cancer 1977;
39:2048-2058.
Kaufman LM, Mafee MF, Song CD. Retinoblastoma and simulating lesions. Role of CT, MR imaging and use of
Gd-DTPA contrast enhancement. Radiol Clin North Am 1998; 36:1101-1117.
Kendler DL, Lippa J, Rootman J. The initial clinical characteristics of Graves' orbitopathy vary with age and sex.
Arch Ophthalmol 1993; 111:197-201.
Kodilinye HC. Retinoblastoma in Nigeria: problems of treatment. Am J Ophthalmol 1967; 63:469-481.
Kyritsis AP, Tsokos M, Triche TJ, Chader GJ. Retinoblastoma--origin from a primitive neuroectodermal cell?
Nature 1984; 307:471-473.
Mafee MF, Goldberg MF, Cohen SB, et al. Magnetic resonance imaging versus computed tomography of
leukocoric eyes and use of in vitro proton magnetic resonance spectroscopy of retinoblastoma. Ophthalmology
1989; 96:965-975; discussion 975-966.
Mafee MF, Goldberg MF, Greenwald MJ, Schulman J, Malmed A, Flanders AE. Retinoblastoma and simulating
lesions: role of CT and MR imaging. Radiol Clin North Am 1987; 25:667-682.
Mafee MF, Goldberg MF. Persistent hyperplastic primary vitreous (PHPV): role of computed tomography and
magnetic resonance. Radiol Clin North Am 1987; 25:683-692.
Mafee MF, Peyman GA. Retinal and choroidal detachments: role of magnetic resonance imaging and computed
tomography. Radiol Clin North Am 1987; 25:487-507.
Mafee MF. The eye. In: Som PM, Curtin HD, eds. Head and neck imaging. 4th ed. St. Louis: MosbyElsevier
Science; 2003
Mafee MF. Uveal melanoma, choroidal hemangioma, and simulating lesions. Role of MR imaging. Radiol Clin
North Am 1998; 36:1083-1099
Margo CE, Katz NN, Wertz FD, Dorwart RH. Sclerosing endophthalmitis in children: computed tomography with
histopathologic correlation. J Pediatr Ophthalmol Strabismus 1983;20:180-184
Pendergrass TW, Davis S. Incidence of retinoblastoma in the United States. Arch Ophthalmol 1980; 98:1204-1210.
Reese AB. Telangiectasis of the retina and Coats' disease. Am J Ophthalmol 1956; 42:1-8.
Rubin RM, Sadun AA. Ocular myopathies. In: Yanoff M, Duker JS, eds. Ophthalmology. St. Louis: Mosby; 1999.
Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J
Neurol Neurosurg Psychiatry 1954; 17:300-302.
Trokel SL, Hilal SK. Recognition and differential diagnosis of enlarged extraocular muscles in computed
tomography. Am J Ophthalmol 1979; 87:503-512.
Weber AL, Romo LV, Sabates NR. Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation.
Radiol Clin North Am 1999; 37:151-168, xi.
Wilder HC. Nematode endophthalmitis. Trans Am Acad Ophthalmol Otolaryngol 1950:99-109.
Yanoff K, Fine BS, Ocular Pathology . Hagerstown: Harper & Row, 1975.
1094
1096
Neuroradiology
Imaging of the Orbit:

Intraconal and Extraconal Lesions
Figure 5-7-1
Intraconal Lesions
Optic nerve glioma

Optic nerve sheath meningioma
Schwannoma
Lymphoma
Fibrous histiocytoma
Varix, arteriovenous malformation
Optic Nerve Glioma
3% of all orbital tumors; 4% of gliomas

Peak age: 28 years (range: birth to 60 years)
50% associated with neurofibromatosis type 1 (NF-1) and
frequently bilateral
10%15% of all NF-1 cases
Optic atrophy on ophthalmoscopy
Arise from glial cells of optic nerve
Slow growth usually; may grow in spurts
Azar-Kia et al, Radiol Clini North Am 1987; 25:561-581
Optic Nerve Glioma
Optic nerve glioma with characteristic

"kinking" of the nerve
[Figure 5-7-1]
Juvenile: pilocytic astrocytoma

Adult: glioblastoma multiforme (non-NF-1 cases)
Fusiform enlargement: kinking, buckling
CT: iso- to hypodense
Calcification rare
T1WI: hypointense, T2WI: hyperintense
Azar-Kia et al, Radiol Clin North Am 1987; 25:561-581; Haik et al, Ophthalmology
1987; 94:709-717
Figure 5-7-2
Optic Nerve Sheath Meningioma
5% of all orbital tumors

Less than 1% of all meningiomas
Extradural meningiomas: associated with blistering of adjacent
bone
Slowly progressive loss of vision, proptosis
Optociliary venous shunts, disk pallor, visual loss: highly
suggestive
Sibony et al, Ophthalmology 1984; 11:1313-1326
Optic Nerve Sheath Meningioma
Well-defined tubular thickening of ON

CT: Calcification common
T1WI: Isointense to ON
T2WI: Iso- to hypoattenuated
Tram-track enhancement
Fat suppression essential
May be eccentric, extend intracranially
[Figure 5-7-2]
Optic nerve sheath meningioma with

tram-track enhancement (upper
Daniels et al, AJNR 1982: 3:181-183; Azar-Kia et al, Radiol Clin North
image)
and calcification (lower image)
Am 1987; 25:561-581
in 2 different patients
Neuroradiology
1097
Imaging of the Orbit: Intraconal and Extraconal Lesions
Cavernous Hemangioma
[Figure 5-7-3]
Most common vascular orbital tumor in adults

Peak age: 25-40 y/o (range 25-70)
(pseudocapsule)
Intraconal (83%)
Benign non-infiltrative hamartoma
Large dilated sinusoid-like spaces
Slowly progressive enlargement
Prominent arterial supply usually
absent
CT
Hyperattenuated
Phleboliths
Bone remodeling
MR
T1WI: mixed
T2WI: iso-to-hyperintense
Hemorrhage occasionally
Bilaniuk, Radiol Clin North Am 1999;

37:169-183; Mafee et al, Radiol Clin North
Am 1987; 25:529-559
Figure 5-7-3
Cavernous hemangioma with pressure erosion of orbital roof
Schwannoma
1% of all orbital tumors: usually intraconal

Arise from cranial nerves, not optic nerve
Isolated or neurofibromatosis association
Benign with slow growth
Well-encapsulated
Painless proptosis
Compresses or engulfs optic nerve
Carroll et al, Radiol Clin North Am 1999; 37:195-202
Schwannoma
[Figure 5-7-4]
Fusiform to oval-shaped mass

CT: Isoattenuated to extraocular muscles
More hyperdense areas = Antoni A cells
T1WI: Iso- to hypodense
T2WI: Hyperintense
Marked enhancement
Figure 5-7-4
Orbital Lymphoma
Lymphoid tumors: 10%-15% of orbital masses

Lymphoma, pseudolymphoma, lymphoid hyperplasia
10% of all lymphomas as primary site
75% have or will have systemic lymphoma
Lacrimal gland: most common site
EOMs rarely involved
Non-Hodgkins (B-cell): majority
Proptosis, ptosis, diplopia
Rubbery firm masses
Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et

al, Radiol Clin North Am 1997; 25:601-612;
Schwannoma
1096
1098
Neuroradiology
Orbital Lymphoma
Figure 5-7-5
[Figure 5-7-5]
Molds itself along margins of normal structures

Bone erosion: late finding
Usually well-defined, round to oval
CT: homogeneous, mildly hyperattenuated
T1WI: hypointense
T2WI: iso-to-hypointense
Mild to marked enhancement
Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et

al, Radiol Clin North Am 1997; 25:601-612;
Fibrous Histiocytoma
[Figure 5-7-6]
Most common primary orbital mesenchymal tumor in adults

1% of all primary orbital tumors
Mean age: 42 y/o
Usually benign (66%)
Malignant: bone erosion, hemorrhage, post-radiation therapy
for retinoblastoma
Well-defined intra-or extraconal mass
Moderate to marked enhancement
Font and Hidayat, Hum Pathol 1982; 13:199; Mafee in Valvassori,

Mafee, and Carter, Imaging of the Head and Neck, Thieme, 1995
Orbital Varix
[Figure 5-7-7]
Most common cause of spontaneous orbital hemorrhage

Focal venous dilatation
Valsalva: stress proptosis
Lobulated mass
Phleboliths
Spontaneous thrombosis common
Orbital lymphoma with characteristic

molding of the tumor against the
orbital globe
Figure 5-7-6
Bilaniuk, Radiol Clin North Am 1999; 37:169-183
Figure 5-7-7
Orbital varix with enlargement upon

Valsalva maneuver on post-contrast
image (bottom)
Neuroradiology
1097
1099
[Figure 5-7-8]
Usually post-trauma
Spontaneous less common (Ehlers-Danlos, osteogenesis
imperfecta, pseudoxanthoma elasticum)
Orbital bruit, proptosis, chemosis
CT/MR: dilated superior ophthalmic vein
Angiography diagnostic
Endovascular occlusion: treatment of choice
Figure 5-7-8
Tan et al, Radiol Clin North Am 1987; 25:849-861
Extraconal Lesions
Lymphangioma*
Metastasis*
Rhabdomyosarcoma*
Dermoid/epidermoid
Paranasal sinus disease
Infection
Neoplasm
*commonly intercompartmental
Lymphangioma
Carotid-cavernous
fistula with enlarged
superior ophthalmic
vein. Lateral view from
cerebral angiogram
shows abnormal flow
through cavernous
sinus, petrosal
sinuses, and superior
ophthalmic vein
[Figure 5-7-9]
Children and young adults

Exophthalmos with viral infection
Lymphoid follicles, dilated spaces
Infiltrative; do not respect fascial planes
Hemorrhage common
Extraconal space primarily
CT/MR: Heterogeneous appearance
Hemorrhage or cystic fluid
Variable enhancement (venous channels)
Bilaniuk, Radiol Clin North Am 1999; 37:169-183; Mafee

et al, Radiol Clin North Am 1987; 25:529-559
Figure 5-7-9
Figure 5-7-10
Lymphangioma with hemorrhage in 2 different patients
Metastatic Lesions
[Figure 5-7-10]
10% of orbital tumors

1/3- bony orbit, 1/3- globe, 1/3- scattered
Increasing incidence (longer survival)
Primary site
Breast: 42%
Lung: 11%
Unknown primary: 11%
Prostate: 8%
Melanoma: 5%
Children: neuroblastoma, leukemia, Ewings
Orbital metastasis from unknown

primary neoplasm
1098
1100
Neuroradiology
Metastatic Lesions
Figure 5-7-11
9 months average survival (lung carcinoma,

melanoma worst)
Hematogenous spread
Diplopia, proptosis, pain, vision loss
Enophthalmos: breast carcinoma
Isolated lateral rectus enlargement metastasis or
pseudotumor
Rhabdomyosarcoma [Figure 5-7-11]
Most common primary orbital malignancy tumor in

children
Most: 2-5y/o; 90% younger than 16 y/o
Rapidly progressive but usually painless
Arise from undifferentiated mesenchyme in orbital
fat (not from extraocular muscles)
Children: embryonal (70%) and alveolar types
Adults: pleomorphic type
90% 5-year survival with complete resection
35% if significant residual disease
Rhabdomyosarcoma with characteristic bone

destruction
Mafee et al, Radiol Clin North Am 1998; 36:1215-1227;
Rhabdomyosarcoma
Figure 5-7-12
Superior orbit predilection

Homogeneous mass
CT: isoattenuated to muscle
Bone destruction common in larger lesions
Necrosis, calcification, hemorrhage uncommon
T1WI: hypointense
T2WI: hyperintense
Moderate to marked enhancement
Mafee et al, Radiol Clin North Am 1998; 36:1215-1227
Dermoid / Epidermoid [Figure 5-7-12]
Most common congenital orbital lesion

Many manifest in 2nd-3rd decades
Superolateral: most comon location

Arise at sutures or diple
Well-defined mass with fat or fluid signal in upper corners of orbit
Remodel bone without destruction
Kaufman et al, Radiol Clin North Am 1998; 36:1149-1163
Orbital Cellulitis [Figures 5-7-13 and 5-7-14]
Dermoid
Classification
Pre-septal cellulitis: eyelid
Post-septal cellulitis
Subperiosteal phlegmon and abscess
Cavernous sinus thrombosis
Usually paranasal (ethmoid) sinusitis
Usually does not extend into intraconal space
Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am
1998; 36:1165-1183
Neuroradiology
1099
1101
Figure 5-7-14
Figure 5-7-13
Orbital cellulitis. Note involvement

along lamina papyracea
Peri-orbital cellulitis
Fungal sinusitis [Figure 5-7-15]
Rhino-orbital mucormycosis
Aspergillosis
Reversal of typical findings in sinus disease
Increasing protein, decreasing water content
CT: hyperattenuated
T1WI: hyperintense
T2WI: hypointense (can mimic air)
Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am
1998; 36:1165-1183
Lacrimal Gland Lesions
50% inflammatory/lymphoproliferative
Sarcoid
Sjogrens
Lymphoma: frequent anterior/posterior extension
Pseudotumor: 15% of all orbital pseudotumor
50%: epithelial tumors
50%: benign (pleomorphic adenoma, benign mixed cell tumor)
50%: malignant (adenoid cystic, malignant mixed, mucoepidermoid,
adeno, squamous cell, anaplastic)
Imaging: pre-op planning
Figure 5-7-15
Zimmerman et al, Int Ophthalmol Clin 1962;

2:337-367; Mafee et al, Radiol Clin North
Am 1987; 25:767-779
Fungal sinusitis
1100
1102
Neuroradiology
Lacrimal Gland Lesions [Figure 5-7-16 and 5-7-17]
Figure 5-7-16
Inflammatory lesions
Oblong mass
Molded enlargment of lacrimal gland
Pleomorphic adenoma
Long duration
Rounded mass
Bone remodeling
Bone destruction: malignant epithelial tumors
Jakobiec et al, Am J Ophthalmol Clin 1962; 2:337-367
Lacrimal Sac Lesions
[Figure 5-7-18]
Malignant: 57%
Epithelial: 75%
Squamous cell
Transitional cell
Mucoepidermoid
Mesenchymal: fibrous histiocytoma
Lymphoid: lymphoma
Neural
Metastasis
Benign: 43%
Diverticulum
Pneumatocele
Mucocele
Papilloma
Polyp
Fibroma
Dermoid
Lacrimal lymphoma
Figure 5-7-17
Stefanyszyn et al, Ophthal Plast Reconstr Surg 1994; 10:169-184;

Peer et al, Ophthalmology 1996; 103:1601-1605
Figure 5-7-18
Lacrimal pleomorphic adenoma
Lacrimal sac sarcoma with bone destruction
Neuroradiology
1101
1103
Summary
Retinoblastoma: most common intraocular malignancy of childhood

Uveal melanoma: most common malignancy of the globe in adults
Uveal metastasis: frequently bilateral
Most common diseases of the orbit
1. Graves: no tendon involvement
2. Lymphoma
3. Pseudotumor: involves tendon
Intraconal lesions
Optic nerve tumors
Glioma
Nerve sheath meningioma
Nerve sheath tumors
Lymphoma
Fibrous histiocytoma
Varix
Carotid-cavernous fistula
Extraconal lesions
Lymphangioma
Metastases: 10% of orbit masses
Rhabdomyosarcoma
Dermoid
Sinus disease
Lacrimal gland lesions
The 50% gland
50% inflammatory/lymphoproliferative
50% neoplasms
50% benign, 50% malignant
Lacrimal sac lesions: most are malignant
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Azar-Kia B, Naheedy MH, Elias DA, Mafee MF, Fine M. Optic nerve tumors: role of magnetic resonance imaging
and computed tomography. Radiol Clin North Am 1987; 25:561-581.
Bilaniuk LT. Orbital vascular lesions. Role of imaging. Radiol Clin North Am 1999; 37:169-183, xi.
Carroll GS, Haik BG, Fleming JC, Weiss RA, Mafee MF. Peripheral nerve tumors of the orbit. Radiol Clin North
Am 1999; 37:195-202, xi-xii.
Chandler JR, Langenbrunner DJ, Stevens ER. The pathogenesis of orbital complications in acute sinusitis.
Laryngoscope 1970; 80:1414-1428.
Daniels DL, Williams AL, Syvertsen A, Gager WE, Harris GJ. CT recognition of optic nerve sheath meningioma:
abnormal sheath visualization. AJNR Am J Neuroradiol 1982; 3:181-183.
Eustis HS, Mafee MF, Walton C, Mondonca J. MR imaging and CT of orbital infections and complications in acute
rhinosinusitis. Radiol Clin North Am 1998; 36:1165-1183, xi.
Flanders AE, Espinosa GA, Markiewicz DA, Howell DD. Orbital lymphoma. Role of CT and MRI. Radiol Clin
North Am 1987; 25:601-613.
Font RL, Hidayat AA. Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases. Hum Pathol 1982;
13:199-209.
Haik BG, Saint Louis L, Bierly J, et al. Magnetic resonance imaging in the evaluation of optic nerve gliomas.
Ophthalmology 1987; 94:709-717.
Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal
fossa lesions. Am J Ophthalmol 1982; 94:785-807.
Kaufman LM, Villablanca JP, Mafee MF. Diagnostic imaging of cystic lesions in the child's orbit. Radiol Clin
North Am 1998; 36:1149-1163, xi.
Mafee MF, Haik BG. Lacrimal gland and fossa lesions: role of computed tomography. Radiol Clin North Am 1987;
25:767-779.
Mafee MF, Pai E, Philip B. Rhabdomyosarcoma of the orbit. Evaluation with MR imaging and CT. Radiol Clin
North Am 1998; 36:1215-1227, xii.
Mafee MF, Putterman A, Valvassori GE, Campos M, Capek V. Orbital space-occupying lesions: role of computed
tomography and magnetic resonance imaging. An analysis of 145 cases. Radiol Clin North Am 1987; 25:529-559.
1102
1104
Neuroradiology
15. Mafee MF. Imaging of the orbit. In: Valvassori GE, Mafee MF, Carter BL, eds. Imaging of the head and neck. New
York: Thieme, 1995; 302-328
16. Pe'er J, Hidayat AA, Ilsar M, Landau L, Stefanyszyn MA. Glandular tumors of the lacrimal sac. Their
histopathologic patterns and possible origins. Ophthalmology 1996; 103:1601-1605.
17. Sibony PA, et al: Optic Nerve Sheath Meningiomas. Ophthalmology 1984, 91(11): 1313-1326.
18. Stefanyszyn MA, Hidayat AA, Pe'er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994;
10:169-184.
19. Tan WS, Wilbur AC, Mafee MF. The role of the neuroradiologist in vascular disorders involving the orbit. Radiol
Clin North Am 1987; 25:849-861.
20. Valvassori GE, Sabnis SS, Mafee RF, Brown MS, Putterman A. Imaging of orbital lymphoproliferative disorders.
Radiol Clin North Am 1999; 37:135-150, x-xi.
21. Zimmerman LE, Sanders TE, Ackerman LV. Epithelial tumors of the lacrimal gland: prognostic and therapeutic
significance of histologic types. Int Ophthalmol Clin 1962; 2:337-367.
Neuroradiology
1103
1105
Patterns of Location: Infratentorial and

Supratentorial
PATTERN ANALYSIS
Basic Approach
Where is the lesion ?
Intraaxial
Extraaxial
Intraventricular
Where is the lesion ?
Supratentorial
Infratentorial
How old is the patient ?
Child
Adult
What about Sex ?
INTRA-AXIAL
Cortex
Gray-white Junction
Deep White Matter
Deep Gray Matter
Glioma
Medulloblastoma
Hemangioblastoma
Metastases
Infarct/hematoma
AVM/congenital
Abscess/inflammation
EXTRA-AXIAL LESIONS
Subarachnoid
Subdural
Epidural
Calvarium (Skull Base)
Subgaleal
Scalp (Soft-tissues)
Meningioma
Pituitary adenoma
Craniopharyngioma
Schwannoma
Chordoma
Dermoid/epidermoid, cyst, lipoma
Hematoma, metastasis, infection
BASIC APPROACH
CLASSIC LOCATIONS
Foramen magnum
Cerebellopontine angle (CPA)
Fourth ventricle/Cerebellum
Sella/parasellar/suprasellar
Basal ganglia/Third ventricle
Lateral ventricle/Pineal region
Deep hemispheric/periventricular
Cortical and subcortical
Convexity Extraaxial
Patterns of Location: Infratentorial and Supratentorial
1104
1106
Neuroradiology
Cranial Nerves
Figure 5-8-1
Olfactory (I)
Optic (II)
Oculomotor (III)
Trochlear (IV)
Trigeminal (V)
Abducens (V)
Facial (VII)
Vestibulocochlear (VIII)
When looking into the IAC

(internal auditory canal) notice
that
7 is UP and Coke (cochlear) is
DOWN
Internal Auditory Canal

S Schwannoma (8th >> > 5th)

A aneurysm, arachnoid cyst
M meningioma, mets
E epidermoid, ependymoma, CPP
CPA MASSES Differential
S Schwannoma (8th >> > 5th)

A aneurysm, arachnoid cyst
M meningioma, mets
E epidermoid, ependymoma, CPP
Figure 5-8-2
CPA MASSES Demographics
7/9 (Schwannoma, 8th > > 5th)

1/9 Meningioma (tentorial/petrous)
1/9 Other:
Epidermoid Cyst (1/18)
Mets, aneurysm, etc.
Glioma (ependymoma, CPP)
Arachnoid cyst
Cystadenoma of endolymph
Glomus tumor
Vestibular Schwannoma begins as an

intracanalicular mass; then it grows out of the
canal into the cerebellopontine angle cistern
Intracanalicular Schwannoma
[Figure 5-8-3]
Vestibular Schwannoma
Figure 5-8-3
[left] T2W image shows CSF, normal nerve, and round mass.
[right] T1WGd image shows enhancement of mass. The
normal 7th and 8th nerves do NOT enhance in this location
Neuroradiology
1105
1107
Young Schwannoma Old Schwannoma [Figure 5-8-4]
Benign Cystic Degeneration
Figure 5-8-4
Vestibular Schwannoma
IAC origin
IAC involved
IAC Enlarged (70%)
Spherical Mass
encapsulated
Heterogeneous if large
> 20 mm
Enhance always
Trigeminal Schwannoma [Figure 5-8-5]

Figure 5-8-5
The larger and older Schwannoma is heterogeneous due to

benign cystic degeneration
Figure 5-8-6
Trigeminal Schwannoma may present as a dumbbell mass,

bilobed, with one lobe in the cavernous sinus and one in the
poster fossa lateral pontine cistern
Bilateral Vestibular Schwannoma
This mass is hemispheric but

does not extend into the canal.
[Courtesy of Bob Peyster, MD]
Meningioma
Hyperostosis
Meningioma
Figure 5-8-7
Tentorial Meningioma
Meningioma [Figures 5-8-6 and 5-8-7]
Tentorium or Dura
IAC Normal
Hemispherical
Enhance Homogeneous
Hyperostosis
15%-40%
Dural Tail
70%-90%
[left] T1W image no enhancement, undulating (wavy) margin.

Note the wispy internal structure.
[right] T2W image isointense to CSF
1106
1108
Neuroradiology
Epidermoid vs. Arachnoid Cyst
CLASSIC LOCATIONS
Figure 5-8-8
Epidermoid Inclusion Cyst [Figure 5-8-8]

CPA most common
Extraaxial CPA Lesion
IAC Normal
Undulating Margin
CSF - like
Not identical
NO Enhancement
Wispy internal structures
Arachnoid Cyst
Middle fossa common
Extraaxial CPA Lesion
IAC Normal
Rounded Mass
Identical to H2O on CT and all MR
sequences
T1, PD, T2, FLAIR, DWI, ADC
NO Enhancement
NO structure
Foramen magnum
Cerebellopontine angle
This epidermoid inclusion cyst

is only isointense to CSF on the T2W image
Figure 5-8-9
Central Posterior Fossa Lesion
Could be Intraaxial
Could be Intraventricular
Could be extending from vermis into ventricle
Could be extending from ventricle into vermis
Fourth Ventricle - Schematic [Figure 5-8-9]

Central Masses [Figure 5-8-10]
Schematic of central posterior fossa mass: Did it

begin in the 4th ventricle, in the medullary velum,
or in the cerebellum?
CHILD - CEREBELLAR/IVth
Medulloblastoma (PNET)
Astrocytoma (usu. Pilocytic)
Ependymoma
Post fossa cysts
Figure 5-8-10
ADULT - CEREBELLAR/IVth
Metastasis
Hemangioblastoma
Hemorrhage, infarct
Glioma
Ependymoma
Astrocytoma
Abscess
Schematic diagram of medulloblastoma and

ependymoma. Copyright 2005
Neuroradiology
1107
1109
Medulloblastoma [Figures 5-8-11 and 5-8-12]
Figure 5-8-11
Homogeneous
finely irregular
Cyst and Hemorrhage are uncommon
<10%
Hyperdense on NCT
up to 75%
densely cellular
sm. Round blue-cells
Center is behind 4th vent
Rounder not angular
Pilocytic Astrocytoma
[Figures 5-8-13 to 5-8-16]
Cyst and Mural Nodule

balanced morphology
Wall may not enhance
Cyst fluid with protein
Nodule low density on CT
may calcify up to 25%
No increase in vascularity
WHO Grade 1
Peak at ~10 yrs
Medulloblastoma, hyperdense on plain CT
Figure 5-8-12
Figure 5-8-13
Pilocytic astrocytoma: Classic cyst-with-nodule

morphology
Medulloblastoma
Figure 5-8-14
Figure 5-8-15

morphology

morphology
1108
1110
Neuroradiology
Good Rules for Practice [Figure 5-8-17]
Figure 5-8-16
Cerebellum GBM is uncommon

Pediatric GBM is uncommon
If I am wrong
Whats the worst that it could be?
Whats the best that it could be?
The Probability of tandem events occurring is the product of
multiplying the chance of the individual events. E.G. 1/1000 x
1/1000 = 1/million
Uncommon X Uncommon = RARE
Hemangioblastoma [Figures 5-8-18 and 5-8-19]

some solid
some nearly pure cyst
Wall may not enhance
Cyst fluid has protein
Nodule high density
No Ca++
Increased vascularity
Flow void
Blood products
WHO Grade 1
Peak at ~ 35 yrs
Multiple in VHL
Pilocytic astrocytoma: Classic cystwith-nodule morphology.

Copyright 2005
Figure 5-8-17
Figure 5-8-18
What is this? Come to the lecture

and find out!
Figure 5-8-19
Hemangioblastoma may be a cyst and nodule in about 1/3 of
cases. Solid forms, and almost completely cystic types occur
Hemangioblastoma may be a cyst

and nodule in about 1/3 of cases.
Solid forms, and almost completely
cystic types occur.
Copyright 2005
Neuroradiology
1109
1111
Expansile Mass in Brainstem
Figure 5-8-20
[Figure 5-8-20]
Intraluminal Mass of IVth

[Figure 5-8-21]
Figure 5-8-21
Expansile lesion of the pons and

medulla, encroaching on the 4th
ventricle, does not enhance
Figure 5-8-22
Ependymoma, arising from the 4th

ventricle floor, and remains within the
lumen of the ventricle
Ependymoma - Schematic [Figure 5-8-22]

Ependymoma [Figures
Intraventricular
Soft plastic lesion
Angular extensions
Luschka
Magendie
Heterogeneous
Cystic areas
Chunks of Ca++
Arise from floor of 4th
5-8-23 and 5-8-24]
Schematic of ependymoma
Copyright 2005
Figure 5-8-24
Figure 5-8-23
Ependymoma.
Copyright 2005
Ependymoma
1110
1112
Neuroradiology
Posterior Fossa Masses [Figure 5-8-25]
Figure 5-8-25
Lhermitte-Duclos [Figure 5-8-26]

CLASSIC LOCATIONS
Foramen magnum
Schematic Localization of posterior fossa masses.
Copyright 2005
SELLA/PARASELLAR REGION
Differential:
Pituitary adenoma
Craniopharyngioma
Aneurysm (ICA , etc.)
Meningioma
Optic/hypothalamic glioma
Chordoma
Granuloma, e.g., hamartoma,
cyst(arachnoid, dermoid/epi)
Germ Cell (Germinoma)
Figure 5-8-26
Pituitary Adenoma [Figure 5-8-27]
Adult Patient
Microadenoma
< 10 mm
Entirely within gland
Endocrine Sx
Prolactinoma
Acromegaly
Gigantism
Cushing Disease
Macroadenoma
> 10 mm
balloon sella
Visual Sx
if >6 mm above sella
bitemporal hemianopsia
Lhermitte-Duclos. T1 and T2 weighted images show a striated

or courduroy appearance, classic for dysplastic gangliocyoma
Figure 5-8-27
Elevated Prolactin
Microadenoma
< 10mm diameter
Entirely intrasellar
Macroadenoma
> 10 mm
Stalk Effect
Blocks Prolactin Inhibitory Factor
40-150 ng PRL vs. 28 for nl.
Hypothyroidism
Cross Reaction from TSH
Exogenous
Pharmacologic
Pituitary macroadenoma with hyperintensity from
old hemorrhage
Neuroradiology
1111
1113
Macroadenoma [Figure 5-8-28]
Figure 5-8-28
Sella and Suprasellar [Figure 5-8-29]

Craniopharyngioma 2 Types
Child
Adam Ant inomatous
enamel organ of tooth
Commonly Cystic
Machine Oil
Commonly Calcified
Adherent to brain
pilocytic astrogliosis
Adult
Squamous and Papillary
Commonly Solid
Calcification less common
Easier to resect
Pituitary macroadenoma with hyperintensity from old

hemorrhage
Craniopharyngioma [Figure 5-8-30]

Figure 5-8-29
Figure 5-8-30
Craniopharyngioma, you barely see

how the hypothalamus is draped over
the top of the mass
Craniopharyngioma,
expansile remodeling of sella
turcica
Craniopharyngioma Machine Oil
Figure 5-8-31
Craniopharyngioma [Figure 5-8-31]
Craniopharyngioma
1112
1114
Neuroradiology
Where is the Hypothalamus ?
Figure 5-8-32
[Figure 5-8-32]
Hypothalamic Glioma Pilocytic astrocytoma
Where is the Clivus?

[Figure 5-8-33]
Chordoma:
Notochord rests
Midline
Bone destruction
Figure 5-8-33
Hypothalamic Glioma Pilocytic astrocytoma
Figure 5-8-34
Chordoma of the clivus
Schematic of location of remnant notochord tissue.

Copyright 2003
Notochord: Chordoma & Thornwaldt

[Figure 5-8-34]
SELLA/PARASELLAR
Figure 5-8-35
Differential Features:
ADULT Pituitary adenoma
CHILD Craniopharyngioma or
Glioma (hypothalamus or optic ) >
EG, etc
SELLA NORMAL NOT pituitary
Ca++ Craniopharyngioma, but...
HYPEROSTOSIS Meningioma (
exp. blistering )
CLIVUS Chordoma, mets, NP Ca
Remember rule out vascular
lesions (aneurysms)
Pulsation Artifact: Phaseencoding direction [Figure 5-8-35]
Giant (> 2.5 cm) cerebral aneurysm
CLASSIC LOCATIONS
Foramen magnum
Neuroradiology
1113
1115
Figure 5-8-36

Colloid Cyst [Figure 5-8-36]
Hydrocephalus: Vents > Sulci
THIRD VENTRICLE [Figure 5-8-37]
Differential:
Colloid cyst
Cysticercosis
Craniopharyngioma
Hypothalamic and thalamic glioma
CPP, ependymoma
Neurocytoma
Basilar tip aneurysm
Colloid cyst
Figure 5-8-37
Colloid Cyst [Figure 5-8-38 to 5-8-40]

Figure 5-8-38
Colloid cyst
Differential Diagnosis 3rd ventricle.

Copyright 2005
Figure 5-8-39
Figure 5-8-40
Colloid cyst. T1-weighted image shows slight

hyperintensity before gadolinium and no definite
enhancement
Colloid Cyst. Marked hypointensity on T2W

image
1114
1116
Neuroradiology
HYDROCEPHALUS
Figure 5-22-41
Over production of CSF (CPP)
Obstruction of CSF flow:
Obstructive/internal hydrocephalus
Communicating/external hydrocephalus
Under reabsorption of CSF: SAH
Compensatory:
Ex vacuo/enlargement
CSF Homeostasis [Figure 5-8-41]

Normal Ventricular System [Figure 5-8-42]
Schematic of CSF Homeostasis
Copyright 2004
Figure 5-22-42
Schematic of Normal Ventricular System

Copyright 2004
Foramen of Monro Obstruction

Aqueduct Obstruction
Non-traumatic hemorrhage
[Figures 5-8-43 and 5-8-44]
Figure 5-22-44
Figure 5-22-43
Non-traumatic hemorrhage in the right thalamus.

Copyright 2006
Non-traumatic hemorrhage in the right thalamus
Neuroradiology
1115
1117
Hypertensive Hemorrhage
Figure 5-8-45
Hemorrhage into a mass
NOTE: Vasogenic Edema
ARTERIOLOSCLEROSIS
What do they have in
Common? [Figure 5-8-45]
Multiple
Bilateral
Symmetric
Anatomic
Basal ganglia
Toxic and/or Metabolic:
Acquired
Congenital
CT medial lenticular lesion Globus Pallidus.

MR lateral lenticular lesion Putamen
CO Poisoning [Figure 5-8-46]
Figure 5-8-46
MetOH Intoxication
Tx for MetOH - Fomepazole
Fomepazole (Antizole, 4-methylperazole) is a

synthetic alcohol dehydrogenase inhibitor for IV
administration
Clear yellow liquid, mw 82.1, mp 25 C (77 F)
INDICATIONS: Antidote for ethylene glycol, or
methanol poisoning of suspected EG ingestion
PRECAUTIONS: Dilute in > 100 mL NS, follow
hepatic enzymes & WBC (eos) during Rx,
interaction with ethanol (compete for ADH)
DOSE: 15 mg/kg load, 10 mg/kg Q 12 h x 4
doses, then 15 mg/kg Q 12 h till EG < 20 mg/dL
Deep Lesions
White Matter:
Leukoencephalopathy
Bad White Matter Disease
Small vessel disease
Hypertension
Glial Neoplasm
Astrocytoma (incl. GBM)
Oligodendroglioma
Deep White and Gray Matter
Lymphoma
Toxoplasmosis
Both occur in HIV/AIDS, multiple lesions
Carbon monoxide toxicity. Notice there are

bilateral medial lenticular (globus pallidus) signal
abnormalities hypointense on T1 and
hyperintense on T2
Figure 5-8-47
Solitary Deep Lesion - Thalamus [Figure 5-8-47]

Glioblastoma WHO Grade 4
A solitary, deep, irregular, heterogeneous, ring-enhancing mass with

vasogenic edema
Glioblastoma multiforme of the

thalamus and temporal lobe
1116
1118
Neuroradiology
Glioblastoma Multiforme [Figure 5-8-48]
Figure 5-8-48
Toxoplasmosis [Figure 5-8-49]
2 patients with typical lesions
BASAL GANGLIA THALAMUS:
BILATERAL SYMMETRIC
(toxic/metabolic):
PUTAMEN Methanol
GLOBUS PALLIDUS CO Poisoning
BILATERAL ASYMMETRIC
(hematogenous):
INFECTION (TOXO, etc.)
UNILATERAL (acquired/neoplastic):
THALAMIC GLIOMA (astrocytoma)
HYPERTENSIVE HEMATOMA
(exclusion)
T2 - T1gad
Glioblastoma multiforme with extensive vasogenic edema
CLASSIC LOCATIONS
Figure 5-8-49
Foramen magnum
Lateral ventricle
Pineal Region
Patterns in Neuroradiology
Cerebello-Pontine Angle
Fourth Ventricle/Cerebellum
Sella/Parasellar
Basal Ganglia/Third Ventricle
Lateral Ventricle
Pineal Region
Deep Hemispheric/Periventricular
Cortical/Subcortical
Intraventricular Neoplasms
Ependymoma (and subependymoma)

Choroid plexus papilloma
Subependymal giant cell astro.
Meningioma
Colloid cyst (3rd)
Medulloblastoma (4th)
Dermoid/epidermoid
Central neurocytoma
Mets, lymphoma, Germ Cell
Toxoplasmosis
Subependymal Giant Cell Astro
Neuroradiology
(From Vince Mathews,

M.D. IU)
1117
1119
Lateral Ventricle @ f. Monro
Figure 5-8-50
Lateral Ventricle/caudate
Subependymal Giant Cell Astro.
TUBEROUS SCLEROSIS, Enhances &
Ca++
Subependymoma
Variant of Ependymoma
No Ca++, no enhancement
Central Neurocytoma
Septum pellucidum
Cyst/Cavum septum pellucidum
Huntington Chorea
Atrophy
Lateral ventricular masses [Figure 5-8-50]

Lateral Ventricle - Trigone
Schematic of lateral ventricular masses.

Copyright 2005
Meningioma
Xanthogranuloma
Metastasis
Lipoma
Choroid Cyst
Trigone or Atrium
Trapped Temporal Horn
Attached to normal Choroid Plexus

Lobulated
Fronds
Papillae
Trigone of lateral vent
Children
Fourth ventricle
Adults
Third ventricle
CPA cistern
Hydrocephalus
Obstruction
Production =/= Resorption
CSF Overproduction?
Central Neurocytoma
Central
Often centered on septum pellucidum
Extension into both lateral ventricles
Hyperdense on CT
Gray matter on MR
Spontaneous Bleed
Calcifications
CLASSIC LOCATIONS
Foramen magnum
1118
1120
Neuroradiology
Figure 5-8-51

Pineal/Quadrigeminal Cistern Region
Pinealomas
Germ cell tumors
Seminoma
Teratoma
Pineal cell tumors
Pineoblastoma
Pineocytoma
Gliomas (regional)
Brainstem, callosum, thalamus
Other
Dermoid, lipoma, arachnoid cyst
Meningioma
Vein of Galen malformations
Pineal region mass. Germinoma.

Copyright 2005
Figure 5-8-52
Pineal Region Mass [Figure 5-8-51]

Germinoma [Figure 5-8-52]
Central
Pineal Region
Suprasellar Cistern
Homogeneous
Hyperdense to GM
Isointense to GM
Uniform Enhancement
CSF Seeding ?
May ENGULF Pineal Ca++
Pineal region Germinoma
Pineal Cyst-Asymptomatic
[Figure 5-8-53]
Figure 5-8-53
T1W sagittal and T1W-Gd axial

Cyst is ovoid and hypointense
Enlargement of pineal
Cyst wall enhances minimally
Quadrigeminal plate not compressed
Pineal Cyst
CLASSIC LOCATIONS
Foramen magnum
Pineal cyst
DEEP AND PERIVENTRICULAR
Glioma (astrocytoma, oligodendro.)

Lymphoma (usually primary in CNS)
Toxoplasmosis, CMV (ependymitis)
Leukoencephalopathy (WM)
Arteriolar sclerosis (HT)
Infarcts (lacunar, tri-watershed)
Hemorrhage
Neuroradiology
1119
1121
Glioblastoma Multiforme
[Figures 5-8-54 and 5-8-55]
Figure 5-8-54
Figure 5-8-55
Expansile lesion of the corpus callosum
Lymphoma
PCNSL [Figure 5-8-56]
Two butterfly lesions. One with peripheral dense

enhancement is a GBM; and, the other with softer more
uniform enhancement is primary CNS lymphoma
Figure 5-8-56
Figure 5-8-57
FLAIR
T2W
T1W Gd+
Primary CNS Lymphoma an expansile
enhancing lesion of the corpus callosum
PCNSL: Immunocompetent
Cytomegalovirus note the thin rim of abnormal
enhancement cause by ependymitis
(Courtesy Vince Mathews, M.D.)
RIM PHOMA
CMV [Figure 5-8-57]
Multiple Sclerosis [Figure 5-8-58]
CLASSIC LOCATIONS
Figure 5-8-58
Foramen magnum
Multiple Sclerosis. Classic Dawson fingers ovoid
lesions perpendicular to the ventricle from
perivenous inflammation
1120
1122
Neuroradiology
CONVEXITY INTRAAXIAL
Figure 5-8-59
Gray-white junction
Hematogenous neoplasm
Hematogenous infection
Hematogenous thrombi (multiple infarcts)
Infarction/ischemia
Vasculitis (infectious,autoimmune)
Hematogenous dissemination [Figure 5-8-59]
Multiple
Cortical/subcortical
Ring Lesions
smooth
round
uniform thickness
CEREBRAL INFARCTION [Figure 5-8-60]
Abrupt Onset
Gray Matter Involved
Little Mass Effect
Vascular Territory & Wedge Shape
Hematogenous dissemination.
Multiple cortical ring-enhancing
lesions necrotic metastases from
breast carcinoma on chemotherapy
Figure 5-8-60
Cerebral infarction. MCA territory, with matching

lesions on DWI and ADC map
Figure 5-8-61
Time is Brain !
Therapeutic Windows:
3 hours for IV tPA
6 hours for IA thrombolysis
9 hours for IV Bat Spit?
an enzyme known as
desmoteplase or DSPA
isolated from the saliva of
Desmodus rotundus
vampire bat, Central and
South America, 1oz
NOTE: Clock starts with last time
patient was observed normal. If
you wake with a stroke, that
might be bedtime unless you
get up at night
PCA Infarct
Lights up like a lightbulb

on MRI DWI
PCA Infarct [Figure 5-8-61]

Neuroradiology
1121
1123
CVA : Progression of CT findings
Figure 5-8-62
Old Infarct
Wallerian Degeneration

(DNET) [Figures 5-8-62 and 5-8-63]
Imaging Features
Cortical, most in temporal lobe
Hypointense T1W, Hyperintense T2W
Nodular Cortical Mass
Multicystic
Megagyric - Assoc. calvarial erosion
No Edema
No or Minimal Mass Effect
+/- Calvarial Erosion
Occasional Enhancement
DNET Usually a cortical lesion, often wedgeshaped
Figure 5-8-63
DDX: TUBEROUS SCLEROSIS
Cortical Hamartomas (Tubers):

Appear to Spare Superficial Cortex
Tend to be Multiple
Accompanying Subependymal Nodules
+ Family History
Generalized Seizures
CLASSIC LOCATIONS
Foramen magnum
DNET Usually a cortical lesion, often wedgeshaped
CONVEXITY EXTRAAXIAL Differential [Figure 5-8-64]
EPIDURAL (sub-periosteal)
(Hematoma, empyema, mets)
(biconvex, acute, limited by sutures)
SUBDURAL (epi-arachnoid)
(Hematoma, empyema, mets)
(Crescentic, subacute, crosses sutures)
MENINGIOMA
(hyperdense, hemispheric,hyperostosis,
homogeneous enhancement)
Figure 5-8-64
Epidural = Subperiosteal [Figure 5-8-65]

Epidural Metastasis
Schematic of epidural (left) and subdural (right)

localization.
Copyright 2006
Subdural = Epi-arachnoid [Figure 5-8-66]

Subdural Metastasis
Meningioma Pre and Post Gd
1122
1124
Neuroradiology
Meningioma - Dural Tail
Figure 5-8-65
MENINGIOMA [Figure 5-8-67]
The 4H+ Tumor

homogeneous
hyperdense
homogeneous enhancement
hemispheric
hyperostosis
hormonally modulated
Figure 5-8-66
Epidural hematoma
Bilateral chronic subdural hematomas
Figure 5-8-67
Meningioma. Hyperdense on plain CT, hemispheric,

homogeneous enhancement, hyperostosis the 4H+ tumor
Neuroradiology
1123
1125
Patterns of Enhancement
Figure 5-9-1
Why Give Contrast?

Contrast Enhancement
Vascularity
Blood Volume (rCBV)
Blood Flow (rCBF)

Arteries & veins > capillary
Permeability
Capillary (leakage)
Perfusion MTT
Blood
Brain
Barrier
Breakdown
Mechanisms of Enhancement [Figure 5-9-1]
Vascularity
Permeability
VASCULAR (intravascular) PHASE

Inc. Blood Flow/Hypervascular
AVM, Meningioma, GBM
TRUE "Luxury" Perfusion
Hyperemic Swelling (malignant brain edema)
INTERSTITIAL (extravascular) PHASE
Blood-brain-barrier breakdown)
Acute inflammation (MS)
Neoplasm, Abscess, granulation tissue
Ischemia, luxury perfusion, contusion
Two mechanisms of Contrast Enhancement:

Increased vascularity (rCBV and rCBF); and,
increased permeability from breakdown of the
blood-brain-barrier (BBBB)
Figure 5-9-2
Types of Radiology Contrast
Barium (BaSO4) for ingestion and enema

Insoluble suspension
Iodine for ingestion and enema
Gastrograffin
I+ and Gd+ Intravascular Contrast Agents
Ionic Contrast
High Osmolarity
- Magnevist a chelate of Gd
- di-N-methylglucamine salt of gadopentetate
(Gd-DTPA)
Iso and Low Osmolar
- Iodixanol
Non-Ionic Contrast
ProHance Gadoteridol
Omniscan - Gadoimide
Time Density Curves [Figure 5-9-2]

Variability in BBB permeability
and perfusion [Figure 5-9-3]
1124
1126
The bolus creates a high intravascular

concentration gradient that pushes contrast
across a permeable membrane into the tissue
interstitial space
Figure 5-9-3
Variable
degrees of
permeability
alteration may
create variable
time-density
curves for
interstitial
(extravascular)
enhancement
Neuroradiology
Double Dose of Contrast
Figure 5-9-4
Steroid Suppression of Enhancement

[Figure 5-9-4]
MR vs. CT [Figures 5-9-5 and 5-9-6]

Figure 5-9-5
Steroids may reduce or completely suppress

visible enhancement
Enhancement on CT and MR are similar except

for intraarterial and pachymeningeal (dural)
enhancement
What makes the BBB?
Figure 5-9-6
Semi-permeable Capillaries
Brain
Blood-brain-barrier
Testicle
Blood-Testicle barrier
Ovary
Blood-Ovary barrier
Ultrastructure of BBB [Figure 5-9-7]
Neural capillary
astrocytic feet
continuous BM
tight junctions
no pinocytosis
Non-neural or ABBB
no astrocytic feet
fenestrated BM
intercellular gaps
pinocytosis
Figure 5-9-7
Enhancement on CT and MR are
similar except for intraarterial and
dural enhancement
Schematic of Ultrastructure of blood-brain-barrier
Neuroradiology
1125
1127
The Berlin Wall and the BBB [Figure 5-9-8]
Figure 5-9-8
Who built the Berlin Wall?

The East Germans
Why?
To keep out the West Germans
But the barrier works in BOTH directions
Some things are kept out
Drugs, Contrast material
Some things are kept in:
Hemosiderin
Vasogenic Edema
CNS: Normal Tissues w/o BBB
DURA (falx and tentorium)

ARACHNOID ? (its avascular)
CHOROID PLEXUS
PINEAL GLAND (epiphysis)
PITUITARY GLAND (hypophysis)
CTZ (area postrema of medulla oblongata)
one of the "Circumventricular Organs"
Hemosiderin cannot be cleared from the brain and

spinal cord because of the blood-brain-barrier
Physiologic Why?
Why do we have a BBB?

To protect the brain
To create the ionic environment for nerve conduction
Why do we have tastebuds?
So that we eat things good for us
Salt
Sweet
Sour
Umami (MSG)
Bitter
Normal Enhancement
Choroid Plexus
Pineal
Pituitary Stalk
Pituitary Gland (anterior and posterior)
Hypophyseal Portal System
Cavernous sinus and dural reflections
Nasal turbinates
Sinonasal mucosa
Extracranial muscles and mucosa
Plain vs. Enhanced

Fat -Suppressed T1W - Gd
Nasal Cycle
Vasocongestion ~/~ vasoconstriction

6 8 hour cycle alternation
Humidify and warm the air
Secrete mucus (1 2 liters/day)
Chronic vasocongestion would cause submucosal edema
Breathe mostly through the vasoconstricted side (~ 75%-85%)
Yogis can control which nostril
So can Tom Cruise (Minority Report)
1126
1128
Neuroradiology
Fat -Suppressed T1W Gd
Figure 5-9-9
Musk Ox Nasal Turbinates

Fat -Suppressed T1W - Gd [Figure 5-9-9]
Cranial Nerve Enhancement
Optic Nerve Never normal

* Seventh Nerve:
Inside facial canal Yes, asymmetric ~70%
Geniculate ganglion 98%
Tympanic > labyrinthine > mastoid
May represent perineural vessels
Eighth Nerve Never normal
Abnormal optic nerve enhancement

optic neuritis
* Neuroradiology 1997 Mar;39(3):207-12.
Figure 5-9-10
Plain vs. Enhanced [Figure 5-9-10]

Contrast Enhancement Phases
ANGIO(I)
R-N (Tc+)
C.T. (I)
MRI (Gd+)
VASCULAR
++++
+ (flow)
+
+/
BBB
+ (static)
+++
+++
Enhancement vs. Edema
Normal pineal enhancement. Visualization

depends on the timing of injection and the
molecular weight of the contrast
Morphologic Patterns
Homogeneous (solid)
Heterogeneous (non-uniform)
Ring (unilocular/multilocular)
Serpentine ("Gyriform)
Serpiginous?
Serpiginous
A creeping skin eruption.
Location
SUPERFICIAL (CORTICAL/GYRAL)
GREY-WHITE JUNCTION
DEEP WHITE MATTER
PERIVENTRICULAR, EPENDYMAL
Neuroradiology
1127
1129
Figure 5-9-11
Cortical/Gyriform
Cerebral Ischemia / Infarction
CSF or sub-pial spread
Meningo-encephalitis
S.A.H.
Leptomeningeal Malformation (SW)
Meningioangiomatosis (NF2)
Listeria Monocytogenes [Figure 5-9-11]

CNS Bacterial Infections
Birth to Four Weeks

2-10 cases / 10k births
Group B streptococcus
E. coli
Listeria monocytogenes
3 mo. to 3 yrs
Haemophilus influenzae (Type B)
Strep pneumoniae
Meningococcus (Neisseria
meningitidis)
Over 3 yrs to Adult
Strep pneumoniae
N. meningitidis
CSF Signal ? [Figure 5-9-12]
Meningitis - Listeria Monocytogenes
Figure 5-9-12
Flair shows dirty CSF from protein and pus in the SAS
(Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)
Figure 5-9-13
Enhancement? [Figure 5-9-13]
Leptomeningeal Enhancement Pneumococcal Meningitis
Pachymeningeal Enhancement
[Figure 5-9-14]
Intracranial Hypotension
Figure 5-9-14
Multiple symmetric areas of abnormal leptomeningeal
enhancement from meningitis Notice the abnormal
ehancement of the entire suprasellar cistern
(Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)
Pachymeningeal
enhancement
(Courtesy Laszlo Mechtler,
DNI)
1128
1130
Neuroradiology
Hemorrhagic Infarction [Figure 5-9-15]
Figure 5-9-15
Ischemic Enhancement
Acute and/or Reperfusion enhancement

True luxury perfusion 2 to acidosis
BBBB after 4-6 hours of ischemia
Subacute to Chronic enhancement
Ingrowth of capillaries from surface
Primarily in GM (cortex and deep)
Peak intensity at 2-3 weeks
Fades away over weeks to months
Atrophy replaces Enhancement
Blood Brain Barrier [Figure 5-9-16]
Hemorrhagic infarction shows early and dense

enhancement due to reperfusion
Cirrhosis
Hyperbilirubinemia
Bilirubin bound to Albumin
Albumin cant cross the Blood-brain-barrier
BBB is abnormal in infarct
Mostly gray-matter
Figure 5-9-16
H&P
Pt is a 25 yo woman, PMHx of BCP, presenting w/

acute mental status changes, afebrile
Actually
Pt is a 34 yo marine stationed at Guantanamo Bay
Cuba, presenting w/ acute mental status changes,
febrile
HSV Encephalitis [Figure 5-9-17]

Figure 5-9-17
Gyral enhancement in Sturge-Weber disease
Figure 5-9-18
Herpes encephalitis
Meningioma Dural Tail [Figure 5-9-18]

Dural Tail
Meningioma - Dural Tail
Curvilinear enhancement
AKA dural flair
First reported w/meningioma
First reported to be neoplastic invasion
What is it REALLY?
Thickening of the dura
Vasocongestion of the dura
Edema of the dura
Neuroradiology
1129
1131
Contrast Enhancement [Figure 5-9-19]
Figure 5-9-19
Ring Lesion
Circumferential or peripheral/marginal
enhancement, surrounding a central nonenhancing region.
In turn, this is often surrounded by a large area
of edema.
May be unilocular or multilocular.
Rules for Ring Enhancing Masses

[Figure 5-9-20]
Abscess
Figure 5-9-20
Differential appearance of ring enhancing lesions
Contrast leaks into interstitium from vessels without BBB

Remains localized within millimeters of where it leaks out
Not simple diffusion but rather BULK FLOW at a very slow rate
(Glacier Not River)
Ring Lesions Differential

M Metastasis, MS
A Abscess (Also Cerebritis)
G Glioblastoma, Granuloma
I Infarct (Esp. Basal Ganglia)
C Contusion (Rare)
A AIDS (Toxo, Etc.)
L Lymphoma (in Aids)
D Demyelination (Active)
R Resolving Hematoma
Radiation Change (Necrosis)
Ring Lesion Features For Infection

ORGANIZED ABSCESS
thin and uniform wall (3-7mm.)
smooth inner margin does not fill in on CT, MR, even after time delay
imaging
CEREBRITIS (infection w/o organization):
variable wall (may be smooth) smooth/variable inner margin
often has fill-in on DDD
(w/o fluid level)
1130
1132
Neuroradiology
Contrast Enhancement- Abscess
2 4 wks. for ORGANIZED WALL

2 LAYERS
inner MESENCHYMAL (capillaries,fibroblasts, collagen)
outer ASTROGLIAL (reactive astrocytes)
WALL facing GM is well formed 3-5 mm
WALL FACING WM IS THINNER/WEAKER (Daughter Abscess)
Ventricular Spill (pyocephalus)
Figure 5-9-21
Abscess [Figures 5-9-21 to 5-9-23]
Round
Smooth
Regular
Convex all around
Rim of Edema
Restricted Diffusion
MRS shows
AA peaks
Acetate
Succinate
Figure 5-9-22
Cerebral abscess in thalamus
Figure 5-9-23
Abscess. Viscous Pus and Coagulation Necrosis

cause restricted diffusion
Figure 5-9-24
Abscess
Contrast Enhancement [Figure 5-9-24]
Ring Lesion Features For Neoplasm

NECROTIC NEOPLASM:
thick and irregular wall
shaggy inner margin (usually)
may fill in heterogeneously on DDD
CYSTIC NEOPLASM:
thin wall +/ MURAL NODULE
PART OF WALL MAY NOT ENHANCE
smooth inner margin
uniform fluid enhancement
or FLUID LEVEL
Neuroradiology
Ring enhancing lesion: Glioblastoma
1131
1133
Figure 5-9-25
Pilocytic Astrocytoma [Figure 5-9-25]
Cyst with mural nodule?
Tumefactive Demyelination
[Figure 5-9-26]
Open (Incomplete) Ring Sign
Demyelinating Disease
Fluid-secreting (Cystic) Neoplasms
Masdeau JC, Moreira J, Trasi S, Visintainer

P, Cavaliere R, Grundman M: The open ring.
A new imaging sign in demyelinating disease.
J Neuroimaging 1996; 6(2):104-107.
Figure 5-9-26
Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D,

Visintainer P: Open-ring imaging sign: highly specific for
atypical brain demyelination. Neurology 2000;
54(7):1427-1433
Contrast Enhancement: Hematoma
EARLY: Hyperdense, round/oval homogeneous

mass of RBCs with proportional mass effect for
volume Edema Halo, not spreading
LATER: Iso-/Hypodense, smaller Reactive capillaries
form outside Uniform rim of enhancement May see
vasogenic edema spreading
Hematoma Halo of serum [Figure 5-9-27]
Tumefactive Demyelination
Figure 5-9-28
Figure 5-9-27
Acute Hematoma - halo of edema

Subacute to chronic may have vasogenic edema
Acute Hematoma - halo of edema

Subacute to chronic may have vasogenic edema
Figure 5-9-29
Reactive Ring Enhancement [Figure 5-9-28]

MCA infarct [Figure 5-9-29]
Ring Enhancing Mass
Benign
Round
Smooth
Thin wall
Malignant
Undulating
Irregular
Thick wall
MCA infarct involving cortex and basal ganglia
1132
1134
Neuroradiology
Post-Operative Enhancement
Figure 5-9-30
RESIDUAL TUMOR
Left behind
RECURRENT TUMOR
It grew back
Infection
Normal Postoperative Change
surgical trauma, healing, gliosis
Radiation Necrosis
Contrast Enhancement Surgical Change

and/or Residual Neoplasm? [Figure 5-9-30]
Surgical Enhancement typically after 24-48 hrs

Scan early (24 hours) or scan late (4-6 wks)
May fade after a few weeks but may last for
months
Gd+ enhancement may begin in 4-6 hours
In the Operative Bed
Mixed w/ residual tumor?
Along the Margins of Resection
Thin and uniform in brain (CT/MR)
LINEAR meningeal/dural enhancement on MR
Not lumpy-bumpy
Small amts of air, blood are normal
No instruments or sponges, etc.!!
Normal enhancement after ventricular shunt

catheter insertion
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Ahmadi J, Hinton DR, Segall HD, Couldwell WT. Surgical implications of magnetic resonance-enhanced dura.
Neurosurgery. 1994 Sep;35(3):370-7;discussion 377.
Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance
of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990 Sep-Oct;11(5):935-8.
Asari S, Yabuno N, Ohmoto T. Magnetic resonance characteristics of meningiomas arising from the falcotentorial
junction. Comput Med Imaging Graph. 1994 May-Jun;18(3):181-5.
Ekinci G, Akpinar IN, Baltacioglu F, et al. Early-postoperative magnetic resonance imaging in glial tumors:
prediction of tumor regrowth and recurrence. Eur J Radiol 2003; 45:99-107.
Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural "tail" associated with meningiomas on Gd-DTPAenhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment.
Radiology. 1990 Aug;176(2):447-50. .
Helie O, Soulie D, Sarrazin JL, Derosier C, Cordoliani YS, Cosnard G. [Magnetic resonance imaging and
meningiomas of the posterior cerebral fossa. 31 cases] J Neuroradiol. 1995 Dec;22(4):252-70. French.
Henegar MM, Moran CJ, Silbergeld DL. Early postoperative magnetic resonance imaging following nonneoplastic
cortical resection. J Neurosurg 1996; 84:174-179.
Hutzelmann A, Palmie S, Buhl R, Freund M, Heller M. Dural invasion of meningiomas adjacent to the tumor
margin on Gd-DTPA-enhanced MR images: histopathologic correlation. Eur Radiol. 1998;8(5):746-8.
Hutzelmann A, Palmie S, Freund M, Buhl R, Heller M. [Dura thickening adjacent to intracranial, para-dural spaceoccupying lesions in MRI. Histologic correlation] Aktuelle Radiol. 1997 Nov;7(6):305-8. German. PMID:
9467021
Hutzelmann A, Palmie S, Zimmer C, Benz T, Leweke F, Freund M. [The meningeal sign: a new appraisal] Rofo.
1996 Apr;164(4):314-7. German.
Ildan F, Tuna M, Gocer AP, Boyar B, Bagdatoglu H, Sen O, Haciyakupoglu S, Burgut HR. Correlation of the
relationships of brain-tumor interfaces, magnetic resonance imaging, and angiographic findings to predict cleavage
of meningiomas. J Neurosurg. 1999 Sep;91(3):384-90.
Kaufman BA, Moran CJ, Park TS. Computer tomographic scanning within 24 hours of craniotomy for a tumor in
children. Pediatr Neurosurg 1995; 22:74-80.
Kawahara Y, Niiro M, Yokoyama S, Kuratsu J. Dural congestion accompanying meningioma invasion into vessels:
the dural tail sign. Neuroradiology. 2001 Jun;43(6):462-5.
Lai PH, Ho JT, Chen WL, et al. Brain abscess and necrotic brain tumor: discrimination with proton MR
spectroscopy and diffusion-weighted imaging. AJNR Am J Neuroradiol 2002; 23:1369-1377.
Neuroradiology
1133
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15. Martin-Duverneuil N, Sola-Martinez MT, Miaux Y, et al. Contrast enhancement of the facial nerve on MRI:
normal or pathological? Neuroradiology 1997; 39:207-212.
16. Masdeau JC, Moreira J, Trasi S, Visintainer P, Cavaliere R, Grundman M: The open ring. A new imaging sign in
demyelinating disease. J.Neuroimaging 1996; 6(2):104-107.
17. Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer P: Open-ring imaging sign: highly specific for
atypical brain demyelination. Neurology 2000; 54(7):1427-1433.
18. Nagele T, Petersen D, Klose U, Grodd W, Opitz H, Voigt K. The "dural tail" adjacent to meningiomas studied by
dynamic contrast-enhanced MRI: a comparison with histopathology. Neuroradiology. 1994 May;36(4):303-7.
19. Nakasu S, Nakasu Y, Matsumura K, Matsuda M, Handa J. Interface between the meningioma and the brain on
magnetic resonance imaging. Surg Neurol. 1990 Feb;33(2):105-16.
20. Nakau H, Miyazawa T, Tamai S, Tsuchiya K, Shima K, Shirotani T, Chigasaki H. Pathologic significance of
meningeal enhancement ("flare sign") of meningiomas on MRI. Surg Neurol. 1997 Dec;48(6):584-90; discussion
590-1.
21. Quekel LG, Versteege CW. The "dural tail sign" in MRI of spinal meningiomas. J Comput Assist Tomogr. 1995
Nov-Dec;19(6):890-2.
22. Sakai K, Tada T, Fukasaku K, Kyoshima K, Kobayashi S. Histological examination of the gadolinium-enhanced
dura mater around meningiomas on magnetic resonance imaging. Neurol Med Chir (Tokyo). 1993 Jul;33(7):42933.
23. Sato M, Matsumoto M, Kodama N. Meningeal enhancement surrounding meningiomas on Gd-DTPA MRI.
Fukushima J Med Sci. 1998 Jun;44(1):1-11.
24. Sato N, Bronen RA, Sze G, et al. Postoperative changes in the brain: MR imaging findings in patients without
neoplasms. Radiology 1997; 204:839-846.
25. Sekiya T, Manabe H, Iwabuchi T, Narita T. [The dura mater adjacent to the attachment of meningiomas: its
enhanced MR imaging and histological findings] No Shinkei Geka. 1992 Oct;20(10):1063-8. Japanese.
26. Takeguchi T, Miki H, Shimizu T, Kikuchi K, Mochizuki T, Ohue S, Ohnishi T. The dural tail of intracranial
meningiomas on fluid-attenuated inversion-recovery images. Neuroradiology. 2004 Feb;46(2):130-5. Epub 2004
Jan 28.
27. Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L, Baert AL. Thickening of dura
surrounding meningiomas: MR features. J Comput Assist Tomogr. 1989 Sep-Oct;13(5):763-8.
28. Yamaguchi N, Kawase T, Sagoh M, Ohira T, Shiga H, Toya S. Prediction of consistency of meningiomas with
preoperative magnetic resonance imaging. Surg Neurol. 1997 Dec;48(6):579-83.
1134
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Neuroradiology
Radiologic Grading of Astrocytoma and

The WHO 2000 Brain Tumor Classification
Brain Neoplasia: Frequency [Figure 5-10-1]
Figure 5-10-1
Childhood CNS Tumor Demographics
367 Syrian children, collected from 1993-2002

Supratentorial - 47%
Infratentorial - 53%
Male 52%:48% Female
Overall Incidence:
Medulloblastoma 27%
Astrocytoma 26%
Craniopharyngioma 14%
Posterior Fossa Only:
Medullo (PNET) 54%
Astrocytoma 23%
Ependymoma 17%
The frequency of the various primary central

Kadri H,Mawla AA, Murad L: Incidence of childhood brain
nervous system tumors ranges from 2% for
tumors in Syria (1993-2002) Pediatric Neurosurgery
meningioma
and mixed oligoastrocytoma to 40%
2005; 41:173-177
for glioblastoma multiforme (GBM) and 42% for
infiltrative astrocytoma
Pediatric Posterior Fossa
454 posterior fossa patients

All under the age of 18
402 tumors:
37.1% Cerebellar astrocytoma (149)
34.6% Medulloblastoma (PNET) (139)
11.4% Brain stem astrocytoma (46)
7% Ependymoma (28)
9.9% "other" (40)
Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst
1998; 14:426-439.
Traditional Tumor Grading
PATHOLOGIST
LOW GRADE
HIGH GRADE
RADIOLOGIST
NON-ENHANCING
ENHANCING
NEUROSURGEON
SUCKABLE
NON-SUCKABLE
Kernohan-Sayre (AFIP) Grading System:
GRADE I BENIGN or Low-Grade

GRADE II BENIGN or Low-Grade
GRADE III ANAPLASTIC
atypia, pleomorphism, mitoses, etc.
GRADE IV- MALIGNANT
Mitoses, Vascularity, Endothelial changes
Necrosis
NOTE: Numerous modifications exist, most into three grades, e.g..: Low Grade
(Benign), Anaplastic, and GBM (w/ NECROSIS).
Neuroradiology
1135
1137
Pathologic Radiologic Correlation

Pathology
Radiology
Cellularity
T2 SI, DWI & ADC
Endothelial proliferation
and Vascularity
Necrosis
Enhancement, PWI, and

Permeability Imaging
Ring Lesion, MRS, DWI & ADC
Hemorrhage
T1 and T2 SI
Labeling Indices
MRS, Th 201 and FDG
Infiltration
T1 and T2 SI, DTI
ASTROCYTOMA: Five Year Survival

Grading Systems: Sem Rad Onc (1991); 1: 2-9
Kernohan
Berger
Benign (1)
Astrocytoma
WHO 2000
1 Pilocytic,SEGA
2 Astrocytoma
Benign (2)
Anaplastic
3 Anaplastic
Glioblastoma
4 Glioblastoma
Anaplastic (3)
Glioblastoma (4)
Define the Problem
Some Low Grade Enhance

Some Low Grade Do Not
Some Low Grade => GBM
WHO Classification
Defines Histologic Subtypes

Grades Biologic Potential
Allows International Cooperation
Ascending scale of Aggression from 1-4
WHO Correlation
Low Grade
Long-Term Survival:
Possible Cure
Stable History (No Progression): Possible Cure
WHO Grading CNS Tumors

GRADE 1
GRADE 2
GRADE 3
GRADE 4
JPA
PXA
PXA
GBM
SGCA
GANG
MENING
HPC
HPC
ANAPLASTIC
CNS NEOPLASM-GLIAL: Prognostic Factors
Location
Age
Histology
1136
1138
Neuroradiology
BENIGN ASTROCYTOMA
Figure 5-10-2
Two types:
Low grade (benign)
Diffuse (Adults)
Low grade special
Circumscribed (Children)
Normal
Diffuse Astrocytoma [Figure 5-10-2]
WHO Gr1 - Pilocytic Astrocytoma [Figure 5-10-3]
Circumscribed vs Diffuse
Circumscribed Astrocytoma
Diffuse astrocytoma: Individual neoplastic cells

spread out through the white matter. In WHO Gr 2,
this is only noted as increased cellularity
Astrocytoma: Circumscribed
Special astrocytomas
Astrocytoma of young
Various locations
Well circumscribed (yet, no capsule)
Do NOT spread along WM
Do NOT change grade (except PXA)
Constellation of findings correlates w/ Histology
Figure 5-10-3
Cystic Cerebellar Astrocytoma

Juvenile Pilocytic Astrocytoma
(PA or JPA)
Synonyms: Polar Spongioblastoma, Cystic

Cerebellar Astrocytoma
Cell of Origin: Astrocyte (bi-polar, hairlike)
Associations: in ON w/ NF-1
Incidence: 3%6% of ALL Cranial, 32% of Child
Age: 515 (Zulch 37) Sex: Slight F (11/9)
Location: Cerebellum, Chiasm/Hypothal, Optic
Treatment: Surgery, patience
Prognosis: 77% at 5 yrs, 75% at 10 yrs, 75% at 15 yrs
Circumscribed astrocytoma, like pilocytic

astrocytoma, have pushing margins and are
often fluid-secreting
Pilocytic Astrocytoma: Radiology

[Figures 5-10-4 to 5-10-6]
Cerebellum, Diencephalon
rare in BS or Cerebrum
Majority have significant cyst
part of lining does NOT enhance
Nodule may be heterogeneous
Exceptional purely solid
Nodule NOT hyperdense
Calcification in 5%25%
Figure 5-10-4
Pilocytic astrocytoma with classic cyst and

nodule morphology
Neuroradiology
1137
1139
Figure 5-10-5
Figure 5-10-6
Gross picture of pilocytic astrocytoma

Pilocytic astrocytoma with classic cyst and
nodule morphology
Figure 5-10-7
WHO Gr1 - Pilocytic Astrocytoma

Pathology
Biphasic pattern
dense pilocytic glia
Rosenthal fibers
loose microcystic areas
No necrosis
Low grade
Abnormal capillaries
allow enhancement
fluid production
Figure 5-10-8
Grading Problems in Gliomas

51 Pilocytic (WHO Gr. 1)
KERNOHAN MAYO-ST.ANNE
1 26%
1
2%
2 69%
2
55%
3 6%
3
35%
4 0%
4
8%
By conventional feature counting most pilocytic
astrocytomas were overgraded.
(Courtesy of Paul Sherman)
Variant Appearance
Variant Location
A Cyst with mural nodule?

Not Always !!!
Figure 5-10-9

Pilocytic Astrocytoma: Locations [Figure 5-10-9]
CEREBELLUM
Chiasm And Optic Nerve
Hypothalamus/thalamus
Cerebral Hemisphere
Spinal Cord (Intramedullary)
Pilocytic astrocytoma of hypothalamus

1138
1140
Neuroradiology
Enhance
Cyst w/ Nodule Cystic
Hypodense nodule
Calcification
NOT vascular
Nodule location varies
Hemangioblastoma
Enhance
Solid <- cyst w/nodule ->
Hyperdense nodule
Never Ca++
Hypervascular, Flow Voids
Nodule is Subpial
Figure 5-10-10
Pilocytic Astrocytoma (Juvenile Pilocytic)
Childhood, Young Adults

Benign, no mitosis/necrosis
Circumscribed Enhancing
Cyst Formation, Mural Nodule
Cerebellum and Diencephalon
(Optic tracts, Hypothalamus)
WHO Grade I
JPA (Pilocytic)
SGCA (Subependymal Giant Cell)
Ganglioglioma
Meningioma
Subependymal Giant Cell Astro [Figure 5-10-10]
Astrocytomas
SPECIAL ASTROCYTOMAS
Circumscribed Growth:
Pilocytic
Pleomorphic Xantho-Astrocytoma
Rare Variant of Astrocytoma

Arises from Subpial Astrocytes
Affects Superficial Cerebral Cortex and Meninges
Skull erosion (scalloped excavation)
Temporal > Frontal > Parietal
WHO Grade 2,3
50% progress over time
IMAGING:
CT APPEARANCE:
Well-Circumscribed Hypodense or Cystic Mass
Often Isodense Solid Nodule that Intensely Enhances
May Mimic Juvenile Pilocytic Astrocytoma
Calcifications Rare
MR APPEARANCE:
Well-Circumscribed Mass of Variable Size
Superficial Cortical Location
T1: Low/Mixed Signal,
T2: High/Mixed Signal
Often with Cystic Component
Solid Portion Intensely Enhances
Adjacent Meninges May Enhance (Tail)
Little or No Mass Effect
Neuroradiology
1139
1141
Astrocytomas
Ordinary Astrocytoma
Diffuse Infiltration of WM by:
Fibrillary Astrocytes
Protoplasmic Astrocytes
Gemistocytic Astrocytes
WHO 2,3,4 (NOT 1)
KS & Mayo Grades 14
Where do Glioblastomas come from?
Progressive Transformation from lower grade diffuse astrocytoma

- OR Arise de novo
Diffuse Astrocytoma too many cells !
Mild cellular atypia
KERNOHAN
(KS)
ANAPLASIA
Min
>1/2
Marked
CELLULARITY
Mild
Mild
Inc
Marked
MITOSIS
Plus
Marked
Min
Min
Marked
ENDOTHELIAL 0
Proliferation
NECROSIS
TRANSITION
ZONE
Marked
<== Broad
Sharp ==>
Figure 5-10-11
Astrocytoma: Diffuse
(Fibrillary, protoplasmic, etc.)
Adult type or Hemispheric Astrocytoma

Diffusely infiltrate brain, along WM tracts
Continuum, from low-grade to high-grade
Genetic Alterations 17 => 9 => 10
Many Progress in Histology over time, changing
from WHO Gr. 2 => Gr. 3 => Gr. 4 (GBM)
Imaging tends to correlate with histology, especially
at the ends of spectrum
Astrocyte Mutation [Figure 5-10-11]
Successive mutations in Astrocytoma
Diffuse Astrocytoma [Figures 5-10-12 and 5-10-13]

Figure 5-10-12
All three grades of

astrocytoma in one
patient
1140
1142
Neuroradiology
Diffuse Astrocytoma
Figure 5-10-13
Astrocytoma
Anaplastic Astrocytoma
The Eastwood Method:
The Good
The Bad
The Ugly
Astrocytoma: Radiologic Grading
TYPE 1 WHO 2, KS Grade 12, Benign

Homogeneous
No Enhancement, No Vasogenic Edema
TYPE 2 WHO Grade 3, Anaplastic
Variable Enhancement, Edema
50% enhance 50% dont
TYPE 3 WHO Grade 4 Glioblastoma
Heterogeneous (Necrosis, Blood)
Ring Enhancement, Edema
Diffuse astrocytoma is a spectrum of tumors,

pathologically and by imaging. The classification
into three grades (WHO 2,3, or 4) may be an
artificial segmentation along a continuum
Benign Astrocytoma: WHO 2, KS 12,

Mayo 1
YOUNGER PATIENT
CHILDHOOD
Young Adults (20s 40s)
NL VESSELS (NO NEOVASCULARITY)
BBB INTACT
NO EDEMA
NO ENHANCEMENT
NO TUMOR VESSELS
Figure 5-10-14
Benign Diffuse
HOMOGENEOUS
NO NECROSIS
NO HEMORRHAGE
INCREASED WATER
DARK and Poorly Demarcated on CT
Dark and Sharp on T1W
BRIGHT and Sharp on T2W
MICROCYST >>> MACROCYST
(macrocysts occur in JPA, etc.)
PD
T2
Gr 2 Fibrillary Astrocytoma
Gr 2 Fibrillary Astrocytoma
[Figures 5-10-14 and 5-10-15]
Figure 5-10-15
T1T1gad
non
Gr 2 Fibrillary Astrocytoma no enhancement
after gadolinium
Neuroradiology
1141
1143
Gr 2 Astrocytoma: PWI [Figure 5-10-16]
Figure 5-10-16
Reduced perfusion
Gliomatosis Cerebri [Figure 5-10-17]

Figure 5-10-17
T1-gad
T2
Female, acute stroke (3 days), MCA occlusion
Figure 5-10-18
Gliomatosis Cerebri a diffuse astrocytoma
infiltrating two or more lobes of the brain
Gliomatosis Cerebri: Diffuse Astrocytoma

2 lobes
[Figure 5-10-18]
Spread along White Matter Tracts

[Figure 5-10-19]
Figure 5-10-19
Gliomatosis Cerebri a diffuse astrocytoma
infiltrating two or more lobes of the brain
Figure 5-10-20
Diffuse Grade 2 astrocytoma spreading through

white matter, including corpus callosum
Astrocytoma: Microcystic change
Gliomatosis Cerebri
Astrocytoma: Microcystic Change [Figure 5-10-20]
Figure 5-10-21
Astrocytoma [Figure 5-10-21]
Pontine astrocytoma WHO Gr 2

1142
1144
Neuroradiology
Modes of Spread
1.
2.
3.
4.
Figure 5-10-22
Natural passages
Along surfaces
Along tracts
Across the meninges
Spread Along Tracts:
Corona Radiata
Peduncles
Corpus Callosum
Anterior Commisure
Arcuate Fibres
Astrocytes Track Along WM
Progressive transformation of WHO Grade 2

astrocytoma to Glioblastoma multiforme
Pontine Astrocytoma
Figure 5-10-23
Pontine Astrocytoma: WHO 2

WHO 2 GBM [Figure 5-10-22]
Expanded Brain
Anaplastic Astrocytoma: Overall
Characteristics
Grade III malignant glioma

Anaplastic astrocytoma no enhancement in this example
Less aggressive than GBM, malignant
about 50% will enhance
with somewhat better prognosis
Frequency: highest in young adults (30 40 years)
Recurrence: often as a higher-grade glioma
Challenge: difficult to remove completely with surgery
Median survival: 3 4 years
Anaplastic Astrocytoma [Figure 5-10-23]

Anaplastic Astrocytoma ( WHO 3 )
Increased Cellularity, +/- minimal vascular changes, no necrosis , no

hemorrhage
GBM - Glioblastoma
Malignant Astrocytoma:
Older patient
40s and up
exceptions (PNET)
~ 1/2 arise from previous low grade (23)
Abnormal Vessels (neovascularity)
BBB abnormality
vasogenic edema
contrast enhancement
irregular vessels, shunting, etc.
HETEROGENEOUS
hemorrhage (old/new)
tumor necrosis
tumor itself
Neuroradiology
1143
1145
Astrocytoma Gr4: Angiogenesis
Figure 5-10-24
Glioblastoma Multiforme [Figures 5-10-24 and 5-10-25]

Figure 5-10-25
T2
T1-gad
Glioblastoma Multiforme. Photomicrograph at

high power shows both angiogenesis and
pseuopalisading necrosis
Figure 5-10-26
(Gr 4) Glioblastoma: PWI-CBV [Figure 5-10-26]

Glioblastoma WHO Grade 4
Mechanisms of Enhancement
Ultrastructure of BBB
Neural capillary
astrocytic feet
continuous BM
tight junctions
no pinocytosis
ABBB or Non-neural
no astrocytic feet
fenestrated BM
intercellular gaps
pinocytosis
Increased perfusion due to angiogenesis in a
glioblastoma multiforme
GBM
Center of Abnl Density/Intensity

variegated necrosis
ENHANCING RIM
hypercellular, fleshy neoplasm
greatest neovascularity
Corona of Abnl Density/Intensity
edematous white matter
areas of microscopic neoplastic infiltration
Figure 5-10-27
GBM - Glioblastoma
Glioblastoma multiforme with pseudopalisading
necrosis
Pseudopalisading Necrosis [Figure 5-10-27]

Ring Lesion and Infiltration [Figure 5-10-28]
Figure 5-10-28
Schematic of Glioblastoma
multiforme there are neoplastic
cells infiltrating into the surrounding
white matter
1144
1146
Neuroradiology
(Surrounding Zone of Infiltration)
Figure 5-10-29
GBM arose from a preexisting low grade

surrounding lower grade neoplasm
may also transform over time
GBM arose de novo
sends cells to invade the brain
Terrorist Cells Infiltrate Brain

GBM - Multifocal [Figure 5-10-29]
DWI of Glioblastoma (Gr 4)
Multifocal glioblastoma multiforme. Microscopic
infiltration does not always produce macrosopic
changes that are visible on CT, MR, or even at
gross pathology
Ring Enhancing Mass

Ring Lesion Differences [Figure 5-10-30]
Figure 5-10-31
Figure 5-10-30
Glioblastoma Multiforme vs. Abscess

(toxoplasmosis). The abscess rim is thinner,
without a shaggy inner margin
Glioblastoma Multiforme vs. Abscess

(toxoplasmosis). The viscous pus and white cell
infiltrate in the abscess causes restricted diffusion
bright on DWI
DWI: Necrosis vs. PUS [Figure 5-10-31]

Glioblastomas: Growth/Spread
Figure 5-10-32
Glioblastoma: Ependymal spread

GBM [Figure 5-10-32]
GBM Thicker on Surface
Two different patients with GBM the tumor

flourishes when it reaches the rich vascularity of
the cerebral cortex
Neuroradiology
1145
1147
X-Ray Perfusion Imaging [Figure 5-10-33]
Figure 5-10-33
MR Perfusion Imaging [Figure 5-10-34]

GBM with increased rCBV
New Tools for Grading and Staging
Radiology
Perfusion Imaging rCBV and rCBF
Diffusion Imaging, ADC and DTI
Spectroscopy
PET/SPECT
Monoclonal Ab.
Pathology
Labeling Index
Chromosome Analysis
Histochemical
Electron Microscopy
Glioblastoma multiforme. The early draining veins

reflect increased perfusion and a shortened mean
transit time (MTT)
Figure 5-10-34
DTI and Tumor Imaging

Astrocytes Track Along WM
Two Port Radiotherapy
Bad News
Cant define full extent of tumor by any current
test
Good News
90% of tumor recurrence within 2cm of
enhancing rim
Glioblastoma multiforme
Figure 5-10-35
Glioblastoma Multiforme [Figure 5-10-35]

Define the Problem
Some Low Grade Enhance*

Some Low Grade => GBM
Some Low Grade Do Not*
*These are the Circumscribed Astrocytomas
The others are the Diffuse Astrocytomas
WHO Astrocytoma Summary

Butterfly glioma Glioblastoma multiforme
References
1.
2.
3.
4.
Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (1993-2002) Pediatric Neurosurgery
2005; 41:173-177
Levin VA, Leibel SA, Gutin PH. Neoplasms of the central nervous system.
In: DeVita VT Jr, Hellman S, Rosenberg SA, eds. Cancer: Principles & Practice of Oncology. Vol 2. 5th ed.
Philadelphia, Pa: Lippincott-Raven Publishers; 1997:2022-2082.
Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439.
Pobereskin LH, Chadduck JB: Incidence of brain tumours in two English counties: a population based study. J
Neurol Neurosurg Psychiatry 2000; 69: 464-471.
1146
1148
Neuroradiology
Non-Astrocytic Gliomas
PRIMARY NEOPLASMS Neuroectodermal
Figure 5-11-1
Neuroectoderm
Embryologic Neural Tube
Neuroepithelial
Broad Categories
Glial Tumors (GLIOMAS)
Embryonal/Immature (P.N.E.T.s)
Neuronal (Neurocytoma)
Mixed (Ganglioglioma)
Neuroectodermal Tumors
Astrocytoma
Circumscribed
Diffuse
Ependymoma
Oligodendroglioma
Atypical Rhabdoid Tumor
Ganglioglioma
Central Neurocytoma
Lhermitte-Duclos
Ependymoma Gross Axial section
Figure 5-11-2
EPENDYMOMAS Brain and Cord
Cell of Origin: Ependyma

Lining of ventricles
Central canal/filum terminale
rests in parenchyma
Subtypes:
Anaplastic/Malignant
Immature (Ependymoblastoma)
Myxopapillary (cauda equina)
EPENDYMOMA
WHO Classification
Ependymoma (WHO grade II)
Variants: cellular, papillary, clear cell, tanycytic, mixed
Anaplastic ependymoma (WHO gr III)
Myxopapillary ependymoma
Subependymoma
Ependymoma heterogeneous
central mass
Figure 5-11-3
EPENDYMOMAS Demographics
5%6% of All Intracranial

70% occur in Males
70% arise in the Fourth Ventricle
70% present in Childhood
70% of All Intramedullary
5 year survival 50%
EPENDYMOMAS Gross Pathology

[Figures 5-11-1 to 5-11-5]
Soft Intracavitary Mass

cast of ventricle
extrude out foramina
invade floor of 4th (pons/medulla)
Neuroradiology
Ependymoma Enhancing and

heterogeneous, small cysts and calcified
chunks
1147
1149
Figure 5-11-4
Heterogeneous
small/large cystic areas
calcification (often chunks)
Spinal Cord sharply circumscribed
Figure 5-11-5
Ependymoma Tumor extends into lateral recess

of 4th ventricle
Figure 5-11-6
Ependymoma Tumor extends into

vallecula of cisterna magna
Schematic Ependymoma [Figure 5-11-6]

Ependymoma [Figure 5-11-7]
Ependymoma CPA Cistern [Figure 5-11-8]
Figure 5-11-7
Schematic of Ependymoma filling lumen of fourth

ventricle. Copyright 2004
Figure 5-11-8
Ependymoma in the cerebellopontine angle cistern

Ependymoma filling lumen of fourth
ventricle
1148
1150
Neuroradiology
Extra-ventricular Ependymoma
Figure 5-11-9
[Figures 5-11-9 and 5-11-10]
More common in children

More common in cerebral hemisphere
Arise from *rests* of Ependymal Cells
Better prognosis
Not infiltrating
Not communicating with CSF
Often partially cystic
CHOROID PLEXUS NEOPLASMS

Introduction
Normal Choroid Plexus (CP)

Makes majority of CSF
Forms papillary fronds:
Vascular core
Ependyma is modified into
CHOROID EPITHELIUM
Neoplasms:
CP Papilloma (benign) WHO Grade 1
CP Carcinoma (malignant) WHO Grade 3
Extra-ventricular Ependymoma Note fluid-fluid

level formed by contrast layering within the cyst
Figure 5-11-10
CHOROID PLEXUS TUMORS
Choroid plexus papilloma (WHO grade I)

Choroid plexus carcinoma (WHO grade III)

Demographics
Tumor of Childhood:
Uncommon (<1% of CNS)
Intrauterine/congenital
40% < 1 year old
86% < 5 years
Location:
TRIGONE in children
4th vent. In adults
Less often 3rd and CPA

Gross Pathology
Extra-ventricular Ependymoma
[Figures 5-11-11 to 5-11-13]
Lobulated intraventricular mass with papillary fronds

Secondary Effects:
Ventricular and SAS Enlargement
Spontaneous Hemorrhage
CSF Seeding
Parenchymal Invasion
Figure 5-11-11
Figure 5-11-12
Choroid plexus papilloma showing

innumerable papillae
Neuroradiology
Photomicrograph of CPP, showing

choroid epithelium and vascular core
1149
1151
Figure 5-11-13
Figure 5-11-14
Choroid plexus papilloma showing innumerable

papillae
Choroid plexus papilloma Note pattern of
bilateral hydrocephalus

CSF Homeostasis [Figure 5-11-15]
Figure 5-11-15
CSF Homeostasis
Normal Ventricular System: Lateral Ventricles

All Ventricles Enlarged?
Over Production vs. Under Resorption
Figure 5-11-16
Congenital CPP
Choroid plexus papilloma [Figure 5-11-16]
OLIGODENDROGLIOMA
Cell of origin
Oligodendrocyte
Makes central myelin
51% - 90% oligos, remainder astrocytes
1%8% of ALL CNS primary
Adults > Children (8:1)
Age peak 35 45 yrs)
Supratentorial 85%
Slow growth, Long Hx (10 years)
Prognosis better with 1p and 19q mutations
Choroid plexus papilloma Note

pattern of bilateral hydrocephalus
1150
1152
Neuroradiology
OLIGODENDROGLIOMA
Figure 5-11-17
Gr 1 - Rare
Gr 2 - Conventional oligodendroglioma
Gr 3 - Anaplastic oligo:
Hypercellularity, atypia, mitoses, endothelial
proliferation, necrosis
Gr 4 - GBM-like (rare)
Not biologically equivalent to Gr 4 fibrillary
astrocytoma
OLIGODENDROGLIOMA Gross Pathology
Arises in White Matter

Grow toward cortex!
Unencapsulated
Not as infiltrating as astro.
Heterogeneous
myxoid areas (cystic)
hemorrhage
C A L C I F I C A T I O. N !
OLIGODENDROGLIOMA Radiology
Heterogeneous Hemispheric Mass

Ca++, Cysts myxoid change, Blood products
Oligodendroglioma. CT shows very dense
Extend to Cortex and infiltrate GM
calcifications, highly suggestive and characteristic
Gyriform or dot-dash Ca++
of oligodendroglioma
Scalloped erosion inner table
Figure 5-11-18
2/3 will enhance
+/ anaplasia
MR +/- special pulse sequences for Ca++ detection
MR Spectroscopy?
Potential for tumor grade, but not subtype or
genetics
MR - What is it?
Oligodendroglioma [Figure 5-11-17]
CT Shows DENSE Ca++
Oligo-astrocytoma
Nothing Specific Looks like Diffuse Astrocytoma
Oligodendroglioma [Figure 5-11-18]
CT Shows DENSE Ca++
Oligodendroglioma Heterogeneous peripheral

mass that involves the cortex with thick curvilinear
calcifications
Chickenwire Vascularity [Figure 5-11-19]
Figure 5-11-19
Fried Egg: round dark nucleus

surrounded by a clear halo this is an
artifact of fixation
Chicken-wire: The capillary vessels
form a "chicken wire pattern around
nests of cells
Neuroradiology
1151
1153
Oligodendroglioma
Figure 5-11-20
Combined 1p/19q loss

Associated with prolonged survival
Response to PVC (procarbazine, vincristine
CCNU [Lomustine]) chemotherapy
50% volume decrease in 100%
Median survival 10 yrs vs 2 yrs
95% 5 yr survival
Most powerful predictor on multivariate analysis
Cairncross et al. J NCI 1998;90:1473
Old Elephants Age Gracefully

Oligo Epend Astro GBM
External granular cell layer normal

in fetus and infants (up to 12 months
old)
90% 50% 25% 15% (Incidence of Ca++)
Oligodendroglioma
MEDULLOBLASTOMA [Figure 5-11-20]
Cell of origin:
medulloblast NOT!
Bi-potential embryologic cells:
Migrate from 4th to form CRBLL
Glial and neuronal differentiation
External Granular Cells (fetus)
Internal Granular Layer (mature)
Primitive Neuroectodermal Tumor - PNET
MEDULLOBLASTOMA Demographics
1st or 2nd most common cerebellar neoplasm in children

1/5 1/3 of ALL pediatric CNS
M:F 1.1 2:1
May be congenital (present at birth up to 60 days)
most (1/2) < 15 yrs.
however, 1/3 present from 1535 yrs.
5 year survival >> 50% => approached 75%-85%
Primitive Neuroectodermal Tumor
Pediatric Posterior Fossa
454 posterior fossa patients

All under the age of 18
402 tumors:
37.1% Cerebellar astrocytoma (149)
34.6% Medulloblastoma (PNET) (139)
11.4% Brain stem astrocytoma (46)
7% Ependymoma (28)
9.9% "other" (40)
Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst
1998; 14:426-439.
Childhood CNS Tumor Demographics
367 Syrian children, collected from 1993-2002

Supratentorial - 47%
Infratentorial - 53%
Male 52%:48% Female
Overall Incidence:
Medulloblastoma 27%
Astrocytoma 26%
Craniopharyngioma 14%
Posterior Fossa Only
Medullo (PNET) 54%
1152
1154
Neuroradiology
Astrocytoma 23%
Ependymoma 17%
Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (19932002) Pediatric Neurosurgery 2005; 41:173-177
Figure 5-11-21
MEDULLOBLASTOMA Gross Pathology

[Figures 5-11-21 and 5-11-22]
Arise from:
post. (inf.) Medullary Vellum
Vermis (midline cerebellum)
Morphology:
expansile, spherical, Unencapsulated
post. 4th ventricle
residual ANT. Crescent of CSF
HOMOGENEOUS
(Ca++. Cyst. Heme are UN-common)
Medulloblastoma (PNET) Rounded
mass in the central posterior fossa
Figure 5-11-22
Figure 5-11-23
Medulloblastoma a small, round,

blue-cell tumor
RADIATION CHEMOTHERAPY
Dividing Cells
Neovascularity
Pharmaceuticals
Tested against murine leukemia
Small round blue cell tumor
Cis-platinum
Dividing cells
Electrical field
Platinum electrodes
MEDULLOBLASTOMA Micro Pathology
Small Round BLUE CELL Tumor

Immature, high Nuclear:Cytoplasm
Both Neuronal and Glial features (occasional
astrocytic differentiation)
Form Rosettes (Homer-Wright) cells arranged
in a circle surround core with linear fibrils
Densely cellular
Necrosis/Hemorrhage are not rare
Medulloblastoma (PNET) Rounded mass in the

central posterior fossa
MEDULLOBLASTOMA Radiology [Figure 5-11-23]
Post. Fossa, Behind/in 4th vent.

HOMOGENEOUS (hes lying!)
grossly uniform,
but, finely heterogeneous
hyperdense on CT (w/o Ca++)
hypo-/isointense to GM on MR (cellularity and high N:C ratio)
(Ca++ and cysts < 15%)
Neuroradiology
1153
1155
Fourth Ventricle - Schematic [Figure 5-11-24]
Figure 5-11-24
Medulloblastoma (PNET) [Figures 5-11-25 and 5-11-26]

Figure 5-11-25
Schematic of posterior fossa masses. Can you

really determine where the mass originated when
it is more than three centimeters in diameter?
Copyright 2004

mass arising in the cerebellum not
from the 4th ventricle roof
Figure 5-11-26
Medulloblastoma
[Figure 5-11-27]
Homogeneous
finely irregular
Cyst and Hemorrhage are uncommon
<10%
Hyperdense on NCT
up to 75%
densely cellular
sm. Round blue-cells
Center is behind 4th vent
Rounder not angular
Medulloblastoma (PNET) Rounded mass in the central

posterior fossa
Figure 5-11-27
Ependymoma - Schematic
POSTERIOR FOSSA [Figure 5-11-28]
INCIDENCE/LOCATION:
Medulloblastoma (PNET) (1/4 - 1/3)
Post. To IVth
Brainstem glioma (1/6)
Ant. To IVth
Ependymoma (1/6)
Inside IVth
Pilocytic Astrocytoma (1/4 - 1/3)
Lat. And/or post. IVth
these are often a cyst w / nodule
Medulloblastoma
Medulloblastoma (PNET) [Figure 5-11-29]
Figure 5-11-28
Zuckerguss or Sugar Icing (CSF dissemination)

CSF DISSEMINATION
Neuroectodermal:
PNET (medulloblastoma)
GBM (reaches ventricle or pia)
Ependymoma
Oligodendroglioma (micro curiosity - no Sx)
CPP and CPC
mass in the central posterior fossa
1154
1156
Neuroradiology
Figure 5-11-29
Non-glial:
Germinoma
Lymphoma (usually secondary)
Leukemia
Carcinomatous Meningitis
CSF Spread - Zuckerguss [Figure 5-11-30]

Lateral Medulloblastoma [Figure 5-11-31]
Medulloblastoma - Desmoplastic
CSF spread of Medulloblastoma (PNET)
Lateral Hemispheric Location

Older Patients More peripheral
Figure 5-11-30
Cerebral Neuroblastoma [Figure 5-11-32]

Summary
Ependymoma
Intraventricular, soft, heterogeneous
Very young, Hydrocephalus
Very Small Papillae and Lobulations
Oligodendroglioma
Superficial, skull remodeling
Dense Calcification: dot-dash and linear
Hyperdense on CT
Central posterior fossa
References
1.
2.
3.
4.
CSF spread of Medulloblastoma (PNET)

Kadri H,Mawla AA, Murad L: Incidence of childhood
brain tumors in Syria (1993-2002. Pediatric Neurosurgery
Figure 5-11-31
2005; 41:173-177
Parizek J, et al: Posterior cranial fossa surgery in 454
children. Childs' Nerv Syst 1998; 14:426-439.
Jenkinson MD, Smith TS, Joyce K, Fildes D, du Plessis
DG, Warnke PC, Walker C MRS of oligodendroglial
tumors: correlation with histopathology and genetic
subtypes. Neurology. 2005 Jun 28;64(12):2085-9.
Cairncross JG, Ueki K, Zlatescu MC, et al. Specific
genetic predictors of chemotherapeutic response and
survival in patients with anaplastic oligodendrogliomas. J
Natl Cancer Inst 1998; 90:1473-1479.
Lateral Medulloblastoma Desmoplastic variant
Figure 5-11-32
Cerebral Neuroblastoma PNET
Neuroradiology
1155
1157
Extraaxial Tumors:
Other Non-Glial Lesions
CHORDOMA [Figure 5-12-1]
Rich Corinthian Leather vs. Physaliphorous Cells Bubble or Vacuolated
CHORDOMA: Normal Notochord
Figure 5-12-1
Nucleus Pulposis of Intervertebral Disk

Ecchordosis of Clivus
Size/Shape like a grain of rice
Dorsal to clivus
Notochord Embryology
Sclerotomes & Notochord [Figure 5-12-2]
Figure 5-12-2
Chordoma - Physaliphorous Cells

(Courtesy of Joe Parisi, M.D.)
Schematic of sclerotomes surrounding the

notochord. Two adjacent sclerotomes fuse into
one vertebral body. The notochord tissue is
extruded into the intervertebral disc, forming the
nucleus pulposis. Copyright 2003
Chordoma
Notochord: Chordoma & Thornwaldt [Figure 5-12-3]
CHORDOMA
ORIGIN: Notochordal Rests
AGE: (3060)
Figure 5-12-3
LOCATION:
Clivus 35%
Spine 15% (esp. Cx)
Sacrum 50%
CHORDOMA: Imaging
Location: midline clivus

Extend lateral, dorsal , ventral
NCT: Bone destruction
Heterogeneous
Cysts, Ca++
ECT: Heterogeneous
Extraaxial Tumors: Other Non-Glial Lesions
Chordoma (left) and Thornwaldt cyst (right). The

orange line is the embryologic location of the
notochord, the green triangle is the clivus
1156
1158
Neuroradiology
Chordoma - Imaging
Figure 5-12-4
Location
Midline Clivus
May extend lateral, dorsal , ventral
Midline Sacrum
Lobulated Heterogeneous Bulky Mass
Bone destruction
NO remodeling
Variable Density/Signal
Bone sequestra
Dystrophic calcifications
Clivus is Missing [Figure 5-12-4]

Chordoma
Chordoma Destruction of the clivus, the basilar
and vertebral arteries are stretched over a large
bulky, hypovascular mass. Air and contrast
remain from an earlier myelogram
Eccentric Growth [Figure 5-12-5]

Figure 5-12-5
Figure 5-12-6
Chordoma with eccentric growth, there is a sharp

margin between the tumor and brain, with a thin
hypointense line the dura
Chordoma [Figures 5-12-6 to 5-12-8]
Chordoma destruction of the sacrum and bulky

soft-tissue mass. This patient presented with
symptoms of constipation and rectal fullness
Figure 5-12-7
Figure 5-12-8
Chordoma destruction of the

sacrum and bulky soft-tissue mass.
There are devitalized fragments of
residual bone sequestra from the
sacrum
Neuroradiology
Chordoma destruction of the

sacrum and bulky soft-tissue mass
1157
1159
T1 pre and post, T2 [Figure 5-12-9]
Figure 5-12-9
Chordoma
Eccentric Mass [Figure 5-12-10]
Chondrosarcoma [Figure 5-12-11]
Figure 5-12-10
Sacrococcygeal chordoma
Figure 5-12-11
Chondrosarcoma low grade.

Notice the dense mineralization and
the eccentric location of the mass
DERMOID/EPIDERMOID
True Cysts
Inclusion Cysts
Lined by an Epithelium
Chondrosarcoma
TRUE CYSTS OF THE CNS
Epidermoid
Dermoid
Colloid
Craniopharyngioma
Rathke Cleft
Ependymal
Endodermal
TRUE CYSTS
A fluid filled space, lined by an epithelium.

Classified by the type of epithelial lining:
Epidermoid
squamous epithelium - ectoderm
Dermoid
squamous and dermal adnexa - ectoderm
Colloid cyst
ciliated cuboidal/columnar epithelium, mucus secreting cells - similar to
endoderm ?
Craniopharyngioma (two types)

Adamantinomatous - children, cystic, calcified
Squamous and papillary - adult, solid
Rathke Cleft cyst
ciliated cuboidal/columnar epithelium - possibly endoderm ?
Teratoma - a neoplasm of multipotential germ cells
NON-GLIAL MASSES
Dermoid and Epidermoid

MYTH OF THE MESODERM
1158
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Neuroradiology
MYTH OF THE MESODERM
Figure 5-12-12
One germ cell layer = epidermoid

Ectoderm
Two germ cell layers = dermoid
Ectoderm and Mesoderm
Three germ cell layers = teratoma
Ectoderm, Mesoderm, Endoderm
DERMOID/EPIDERMOID: Histology
1. Epidermoid Squamous Epithelium - ONLY

2. Dermoid Sq. Epi PLUS Dermal Appendages
(hair, sebaceous, sweat glands, etc.)
3. Teratoma Complex tissues, 2 or more germ
layers
(often mainly ectoderm, benign cystic)
EPIDERMOID [Figure 5-12-12]
AGE: 4 6TH Decade

Location: Midline or lateral (CPA)
Composition: Sq. epithelium, keratin
Thin wall, no Ca++ or Vascularity
NCT: Lipid to Brain
Ca++/enhance. rare
MRI: Hetero., CSF to Brain
NOT bright on T1W
**Fluid/Fluid Level RARE
Epidermoid Inclusion Cyst. Note: the lesion is

nearly, but not exactly, like CSF in signal. The
sagittal image demonstrates internal layers of
keratin
Figure 5-12-13
Pearly Tumor
Epidermoid - Dry Keratin [Figures 5-12-13 and 5-12-14]
Epidermoid [Figures 5-12-15 and 5-12-16]
Epidermoid Inclusion Cyst - Dry Waxy

Keratin
Figure 5-12-14
Figure 5-12-15
Squamous epithelium and flaky or dry

keratin
Epidermoid Inclusion Cyst. On T2W images the
lesion is hyperintense like CSF. However, the
axial image on the right shows some internal
structure
Figure 5-12-16
Epidermoid Gd+ T1W
Midline posterior fossa/fourth ventricle
Neuroradiology
1159
1161
Epidermoid [Figures 5-12-17 and 5-12-18]
Figure 5-12-18
Figure 5-12-17
T1W Gd
FLAIR
Epidermoid Inclusion Cyst
Figure 5-12-19
Epidermoid Inclusion Cyst. Faint peripheral

enhancement from gliosis. Internal wisps from
layers of keratin, and marked hyperintensity on the
FLAIR image
DERMOID
AGE: 3rd Decade

Location: Midline
Composition: Sq. epi. & appendages
Thick wall, Ca++ & Vascularity
NCT: Lipid to Brain, Fluid/Fluid
Ca++/enhance. often
MRI: Hetero., Lipid to Brain
Bright on T1W
**Dysraphism, Sinus tract
Dermoid with hair and sebaceous

material
Figure 5-12-20
Dermoid Inclusion Cyst

Dermoid [Figures 5-12-19 and 5-12-20]
Ruptured Dermoid
Dermoid - rupture [Figure 5-12-21]
Figure 5-12-21
Epidermal surface, but with

sebaceous glands, hair shafts and
follicles making this a dermoid
inclusion cyst
Ruptured Dermoid Inclusion Cyst. Notice the

hairball at the lipid-CSF interface
1160
1162
Neuroradiology
Body Soil
As gross as it is, the average person excretes up to 50 grams of body soil per
day! This is because on a normal day we each use 1 liter of sweat, eliminate 1
billion dead skin cells, and run off 10 grams of sebum, otherwise known as body
oil.
Figure 5-12-22
Clorox Commercial, May 2005

(http://www.clorox.com/health_body_soil.html)
Intradiploic Epidermoid [Figure 5-12-22]

COLLOID CYST
A benign mass, in a Malignant Location
COLLOID CYST [Figure 5-12-23]
Paraphyseal cyst ependymal cyst, choroid cyst

Congenital lesion
Cuboidal, low columnar epithelium
Scant connective tissue
Foramen of Monro
Intradiploic Epidermoid Inclusion Cyst
Figure 5-12-23
COLLOID CYST [Figures 5-12-24 to 5-12-26]
Location: Foramen of Monro

CT: sharply demarcated
hyperdense to hypodense
< half enhance
MR: sharply demarcated
T1W iso. to bright
T2W bright to dark
NOTE: Dark Cysts are too thick for
Stereotactic Aspiration
Colloid cyst
Figure 5-12-25
Figure 5-12-24
Colloid Cyst in the characteristic anterior 3rd

ventricle location, and causing obstructive
hydrocephalus
Colloid cyst
Figure 5-12-26
Aqueous Protein Solution

Colloid Cyst Black Hole
Colloid Cyst: two different patients one cyst is

markedly hyperdense, the other is hypodense.
Why? Variable protein and viscosity
Neuroradiology
1161
1163
Neoplasms of the Meninges

Educational Objectives
Meningioma is the most common non-glial primary tumor

Meningioma is the most common extraaxial neoplasm
Most meningiomas have typical imaging
Hemispheric, homogeneous, broad based on the dura, hyperostosis,
hormonally sensitive
Some meningiomas have atypical imaging
Hemangiopericytoma is NOT a meningioma
Meningioma
75% are histologically typical

75% are radiologically typical
Not the same 75%
CT
MR
Angiography
Atypical Imaging =/= Atypical Histology
The atypical appearance of a common lesion may be seen more often than
the classic appearance of an uncommon lesion.
Primary Meningeal Neoplasms
MENINGIOMA:
Meningioma (typical and metaplastic
Atypical Meningioma
Anaplastic (Malignant) Meningioma
Papillary Meningioma
MESENCHYMAL (non-meningothelial)
Primary MELANOCYTIC Lesions
UNCERTAIN Origin
Hemangiopericytoma (pericyte)
Hemangioblastoma (mesenchyme)
Meningeal Tumors: WHO Grades
91% of Meningioma - Grade 1

Includes most subtypes / metaplastic changes
Transitional, fibroblastic, meningothelial
8.3% are ATYPICAL Meningioma - Grade 2
HEMANGIOPERICYTOMA Grade 2/3
PAPILLARY Meningioma - Grade 3
<1% are ANAPLASTIC Meningioma - Grade 3
Sandhyamani, Rao, Nair, Radhakrishan: Atypical Meningioma:

A Clinicopathological Analysis.Neurology India 2000; 48: 338-342
Meningioma Benign Subtypes WHO I
SYNCYTIAL (Meningothelial)
FIBROBLASTIC (Fibrous)
TRANSITIONAL (Features of Both)
PSAMMOMATOUS
Microcystic (Humid), Secretory
METAPLASTIC FEATURES
Lipoblastic, Osteoblastic, Chondroblastic
Myxoid, Xanthomatous, Melanotic
1162
1164
Neuroradiology
Meningiomas Cell of Origin
Dural Fibroblast ? No
Arachnoid Cap Cell
Meningothelial cell
Arachnoid granulations
Dural sinuses
Sup. Sag.
Sphenoparietal
Meningioma Etiologic Factors
Trauma
Radiation
Viruses
Familial (Non-NF2) Meningioma
Neurofibromatosis Type - 2
MISME Syndrome
Meningioma Radiation
Low Dose (<800 cGray)

Immigrants to Israel (1940s)
Tinea Capitis (ringworm)
Superficial radiation
High Incidence of Meningioma
High Dose (>2000 cGray = 2000 RADS)
Used for Skull Base Tumors
Pituitary Adenoma
Meningioma Molecular Biology
Postulate Tumor suppressor Gene

Chromosome 22 deletion in tumor cells
both sporadic and inherited
w/ or w/o NF-2
Homozygous for TWO wild-type copies is normal
Heterozygous for 22 develops neoplasm
Because there is a subsequent loss of the OTHER wild-type gene
Inherited (germline) deletion of 22
W/Schwannoma = NF2
Meningiomas
1/7 to 1/4 of all Intracranial Primary

~ 6/ 100k / year
small ones in ~ 1.4% of autopsies
1/4 1/3 of all Intraspinal Tumors
Middle age (4060) Your current Age + Ten Years
Female > Male
Cranial 24:1
Spinal 48:1
Progesterone receptors in 2/3
Estrogen receptors less common
Figure 5-13-1
Morphology [Figure 5-13-1]
Globose (spherical, hemispherical)

En plaque (like a flat bread)
Pancake
Crepe
Wonton wrapper
Tortilla
Pita (Greece and Middle East)
Naan (India)
Injera (Ethiopia)
Bolo de milho (Brazil)
Neuroradiology
Meningioma - parasagittal
1163
1165
Incidental Meningioma [Figure 5-13-2]
Figure 5-13-2
Not Incidental
En Plaque Meningioma [Figure 5-13-3]
Meningioma Location
Parasagittal
Convexity
Ant. Basal
Sphenoid
Olfactory
Suprasellar
Tentorial
Ventricular
25%
20%
40%
10%
5%
20%
10%
10%
Small incidental meningioma of the

tentorium cerebelli
Figure 5-13-3
Meningioma CT Imaging [Figure 5-13-4]
Non-Contrast
Sharply Circumscribed
Homogeneous
Hyperdense (+/ Ca++)
NOT from psammoma bodies !
Broad Dural Surface
Bone Changes (Hyperostosis)
Enhanced CT
Homogeneous Enhancement
Figure 5-13-4
En plaque meningioma, on the CT this blends into

the bone
Figure 5-13-5
Meningioma with classic features of

hyperdensity and hyperostosis
Meningioma CT Findings (193 pts)
BENIGN Meningiomas:
Homogeneous Enhancement
Heterogeneous Enhancement
Calcification
Hyperostosis
Midline Shift (large)
Mushrooming
72%
23%
27%
18%
72%
0%
J. Neurosurg 71:665672,1989
Psammomatous Meningioma
Psammomatous Meningioma [Figure 5-13-5]
1164
1166
Neuroradiology
Spreading Vasogenic Edema
Figure 5-13-6
Meningioma Vasogenic Edema
VASCULAR
Parasitization of MCA, etc.
Compression of cortical aa./vv.
COMPRESSIVE TRAUMA
SECRETORY EFFECT Evil Humors
TRANSCORTICAL FLOW
Close apposition of tumor to brain
Thinned cortex
+/- infiltration of brain
Fluid gradient from meningioma into
brain
Meningioma. WHO Grade 1 benign, yet with extensive

vasogenic edema
Meningioma and Edema [Figures 5-13-6 and 5-13-7]
Figure 5-13-7
Edema and Prognosis
Edema =/= Histology

Edema =/= Size
Edema =/= Vascularity
Edema IS Related to Resectability
Smaller pseudocapsule
Surgical cleavage plane
Tumor sticks to underlying brain
Resectability IS Related to Prognosis
Edema IS INDIRECTLY Related to Prognosis
Meningiomas MR Imaging
Meningiomas are ISO-intense.

Usually on T1W
Vary pulse sequence to see
EXTRA-axial Features
Gray-matter buckling
Pseudo-capsule of vessels
Meningeal/dural TAIL
GADOLINIUM ENHANCEMENT
Bar graphs showing high frequency of

vasogenic edema with meningioma
Meningioma Isointense to GM
[Figure 5-13-8]
Figure 5-13-9
Figure 5-13-8
Small peripheral round lesions, nearly isointense

to gray-matter on T1W MR
Meningioma - CT
Meningioma w/Gd+ [Figure 5-13-9]
Neuroradiology
Meningioma - Small peripheral round lesions, nearly

isointense to gray-matter on T1W MR
1165
1167
Meningioma [Figure 5-13-10]
Figure 5-13-10
Meningioma
Tentorium or Dura
IAC Normal
Hemispherical
Homogeneous
Hyperostosis
Tentorial Meningioma [Figure 5-13-11]

Meningioma - Pseudocapsule
Meningioma Pre and Post Gd
Meningioma. Cerebellopontine angle cistern.

This mass is hemispheric, and the enhancement
does not involve the IAC (internal auditory canal)
(Courtesy of Bob Peyster, MD)
MR Signal and Meningioma Types
Most are Isointense to GM

On Both T1W & T2W
Hyperintense to GM on T1W
Lipoblastic (Fatty) Meningioma
Hemorrhage into Meningioma
Hypointense on T2W
Fibroblastic
Transitional
Hyperintense on T2W
Meningothelial
Angioblastic
Microcystic (Humid)
Some Good, Some bad
Figure 5-13-11
Meningioma - Pseudocapsule [Figure 5-13-12]

Meningioma Dural Tail [Figure 5-13-13]
Figure 5-13-13
Figure 5-13-12
Meningioma Pseudocapsule of CSF and

vessels; and internal serpentine hypointensities
TMeningioma Pseudocapsule of CSF and

vessels; and internal serpentine hypointensities
dural tail
1166
1168
Neuroradiology
DURAL TAIL
Curvilinear enhancement
dural flair
First reported w/meningioma
First reported to be neoplastic invasion
What is it REALLY?
Thickening of the dura
Vasocongestion of the dura
Edema of the dura
Dural tail: Histology

Dural Tail: Differential Dx
Figure 5-13-14
Extraaxial Lesions
Meningioma
Most common lesion w/dural tail
Most Common Lesions Overall
Schwannoma
Hemangiopericytoma
Sarcoidosis
Gumma (syphilis)
Intraaxial Lesions
Superficial cerebral astrocytoma
GBM (rare)
Cavernous Sinus Meningioma with extensive

dural enhancement along the tentorium
Whorls of Spindle Cells

Cavernous Sinus Meningioma [Figure 5-13-14]
Meningioma: Named by region
Tentorial / Pineal
Clival
Dural Tail - Schwannoma

MENINGIOMA *Imaging Features: CT vs. MR
Mass effect
Extraaxial Location
Broad Dural Attach.
Typical. Dense./Intensity
Hyperostosis
Homogeneous
Enhancement
(Homogeneous)
Meningeal. Enhance (tail)
Capsule
CT
88%
42%
74%
92%
10%
76%
MR
90%
70%
98%
74%
14%
76%
96%(78%)
2%
14%
96%(80%)
50%
68%
*Neuroradiology 1990;32:467473
Meningioma - Transdural
Other Locations for Meningioma
Intraventricular
Orbit
Intraconal
Periorbital
Nasal Cavity
Neuroradiology
1167
1169
Intraventricular: ~ 5% [Figures 5-13-15 and 5-13-16]
Usually Adult
F>M
Usually Lateral Ventricle
Usually Trigone/Atrium
ALWAYS attached to Choroid Plexus
Vascular pedicle from choroid
Figure 5-13-15
Optic Nerve Meningioma

Meningioma Angiography - Supply
External Carotid
85%
Some have dual supply
Internal Carotid
63%
Tumor Blush
95%
Figure 5-13-16
Meningioma Angiography
Transit Time [Figure 5-13-17]
Blush or Stain
early arterial
prominent in VENOUS phase
capillaries/sm. arterioles
(too small to see individually)
Venous Filling
characteristic if delayed
may fill with/ before NI. veins
Normal Choroid Plexus: Nests of
arachnoid cap cells
Spoke Wheel Vessels

Meningioma Angiography Transit Time
[Figures 5-13-18 and 5-13-19]
Figure 5-13-17
Venous Filling (Stattin, 1996)

170 Meningiomas
delayed in 136 (80%)
with Nl. vv. In 10 (6%)
earlier in 24 (14%)
(8 in early arterial phase)
Leeds & Taveras (1969)
EDV in 6/36 (16%)
Figure 5-13-18
Meningioma Supplied by branches of the ECA,

showing classic spoke-wheel vascularity
Meningioma Venous phase,

showing persistent and dense tumor
blush
1168
1170
Neuroradiology
Meningioma Pre-Op Embolization
Figure 5-13-19
Gd Pre Embo
Gd Post Embo
AJNR Editorial - September 2003; 24: 1499 - 1500
Meningioma Angiography Tumor Blush
May come early

Usually very DENSE
Stays way too long
(Persistent!)
Derek Harwood-Nash:The In-Law Effect
Meningioma Effect on Skull
Hyperostosis (15%-25%)
w or w/o micro invasion
Pressure Erosion
Periosteal remodeling
Bone Destruction
microscopic invasion
HYPEROSTOSIS IN MENINGIOMAS
MR Perfusion study, showing delayed washout

from meningioma.
(Courtesy of Dra. Perla Salgado, Mexico City,
Mexico)
[Figures 5-13-20 to 5-13-22]
Figure 5-13-20
Figure 5-13-21
Variable patterns of hyperostosis from

meningioma
Meningioma Hyperostosis
Variable patterns of hyperostosis from

meningioma
Does NOT mean invasion of bone

Implies CHRONICITY
and benign behavior
Skull Base
Invasion via HAVERSIAN CANALS
Figure 5-13-22
Hyperostosis from meningioma

Neuroradiology
1169
1171
Meningioma Atypical Imaging

(Yet, typical Histology) [Figure 5-13-23]
Figure 5-13-23
Focal Lucency Outside (arachnoid cyst)

Focal Lucency Inside (necrosis, cyst)
Hypodensity (humid, lipoblastic)
Focal Hyperdensity (metaplasia, hemorrhage)
Heterogeneous
Hyperintensity on T1W
Hyperintensity on T2W
Fatty Metaplasia
Fatty (Lipoblastic) Meningioma
Fatty Metaplasia [Figure 5-13-24]
Meningioma with typical histology yet aggressive

radiologic appearance
Meningioma Cysts
Figure 5-13-24
Inside of neoplasm
(rim enhancement)
Between tumor and brain
(arachnoid cysts)
Inside Brain
PIA separates tumor from brain
?? Results of chronic Edema
Vacuolization of White Matter

Meningioma Atypical Histology
WHO Grade 2 Meningioma with Fatty Metaplasia
Atypical Meningioma ~ 5%7%

Anaplastic Meningioma ~ 1%3%
~ 0.2% / 100k per year
Higher incidence of Recurrence
Shorter time to Recurrence
Atypical Histology
necrosis
excessive mitoses
invasion into brain
Figure 5-13-25
Malignant Meningioma
< 3% of all Meningioma

Anaplastic (Malignant) Meningioma
Papillary Meningioma
Benign Metastasizing Meningioma
Hemangio-Peri-Cytoma (HPC)
Malignant Fibrous Histiocytoma (MFH)
Meningioma One level shows typical

hemispheric shape, the next shows a peritumoral
cyst
1170
1172
Neuroradiology
Hemangiopericytoma [Figure 5-13-26]
Syn: angioblastic meningioma

Cell of Origin perivascular pericyte of Zahn and/or
Zimmerman
< 1% of primary CNS
M 1.4:1 F
Age 40s
Dural based, bone destruction, lobulated
Figure 5-13-26
Hemangiopericytoma (HPC)
Narrow dural base (Mushrooming)

No Hyperostosis
No Calcification in tumor
Lobulated (not hemispheric)
Internal Signal Voids (on MRI)
irregular and multiple
Hypervascular on Angio
irregular patterns
Meningioma Radiologic Features CT
Feature
Benign / Malignant
Homogeneous
Enhancement
72%
/ 36%
Heterogeneous
Enhancement
23%
/ 64%
Hyperostosis
18%
/ 7%
Calcification
27%
/ 0%
Mushrooming
0%
/ 57%
Narrow attachment and larger cap
invaginating into brain
Hemangiopericytoma
Hemangiopericytoma vs. Meningioma

Hemangiopericytoma
Narrow Base
Lobulated
Heterogeneous
Bone Destruction No Ca++
Irregular Vessels
Meningioma
Broad Base
Hemispheric
Homogeneous
Hyperostosis Psammomatous Ca++
Spoke Wheel Vessels
Meningioma
The 4H+ Tumor

homogeneous
hyperdense
homogeneous enhancement
hemispheric shape
hyperostosis
hormonally modulated
Meningioma
75% are histologically typical

75% are radiologically typical
Not the same 75%
CT
MR
Angiography
Atypical Imaging =/= Atypical Histology
Neuroradiology
1171
1173
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
Ahmadi J, Hinton DR, Segall HD, Couldwell WT. Surgical implications of magnetic resonance-enhanced dura.
Neurosurgery. 1994 Sep;35(3):370-7;discussion 377.
Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance
of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990 Sep-Oct;11(5):935-8.
Asari S, Yabuno N, Ohmoto T. Magnetic resonance characteristics of meningiomas arising from the falcotentorial
junction. Comput Med Imaging Graph. 1994 May-Jun;18(3):181-5.
Berger MS. Perfusion MR and the evaluation of meningiomas: is it important surgically? AJNR Am J Neuroradiol
2003; 24:1499-1500. (1)
Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural "tail" associated with meningiomas on Gd-DTPAenhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment.
Radiology. 1990 Aug;176(2):447-50.
Helie O, Soulie D, Sarrazin JL, Derosier C, Cordoliani YS, Cosnard G. [Magnetic resonance imaging and
meningiomas of the posterior cerebral fossa. 31 cases] J Neuroradiol. 1995 Dec;22(4):252-70. French.
Hutzelmann A, Palmie S, Buhl R, Freund M, Heller M. Dural invasion of meningiomas adjacent to the tumor
margin on Gd-DTPA-enhanced MR images: histopathologic correlation. Eur Radiol. 1998;8(5):746-8.
Hutzelmann A, Palmie S, Freund M, Buhl R, Heller M. [Dura thickening adjacent to intracranial, para-dural spaceoccupying lesions in MRI. Histologic correlation] Aktuelle Radiol. 1997 Nov;7(6):305-8. German.
Hutzelmann A, Palmie S, Zimmer C, Benz T, Leweke F, Freund M. [The meningeal sign: a new appraisal] Rofo.
1996 Apr;164(4):314-7. German.
Ildan F, Tuna M, Gocer AP, Boyar B, Bagdatoglu H, Sen O, Haciyakupoglu S, Burgut HR. Correlation of the
relationships of brain-tumor interfaces, magnetic resonance imaging, and angiographic findings to predict cleavage
of meningiomas. J Neurosurg. 1999 Sep;91(3):384-90.
Kawahara Y, Niiro M, Yokoyama S, Kuratsu J. Dural congestion accompanying meningioma invasion into vessels:
the dural tail sign. Neuroradiology. 2001 Jun;43(6):462-5.
Maiuri F et al: Intracranial meningiomas: correlations between MR imaging and histology. Eur J Radiol. 1999; 31:
69-75
Nagele T, Petersen D, Klose U, Grodd W, Opitz H, Voigt K. The "dural tail" adjacent to meningiomas studied by
dynamic contrast-enhanced MRI: a comparison with histopathology. Neuroradiology. 1994 May;36(4):303-7.
Nakasu S, Nakasu Y, Matsumura K, Matsuda M, Handa J. Interface between the meningioma and the brain on
magnetic resonance imaging. Surg Neurol. 1990 Feb;33(2):105-16.
Nakau H, Miyazawa T, Tamai S, Tsuchiya K, Shima K, Shirotani T, Chigasaki H. Pathologic significance of
meningeal enhancement ("flare sign") of meningiomas on MRI. Surg Neurol. 1997 Dec;48(6):584-90; discussion
590-1.
Quekel LG, Versteege CW. The "dural tail sign" in MRI of spinal meningiomas. J Comput Assist Tomogr. 1995
Nov-Dec;19(6):890-2.
Sakai K, Tada T, Fukasaku K, Kyoshima K, Kobayashi S. Histological examination of the gadolinium-enhanced
dura mater around meningiomas on magnetic resonance imaging. Neurol Med Chir (Tokyo). 1993 Jul;33(7):42933.
Sandhyamani, Rao, Nair, Radhakrishan: Atypical Meningioma: A Clinicopathological Analysis.Neurology India
2000; 48: 338-342
Sato M, Matsumoto M, Kodama N. Meningeal enhancement surrounding meningiomas on Gd-DTPA MRI.
Fukushima J Med Sci. 1998 Jun;44(1):1-11.
Sekiya T, Manabe H, Iwabuchi T, Narita T. [The dura mater adjacent to the attachment of meningiomas: its
enhanced MR imaging and histological findings] No Shinkei Geka. 1992 Oct;20(10):1063-8. Japanese.
Takeguchi T, Miki H, Shimizu T, Kikuchi K, Mochizuki T, Ohue S, Ohnishi T. The dural tail of intracranial
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Jan 28.
Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L, Baert AL. Thickening of dura
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1174
Neuroradiology
PINEALOMAS and other

Pineal Region Masses
Pineal Gland Introduction
Seat of the Soul

Daily (Diurnal) Biorhythms
Life-Cycles (Puberty, Migration)
Responds to Light/Dark
Melatonin levels
Accessory Optic Pathway
Retinohypothalamic tract, RAS, Sympathetics
Third Eye
Phylogenetically
Developmentally
Embryologically
Pineal Third Eye
Iguana
Third Eye
Photoreceptor
Transparent scale
Hole in skull
Radiometer for sunlight
In lower vertebrates it may have a lens and a retina
Figure 5-14-1
The normal pineal gland is ~10-14

mm in maximum sagittal diameter
Figure 5-14-2
Biological Clock
Day-Night Diurnal Rhythms

Pineal Melatonin Suppresses GnRH
Longer daylight decreases melatonin and leads
to increased Gonadotropin Releasing Hormone
GnRH => LH and FSH
Increased sexual drive and activity in the Spring
and I thought it was the Pollen
Normal Pineal [Figures 5-14-1 and 5-14-2]

Normal Pineal Calcification
Pineal gland and surrounding region: Third

ventricle, quadrigeminal plate and cistern, bilateral
thalami
Figure 5-14-3
725 Normal Patients

Youngest was 6,5
8%11% from 8 to 14 yrs.
30% for 15 y.o.
39%40% from 17 to 29 yrs.
Radiology 142:659662, 1982
Pineal Region Normal Anatomy [Figure 5-14-3]

Pineal Region Mass: Symptoms and Signs
Parinaud Syndrome
NOT Paranoid
Precocious Puberty
Headache, Nausea, Vomiting
Non-specific mass effect
+ICP
Neuroradiology
Pineal Region Normal Anatomy:

The internal cerebral veins are
important landmarks
1173
1175
PINEALOMAS and, other Pineal Region Masses
Parinaud Syndrome: Aqueduct/Tectal Syndrome
Failure of conjugate vertical eye movement

Upward >> downward
Mydriasis, fixed pupils
Failed ocular convergence
Lateral midbrain tegmentum
Blepharospasm
Eyelid spasm
Pineal Region: Differential
Germ Cell Neoplasms

Pineal Cell Neoplasms
Gliomas
Non-neuroglial Masses
Germ Cell Tumors: WHO Classification
Germinoma
Embryonal Carcinoma
Yolk Sac
Endodermal sinus
Choriocarcinoma
Teratoma
Immature, Mature, Malignant
Mixed Germ Cell
Intracranial Germ Cell Tumors
Usually primary in the CNS

Arise from Germ Cell Rests
Pineal/Quadrigeminal and suprasellar cistern
Exceptional caese metastatic to CNS
Usual testicular drainage to renal hilus
Para-artic nodes
Rare cases of testicular seminoma CNS mets
Lance Armstrong had mixed Chorio/embryonal
Pineal Region Mass
369 pts. Hoffman series
Tumor
GERM CELL
59
Germinoma
59
Teratoma Malignant
11
Teratoma Benign
Yolk Sac
Choriocarcinoma
Embryonal CA
Mixed Germ Cell
PINEAL REGION MASS
369 pts. Hoffman

EPIDERMOID
TRUE PINEAL
Pineoblastoma
Pineocytoma
OTHER NEOPLASMS
Gliomas
Non-glial (meningioma, etc)
1+
14
12
2
27
26
1174
1176
Neuroradiology
Dr. Hoffmans Series
Dr Harold J Hoffman (1932 2004)

Arguably the most famous pediatric neurosurgeon in
the world.
Harold joined the Neurosurgical Staff at the Hospital
for Sick Children in 1964
1998, the Harold J Hoffman/Shoppers Drug Mart
Chair in Pediatric Neurosurgery was established at
the Hospital for Sick Children
Figure 5-14-4
Pineal Neoplasms: Demographics

Germ Cell Tumors-AFIP Series [Figure 5-14-4]
Pineal Region Germ Cell Tumors - AFIP Series
Basic Approach to Pineal Region
Figure 5-14-5
Intracranial Germinoma
Synonyms: Pinealoma, Seminoma, Dysgerminoma,

Atypical Teratoma
Cell of Origin: Germ Cell Rests, 2-cells pattern
Incidence: 1%2% of ALL Cranial neoplasms
2%4% of Child.
9%15% in Japan
Age: 535 (remember precocious puberty)
Sex: 27M/F
Location: 6080% Pineal, 22% Suprasellar
Treatment: Bx, Radiation, ChemoTx
Prognosis: >50% at 5yrs
Radiosensitive and chemosensitive tumor
Median survival ~19 yrs
Pineal Region Germinoma Two cell pattern, one

cell resembles a lymphocyte
Figure 5-14-6
Intracranial Germinoma
Central:
pineal (para-pineal)
Suprasellar cistern
Homogeneous Solid
Hyperdense on NCT
Isointense on T1W
Hormonally silent
no AFP/HCG but PLAP+
Germinoma Imaging [Figures 5-14-5 to 5-14-8]
Pineal region seminomas are usually hyperdense

on plain CT
Sharply circumscribed, midline mass

Surrounds/Engulfs Pineal Ca++
Alternate locations
thalamus, 3rd vent., suprasellar cistern
NCT Homogeneous Hyperdense
ECT Homogeneous Enhancement
MR Isointense to gray matter
+/ CSF Spread, tumor Ca++
Figure 5-14-7
Surgical Planning
Find the Internal Cerebral Veins and the VOG

If Tumor is BELOW these veins
Suboccipital Infratentorial Approach
If Tumor is ABOVE these veins
Interhemispheric Approach
Sub Temporal Approach
Various other techniques
Neuroradiology
Pineal region germinoma
1175
1177
Pineal Region Neoplasms
Figure 5-14-8
Germinoma:
Iso on T1W
slightly Hyper on T2W
Choriocarcinoma:
Hyper on T1W (blood)
Dermoid, Teratoma:
Hyper on T1W (lipid)
MR AJNR 11:557565,1990

Pineal Region [Figures 5-14-9 and 5-14-10]
Teratoma
Sharply circumscribed
Lobulated and Loculated
HETEROGENEOUS
(mixture of lipid, soft-tissue, Ca++)
Enhancement of solid areas
Pineal region germinoma, extending

below the tentorium
Figure 5-14-10
Figure 5-14-9
Ruptured Pineal region teratoma. Note the

lipid/fluid levels in the frontal regions of both lateral
ventricles. The primary tumor is seen in the
midline pineal region
Pineal region teratoma. Note the
peripheral rim of T1-shortening from
lipid
Teratoma vs. Dermoid
Teratoma is a Neoplasm
From Multipotential Cells/Tissues
Included Twin from embryo/fetus
Ectoderm (Skin, Occ. Brain) Common
Lipid (Mesodermal FAT or Sebaceous)
Multiloculated, Lobulated
Dermoid is an Inclusion Cyst
Only Skin (Ectoderm)
Water and/or Sebaceous Lipid
Unilocular
Famous Quote
Aunt Voula:
You family now, so I tell you a story. All my life ... I have this lump on the back of
my neck. When I reach the menopause, the lump get bigger. I go to the doctor,
and he performs a...bo-bobopsy. And inside the lump, he finds teeth, and a spinal
column.
1176
1178
Neuroradiology
Two Theories for Teratoma
Progressive differentiation from multi-potential (omnipotential)

Germ Cells primordial germ cells
Inclusion of a twin during early gestation - embryogenesis
Figure 5-14-11
Dermoid Inclusion Cyst [Figure 5-14-11]

Pineal Region Mass
Endodermal Sinus Tumor [Figure 5-14-12]
Figure 5-14-12
Dermoid Inclusion Cyst in the pineal

region. Histology only revealed
ectodermal elements
Non-seminomatous germ cell tumor NOT
hyperdense on plain CT, but does engulf a central
calcification
Figure 5-14-13

Germinoma
Germinoma - seeding [Figure 5-14-13]
CSF Dissemination
Pineal Neoplasms Laboratory Tests:
Serum and CSF
Neoplasm
Germinoma
Yolk-sac
Chorio Ca.
Embryonal
BHCG
--inc.
inc.
AFP
-inc.
-inc.
PLAP
inc.
----
Germinoma CSF seeding along the

edge of the tentorium (arrows)
BHCG = Beta HCG

AFP = Alpha Feto Protein
PLAP = Placental Alkaline Phosphatase
Neuroradiology
1177
1179
Pineal Parenchyma
Figure 5-14-14
Pineoblastoma (PNET)
Young Patients (1st two decades)
Tumor ITSELF Calcifies
Exploded Pineal Ca++
Pineocytoma (Mature pineal cells)
Young or Old
Trilateral Retinoblastoma
Inherited Rb (chromosome 13)
1/3 inherited but 2/3 heritable
Look at ORBITS for signs of Tx
Pineal Parenchyma Mass [Figure 5-14-14]
Exploded Pineal Calcifications

Pineoblastoma
Some hyperdense on CT
Pineocytoma
Isodense on CT
Pineal Parenchyma Mass Schematic:

exploded calcifications from a mass
arising inside the pineal gland. This
pattern is seen in both pineal cysts
and neoplasms
Figure 5-14-15
Figure 5-14-16
Pineoblastoma exploded calcifications around

the outside rim of the tumor
Pineoblastoma
Figure 5-14-17
Figure 5-14-18
Pineoblastoma with seeding along the edge of the

tentorium. Using this T1W MR alone, this mass is
indistinguishable from a pineal region germinoma
Pineoblastoma. Note how the lesion extends

below the tentorial hiatus into the posterior fossa
1178
1180
Neuroradiology
Pineoblastoma [Figures 5-14-15 to 5-14-18]
Figure 5-14-19
Pineal Cysts
Autopsy
~ 5% of Adults
< 2 mm in a Normal Size Gland
MR Visible
2%-8% of Adults
2-7 mm common
14-25 mm cysts are not rare
May expand the gland
Ring enhancement should be smooth and thin 1-2 mm
Why do they grow? Unknown
Pineal Cyst
Schematic for Vein of Galen

malformations: Straight sinus
obstruction, sinus hypoplasia, AVM or
dural fistula draining into the VOG
Typical cysts:
Round or Oval
T1 ~ WM
T2 ~ CSF
T2 Homogeneous
Rim Enhancement< 2mm
No nodularity
These findings suggest that typical pineal cysts
may be followed up on a clinical basis alone rather
than on imaging.
Figure 5-14-20
Pineal Region Mass: Other Lesions
Glial - Astrocytoma
Splenium Of Corpus Callosum
Tectum Of Midbrain
Thalamus
Congenital
Lipoma
Inclusion Cyst (Epidermoid/Dermoid)
Vein of Galen Malformation
Non-Glial - Meningioma
Vein of Galen Malformation
Vein Of Galen Malformation: Symptoms, Signs
Figure 5-14-21
Childhood Large shunt

High Output Failure
Persistent Ductus
Hydrocephalus
Cranial Bruit/thrill
Adult Small shunt
Asymptomatic
Pineal Symptoms
Vein Of Galen Malformation: Types and

Causes
[Figures 5-14-19 and 5-14-21]
Vein of Galen Malformation. Persistence of the

falcine vein
Parenchymal Avm (Shunt)

Direct Fistulae To Vein
Dural Fistula (Drains To Vein)
Sinus Thrombosis (Fetal)
Hypoplastic Straight Sinus
Hydrocephalus
Mechanical
Aqueductal Obstruction
Impaired CSF Resorption
Venous Hypertension
Neuroradiology
1179
1181
Intracranial Lipoma [Figure 5-14-22]
Figure 5-14-22
Congenital, NOT a true neoplasm

MIDLINE (usually)
Usually around Corpus Callosum
Occasional Tectal, Hypothalamic, CPA
Abnormal Differentiation
Meninx Primativa Into Fat
Meningioma [Figures 5-14-23 and 5-14-24]

Figure 5-14-23
Intracranial Lipoma of the quadrigeminal plate.

Notice how the mass presents below the
tentorium because of the herniation caused by
obstructive hydrocephalus
Figure 5-14-24
Meningioma of the quadrigeminal plate
Glioblastoma multiforme [Figure 5-14-25]

Figure 5-14-25
Meningioma of the quadrigeminal

plate see the dural tail
Figure 5-14-26
Mass in the splenium of the corpus callosum
Glioblastoma multiforme
Astrocytoma - Splenium [Figure 5-14-25]

Headaches and Parinaud Syndrome [Figure 5-14-26]
Astrocytoma of Tectum
Astrocytoma of the quadrigeminal

plate
1180
1182
Neuroradiology
Pineal Region
60% Germ Cell Neoplasm

Seminoma (2/3)
Teratoma
Other non-germinoma GCT
15% Pineal Parenchymal
Pineocytoma
Pineoblastoma (PNET)
OTHER Lesions
Astrocytoma
Splenium, Tectum, Thalamus
Meningioma, Lipoma
VOG Malformations
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
Barboriak DP, Lee L, Provenzale JM. Serial MR imaging of pineal cysts: implications for natural history and
follow-up. AJR Am J Roentgenol. 2001 Mar;176(3):737-43.
Barbouriak DP, Lee L, Provenzale JM: Serial MR Imaging of Pineal Cysts: Implications for Natural History and
Follow-Up.AJR 2001; 1737-743.
Fain JS, Tomlinson FH, Scheithauer BW, Parisi JE, Fletcher GP, Kelly PJ, Miller GM. Symptomatic glial cysts of
the pineal gland. J Neurosurg. 1994 Mar;80(3):454-60.
Fleege MA, Miller GM, Fletcher GP, Fain JS, Scheithauer BW. Benign glial cysts of the pineal gland: unusual
imaging characteristics with histologic correlation. AJNR Am J Neuroradiol. 1994 Jan;15(1):161-6.
Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O, Tamura A, Sano K.J Neurooncol. CT and
MRI features of intracranial germ cell tumors. 1994;19(3):217-26.
Hayashida Y, Hirai T, Korogi Y, Kochi M, Maruyama N, Yamura M, Yamashita Y. Pineal cystic germinoma with
syncytiotrophoblastic giant cells mimicking MR imaging findings of a pineal cyst. AJNR Am J Neuroradiol. 2004
Oct;25(9):1538-40.
Jinkins JR, Xiong L, Reiter RJ. The midline pineal "eye": MR and CT characteristics of the pineal gland with and
without benign cyst formation. J Pineal Res. 1995 Sep;19(2):64-71.
Koenigsberg RA, Faro S, Marino R, Turz A, Goldman W. Imaging of pineal apoplexy. Clin Imaging. 1996 AprJun;20(2):91-4.
Korogi Y, Takahashi M, Ushio Y. MRI of pineal region tumors. J Neurooncol. 2001 Sep;54(3):251-61.
Lee DH, Norman D, Newton TH. MR imaging of pineal cysts. J Comput Assist Tomogr. 1987 Jul-Aug;11(4):58690.
Mamourian A, Towfighi J. MR of pineal cysts. AJNR Am J Neuroradiol. 1994 Oct;15(9):1796-7.
Mamourian AC, Towfighi J. Pineal cysts: MR imaging. AJNR Am J Neuroradiol. 1986 Nov-Dec;7(6):1081-6.
Mamourian AC, Yarnell T. Enhancement of pineal cysts on MR images. AJNR Am J Neuroradiol. 1991 JulAug;12(4):773-4. No abstract available.
Osborn RE, Deen HG, Kerber CW, Glass RF. A case of hemorrhagic pineal cyst: MR/CT correlation.
Neuroradiology. 1989;31(2):187-9.
Reis F, Faria AV, Zanardi VA, Menezes JR, Cendes F, Queiroz LS. Neuroimaging in pineal tumors. J
Neuroimaging. 2006 Jan;16(1):52-8.
Sener RN. The pineal gland: a comparative MR imaging study in children and adults with respect to normal
anatomical variations and pineal cysts. Pediatr Radiol. 1995;25(4):245-8.
Welton PL, Reicher MA, Kellerhouse LE, Ott KH. MR of benign pineal cyst. AJNR Am J Neuroradiol. 1988 MayJun;9(3):612. No abstract available.
Neuroradiology
1181
1183
The Phakomatoses
Phakomatoses or Neurocutaneous Syndromes
An Introduction
The Phakomatoses
Neuro-ectodermal
or
Nerves and Skin
Phakomatoses: Why Study Them?
They are COMMON diseases

DIAGNOSED by Imaging
GENETIC Implications
SCREEN Relatives
SURVEILLANCE of Affected
Phakomatoses Mnemonic Tool
NF-1 (von Recks)

TRUE Neurofibromatosis #17
NF-2 (Bil. VS Syndrome)
M.I.S.M.E. #22
STURGE-WEBER (Dimitri) Syndrome
Congenital Vascular Lesion
Perhaps NOT inherited
Tuberous Sclerosis
Pringles HAMARTOMA Disease
Von HIPPEL-LINDAU Syndrome
NO cutaneous lesions
Hemangioblastomas and Visceral Lesions
Phakomatoses Plan
Demographics
Diagnostic Criteria
Ocular/Orbit Lesions
Skin
Brain
Visceral Manifestations
Complications of Disease
CNS Neoplasms: Chromosomes
Loss of Heterozygosity (LOH)

Schwannoma - 22q
Meningioma - 22q (Long Arm)
Ependymoma - 22
Medulloblastoma - 17p (Short Arm)
Neurofibrosarcoma - 17p
Retinoblastoma - 13q
Pilocytic Astrocytoma - None !
TUMOR SUPPRESSOR GENES
Molecular Biology [Figures 5-15-1]
Genetic Two Hit Theory
The Phakomatoses
1182
1184
Neuroradiology
Phakomatoses
NEUROFIBROMATOSES
Type 1, Chromosome 17q11
Type 2, Chromosome 22q12
Tuberous Sclerosis
Chromosome 9q, 16p
STURGE-WEBER (? not inherited)
von Hippel-Lindau
Chromosome 3p25
Figure 5-15-1
Neurofibromatosis
NF-1, von Recklinghausen (peripheral bad term)

NF-2, Bilateral Acoustic (central bad term)
NF-3, Overlap of 1 and 2
NF-5, Segmental (e.g. a quadrant)
NF-6, Cafe-au-lait, w/o CNS/PNS
NF-7, Late Onset
NF-8, Other
Neurofibromatosis Types
Neurofibromatosis Type 1 (NF-1)

von Recklinghausen Disease
True Neurofibromatosis
Prominent Cutaneous Signs
Chromosome 17q
Neurofibromatosis Type 2 (NF -2)
Bilateral Acoustic Schwannoma
Central Neurofibromatosis
Minimal Skin Manifestations
Chromosome 22q
Neurofibromatosis Type 1 or von

Recklinghausen Disease
Tumor Suppressor Gene: Two Hit Hypothesis
Chromosome 17
Neurofibromatosis
1768 MARK AKENSIDE (New York)

1793 TILESIUS (Leipzig)
1849 R.W. SMITH (England)
1822 WISHART (Edinburg) NF-2
1882 von RECKLINGHAUSEN (Germany)
Neurofibromatosis - 1
Clinical
Incidence: 1/2,500 births
Inheritance: Autosomal Dominant
Age at Presentation: Birth to Death
Sx at Presentation: Spots, NFB
Diagnostic Criteria: Cutaneous, PNS
Chromosome Abnl.: 17
Ocular Findings: Myelinated retina
Cutaneous Findings: cafe-au-lait, neurofibroma
CNS Findings: Optic N. Glioma, Hamartoma, Heterotopia, macrocephaly,
mentation problems
NIH Diagnostic Criteria: 2 from list
Cafe-Au-Lait spots
6 or more
5 mm child, 15 mm adult
Neurofibromas 2 or more
Plexiform Neurofibroma 1
Neuroradiology
1183
1185
The Phakomatoses
Figure 5-15-2
Axillary (Intertriginous) Freckling-1

Optic Glioma
Lisch Nodules (Iris) 2 or more
Distinctive Bone Lesions
1st degree relative with NF-1
Neurofibromatosis 1
Clinical
Ocular: Myelinated retina
Cutaneous: cafe-au-lait, neurofibroma
CNS: Optic N. Glioma, Hamartoma, Heterotopia,
Macrocephaly, Mentation
NF-1: Eye Manifestations
Lisch Nodules Named for the 19th century

German physician who first described them, Dr.
Augustus Nodule
LISCH Nodules (Iris Hamartomas)

[Figure 5-15-2]
Penetrance > 90%

Specificity > 90%
Translucent/pigmented
Small ( < 3mm.), Slit-Lamp Exam
OPTIC GLIOMA [Figures 5-15-3]
Up to 15% of patients
Pilocytic Astrocytomas
Benign (Hamartoma -like), Tx?
True Neoplasms, spread along SAS
up to 1/2 of Childhood ONG w/NF -1
Figure 5-15-3
Neurofibromatosis 1
Cutaneous Manifestations
Cafe-au-Lait spots [Figure 5-15-4]
Intertriginous Freckling
Neurofibromas (Skin and SubQ)
Fibroma Molluscum (TNTC NFB)
Elephantiasis Neuromatosa
diffuse skin thickening/plexiform NFB
or focal gigantism
Neurofibromatosis 1
Bone Dysplasia and Remodeling

Macrocephaly
Craniofacial dysplasia
especially sphenoid
Vertebrae (scalloping, scoliosis)
Pseudoarthrosis
especially congenital
Genu Valgum/Varum
Twisted Ribbon Ribs
Optic Nerve Glioma
Figure 5-15-4
Neurofibromatosis 1 [Figures 5-15-5 to 5-15-8]
Skull and Spine Dysplasia

Sphenoid Bone (absent orbit)
Lambdoid Suture at Temporal Bone
Optic and Auditory Canals (enlarged)
Scoliosis
Simple or Acute Cx Kyphosis
Vertebral Scalloping (usu. Lumbar)
Enlarged Spine Neural Foramina
Caf-au-lait spot a macular (flat)

area of hyperpigmentation
The Phakomatoses
1184
1186
Neuroradiology
Embryo Differential Development
Molecular biology
Chemical gradients
Medial <> Lateral
Proximal <> Distal
Anterior <> Posterior
Superior <> Inferior
Sonic Hedgehog Gene (shh)
Drosophila embryo spiked like a hedgehog
Desert hedgehog (dhh), Indian hedgehog (ihh)
Needed a new name scientists like videogames so
Figure 5-15-5
Nerve Sheath Tumors
Schwannoma (Sporadic >> NF-2 > NF-1)

focal mass
usually sensory root, cranial and spinal nerves
Neurofibroma
usually NF-1, esp. if spinal or paraspinal
spindle or dumb -bell lesion
Plexiform Neurofibroma (usually NF-1)
diffuse or fusiform enlargement
NF-1 or Sporadic
Empty Orbit from sphenoid

dysplasia. The left orbit shows the
outline of normal sphenoid lesser
(superior) and greater (inferior)
densities
Figure 5-15-6
Figure 5-15-7
Sphenoid Dysplasia
Figure 5-15-8
Progressive Pseudoarthrosis
Focal Gigantism the overgrowth may affect all

elements, bone, muscle, fat, vessels, etc.
Neuroradiology
1185
1187
The Phakomatoses
Figure 5-15-9
Figure 5-15-10
Neurofibroma a diffuse lesion, even at the

microscopic level
Schwannoma a focal mass, even at the

microscopic level
Neurofibroma vs. Schwannoma
Neurofibroma [Figure 5-15-10]

Schwann cells
Fibroblasts
Acellular material
Infiltrating
Resect Parent Nerve
Schwannoma [Figure 5-15-9]
Schwann Cell Neoplasm
Secondary vascular changes
Mostly cellular
Encapsulated
Nerve Sparing Surgery
Distribution of Nerve Sheath Tumors
Intra-Cranial Schwannoma
Sporadic >> NF-2
Spinal Both Types (S >> N)
Dumbbell Both (N >> S)
PNS Both
Cutaneous Neurofibroma
Usually N in NF-1)
Figure 5-15-11
Intraspinal Neoplasms
68 Pts.
86 Spinal Nerve Sheath neoplasms
Sporadic: 42 pts. (65%)
40 Schwannoma and 2 neurofibroma
NF-1: 12 Pts. (18%)
All were Neurofibroma
NF-2: 7 Pts (11%)
6 Schwannoma/1 mixed tumor
Unknown - 5 Pts.
Acute Cervical Kyphoscoliosis one of the

characteristic lesions of NF-1
Neurofibromatosis : Spine [Figure 5-15-11]
Scoliosis (NF-1, only?)

Simple ("idiopathic")
Acute Cervical Kyphosis
Dural Ectasia (NF-1, only?)
Vertebral Scalloping
Arachnoid "cysts"
Lateral Thoracic meningocele
The Phakomatoses
1186
1188
Neuroradiology
Neurofibromatosis : Spine
Figure 5-15-12
Neurofibroma (NF-1)
Osteoporosis (NF-1, only?)
Idiopathic
Parathyroid Adenoma
Schwannoma (NF-2)
Meningioma (NF-2
Ependymoma (NF-2)
Enlarged Neural Foramen
DDx:
Nerve Sheath Tumor
Neurofibroma
Schwannoma
Arachnoid Cyst
Bone Dysplasia
Enlarged neural foramina from multiple plexiform

neurofibromata
Neurofibromatosis: Enlarged Neural

Foramen [Figures 5-15-12 to 5-15-14]
Nerve Sheath Tumor

Neurofibroma
NF-1 >> sporadic
"dumbbell shape
Schwannoma
sporadic >> NF-2
Mesodermal Defect
NF-1 only?
Dural weakness
Bone weakness
Figure 5-15-13
Rib Notching [Figure 5-15-15 ]
Aortic Coarctation
Older than 5-6 years
3-9 possible
Ribs 5-8 most often
1-2 arise from subclavian artery
Usually Bilateral
Unilateral on the Right
if Coarctation involves Left Subclavian origin
A-V Fistulae
Nerve Sheath tumors
Multiple Dumbbell Lesions - neurofibromas
Figure 5-29-14
Figure 5-15-15
Neurofibroma vs. Schwannoma
Rib Notching from extensive plexiform

neurofibromas involving all of the intercostal
nerves
Neuroradiology
1187
1189
The Phakomatoses
Plexiform NF [Figure 5-15-16 ]
Figure 5-15-16
Plexiform neurofibromas
Neurofibromatosis - 1: Spine
Figure 5-15-17
[Figures 5-15-17 and 5-15-18]
Scoliosis (Acute Cx Kyphoscoliosis)

Vertebral Scalloping
Enlarged Neural Foramina
Neurofibromatosis - 1 [Figure 5-15-19]
Posterior Meningocele (sporadic)

dorsal dysraphism, closure of tube
Anterior Meningocele (sporadic)
neurenteric canal/cyst
anterior vertebral cleft
Lateral Thoracic Meningocele (NF-1)
pulsion diverticulum of SAS
negative intrathoracic pressure
no overlying paravertebral MM.
Vertebral Body Scalloping and one

neurofibroma. Both lesions may also
cause enlargement/erosion of the
neural foramna
Figure 5-15-18
Figure 5-15-19
Arachnoid Cyst & Neurofibroma

The Phakomatoses
1188
1190
Neuroradiology
Neurofibromatosis 1: DBOs MR Signal

Abnormalities
Figure 5-15-20
[Figure 5-15-20]
T1W Bright Foci

globus pallidus
T2W Bright Foci
w/o mass, dont enhance
Cerebellar peduncles, Pons, midbrain
globus pallidus, thalamus, optic radiations
What in the heck are they??
Ectopic Schwann cells, Melanocytes??
Dysmyelination??
Intracellular proteinaceous fluid?
DBOs and NF-1
Incidence: A considerable body of knowledge suggests that

these Unidentified Deep Bright Objects or DBOs are very
common in children with NF-1. (>90% in some series)
Age: They are most frequent from 4 12 years of age. They are
uncommon under the age of 4, and begin to fade away over the
age of 16.
Location:
Globus Pallidus 30%
Cerebellum 23%
Deep Bright Objects
Midbrain 16%
DBOs of NF-1: Globus pallidus
Neurofibromatosis
Objects
of Uncertain
Significance
NOUS
Neurofibromatosis
Malignant Nerve Sheath

Tumor (malignant PNST, neurofibrosarcoma,...)
Embryonal Malignancies:Wilms,
Rhabdomyosarcoma
Leukemia (CML)
Melanoma, Medullary Thyroid Ca.
Low Incidence of Lung Cancer
Neurofibromatosis Deep Bright Objects DBOs

Neurofibromatosis Type 2 or Wishart Disease
Chromosome 22
Bilateral Acoustic Schwannoma
"Central Neurofibromatosis"
Minimal Skin Manifestations
Neurofibromatosis Type 2
Incidence: 1/50,000
Inheritance: Autosomal Dominant
Age at Presentation: Birth to 40s (peak in 20s)
Sx at Presentation: Hearing loss from VS
Diagnostic Criteria: VIII masses
Cutaneous Findings: minimal (skin tags)
CNS Findings: Schwannoma, Meningioma, Ependymoma (intramedullary
spinal cord)
Neuroradiology
1189
1191
The Phakomatoses
CNS Neoplasms Chromosome Loss of

Heterozygosity
Figure 5-15-21
NF-2
SCHWANNOMA 22q
MENINGIOMA 22q (long arm)
EPENDYMOMA 22
NOT Neurofibroma
NOT Astrocytoma
NOT Optic Glioma
NF-2 (Central), 1 or More
Intracanalicular Schwannoma they all begin

Bilateral VIIIth Masses
inside the IAC because that is where the
Relative with NF-2 and either:
Schwann cells are. The cisternal segment of the
Unilateral VIIIth Mass
nerve has oligodendrocytes
Any Two:
Neurofibroma, Meningioma, Glioma, Schwannoma, (Congenital)
Lens Opacity
Figure 5-15-22
Neurofibromatosis Type 2
NEJM 319:278-83, 1988 (Gulf of Mexico)

23 Pts. (15M/8F), Kindred of 137
0.95 Penetrance
18 Acoustic Schwannoma (17 bil.)
8 Meningioma (3 mult.)
4 Ependymoma
2 Spinal Neurofibroma
Schwannoma [Figures 5-15-21 and 5-15-22]
5%-10% of All CNS Tumors

Benign, Slowly growing
F > M (Intracranial), M > F (Spinal)
30s 60s, w/NF-2 10s 30s
Sensory Nerves (usually):
CNN VIII (Sup.Vestibular), V, X
Spine: Dorsal Roots
Majority (>90%) are Sporadic
Multiple in NF-2, Bilat.VIII Pathognomonic
Neurofibromatosis 2 [Figure 5-15-23]
Bilateral Vestibular Schwannoma
Meningiomas:
multiple transitional type meningioma
NOT meningothelial
Meningioangiomatosis:
cortical (intracortical) vascular tissue
resembles a vascular malformation
meningothelial and fibroblast -like cells
Figure 5-15-23
Multiple Schwannomas and Meningiomas and

Ependymomas [Figure 5-15-24]
Neurofibromatosis 2 [Figure 5-15-24]
Multiple Meningiomas (up to 45%)

Intraventricular Meningiomas
Childhood Meningiomas
Multiple Meningiomas
1%-10% of all patients with meningioma
SPORADIC in 80%-90%
Intraventricular Meningiomas
SPORADIC in 90%
Childhood Meningiomas
SPORADIC vs. Inherited (NF-2 or Not)
The Phakomatoses
Multiple Schwannomas and

Meningiomas
1192
Neuroradiology
Neurofibromatosis Type - 2=> MISME
Figure 5-15-24
M ultiple
I nherited
S chwannomas
M eningiomas
E pendymomas
Sturge-Weber Disease or EncephaloTrigeminal Angiomatosis
Inheritence ??
Autosomal Dominant ?
Autosomal Recessive ?
Sex-Linked ?
Multiple Schwannomas, Meningiomas, and

Ependymomas the MISME syndrome
STURGE-WEBER: Definition
A telangiectatic venous angioma of the

leptomeninges, face, and choroid of the eye.
Figure 5-15-25
STURGE-WEBER SYNDROME: History
1879 STURGE, Clinical description

1897 Kalischer, Vascular nature
1922 Weber, published radiography
1923 Dimitri, tram-track Ca++
1934 Krabbe, Ca++ in cortex
1937 van der Hoeve, Phakomatosis
STURGE-WEBER SYNDROME: Classic Triad
Facial Neveus Flammeus

Port-Wine Stain
Seizures
Mental Deficiency
STURGE-WEBER: Manifestations
Seizures, Mental Decline

Usually begins in first 24 months
Facial Angioma
At birth
Angiomatous Overgrowth of soft-tissue and bone
Leptomeningeal Angioma
Cortical Atrophy w/Ca++
Trigeminal Angiomatosis in SWS
Figure 5-15-26
STURGE-WEBER: Variants
Facial and Intracranial w/o Eye

Intracranial and Eye w/o Face
Intracranial Alone
(Cerebral and Leptomeningeal)
Klippel-Trenaunay (KT Weber)
Extracranial soft-tissue angiomas)
STURGE-WEBER SYNDROME:
Port Wine Stain (PWS) [Figures 5-15-25 and 5-15-26]
Facial Neveus Flammeus

Blanches w/ pressure
Trigeminal Dermatome
V1 Ophthalmic
V2 Maxillary
V3 Mandibular
Sturge-Weber: Facial overgrowth and

extensive, bilateral, port-wine stain.
Neuroradiology
1191
1193
The Phakomatoses
Association of PWS with SWS
Figure 5-15-27
All 3 >> 1+2 >> 1 or 2 alone >> other

medial aspect of eyelid (V1 or V2)
Medullary Veins [Figures 5-15-27 and 5-15-28]

STURGE-WEBER: Vascular
Absence of cortical veins

Poor filling of sagittal sinus
Persistent Primitive Plexus (SAS)
Recruitment of Medullary Veins
Prominent Choroid Plexus
Persistence of Primitive Plexus
Medullary Veins are prominent, bridging veins are

absent over the occipital and posterior parietal
lobes
Persistence of primitive vascular plexus

Absence of cortical veins
Deoxygenated blood
Figure 5-15-28
Cranial Vascular Development
Begins with primitive plexus

Progressive Differentiation
Arteries
Capillaries
Veins
Progressive Lamination
Cerebral (brain) circulation
Dura and Bone circulation
Scalp Circulation
Connect via bridging

veins
Sequential Induction: Eye Development
Optic nerve induces lens development

Lens induces cornea development from surface ectoderm and
adjacent mesenchyme
STURGE-WEBER: Etiology
Abnormal Development of Capillaries

Poor cortical venous drainage
Absent cortical veins
Prominent veins in SAS
Prominent deep (medullary) veins
Enlarged choroidal vessels
Persistence of Primitive Plexus
T1W MR with contrast. Prominent

medullary veins in SWS. Also note
the widened diploic space of the
frontal bone and gyriform surface
enhancement posteriorly
Figure 5-15-29
STURGE-WEBER: Orbit/Eye [Figure 5-15-29]
Buphthalmos (Ox Eye)

congenital glaucoma
enlarged globe
Choroidal Angioma
Episcleral Telangiectasia
Angiomatous Overgrowth of EOMs
STURGE-WEBER: Manifestations
Seizures, Mental Decline

Facial Angioma
Angiomatous Overgrowth
Leptomeningeal Angioma
Cortical Atrophy w/Ca++
The Phakomatoses
Buphthalmos or Ox eye. Congenital

glaucoma, caused by a choroidal
angioma, has led to enlargement
of the ocular globe
1192
1194
Neuroradiology
Vessels in SAS [Figure 5-15-30]
Figure 5-15-30
Normal vs. Venous Outflow Obst. [Figure 5-15-31]

Figure 5-15-31
Multiple small vessels in SAS in SWS
Figure 5-15-32
Impaired venous drainage leads to chronic

cerebral ischemia
STURGE-WEBER: Calcification [Figure 5-15-32]
Abnormal (sluggish) circulation

Chronic Cerebral Ischemia
Progressive Cell Loss (Atrophy)
Progressive Cerebral calcification
early subcortical WM (?)
Later middle layers of cortex
DYKE, DAVIDOFF, MASSON
Cerebral Hemiatrophy with Homolateral

Hypertrophy of the Skull and Sinuses
Surgery, Gynecology, & Obstetrics 1933 pp. 589-600 Sturge-Weber Disease. Cerebral hemiatrophy and
calcification
Sturge-Weber- Hemiatrophy [Figure 5-15-33]

Figure 5-15-33
Noncontrast and postcontrast axial CT (Images 1,

2, 3) sections show prominent subarachnoid
spaces overlying atrophic left frontal lobe.
Cortical calcification and hypodense white
matter of the ipsilateral forceps minor are well
shown also
Neuroradiology
1193
1195
The Phakomatoses
Progression in SWS [Figure 5-15-34]
Figure 5-15-34
Dyke, Davidoff, Masson [Figure 5-15-35]

Figure 5-15-35
Dyke, Davidoff, Masson changes from cerebral

hemiatrophy in SWS
STURGE-WEBER [Figures 5-15-36
to 5-15-38]
Progression in SWS
same patient two years apart
Gyral Gadolinium Enhancement

Abnormal BBB in Cortex
(Chronic ischemia)
Epi-Cortical enhancement
(slow flow in superficial veins)
Figure 5-15-36
STURGE-WEBER: Treatment
Symptomatic (anticonvulsants)
Cosmetic Tattooing
Laser Treatment of Skin Lesions
Hemispherectomy
Aspirin (mild antiplatelet)?
Figure 5-15-37
Sturge-Weber Disease: Gyral Enhancement and

Choroid Plexus enlargement
Figure 5-15-38
Sturge-Weber Disease. Gyral hypointensity from

dense calcifications. Angiomatous overgrowth
of the temporalis muscle (arrow)
The Phakomatoses
Sturge-Weber Disease. T2W MR and CT in the

same patient show changes from gyral
calcification
1194
1196
Neuroradiology
Half a Brain
Uncontrolled Sz
Under age 2
Up to age 5-7
Plasticity
Uncrossed tracts
5%15%
Tuberous Sclerosis or Bourneville Disease
Chromosomes 9 and 16
Tuberous Sclerosis
Original VOGT TRIAD

FACIAL NEVUS (ADENOMA SEBACEUM)
SEIZURES
MENTAL DEFICIENCY
Tuberous Sclerosis
AUTOSOMAL DOMINANT
No Racial/Sexual
High Spontaneous Mutation
High Penetrance
SPORADIC over-reported
Multiple Genes
TSC1 9q
TSC2 16p
Tuberous Sclerosis, NIH Consensus Conference
Major Features:
Facial angiofibroma or forehead

plaque
Ungual or Periungual fibroma
>3 Hypomelanotic macules
Shagreen patch
Multiple retinal nodular
hamartomas
Cortical Tuber
Subependymal Nodule
Astrocytoma
Cardiac rhabdomyoma
Lymphangiomyomatosis
Renal angiomyolipoma
Minor Features:
Multiple dental enamal pits
Hamartomatous rectal polyps
Bone cysts
White matter migration lines
Gingival fibromas
Non-renal hamartoma
Retinal achromic patch
"Confetti" skin lesions
Multiple renal cysts
Definite TS - (2 Major) or (1 Major + 2 Minor)

Probable TS - 1 Major + 1 Minor
Possible TS - (1 Major) or (2 Minor)
Hyman MH, Whittemore VH:"National Institutes of Health Consensus
Conference:tuberous sclerosis Complex" Arch Neurol 2000; 57: 662-665
Tuberous Sclerosis
Definitive (need 1)
(1) facial angiofibroma
(2) ungual fibroma
(3) retinal hamartoma
(4) cortical tubers
(5) subependymal nodules
(6) multiple renal AML
Neuroradiology
1195
1197
The Phakomatoses
Presumptive (need 2)
(1) hypomelanotic nodules
(2) shagreen patch
(3) single renal AML
(4) multicystic kidney
(5) cardiac rhabdomyoma
(6) pulmonary lymphangiomyomatosis
(7) radiographic honeycomb lung
(8) first degree relative with TS
Tuberous Sclerosis:
Seizures 90%
Adenoma Sebaceum 60%-90%
Retardation 40%-60%
Retinal Phakoma 50%
Xr: Intracranial Ca++ 50%
Ungual Fibromata 17%
Giant Cell Astrocytoma 15%
INCIDENCE Of Tuberous Sclerosis:
CLASSIC TRIAD
VARIABLE Incidence
1 In 10K- 500K
1 In 150K In HONG KONG
MAYO Clinic Criteria
1 IN 10,000 AT MAYO CLINIC
Local Population Olmsted Cty
FORME FRUSTE
Five Times More Common Than Classic
Tuberous Sclerosis
Hamartomas
CNS (Cortical Ventricular)
Retina (Phakoma)
Kidney (Angio Myo Lipoma AML)
Angiofibromas
Face (Adenoma Sebaceum)
Nail Bed (Fibromas)
Tuberous Sclerosis:
Rhabdomyomas Heart
Hamartomas
Angiomyomatosis Lung
smooth muscle proliferation
Tuberous Sclerosis: Cutaneous
Adenoma Sebaceum
Peau Dorange
Ash-Leaf Macule
Ungual Angiofibromas
Adenoma Sebaceum
AKA PRINGLES DISEASE

NOT present at birth
develop before puberty
nasolabial fold ->bi-malar
papules of angiofibroma
The Phakomatoses
1196
1198
Neuroradiology
Pringles Disease [Figure 5-15-39]
Figure 5-15-39
Pringles Name
Entire Disease
Facial lesion only
Mild Mental Retardation
Seizures
Hard Potatoes
Tubular Can Tuberous
Subungual/Periungual Fibroma [Figure 5-15-40]

Figure 5-15-40
Pringle Disease: A papular (raised)

reddish lesion, often in a bimalar
pattern, caused by
angiofibromata of the skin in
Tuberous Sclerosis
Subungual (right) and Periungual (left) Fibroma in

Tuberous Sclerosis. These are
angiofibromata, similar to the Pringle facial
lesion
Depigmentation:
Ash-Leaf Spots
(Lance- Ovate Shape)
Confetti Like Hypopigmentation
(Inverse Freckle)
Figure 5-15-41
Other Cutaneous Manifestations
Subepidermal Fibrosis:
Dorsal Surfaces
Shagreen Patch
Peau Dorange
Pigskin
Elephant Hide
Tuberous Sclerosis: Ocular

[Figure 5-15-41]
PHAKOMA
benign astrocytic hamartoma
LEUKOKORIA
White light reflex
Calcification Common
Especially over Optic Nerve
Tuberous Sclerosis. Astrocytic hamartoma of the

retina the original phakoma of van der
Hoeve
Tuberous Sclerosis BRAIN:
HETEROTOPIAS AND HAMARTOMAS

in white and gray matter
CORTICAL TUBERS
HAMARTOMAS
but with abnormal N cells
neither Astrocyte nor Neuron
Decreased Myelination
No laminar architecture
Neuroradiology
1197
1199
The Phakomatoses
Figure 5-15-42
SUBEPENDYMAL NODULES (almost 100%)

hamartomas vs. neoplasia
Caudothalamic groove
Polypoid Candle Gutterings
DILATED VENTRICLES
variable
obstructive, atrophic vs. idiopathic
TUMORS 15%
Sub-ependymal Giant Cell Astrocytoma
True neoplasm, Benign WHO Grade I
Cortical Tubers
[Figure 5-15-42]
Subependymal Nodules
[Figures 5-15-43 and 5-15-44]
Figure 5-15-43
Cortical Tubers in Tuberous Sclerosis
Figure 5-15-44
Neonatal sonogram. Subependymal Nodules in

Tuberous Sclerosis
Tuberous Sclerosis [Figures 5-15-45 to 5-15-47]
Renal
Angiomyolipoma
Multiple Simple Cysts
Another cause of APCKD
RCC Reported
Figure 5-15-45
Subependymal nodules may enhance without

neoplastic transformation
Tuberous Sclerosis is a
disorder of neuronal
migration and maturation
The Phakomatoses
1198
1200
Neuroradiology
Figure 5-15-46
Figure 5-15-47
Tuberous Sclerosis. Cortical tubers

and white matter hyperintensities
from the abnormal migration and
maturation of the brain
Subependymal Giant Cell Astrocytoma in

Tuberous Sclerosis. This is a low grade
WHO 2 neoplasm
Figure 5-15-48
Angiomyolipoma
[Figure 5-15-48]
10% w/enough FAT for plain film

1/6 OF Solitary AML Pts. Have TS
1/3-1/2 of solitary AML Pts. Have other stigmata of TS
50-80% of Pts. W/TS will have AML
3/4 MULTIPLE
1/3 1/2 BILATERAL (probably more)
variable amts. of FAT, Smooth mm., and vessels
Renal Cysts
[Figure 5-15-49]
Figure 5-15-49
Multiple renal angiomyolipomas in
Tuberous Sclerosis
Tuberous Sclerosis. In addition to angiomyolipoma, the patients may

also develop multiple and bilateral renal cysts
[different patients]
Neuroradiology
1199
1201
The Phakomatoses
Angiomyomatosis vs. Lymphangiomyomatosis
Figure 5-15-50
[Figure 5-15-50]
sporadic cases, all are female

50% chylothorax
Perilymphatic smooth mm.
May have abdominal LN involvement
In TS, males can be affected
chylothorax is rare
Periarterial smooth mm around pulmonary aa
Bone Islands [Figure 5-15-51]

Hemangioblastomatosis or Von Hippel-Lindau
Disease
Chromosome 3
Pulmonary
Lymphangioleiomyomatosis in
Tuberous Sclerosis
von Hippel-Lindau
Incidence of 1/35K 40K

6-7K pts in USA
AUTOSOMAL DOMINANT
NO RACIAL/SEXUAL PREDILECTION
VARIABLE PENETRANCE / EXPRESSIVITY
Chromosome 3p25-26
von Hippel-Lindau Syndrome: History
1864 scattered reports of angiomatous lesions of both retina and cerebellum

1894 Collins (England)
two sibs with retinal angioma
1904 von Hippel (Germany)
familial retinal hemangioblastoma
1926 Lindau (Sweden)
familial retinal and cerebellar hemangioblastomas
1964 Melmon and Rosen
von Hippel-Lindau
1.
2.
3.
CNS and Retinal hemangioblastoma

Hemangioblastoma and one:
a. renal, pancreatic, hepatic, epididymal cyst
b. pheochromocytoma
c. renal cancer
Family history and one:
a. hemangioblastoma
b. viscera
c. pheochromocytoma
d. renal cancer
Figure 5-15-51
von Hippel-Lindau
SYNDROME: NIH
Classification
Type I VHL w/o Pheo

Renal/Pancreatic cysts, RCC
most common type
Type II VHL with Pheo
IIA Islet cell tumors (no cysts)
IIB Renal/Pancreatic Disease
least common type
Tuberous Sclerosis Multiple bone islands. These could also be

considered as hamartomas.
The Phakomatoses
1200
1202
Neuroradiology
von Hippel-Lindau
Hemangioblastoma
Cerebellum
Retina
Medulla, Cord
Cysts/Tumor
Kidney
Liver
Pancreas
Epididymis and Endolymphatic Cystadenoma
Pheochromocytoma -Adrenal (Certain Families -Type II)
von Hippel-Lindau: Six Classic Lesions [Figure 5-15-52]
Hemangioblastoma
Retinal Angioma (Hemangioblastoma)
Pancreatic Cyst
Renal Cysts and Ca
Pheochromocytoma
Epididymal Cystadenoma
Endolymphatic sac tumor
Figure 5-15-52
von Hippel-Lindau Syndrome. Retinal

angioma demonstrated on
flourescein angiogram
Figure 5-15-53
Hemangioblastoma:
True Neoplasm Endothelial Origin

Hypervascular
capillary to sinusoidal
dilated feeding artery
dilated draining vein
slow flow
Stromal Cells
foamy, lipid -laden
Hemangioblastoma the classic
cyst with nodule morphology
von Hippel-Lindau: Hemangioblastoma
Cerebellum 66%
Retina (angiomas) 58%
Spinal Cord / Roots 28%
Medulla 14%
Figure 5-15-54
Hemangioblastoma [Figures 5-15-53 to 5-15-60]
Figure 5-15-55
Hemangioblastoma with capillaries and stromal

cells
Hemangioblastoma Sporadic on left, VHL on

right (multiple lesions).
Neuroradiology
1203
The Phakomatoses
Figure 5-15-56
Figure 5-15-57
Hemangioblastomas span a spectrum from largely

cystic to mostly solid
Hemangioblastoma densely enhancing nodule

persists into venous phase
Figure 5-15-58
Figure 5-15-59
Hemangioblastoma in the medulla

oblongata
Hemangioblastoma densely enhancing nodule

persists into venous phase
Figure 5-15-60
Hemangioblastoma AND VHL
1/6-1/5 of solitary cerebellar hemangioblastomas are

associated w/ VHL
up to 1/2 of medullary HBL occur in VHL
ALL Multiple HBL are VHL
there was one family w/o VHL
Erythropoietin
in cyst fluid
Elevated ESR
Elevated Hct
von Hippel-Lindau. Syringohydromyelia with

multiple enhancing nodules of
hemangioblastoma
The Phakomatoses
1202
1204
Neuroradiology
von Hippel-Lindau: Renal Manifestations

CYSTS
ANGIOMAS
ADENOMAS
CLEAR CELL CA
Figure 5-15-61
25%63%
7%
14%
15%50%
von Hippel-Lindau: Kidney

[Figure 5-15-61]

Multiple
Bilateral
Conservative Surgery
von Hippel-Lindau. Multicystic renal cell carcinoma
von Hippel-Lindau: Pancreas [Figure 5-15-62]
Pancreatic cysts 18%-72%

Pancreatic adenoma 7%
microcystic (glycogen rich)
Pancreatic Ca
reported in single family
Islet Cell Tumors
Figure 5-15-62
Pancreatic Adenoma In VHL
Microcystic (Not Macrocystic)

Serous (Not Mucin Producing)
Not Pre-Malignant
Glycogen Rich
Stellate Scar
which may be visible, have Ca++
VHL Visceral Manifestations
von Hippel-Lindau. Pancreatic Microcystic Adenoma

also called glycogen-rich adenoma - Note
central stellate scar on gross image.
Pheochromocytoma and VHL
20% of ALL Pheochromocytoma are VHL

Typically in Adrenal
Present YOUNGER w/VHL
Multiple with VHL
Mortality (5% of VHL DIE from catecholamines)
Workup: MR and MIBG (95% sensitive)
24hr NOREPINEPHRINE
VMA (53% sensitive)
US (40% sensitive)
Figure 5-15-63
Endolymphatic Sac Tumor [Figure 5-15-63]
Petrous Apex Mass

Cholesterol granuloma
Glomus tumor
Vascular variant
Cystadenoma (endolymphatic sac tumor)
Von Hippel-Lindau
Hemangioblastoma
Cerebellum
Retina
Medulla, Cord
Cysts/Neoplasms
Kidney
Liver
Pancreas
Epididymis
Endolymphatic sac
Pheochromocytoma -Adrenal
Neuroradiology
Endolymphatic Sac Tumor

1203
1205
The Phakomatoses
VHL - Multiple hemangioblastomas [Figure 5-15-64]
Figure 5-15-64
VHL - Multiple hemangioblastomas
Educational Objectives
Describe why NF-1 is truly Neurofibromatosis

Describe three neoplasms caused by the chromosome 22 mutation in NF-2
Describe the vascular abnormalities of Sturge Weber Syndrome
Explain why Tuberous Sclerosis is a disorder of neuronal migration
Distinguish von Hippel-Lindau from the neurocutaneous phakomatoses
Summary - Phakomatoses Mnemonic Tool
NF-1 (von Recks)

TRUE Neurofibromatosis #17
NF-2 (Bil. VIII Syndrome)
M.I.S.M.E. #22
STURGE-WEBER (Dimitri) Syndrome
Congenital Vascular Lesion
perhaps NOT inherited
Tuberous Sclerosis
Pringles HAMARTOMA Disease
von Hippel-Lindau Syndrome
NO cutaneous lesions
Hemangioblastomas and Visceral Lesions
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2.
3.
4.
5.
6.
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Choyke PL, Glenn GM, McClellan WM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau Disease:
Genetic, Clinical, and Imaging Features. Radiology 1995; 194:629-642.
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Feghali JG, Levin RJ, Llena J, Bradley MK, Kantrowitz AB. Aggressive Papillary Tumors of the Endolymphatic
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The Phakomatoses
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Neuroradiology
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1209
The Phakomatoses
Subarachnoid Hemorrhage and

Intracranial Aneurysms
Mary E. Jensen, MD
Epidemiology
Most common cause of non-traumatic SAH is aneurysmal rupture

Annual incidence of aneurysmal SAH: 1:10,000
Higher than primary brain tumors, MS
Remained stable over last 30 yrs
Females 1.6 x over males
Blacks 2.1 x over whites
Higher in Japan and Finland
Accounts for 2-5% of all new strokes each yr
21,000 33,000 Americans
Average age much lower than other strokes
Peaks in 6th decade
Epidemiology - Prevalence of Unruptured Aneurysms
Common incidental finding

3.6% on prospective autopsy series
6% on prospective angiography series
Highest in patients with AD polycystic kidney disease, familial
predisposition, atherosclerosis
Multiplicity
20%-30% of patients have multiple aneurysms
Usually 2 or 3
Figure 5-16-1
Types of Aneurysms
[Figures 5-16-1 and 5-16-2]
Saccular (berry)
90% of all aneurysms
Fusiform
Serpentine: Partially thrombosed
aneurysm containing tortuous
vascular channels
Cirsoid: Dilated, elongated and
tortuous
Dissecting
Blister/bleb
Saccular, paraophthalmic (left) Fusiform, vertebral (center)

Serpentine, MCA (right)
Figure 5-16-2
Non-Modifiable Risk Factors
Personal history of SAH

Risk of developing a new aneurysm: 2%
Annual incidence of SAH: 6:10,000
Family history of SAH
5%-20% of patients with SAH have a positive
family history
First-degree relatives of pts with SAH have 37X increased risk
Second-degree relatives have same risk as
general population
Probably an autosomal transmission that does
not follow a specific mendelian model
Female gender
Dissecting, PICA (left) Blister-bleb, AComA (right)
Before the 5th decade, the risk is higher in men
Risk is greater in post-menopausal women than men
Supplemental low-estrogen hormones may impart some protection
Subarachnoid Hemorrhage-Intracranial Aneurysms
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Neuroradiology
Non-Modifiable Risk Factors
Age
Very rare in children
Heritable disorders of connective tissue
AD polycystic kidney disease
Ehlers-Danlos disease IV
Neurofibromatosis type 1
? Fibromuscular dysplasia, Marfans
syndrome
Associated with anatomic variants
Persistent trigeminal artery
Fenestrations
Azygous ACA
Aneurysms in Children
Figure 5-16-3
Mid-basilar aneurysm with severe distal stricture (arrows)
Figure 5-16-4
Incidental aneurysms rarely found at autopsy
Etiology: trauma, infection, congenital
Location, size, age and presentation are
different from adults
Age of presentation bimodal
0-6 yrs, then 8-adolescence
Posterior circulation aneurysms 3x more
prevalent
Large/giant aneurysms more common
greater proportion of giant
aneurysms, pts < 2 yrs
47 y.o. female with Marfans and rapidly growing mid-basilar
Fusiform Basilar Aneurysm in a
aneurysm
Child [Figure 5-16-3]
Aneurysm Associated with Connective Tissue Disorder

[Figure 5-16-4]
Aneurysm Associated with Anatomical Variants
[Figure 5-16-5]
Modifiable Risk Factors
Smoking
Only risk factor that has been consistently identified in all populations
studied
Risk is 3-10X higher than non-smokers
Risk is proportional to number of cigs
Increased risk of new aneurysm formation in pts with SAH who continue to
smoke
Hypertension
Probably a risk factor for both SAH and aneurysm formation
Alcohol consumption
Heavy, binge drinking
Cocaine use
? Hypercholesterolemia, DM, obesity
Figure 5-16-5
Bilobed vertebrobasilar junction aneurysm

associated with fenestration (left).
Large aneurysm associated with an azygous ACA
(right)
Neuroradiology
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Pathogenesis: Saccular Aneurysms
Congenital defect in tunica media no longer considered the cause of aneurysm

formation
Gaps in muscles layers are also seen in unaffected individuals
In aneurysms, the gap is not at the neck but in the sac
Acquired changes due to environmental factors (HTN, smoking, EtOH abuse)
are more likely
Formation of intimal thickening proximal and distal to branch points
May cause increased strain in the more elastic portion of the wall
Structural abnormalities in structure proteins of the extracellular matrix
Pathogenesis: Saccular Aneurysms
Figure 5-16-6
Hemodynamic factors
Wall shear stress
Frictional force of viscous blood
High WSS fragments the internal elastic lamina
- Initiation of aneurysm formation
Low WSS degenerates endothelial cells via apoptosis
- Responsible for aneurysm growth/rupture
Increased flow
10%-20% of patients with brain AVMs have aneurysms
Pathogenesis: Fusiform Aneurysms
Fusiform
Four major histological findings
Fragmentation of internal elastic lamina
Double channel appearance of
Neoangiogenesis within the thickened intima
dissecting aneurysm aneurysm
Intramural hemorrhage and thrombus formation
Repetitive intramural hemorrhage from neovascularity within the
Figure 5-16-7
thrombus
Similar histologic features seen with ASVD
ASVD starts with lipid deposition, not fragmentation of
the IEL
Pathogenesis: Dissecting Aneurysms
Cystic medial necrosis

Widespread disruption of the arterial wall
Medial disruption with subadventitial
dissecting hemorrhage causing true lumen
stenosis
Formation of a dilated pseudoaneurysm
covered only by thin adventitia
AP and lateral views show pearl and string

appearance of dissecting aneurysm
CTA and MRA of Dissecting Vertebral Aneurysm [Figure 5-16-6]

DSA of Dissecting Vertebral Artery Aneurysm [Figure 5-16-7]
Subarachnoid hemorrhage
Unique headache
Nausea/vomiting
Meningeal irritation
Photophobia
Focal or global neurological deficits
Subhyaloid hemorrhages
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Neuroradiology
Subarachnoid Hemorrhage Patterns [Figure 5-16-8]
Figure 5-16-8
Mass effect
Headache
Focal neurological findings
Seizures
Risk of rupture of unruptured aneurysm causing
mass effect is 6%/yr
Cerebral ischemia
Distal embolization from intra-aneurysmal
thrombus
Giant ICA Terminus Aneurysm [Figure 5-16-9]

Outcomes of SAH
12% die before reaching hospital

40% of hospitalized patients die within first month
>1/3 of survivors have major neurological deficits
Unable to live independently
Many good outcomes have persistent cognitive
deficits
AComA pattern (left images) - Basilar tip pattern

(right images)
Outcomes of SAH - Major factors associated with poor outcome
Patients level of consciousness on

admission
Based on the sum score of the
Glasgow Coma Scale
Eye opening (4 pts), best motor
response (6 pts), best verbal
response (5 pts)
Age
Amount of blood on CT
Predicts risk of delayed cerebral
ischemia using two parameters
Amount of subarachnoid clot
Ventricular hemorrhage
Risk is additive
Figure 5-16-9
59 y.o. female evaluated for headache and cognitive

dysfunction
Clinical Grading Scale [Figure 5-16-10]

Radiologic Grading Scale [Figure 5-16-11]
Figure 5-16-10
Neuroradiology
Figure 5-16-11
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SAH-induced Vasospasm
Figure 5-16-12
Occurs angiographically in 30%-70% of patients

Clinical symptoms seen in 20%-45% of patients
Adds 10%-20% significant morbidity/mortality
Smooth muscle constriction and vessel wall edema,
infiltration and fibrosis leads to luminal narrowing
and decreased compliance
Time course
Range: 4-14 days
Peak: 7-10 days
Treatment
Fluid status, blood pressure, calcium channel
Severe vasospasm of the supraclinoid ICA and M1
blockers
segment leading to poor perfusion of the right MCA
Induced hypertension
territory
Angioplasty, intra-arterial infusion of vasodilators
Figure 5-16-13
SAH-induced Vasospasm [Figure 5-16-12]

Risk of Unruptured Aneurysm [Figure 5-16-13]
Diagnostic Testing: Non-contrast Head CT
First imaging study

Detect 98%-100% of cases in first 12 hours
Sensitivity goes down to 30% at 2 wks
Reportable findings
Presence, amount, location of blood
Increased density in subarachnoid space
Predominately in basilar cisterns
Intraparenchymal hematoma
Hydrocephalus
Cerebral edema, herniation
Negative image of aneurysm in
SAH
Figure 5-16-14
CT of Subarachnoid Hemorrhage
[Figure 5-16-14]
CT Findings of Ruptured
Aneurysm [Figure 5-16-15]
Diagnostic Testing:
Lumbar puncture
Usually done when CT is negative or

equivocal
Should be done 6-12 hours after event
Time it takes for xanthochromia to
occur
Xanthochromia is diagnostic
Detectable in all patients
between 12 hrs and 2 wks
Findings
Elevated opening pressure
Elevated RBCs that do not clear
Unreliable way to r/o traumatic
tap
Xanthochromia
Acute SAH (left image)-SAH 5 days post bleed with CTA

showing PComA aneurysm (right two images)
Figure 5-16-15
NCCT shows extensive SAH, intraparenchymal hematoma with

fluid-fluid level, uncal and subfalcine herniation, small amount
of intraventricular blood, and an MCA aneurysm (arrow)
1212
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Neuroradiology
Diagnostic Testing: MRI
FLAIR detects early SAH as well as CT

Impractical
Equipment availability
Patient motion
Better than CT for detecting older SAH
Great for patients with negative CT, positive
LP who are not referred immediately
Figure 5-16-16
SAH on CT and MRI FLAIR [Figure 5-16-16]

Diagnostic Testing: CTA
Advantages
High sensitivity (97%-100%)
Equal to IA DSA
Good even if SAH present
Source images, reconstructions
Disadvantages
Sensitivity varies with size
Use of iodinated contrast and
radiation
Negative study requires IA DSA
Technical issues
Post-processing time is
substantial
Venous contamination with poor
cardiac output
CTA with Active Extravasation

[Figure 5-16-17]
CTA of Small Aneurysms
CT three days and MR four days post-SAH

(arrows) with interval development of
intraventricular hemorrhage seen on MR
Figure 5-16-17
CTA shows the location, size and relationship of the aneurysm

to the surrounding branches, and active extravasation into the
hematoma
Figure 5-16-18
[Figure 5-16-18]
Diagnostic Testing: MRA
Advantages
Non-invasive
High sensitivity
86%-100% in aneurysms 3-5 mm
81%-100% in cases of SAH
Multiplanar viewing
Disadvantages
Complex/disturbed flow degrades image
Availability of equipment
Patient movement
Anesthesia needed for uncooperative patients
MRA of Multiple Aneurysms [Figure 5-16-19]
CTA of Small Aneurysms
Figure 5-16-19
Diagnostic Testing: DSA
Gold standard
Carries risk of complication
1.8% transient or permanent
SAH patients hypercoagulable
Detects smallest aneurysms
Gives important information for treatment decisions
Following coiling of the three aneurysms larger
than 2 mm, the MRA shows no change in the two
untreated aneurysms
Neuroradiology
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DSA: Evaluation of Aneurysm
Figure 5-16-20
Location and number

Size and configuration
Characteristics of aneurysm neck
neck to dome ratio; absolute size of neck
slit, oval, round, wide
relationship to adjacent vessels
Suspected rupture site (tits)
Collateral circulation distal to aneurysm
Hemodynamics
Calcification
Intraluminal thrombus
Location
Anterior circulation (80%-85%)

ICA/PComA junction
AComA complex
MCA trifurcation
Posterior circulation (15%-20%)
Basilar terminus (5%)
PICA
SCA
Aneurysm Locations: Anterior Circulation
Common locations for aneurysms on the anterior

circulation
[Figure 5-16-20]
Aneurysm Locations: Posterior Circulation
Figure 5-16-21
[Figure 5-16-21]
Aneurysm Measurements
Aneurysm
Small: less than 10 mm
Large: 10-25 mm
Giant: > 25 mm
Aneurysm neck
Absolute size
Small: 4mm or less
Dome to neck ratio
Small: 2:1 or greater
Common locations for aneurysms on the posterior circulation
Aneurysm Sizes [Figure 5-16-22]

Figure 5-16-22
Small superior hypophyseal (left)

Large paraophthalmic (center)
Giant ICA terminus (right)
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Neuroradiology
3D Angiography [Figure 5-16-23]
Figure 5-16-23
Rupture Sites [Figure 5-16-24]

Imaging Algorithm for Suspected
SAH
Non-contrast head CT
SAH
CTA or DSA
If negative, repeat CTA in 1-3 weeks
If negative, image the brain and spinal cord
No SAH
LP
Abnormal or equivocal, go to SAH algorithm
Imaging Algorithm for Screening
3D angiography better defines the aneurysm neck than biplane

DSA
Figure 5-16-24
For high risk patients

Family history of intracranial
aneurysms
Asymptomatic individuals with
two or more affected members
Non-invasive imaging every >1
year, < 5 years
Asymptomatic adults with
autosomal dominant polycystic
kidney disease
5%-10% asymptomatic patients
have aneurysms
Clustering in some families with
20%-25% with aneurysms
Paraophthalmic (left)
AComA (center)
Basilar tip (right)
Other Causes of SAH
Non-aneurysmal perimesencephalic
SAH
Infectious intracranial aneurysms
Brain AVMs
Flow related aneurysms
Intra/perinidal aneurysms
Dural AVMs
Intracranial dissections
Spontaneous
Iatrogenic
Spinal vascular malformations
Non-aneurysmal Perimesencephalic Hemorrhage
Responsible for 10% of non-traumatic SAH

Defined only by the characteristic location of blood and lack of aneurysm
Blood confined to perimesencephalic cistern
Centered anteriorly
May have small amount of sedimentation in posterior horns
2.5%-10% of posterior fossa aneurysm hemorrhages mimic this pattern
Clinical findings
Gradual headache
Focal findings, LOC uncommon and transient
Seizure at presentation essentially excludes the diagnosis
1/3 with transient amnesia
Outcomes
Short convalescence
No rebleeding
No symptomatic vasospasm
Neuroradiology
1215
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Non-aneurysmal Perimesencephalic Hemorrhage [Figure 5-16-25]
Figure 5-16-25
Infectious Intracranial Aneurysms
Rare- 2%-6% of all brain aneurysms

Presentation: stroke,seizure, SAH
Pathology
Septic embolization of lumen or vasovasorum
Focal arteritis, necrosis, and aneurysm
formation
Location
Distal MCA>PCA and ACA
Treatment
Resection, parent artery occlusion
Antiobiotic therapy
Infectious Intracranial Aneurysms [Figure 5-16-26]

Other Causes of SAH
Non-aneurysmal perimesencephalic hemorrhage
Figure 5-16-26
Saccular aneurysm of spinal artery

Atrial myxoma
Pituitary apoplexy
Coagulation disorders
Superficial siderosis of the CNS
Aneurysms: Treatment Options
Nothing
Follow
Very small unruptured aneurysms
Unruptured aneurysms in cavernous
carotid/carotid cave
Surgical clipping
Endovascular treatment
Reconstructive: coiling +/- stent
placement/balloon remodeling
Saccular aneurysms
Deconstructive: parent artery
occlusion
Giant, dissecting, septic
Separate patients with septic aneurysms

(Courtesy A. Brooks, M.D.)
Figure 5-16-27
Treatment Outcomes
ISAT trial
Permanent occlusion of the left ICA with detachable balloons
Comparative study of coiling vs.
results in flow perpendicular to the neck of the aneurysm and
clipping
clotting of the dome
23.9% relative/7.4% absolute
risk reduction for coiling vs. clipping
Figure 5-16-28
Controversial study
Should patients be offered the option of
coiling vs. clipping in the acute setting?
Deconstructive Therapy
[Figures 5-16-27 and 5-16-28]
A paraophthalmic aneurysm treated by endosaccular occlusion

with coils
1218
Neuroradiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
Aryan H, Giannotta SL, Fukushima T, et al. Aneurysms in children: review of 15 years experience. J Clin
Neurosci 2006;13(2):188-92.
Claassen J, Bernardini GL, Kreiter K, et al. Effect of cisternal and ventricular blood on risk of delayed cerebral
ischemia after subarachnoid hemorrhage: the Fischer scale revisited. Stroke 2001; 32:2012-20.
Hamada Y, Mannoji H, Kaneko Y. A ruptured dissecting aneurysm of the vertebral artery: comparison of the
angiographic and histological findings. Neuroradiology 2001; 375-8.
Hoh BL, Cheung AC, Rabinov JD, et al. Results of a prospective protocol of computed tomographic angiography
in place of catheter angiography as the only diagnostic and pretreatment planning study for cerebral aneurysms by
a combined neurovascular team. Neurosurgery 2004; 54(6):1329-40.
Molyneux A, Kerr RS, Yu LM, et al. International subarachnoid aneurysm trial (ISAT) of neurosurgical clipping
versus endovascular coiling in 2143 patients with ruptured intracranial aneurysms: a randomised comparison of
effects on survival, dependency, seizures, rebleeding, subgroups, and aneurysm occlusion. Lancet 2005;
366(9488):809-17.
Nakatomi H, Segawa H, Kurata A, et al. Clinicopathological study of intracranial fusiform and dolichoectatic
aneurysms: insight on the mechanism of growth. Stroke 200; 31:896-900.
Schievink W. Intracranial aneurysms. [Review] NEJM 1997; 336(1):28-40.
Shojima M, Oshima M, Takagi K, et al. Magnitude and role of wall shear stress on cerebral aneurysm:
computation fluid dynamic study of 20 middle cerebral artery aneurysms. Stroke 2004; 35:2500-05.
Suarez JI, Tarr RW, Selman WI. Aneurysmal subarachnoid hemorrhage. Review NEJM 2006; 354:387-96.
Van Gijn J, Rinkel GJ. Subarachnoid hemorrhage: diagnosis, cause and management. [Review Article] Brain
2001; 124:249-78.
Velthuis BK, Rinkel GJ, Ramos LM, et al. Subarachnoid hemorrhage: aneurysm detection and preoperative
evaluation with CT angiography. Radiology 1998; 208: 423-430.
Wani AA, Behari S, Sahu RN, et al. Paediatric intracranial aneurysms. J Pediatr Neurosci 2006; 1:11-15.
Wiebers DO, Whisnant JP, Huston J 3d, et al. Unruptured intracranial aneurysms: natural history, clinical
outcome, and risks of surgical and endovascular treatment. Lancet 2003; 362(9378):103-110.
Neuroradiology
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Intracranial Vascular Malformations

Mary E. Jensen, MD
Classification of IVMs
Traditional (path-based)
Arteriovenous malformations
Pial
Dural
Venous vascular malformations
Venous angioma
Capillary telangectasias
Cavernous angiomas
Functional (flow-based)
AV Shunting
Pial AVM
Cerebral AVF
Dural AVF
Non-shunting
Capillary malformations
Venous malformations
Cavernous malformations
Epidemiology
Detection rate of AVM

1.1:100,000 (excluding autopsy cases)
2.1:100,000 (including autopsy cases)
1.2:100,000 person-years (symptomatic)
Prevalence
0.1% of US population (300,000 people)
Point prevalence 18:100,000 adults
Account for
1%-2% of all strokes
3% of strokes in young adults
9% of subarachnoid hemorrhage
4% of all intraparenchymal hemorrhages
1/3 of young adult hemorrhages
Etiology
Congenital
Sporadic
Genetic
Hereditary hemorrhagic telangectasia
4%-13% with cerebral AVMs
?ENG, ACVR1 gene mutations
Encode proteins in TGFB1 receptor complexes
Neurocutaneous disorders
Pathogenesis
Wedge-shaped abnormal tangle of arteries and veins

Arteries and veins linked by fistulae
No normal capillary bed
Deficient muscularis in small arteries
Hemodynamic effects
Fistulous effects
High flow, rapid shunting
Induced hypotension in feeders and adjacent areas (arterial steal)
Opening of collateral pathways, angiomatous change
Dysplastic appearance of feeding pedicles, flow-induced angiopathy
Luminal dilatation or stenosis
Aneurysms
1218
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Neuroradiology
Usually present before 40 years of age

Men and women equally affected
Hemorrhage is #1 complication (>50%)
2%-4% yearly rupture rate
6%-18% annual rate after first bleed
- Increased rate for the first year reported but not consistently noted
10%-18% mortality from hemorrhage
1.0%-1.5% annual rate
Seizures: 20%-25%
Generalized more common than focal
Headache: 15%
Focal neurologic deficit: <5%
Steal phenomenon is uncommon
Pulsatile tinnitus
Children (< 2 yr)
Large head from hydrocephalus
seizures
Location
Supratentorial (85%), infratentorial (15%)

Cortical AVMs (72%)
Gyral, sulcal, mixed
Subcortical AVMs (1%)
Deep AVMs (27%)
Subarachnoid, parenchymal,
plexal, mixed
Figure 5-17-1
Hemorrhage Predictors
Many are not consistent

Radiologic findings
Prior hemorrhage
Small AVM in diameter or volume
Increased feeding artery pressures
Periventricular/intraventricular
location
Radiologic Predictors
An enlarged lenticulostriate running through the ventricle is the

presumed source of hemorrhage
Features that may result in a higher risk

of bleeding
Arterial aneurysms (10%)
Intranidal aneurysms (20%-58%)
Arterial supply from perforators
Location
Intraventricular/periventricular
Basal ganglia, thalamus
Deep venous drainage
Single venous drainage outlet
Venous stenosis
Intraventricular Hemorrhage
[Figure 5-17-1]
Intraparenchymal Hemorrhage
Figure 5-17-2
CTA, MRA and DSA all show intranidal aneurysm within a

small AVM
[Figure 5-17-2]
Neuroradiology
1219
1221
Hemorrhage Risk: Summary
Lowest-risk group (1%)

No history of prior hemorrhage
>1 draining vein
Highest-risk group (8.9%)
History of prior hemorrhage
Single draining vein
Diffuse nidus
AVM Grading
There are many grading systems

The most commonly used is the Spetzler-Martin system
Size (<3 cm = 1, 3-6 cm = 2, >6 cm = 3)
Eloquence of surrounding brain
(non-eloquent = 0, eloquent = 1)
Pattern of venous drainage (superficial = 0, deep = 1)
Spetzler-Martin Grading System
This system is used to stratify surgical outcome

Grades I through II
Extremely low rates of surgically related morbidity and mortality
Grade IV and V
High risk
Spetzler-Martin Grading Scale
Grade III
Heterogeneous group
S1V1E1 same risk as I/ II (III-)
- Microsurgery
S2V0E1 same risk as IV/V (III+)
- Manage conservatively
S2V1E0
- Intermediate risk
- Judicious selection for
surgery
Figure 5-17-3
Radiographic Evaluation
CT
Usual first study
Hemorrhage, calcifications,
parenchymal changes, iso- or
hyperdense serpentine structures
Contrast study outlines boundaries
CTA
Vascular elements
Location of feeding
arteries/draining veins
Associated aneurysms
Volumetric determination
NCCT shows enlarged MCA trunk and sylvian arteries,

intraventricular hemorrhage, focal atrophy and hyperdense
vessels
Figure 5-17-4
CT of Brain AVM [Figure 5-17-3]

CTA of Brain AVM [Figure 5-17-4]
CTA shows AVM components and their relationship to the

parenchymal hemorrhage
1220
1222
Neuroradiology
Figure 5-17-5
MR
Location and topography
Presence or absence of acute, subacute or
chronic hemorrhage
Associated parenchymal changes such as
edema, ischemia, gliosis, atrophy, mass effect,
radiation effects
MRA
Same as CTA
Useful in stereotactic radiosurgery planning
MRI of Brain AVM [Figure 5-17-5]

MRA of Brain AVMs [Figure 5-17-6]
Angiography
Arterial supply, regional and individual
High flow arteriopathy
stenosis
dolichoectasia
flow related aneurysms (10%)
Arterial supply to the brain
Pial
Dural
Assessment of nidus
plexiform vs. fistulous
intra-nidal aneurysms and ectasias
true nidus vs. angiomatous change
T1 images without and with Gd (left images)

T2 and FLAIR images (right images)
Figure 5-17-6
DSA of Cerebral AVMs [Figure 5-17-7]

Nidal Components [Figure 5-17-8]
En Passage Vascular Supply [Figure 5-17-9]
AVM Associated Aneurysms [Figure 5-17-10]
MRA of shows extent of parietal AVM, and the

enlarged feeding arteries and draining veins
Angiomatous Change [Figure 5-17-11]
Figure 5-17-8
Figure 5-17-7
Superselective
injections of a
temporal lobe AVM
(A) show different
nidal components
including a
macrofistula (B) and a
racemose nidus (C)
Typical appearance of gyral (left) and sulcal (right)

AVM
Neuroradiology
1221
1223
Figure 5-17-9
Angiography
Venous drainage, regional and individual
High-flow venopathy
dural sinus high-flow
venous thrombosis
venous enlargement, stenoses, varix
Normal drainage of the brain
Venous Abnormalities [Figure 5-17-12]

Treatment
Controversial
Observation
Microsurgery
Embolization
Stereotactic radiosurgery
Combination therapy
Superselective study (right) of temporal AVM

shows multiple en passage feeders with distal
arterial supply to normal brain
Figure 5-17-10
Arterial feeder (left)
Perinidal (center)
Intranidal (right)
Figure 5-17-11
Figure 5-17-12
Difference between angiomatous change (circle)

and nidus (box)
Two patients with high-flow venopathya venous

aneurysm in a deep AVM (left) and a venous varix
with a stricture (right, arrow)
1222
1224
Neuroradiology
Pre-Radiosurgery Embolization [Figure 5-17-13]
Figure 5-17-13
Intracranial AVFs
Dural
Most common type of cerebral AV fistula
Shunt occurs primarily from dural arteries to
dural sinuses or cortical veins
Cerebral
Rare
Vein of Galen malformation
Pial
Pre- and post-embolization of distal pericallosal

artery with coils and NBCA
Etiology/Pathogenesis
Adults (Dural AVF)

Acquired lesions
Present in later life (40s-60s)
Shunting within dural/venous wall
Sinus thrombosis
- Environment conducive to the
development of DAVF
- Triggered by factors which stimulate
angiogenesis
Children (Pial AVF)
Congenital lesions
?vascular remodeling of the venous
endothelial cells in the capillary bed caused
by a trigger such as hypoxia
Pial A-V Fistula [Figure 5-17-14]

Location of DAVFs
Transverse - sigmoid sinus:

Cavernous sinus:
Tentorial - incisural:
Convexity - sagittal sinus:
Orbital - anterior falx area:
Sylvian - middle fossa area:
Others: marginal sinus, etc
Figure 5-17-14
Newborn with congestive heart failure and cranial

bruit
62.6 %
11.9 %
8.4 %
7.4 %
5.8 %
3.7 %
Classification of DAVFs
Borden, Djindjian and Merland, Cognard

Venous drainage
Sinus vs. cortical veins
Direction of flow
Antegrade, retrograde, both
Involvement of cortical venous drainage
Benign vs. aggressive
Determines risk
Borden Classification
Neuroradiology
1223
1225
Benign: All Classifications [Figure 5-17-15]
Figure 5-17-15
Aggressive: All Classifications [Figure 5-17-16]

Symptoms and signs vary with location

Tinnitus, bruit
Orbital congestion
Focal neurological deficits
Global neurological deficits
Hemorrhage
ICH: 35%-42% overall;
75%-95% in tentorial, ACF
Benign transverse sinus DAVF with fistulous flow

only to sinus
Figure 5-17-16
Hemorrhage Associated with DAVF

[Figure 5-17-17]
Clinical Outcomes
Without cortical venous involvement

Symptom improvement or resolution
No treatment: 81%
Treatment: 86%
With cortical venous involvement
non-hemorrhagic neurological deficit: 6.9%/yr
Hemorrhage: 8.1%/yr
Mortality: 10.4%/yr
Malignant transformation of benign lesion: <1%
Acute symptoms
CT to rule out hemorrhage
MRI/A, CTA to show prominent vessels
Not adequate for demonstration of shunt
DSA
Confirm diagnosis
Identify angioarchitecture
Evaluate hemodynamics
Chronic symptoms
Contrast CT/MR
DSA
Figure 5-17-18
Treatment
Benign
Observation
Aggressive
Endovascular therapy
Transvenous, transarterial,
combined
Surgery
Failed endovascular therapy
Isolated sinus that required
direct puncture/exposure
Developmental Venous Anomaly
Most common type of vascular

malformation
Figure 5-17-17
Tentorial DAVF with SAH (left) and transverse

sinus DAVF with ICH (right)
Aggressive DAVF [Figure 5-17-18]
Aggressive transverse sinus DAVF with all fistulous

flow into the cortical veins
Diffusion study shows temporal lobe edema and old

hemorrhage; Gd MRI shows marked venous congestion and a
multi-channel left transverse sinus; DSA confirmed
development of a DAVF
1224
1226
Neuroradiology
Accounts for 60% of CVM

2.5%-9% prevalence on enhanced MR
Congenital
Develop during 30th to 45th day as embryo
Focal underdevelopment of superficial or deep adult veins
Anomalous medullary veins converge into a centrally located collector vein
Caput medusa
Pathology
Veins are closely aggregated and dilated

lack muscular and elastic fibers
abnormally hyalinized
Normal adjacent arteries
Adjacent parenchyma
Normal
Gliosis, neuronal degeneration, demyelination
May be associated with focal neuronal migrational abnormalities
Incidental finding in most cases

Associated with H/N vascular malformations
Rarely cause neurologic symptoms
Posterior fossa
Dizziness, ataxia, diplopia
Cerebrum
Seizures, headaches, focal deficits
Hemorrhage
Not the DVA that hemorrhages, but the associated cavernoma
Treatment
Surgery may be required to remove hemorrhage/cavernoma
Leave DVA
Imaging Findings
NCCT
Normal
Ill-defined hyperdense area without edema or mass effect
CECT
Diffuse enhancement of linear vessels adjacent to the ventricle
Stellate pattern
Converge on collector vein
Well-visualized on CTA
MR
Variable degrees of T2 and T1 prolongation in the adjacent parenchyma
Due to increased blood pool
Signal intensity void in draining vein on T2
Occasionally gliosis
Associated with cavernomas in 8%-33% of DVAs
CE MR
Dilated deep veins converge on a collector vein
Follows a transhemispheric course to a normal vein
Angiography
Pathognomonic
Normal arterial phase
Radially oriented dilated medullary veins converge on an enlarged
transcortical draining vein
Caput medusa opacifies at the same time as normal veins
Collector vein seen on late venous phase
Location
In the deep white matter near the margin of an adjacent ventricle
Frontal>parietal>brachium pontis/dentate
Frontal lobes may opacify earlier and show a capillary blush
Neuroradiology
1225
1227
Developmental Venous Anomaly [Figure 5-17-19]

DSA of Developmental Venous Anomaly [Figure 5-17-20]
Cavernous Angioma
Prevalence: 0.5%-0.7% on MR/autopsy

Account for 5%-13% of cerebral VMs
Sporadic
Familial form
Autosomal dominant, Hispanic
Multiple lesions
Increased number of lesions with aging
Correlation of symptoms with the presence of high signal intensity lesions
on MR
Figure 5-17-19
Pathology
Gross findings
Small, reddish-purple (mulberry)
lesions
Few mms to several cms
Multiple or single
Often encapsulated and multilobar
Occasionally calcified
Often associated with DVA
Found throughout the CNS
Pathology
Histologic findings
Thin-walled vascular sinusoids
Endothelium lacks smooth muscle,
elastin, and intervening parenchyma
Lacks blood brain barrier
Surrounded by hemosiderin
deposits and gliosis
May or may not be thrombosed
NCCT and T1 and T2 weighted MRI shows dystrophic

microcalcifications and/or hemosiderin staining in the right
basal gangliar region
Usually present between age 30-50

years
Symptoms
Seizures
Lesions usually in frontal or temporal lobe
Focal neurologic deficits
Acute hemorrhage
Risk is 0.25%-6% per year
Increased risk with previous hemorrhage,
pregnancy
Headache
Figure 5-17-20
Imaging Findings
CT
Variable density
Hemorrhage
Calcification
- Rim, coarse, stippled, granular
DSA
Cavernous angioma not visualized
Associated DVA
DSA shows caput medusa in early venous phase

followed by filling of the collector vein
1226
1228
Neuroradiology
CT of Cavernous Angioma [Figure 5-17-21]
Figure 5-17-21
Imaging Findings - MRI
Type 1
Hyperintense core on T1
Hyper or hypointense core on T2
Corresponds to subacute
hemorrhage
Type 2 popcorn
Reticulated mixed signal on T1
Reticulated mixed signal on T2 with
hypointense rim
CT findings include a hypodense center with rim calcification
Corresponds to lesions with multiple
(left) and fine granular calcifications throughout the lesion
hemorrhages of various age
(right)
Type 3
Iso or hypointense on T1
Hypointense lesion with hypointense rim on T2
Corresponds to chronic hemorrhage with hemosiderin staining
Type 4
Not visible on T1 or T2
Punctate hypointense lesion on GRE
Corresponds to tiny lesion or telangiectasia
Figure 5-17-22
MRI of Cavernous Malformations

[Figure 5-17-22]
Cavernous Malformation with DVA

[Figure 5-17-23]
Treatment
Stereotactic microsurgery
Reasons for surgery
Repeat hemorrhage
Mass effect from enlarging
lesion
Seizure focus
Radiosurgery
Controversial
Associated with hemorrhage and
radiation-induced mass effect
Follow
Deep lesions
Familial lesions
T1, T2 and GRE images show a Type 2 lesion in the right

frontal lobe and a Type 4 lesion in the left occipital lobe
Figure 5-17-23
Capillary Telangiectasia
Represents 16%-20% of all CVMs

Incidental finding
Nearly always asymptomatic
Sx: headache, vertigo, ataxia, hearing loss
Congenital vs. acquired lesions
Obstructed venous drainage; radiation
May be associated with VA, CM, AVM
Transitional malformations
Gd MRA shows DVA associated with a cavernous

malformation
Neuroradiology
1227
1229
Pathology
Location
Pons, cerebral/cerebellar hemispheres, spinal cord
Histology
Thin-walled, capillary-type, ectatic blood vessels
Interspersed with normal brain
Size
Few millimeters to 2 cm
Hemorrhage
Rare
Usually from an associated vascular malformation
Imaging [Figure 5-17-23]
Figure 5-17-24
CT
Usually normal
MR
T1: hypo- or isointense
T2: iso- or slightly hyperintense
GRE: hypointense
Most consistent finding
Due to intravascular
deoxyhemoglobin
CE-MR
Faintly enhance in a stippled
or brushlike pattern
2/3d with enlarged vessel
Typical MR findings in a capillary telangiectasia
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
Al-Shani R, Warlow C. A systematic review of the frequency and prognosis of arteriovenous malformations of the
brain in adults. Brain 2001; 124:1900-26.
Augustyn GT, Scott JA, Olson E, et al. Cerebral venous angiomas: MR imaging. Radiology 1985; 156:391395
Camacho DL, Smith JK, Grimme JD, et al. Atypical MR imaging perfusion in developmental venous anomalies.
AJNR Am J Neuroradiol 2004; 25(9):549-52.
Gault J, Sarin H, Awadallah N, et al. Pathobiology of human cerebrovascular malformations: basic mechanisms
and clinical relevance. Neurosurgery 2004; 55(1):1-17.
Lai CW, Agid R, van den Berg R, ter Brugge K. Cerebral arteriovenous fistulas induced by dural arteriovenous
shunts. AJNR J Neuroradiol 2005; 26:1259-62.
Lawton MT, UCSF Brain Arteriovenous Malformation Study Project. Spetzler-Martin Grade II arteriovenous
malformations: surgical results and a modification of the grading scale. Neurosurgery 2003; 52(4):740-8.
Mast H, Young WL, Koennecke HC, et al. Risk of spontaneous haemorrhage after diagnosis of cerebral
arteriovenous malformation. Lancet 1997; 350:1065-8.
Ogilvy C, Steig P, Awad I, et al. Recommendations for the management of intracranial arteriovenous
malformations. A statement for health care professionals from a special writing group of the Stroke Council,
American Stroke Association. Circulation 2001; 103:2644-57.
Peebles TR, Vieco PT. Intracranial developmental venous anomalies: diagnosis using CT angiography. J Comput
Assist Tomogr 1997; 21(4): 582-6.
Sakata N, Takebayashi S, Kojima M, et al. Different roles of arteriosclerosis in the rupture of intracranial
dissecting aneurysms. Histopathology 2001; 38(4):325-37.
Stapf C, Mohn JP, Choi JH et al. Invasive treatment of unruptured brain arteriovenous malformations is
experimental therapy. Current Opinion in Neurology 2006; 19(1):63-8.
The Arteriovenous Malformation Study Group. Arteriovenous malformations of the brain in adults. NEJM 1999;
340(23):1812-18.
The Scottish Intracranial Vascular Malformation Group. Prospective, population-based detection of intracranial
vascular malformations in adults. Stroke 2003; 34:1163-69.
1228
1230
Neuroradiology
Imaging of Intracranial Infections

Summary
Meningeal Disease
Epidural abscess
Subdural empyema
Meningitis
Parenchymal Infection
Cerebritis to abscess
Encephalitis HSV, CJD, ADEM
Parasitic Cysticercosis, Lyme disease
Tuberculosis
Mycotic
AIDS related infections
Intracranial Infection
Location
Intra-axial Parenchymal
Extra-axial Epidural, subdural, leptomeningeal
Response to infection
Edema and swelling
Mass and mass effect
Abnormal enhancement
Chronic Atrophy
Figure 5-18-1
Blood-brain barrier
Tight junctions (no gaps) exist between normal endothelial cells

Little communication between capillary & extracellular space or
neurons
Infection results in loss of tight junctions, with increased
permeability of endothelial membranes = LOSS OF BBB
Loss of BBB + increased local blood volume = ABNORMAL
ENHANCEMENT
Intracranial infection Location Meninges
Dura mater
2 layers
Outer layer is calvarial periosteum
Inner layer is separation between dura mater and arachnoid
Collection between inner table of skull & dura is EPIDURAL
Intracranial infection Location Meninges [Figure 5-18-1]
Subdural empyema
(courtesy of Amirsys, Inc.)
Figure 5-18-2
Arachnoid Mater
Thin connective tissue
Parallels dura mater
Collection between dura & arachnoid is SUBDURAL
Subdural space is potential space w/ bridging veins
Intracranial infection Location Meninges[Figure 5-18-2]
Pia mater
Blood vessels
Covers surface of brain
Space between arachnoid & pia is SUBARACHNOID space
LEPTOMENINGES Arachnoid + Pia
Leptomeningeal pattern of infection

(courtesy of Amirsys, Inc)
Neuroradiology
1229
1231
Extra-axial Infection Epidural [Figures 5-18-3 and 5-18-4]
Usually starts in paranasal sinuses or mastoids

Low density on CT
Enhancing periphery
? SI on T1
? SI on T2, FLAIR
Enhancing dura
Check for osteomyelitis of calvarium
Figure 5-18-3
Acquired CNS infections
Meningitis and complications

Cerebritis and abscess
Encephalitis
Parasitic, rickettsial, spirochetal infections
TB, other granulomatous infections
Immunocompromised host, especially AIDS
Epidural abscess with typical

enhancing dural surface
Acute Bacterial Meningitis
Figure 5-18-4
Organisms
Neonates: group B streptococcus
Children: H. influenza
Adults: Streptococcus pneumoniae
Pathogenesis
Hematogenous seeding, choroid, leptomeninges
Contiguous spread from sinusitis, mastoiditis
Neonatal meningitis - maternal GU infxn, PROM
Clinical - H/A, neck stiffness, photophobia, cranial
nerve dysfunction, lethargy
Meningitis role of imaging
Epidural collection crosses midline. Subdural

abscess does not cross midline, and can extend
along interhemispheric fissure
Complications
Hydrocephalus (especially communicating)
Subdural effusions, empyema
Venous sinus thrombosis/infarction
Arterial infarction
Vasculitis
Cerebritis or abscess
Ventriculitis/ependymitis
Figure 5-18-5
ACUTE MENINGITIS: Patterns [Figure 5-18-5]
Dura Arachnoid
Pachymeningitis
Pia-Subarachnoid Space
Leptomeningitis
Two patterns of meningitis

(courtesy of Amirsys, Inc).
Meningitis: CT Findings
Figure 5-18-6
Early on, CT usually normal

Sulcal or cisternal effacement
Pia/subarachnoid enhancement
Hydrocephalus
? Source Paranasal sinus, mastoid
Acute Meningitis: MR Findings [Figure 5-18-6]
T1 and T2 images may be normal

FLAIR ? SI in SAS
Gd - Leptomeningeal enhancement
? Hydrocephalus
? Infection source
Meningeal infection best

detected on FLAIR image, with
increased SI in subarachnoid
1230
1232
Neuroradiology
Acute Meningitis: MR Findings [Figure 5-18-7]
Figure 5-18-7
T1 and T2 images may be normal

FLAIR ? SI in SAS
Gd - Leptomeningeal enhancement
? Hydrocephalus
? Infection source
Meningitis, complications
Hydrocephalus
Ependymitis
Subependymal enhancement
In HIV disease, diff dx is lymphoma
Meningitis, complications [Figure 5-18-8]
Subdural effusions
Often sterile
If infected, restricted diffusion on DWI
H. flu
More common in children
Bilateral, resolve spontaneously
CT/MR similar to CSF
Membranes may enhance
Meningitis with subarachnoid pattern

of enhancement on T1 gad. Image
Figure 5-18-8
Meningitis, complications
Infarction
May be arterial or venous
Well-demarcated best way to differentiate from cerebritis
Typical CT/MR features with restricted diffusion on DWI
Diffusion weighted imaging (DWI)
Powerful tool, physiologic information

Differentiates unrestricted from restricted free water diffusion
Restricted diffusion
Acute and subacute infarction
Intracranial abscess
Rare MS plaque
Epidermoid (vs. arachnoid cyst)
Meningitis, complications [Figure 5-18-9]
Figure 5-18-9
Summary
Meningeal Disease
Epidural empyema
Subdural empyema
Meningitis
Tuberculosis
Mycotic
Thalamic and basal ganglia infarctions due to

acute meningitis
Cerebritis to brain abscess
Bilateral sterile effusions after H. flu

meningitis
Early and late cerebritis

Early and late capsule/abscess
Etiology
Direct spread (sinus, mastoid, odontogenic)
Hematogenous
Surgery or trauma
25% - no source found
Neuroradiology
1231
1233
Early cerebritis
3-5 days after infection
Unencapsulated white cells, edema, necrosis,
petechial hemorrhage
Late cerebritis
4-14 days after infection
Poorly delineated rim, necrotic core, white
cells/inflammatory cells, granulation tissue
Figure 5-18-10
Early capsule
2-4 weeks
Collagenous capsule +/- daughter abscesses
Necrotic core
Mild mass effect
Late capsule
Weeks/months
Thick capsule
Edema, mass effect resolve
Abscess is typically associated with vasogenic

edema and rim enhancement
Figure 5-18-11
Abscess - pyogenic [Figure 5-18-10]
Supratentorial is most common

Usually solitary (may have small surrounding cysts)
Streptococcus, Staphylococcus
Infants Citrobacter, Proteus, Pseudomonas,
Serratia
Abscess [Figures 5-18-11 and 5-18-12]
Mass effect, edema, enhancement

T2 WI ? SI rim
DWI/ADC
Restricted diffusion
MR spectroscopy
Lactate (1.3 ppm)
Complications
Herniation
Intraventricular rupture
Choroid plexitis
Leptomeningitis
Abscess with restricted diffusion, confirmed on

ADC map
Figure 5-18-12
Encephalitis Inflammation of the brain
Diffuse infection
Post-infectious/immunization
Acute disseminated encephalomyelitis
Viral
Herpes simplex virus
Others: measles, mumps, etc.
Herpes encephalitis
Adults - HSV-1 (oral)

Frontal sinusitis resulting in frontal abscess, with
Neonates HSV-2 (genital)
low signal rim on T2, and enhancement
Confusion, progressing to coma
50%-70% mortality, especially when delay to diagnosis (most common cause
of fatal encephalitis)
Necrotizing hemorrhagic encephalitis, often resulting in diffuse atrophy
Axonal spread from reactivation in trigeminal ganglion
1232
1234
Neuroradiology
Herpes encephalitis [Figure 5-18-13]
Figure 5-18-13
Predilection for limbic system

Temporal lobes
Insula
Cingulate gyrus
Subfrontal region
Herpes encephalitis
Imaging may be normal early in disease course

MR > CT, especially early
MR
Inc. SI on T2 & FLAIR
Mass effect
+/- enhancement
60% bilateral
May have small punctate hemorrhage
Herpes encephalitis of left temporal lobe
Figure 5-18-14
Herpes encephalitis [Figure 5-18-14]
Subtle, gyral enhancement

Unilateral early, then bilateral
Temporal lobes, insular cortex, inferior frontal lobes,
cingulate gyri
Creutzfeldt-Jakob Disease
Transmissible spongiform encephalopathy (TSE)

Prion: proteinaceous infectious particle
Sporadic most common (85%-90%)
Genetic (10%-15%)
Infectious (rare)
Creutzfeldt-Jakob Disease
CLASSIC CJD
Older (mean 68 yrs)
Sporadic
Shorter duration
Dementia
Rare pulvinar sign
VARIANT CJD
Younger (mean 28 yrs)
BSE contaminated food
Longer duration
Behavior changes
Pulvinar sign on MR >
75%
Variable amounts of PrPres Lots of PrPres
Herpes encephalitis, with high SI in temporal

lobes and patchy enhancement
Creutzfeldt-Jakob Disease: Imaging
Symmetric high signal on T2 & FLAIR

Caudate head, basal ganglia, thalamus (pulvinar sign)
Diff. restriction > 2 weeks (c/w infarct)
Cortical, limbic system involvement 1/3
Occipital lesions: 20%
Rapidly progressive atrophy
CNS Tuberculosis
Increasing incidence homeless, prisoners (2%-5% of pts. w/ TB)

AIDS population
Hematogenous dissemination from lungs
Most have pulmonary TB
Drug resistance increasing & ominous
Either meningitic or tuberculoma, rarely both forms found together
CNS Tuberculosis Leptomeningeal
Cisterns fill with gelatinous exudate

Hydrocephalus, infarctions, CN palsies
Leptomeningeal enhancement, hydrocephalus
Disease at COW arteritis & infarctions, esp. in children
Neuroradiology
1233
1235
CNS Tuberculosis Cerebritis [Figure 5-18-15]
Figure 5-18-15
CNS TuberculosisTuberculoma
Most common parenchymal form(may

be extra-axial)
Supratentorial > infra
Dense fibrous capsule with caseous
necrotic core
CNS Tuberculosis Tuberculoma

[Figure 5-18-16]
TB cerebritis with typical pattern of right temporal edema and

enhancement
Small or coalesced larger nodules

Edema, mass effect
Wall may have increased SI on T1, decreased SI on T2
Old/treated lesions may Ca+
Figure 5-18-16
Neurocysticercosis
Taenia solium (Pork tapeworm)

Worldwide, most common CNS infection
Worldwide, most common cause of epilepsy
Central/South America; East/SE Asia; India; Africa
CNS disease: 60%-90%
Seizures, intracranial hypertension, focal deficit
Four variants
1. Parenchymal
2. Intraventricular
3. Cisternal
4. Spinal
Multiple tuberculomas, with low signal intensity rim

on T2 and enhancement
Parenchymal Cysticercosis
Vesicular stage
Eccentric nodule (scolex), no edema or enhancement
Colloidal stage
Dying scolex, capsule thickens, extensive edema & enhancement
Granular nodular stage
Cyst ? in size, small enhancing nodules, no edema
Nodular calcified stage
Cyst involutes, calcifies, no edema or enhancement
Figure 5-18-17
Vesicular stage
Larva is fully grown,with thin intact capsule

surrounding distended bladder
Fluid in bladder is clear, no surrounding inflammatory
reaction.
Well-defined cyst, scolex enhances (mural nodule)
Wall does not enhance, no edema
Colloidal stage [Figure 5-18-17]
Colloidal stage of CNS cysticercosis, with thick

capsule, edema, and mass effect
Larva degenerates, cyst fluid turbid

Cyst wall thickens
Vasogenic edema
Cyst fluid ? SI on T1WI
Cyst wall ? SI on T2WI
Vasogenic edema & enhancement
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Neuroradiology
Granular Nodule Stage
Figure 5-18-18
Cyst retracts, capsule thickens, and scolex calcifies

+/- perifocal edema
CT: Isodense cyst, calcified scolex
MR: T1WI isointense to brain
T2WI hypointense to brain
Intraventricular Cysticercosis [Figure 5-18-18]
10-20% of neurocysticercosis
Fourth ventricle - most common site
+/- hydrocephalus
Cyst can parallel CSF density and SI
Does not calcify
Cisternal Cysticercosis
Basilar cisterns or sylvian fissures

MRI more sensitive than CT
Can incite leptomeningeal inflammatory response
Hydrocephalus, infarctions
Intraventricular and subarachnoid

cysticercosis, with hydrocephalus
CNS Lyme Disease
Borrelia burgdorferi
Common tick borne disease in NE US
Presents as meningitis, neuritis (incl. CN), vasculitis
Multifocal wm lesions, +/- enh
Fungal Disease in CNS
Figure 5-18-19
Cryptococcosis
Coccidioidomycosis
Mucormycosis
Aspergillosis
Others
AIDS Related Conditions
HIV Encephalitis
Toxoplasmosis (vs lymphoma)
Cryptococcal meningitis
Progressive multifocal leukoencephalopathy (PML)
Human Retroviruses
HIV (HIV-1 and HIV-2) and HTLV-1

Patchy symmetric abnormal signal
HIV-1 found in CNS in AIDS (neurotrophic)
intensity in patient with HIV
Encephalopathy, myelopathy, peripheral neuropathy, myopathy
encephalitis
HIV replicates in multinucleated giant cells & macrophages in
CNS
Oligodendrocytes, astrocytes, neurons less frequently directly infected
Subacute HIV encephalitis AIDS Dementia Complex (ADC)

[Figure 5-18-19]
Most common CNS complication (30%)

Dementia, behavior changes, headache
Virus in MNGCs
CT normal
MR atrophy
PVWM lesions
Grey matter (late)
Neuroradiology
1235
1237
HIV Leukoencephalopathy
Figure 5-18-20
Disease on MR parallels clinical progression

NAA/Cho & NAA/Cr ratios reduced due to neuronal
loss
PML [Figure 5-18-20]
Papovavirus (JC virus)
Patchy non-enhancing white matter lesions
No mass effect
Hypointense on T1
Asymmetric
HIV Encephalitis vs PML

HIV
Diffuse
Central
T1 images nl
PML has low SI on T1 weighted images, with no

enhancement
PML
Asymmetric
Peripheral
Hypointense T1
Figure 5-18-21
Protozoan Toxoplasma gondii

Soil organism
Endemic in US
Reactivates in CNS in immunocompromised
Most common opportunistic infection
CD4 < 100 cell/mm3
Fever, HA, neurologic deficits, seizures
Basal ganglia, cerebral hemispheres
Single large toxoplasmosis abscess
Toxoplasmosis
Figure 5-18-22
Necrotic debris, inflammatory cells, organisms

Vasogenic edema
Robust enhancement
15% are solitary
Pyrimethamine + sulfa or clindamycin
Life-long maintenance abs
With therapy, lesions may calcify

Resolution of vasogenic edema
New lesions or new edema develop if medication is
stopped
Old, treated toxoplasmosis abscesses may calcify
Cryptococcus neoformans
Ubiquitous fungi within contaminated soil

Inhaled, then hematogenous spread immunocompromised hosts
Most common fungus in AIDS (6%-7%)
Subacute meningitis, HA, AMS, fever
DX India ink preparation, antigen in CSF, fungal culture of CSF
Perivascular spaces distended with mucoid material and fungus
Figure 5-18-23
Cryptococcus meningitis
Basal ganglia,often bilateral

CT may be normal
Non-enhancing low density lesions in Virchow-Robin
spaces gelatinous pseudocysts
Cryptococcus meningitis [Figure 5-18-23]
On MR, pseudocysts are decreased SI on

T1,increased SI on T2
No significant enhancement
Dilated peri-vascular spaces in cryptococcosis

1236
1238
Neuroradiology
Highly Active Antiretroviral TX (HAART) Immune reconstitution syndrome
AIDS treatment regimen that includes protease inhibitor & reverse transcriptase inhibitor
Suppresses viral replication
Increase in CD4 counts, decrease in viral load
Increased survival
May result in unexpected imaging findings
HAART
Ability to mount immune response may change imaging findings, especially enhancement patterns
Enhancement in crypto. meningitis
Summary
Meningeal Disease
Epidural empyema
Subdural empyema
Meningitis
Tuberculosis
Mycotic
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
Chang L, Ernst T. MR spectroscopy and diffusion-weighted MR imaging in focal brain lesions in AIDS.
Neuroimaging Clin N Am. 1997 Aug;7:409-26.
Collie DA, Summers DM, Sellar RJ, et al. Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign:
MR imaging findings in 86 neuropathologically confirmed cases. AJNR Am J Neuroradiol. 2003l 24:1560-9.
Enzman DR, Britt RH, Obana WG, et al. Experimental staphylococcus aureus brain abscess. AJNR 7:395-402,
1986
Fernandez RE, Rothbert M, Ferencz G, et al. Lyme disease of the CNS: MR imaging findings in 14 cases. AJNR
1990;11:479-481
Filippi CG, Sze G, Farber SJ, et al. Regression of HIV encephalopathy and basal ganglia signal intensity
abnormality at MR imaging in patients with AIDS after the initiation of protease inhibitor therapy. Radiology
1998;206:491-498
Galassi W, Phuttharak W, Hesselink JR, et al. Intracranial meningeal disease: comparison of contrast-enhanced
MR imaging with fluid-attenuated inversion recovery and fat-suppressed T1 weighted sequences. AJNR 2005; 26:
553-9
Gaviani P, Schwartz RB, Hedley-Whyte ET, et al. Diffusion-weighted imaging of fungal cerebral infection. AJNR
Am J Neuroradiol. 2005 May;26(5):1115-21.
Han XY, Weinberg JS, Prabhu SS, et al. Fusobacterial brain abscess: a review of five cases and an analysis of
possible pathogenesis. J Neurosurg 2003; 99: 693-700
Kramer LD, Locke GE, Byrd SE, et al. Cerebral cysticercosis: documentation of natural history with CT.
Radiology 1989;171:459-462
Lai PH, Li KT, Hsu SS, et al. Pyogenic brain abscess: findings from in vivo 1.5 T and 11.7 T in vitro proton MR
spectroscopy. AJNR 2005; 26:279-88
Murata T, Shiga Y, Higano S, et al. Conspicuity and evolution of lesions in Creutzfeldt-Jakob disease at diffusionweighted imaging. AJNR Am J Neuroradiol. 2002; 23:1164-72
Nadal Desbarats L, Herlidou S, de Marco G, et al. Differential MRI diagnosis between brain abscess and necrotic
or cystic brain tumors using the apparent diffusion coefficient and normalized diffusion-weighted images. Magn
Reson Imaging 2003; 21: 645-650
Post MJD, Tate LG, Quencer RM, et al. CT, MR, and pathology in HIV encephalitis and meningitis. AJNR 1988;
9:469-476
Prusiner SB. Prions and neurodegenerative diseases. N Engl J Med. 1987; 317:1571-81.
Sze G, Zimmerman RD. The magnetic resonance imaging of infections and inflammatory diseases. Radiol Clin
North Am. 1988: 26:839-59.
Tien RD, Felsberg GJ, Osumi AK. Herpesvirus infections of the CNS: MR findings. AJR Am J Roentgenol. 1993
Jul;161:167-76.
Wehm SM, Heinz ER, Burger PC, et al. Dilated Virchow-Robin spaces in cryptococcal meningitis associated with
AIDS: CT and MR findings. J Comput Assist Tomogr 1989;13:756-762
Neuroradiology
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The Paranasal Sinuses

Summary
Development
Anatomy, especially for endoscopic sinus surgery (ESS)
Infection
Acute
Chronic
Complications
Neoplasms
Benign, including tumor-like lesions
Malignant
Figure 5-19-1
Sinus Anatomy & Development
Nose and nasal cavities - Functions

Respiration - humidifies and warms inspired air
Defense mucous blanket
Olfaction fibers pass through cribriform plate to CN I
Nose external nose, pyriform aperture
Nasal septum cartilage, ethmoid bone, septum
Nasal cavities internal nasal airways
Inferior turbinate nasolacrimal duct
Medial turb. max, frontal, ant ethmoid sinus
Superior turb post. ethmoid, sphenoid sinus
Normal ethmoid anatomy in coronal

plane
Figure 5-19-2
Sinus Anatomy & Development
Lined by mucosa w/ serous and mucinous glands

Mucoperiosteum mucosa attached to bone
Sinus functions protect CNS (collapsible) is only definite
function
Ethmoid sinus
Bilateral
Groups of cells formed by septa and lamella
Anterior ethmoids
Multiple small cells
Middle turbinate
Posterior ethmoids
Fewer but larger cells
Basal lamella
Lateral insertion of middle turbinate
Separates ant from post ethmoids
Pneumatized agger nasi cell on left

on coronal CT
Figure 5-19-3
Ethmoid sinus - anatomy [Figure 5-19-1]
Lamina papyracea
Roof of the ethmoids
Drainage
Infundibulum, lateral to uncinate process
Ethmoid bulla
Ethmoid sinus - variants [Figures 5-19-2 and 5-19-3]
Agger nasi cell

Most ant. ethmoid
Supraorbital air cell
Needs to be differentiated from frontal sinus
Haller cell
May obstruct max. sinus outflow
Paranasal Sinuses
Bilateral Haller cells, inferomedial

ethmoid cells, between uncinate
process and orbital wall
1238
1240
Neuroradiology
Figure 5-19-4
Onodi cell
Close to optic nerve
Maxillary sinus [Figure 5-19-4]
First to form in utero

Rudimentary at birth, final form during 2nd decade
Recesses
Zygomatic
Palatine
Alveolar
Maxillary sinus
Drainage
Ostium is at superomedial portion
Drains into infundibulum
Uncinate process
Medial wall of ostium
Located at insertion of inferior turb on lateral nasal wall
Contiguous with lacrimal bone anteriorly
Maxillary sinus cilia beat secretions

up and medial, drain via maxillary
sinus ostium into infundibulum
Figure 5-19-5
Maxillary sinus - variants [Figure 5-19-5]
Hypoplasia
Sclerotic walls chronic inflammation
Atelectatic
Lateralized medial wall
Maxillary sinus - variants [Figure 5-19-6]
Hypoplasia
Sclerotic walls chronic inflammation
Atelectatic
Silent sinus syndrome
Lateralized medial wall
CT finding of small sinus with thick

sclerotic walls found in chronic sinus
inflammation on right
Frontal sinus
Absent at birth, finish pneumatizing in 2nd decade

Essentially anterior ethmoid cell
Drainage is variable
Ethmoid infundibulum
Frontal recess
Usually asymmetric, may be septated
Figure 5-19-6
Ostiomeatal complex [Figure 5-19-7]
Lateral nasal wall

3 projections
turbinates
Divide nasal cavity
into 3 separate
passages - meatus
Figure 5-19-7
Right maxillary sinus is severely
atelectatic, with depressed walls
consistent with silent sinus syndrome
Ostiomeatal complex
Neuroradiology
1239
1241
Paranasal Sinuses
Normal OMU [Figure 5-19-8]
Figure 5-19-8
3 turbinates/meati
Frontal recess
Infundibulum and middle meatus
Normal OMU Frontal Recess
Ant. coronal images

Drains frontal sinus to ant. middle meatus
Contents
Frontal sinus
Agger nasi cell
Frontal cells
Frontal recess
Middle meatus
Normal OMU Infundibulum [Figure 5-19-9]
Uncinate process
Infundibulum connects max sinus ostium to middle meatus
Ethmoid bulla largest ethmoid cell
Hiatus semilunaris space between uncinate process and
ethmoid bulla
Normal OMU on coronal CT shows

maxillary sinus, ostium, ethmoid
infundibulum, uncinate process,
middle turbinate and middle meatus.
Figure 5-19-9
Normal OMU - Checklist
Anterior
Frontal sinus, recess
Agger nasi cell, frontal cells
Posterior
Uncinate process
Maxillary sinus, ostium, infundibulum
Ethmoid bulla (retrobullar recess)
Middle turbinate/meatus
Hiatus semilunaris
Uncinate process marks lateral

aspect of infundibulum
Sphenoid sinus
Extremely variable pneumatization

Dev. complete by 2nd decade
Planum sphenoidale, posterior wall, anterior wall, inferolateral & pterygoid
recesses
Ant. wall is roof of nasopharynx
Rarely non-pneumatized, consider anemia or ciliary dysmotility syndrome
Sphenoethmoidal recess
Endoscopic sinus surgery
Theory: mucosa is secondarily involved by inflammatory disease, usually due

to sinus ostial obstruction
Surgically relieve obstructing lesion (polyp, anatomic
variant, etc) will allow sinus to drain normally and
mucosal edema & inflammation will improve
Recurrent or chronic sinusitis should improve
Figure 5-19-10
Ostiomeatal Complex Pattern [Figure 5-19-10]
Middle meatus
Maxillary sinus ostia
Ethmoid infundibulum
Anterior ethmoid cells
Hiatus semilunaris
Ostiomeatal complex
(Courtsey of Amirsys, Inc)
Paranasal Sinuses
1240
1242
Neuroradiology
Infection Acute Sinusitis
Figure 5-19-11
No indications for imaging common cold

Secondary bacterial sinusitis
Strep. pneum, H. flu, beta-hemolytic strep
Facial pain, fever, discharge
Clinical diagnosis
Acute Sinusitis CT/MR
Ethmoids often primary source

Asymmetric
Mucosal thickening
Moderate or severe
Non-specific
Thickened enhancing mucosa with submucosal
edema
Air/fluid levels
Frontal = sinusitis
Maxillary = sinusitis in correct setting
Ethmoid rare
Sphenoid - nonspecific
Orbital sub-periosteal infection. Graphic and axial
CECT show ethmoid opacification, and
peripherally enhancing mass in medial extra-conal
location. (courtesy of Amirsys, Inc.)
Acute sinusitis - complications
Local extension
Orbital sub-periosteal abscess
Intra-cranial epidural empyema
Venous occlusion cavernous sinus, transverse sinus (mastoid), superior
sagittal sinus
Chronic sinusitis
Recurrent acute
Chronic patient has no periods without disease
Figure 5-19-12
Sinusitis subperiosteal infection

[Figure 5-19-11]
Most common local complication

Pre-septal swelling clinical dx
Post-septal infection best detected with imaging
CECT is test of choice, to exclude post-septal, subperiosteal abscess
Sinusitis Complications - Local extension
Axial CECT shows small interhemispheric

subdural abscess. Note posterior frontal wall is
intact
[Figure 5-19-12]
Noncontrast sinus CT not enough

Enhanced CT and MR are indicated for complicated
sinusitis
Intracranial infection can occur without
bone defect
Figure 5-19-13
Frontal Sinusitis Intracranial

abscess [Figure 5-19-13]
Sinusitis Mimics Incidental
lesions found on screening sinus T2, sagittal and axial post-Gd T1 MR images show right frontal
abscess, with surrounding edema. Note thin dural
CT
Subarachnoid hem.
GBM
Colloid cyst
Hydrocephalus
Subdural hematoma
Esthesioneuroblastoma
Neuroradiology
enhancement, opacified frontal sinuses and soft tissue

inflammation
1241
1243
Paranasal Sinuses
Figure 5-19-14
Lymphoma of maxillary antrum

SCCa maxillary sinus
Adenoid cystic ca
Sinusitis - Fungal
Two distinct forms

Acute, fulminant invasive fungal sinusitis
Allergic fungal sinusitis with sino-nasal polyps
Imaging appearance variable for both
Allergic fungal sinusitis with polyps (AFS-SNP)

[Figure 5-19-14]
Immune-competent
Chronic nasal obstruction, recurrent sinusitis
Cycle of sinusitis, mucosal edema, polyp formation, ostial
stenosis, sinusitis
Polyps
Coronal non-contrast CT shows

massive expansion of ethmoid and
frontal sinuses, with contents both
low and high density
[Figure 5-19-15]
CT
Pansinus/nasal cavity opacification

Expanded airless sinuses
Thin deossified sinus walls
Sinus contents variable in density
Mixed low and high density on noncontrast CT
Figure 5-19-15

[Figure 5-19-16]
MRI
Extremely complex SI
May be increased or decreased on T1
Variable on T2, regions of frank signal void
Marked expansion may encroach on surrounding structures,
including orbit & skull base
Fungal sinusitis - invasive [Figure 5-19-17]
Immune deficient
Early non-spec. presentation
CT early
Mucosal disease
Nasal cavity soft tissue due to mucosal or turbinate necrosis
Axial non-contrast CT shows marked

thinning of posterior frontal sinus
walls, without destruction
Figure 5-19-16
Figure 5-19-17
Complex, mixed signal intensity

within expanded airless sinuses on
T2 WIs.
Early invasive fungal

sinusitis may have only a
benign appearing nasal
cavity mass
Paranasal Sinuses
1242
1244
Neuroradiology
Figure 5-19-18
CT/MR late
Local invasion
Dirty retro-antral space
Intracranial/orbitalspread
Bone destruction
Variable SI
May have dramatic decrease of SI on T1 & T2
Heterogeneous enhancement pattern
Figure 5-19-19
CECT shows complete left nasal
cavity obstruction, moderate left
maxillary sinus mucosal thickening,
and severe facial swelling
Complex sinus contents on T2 WIs

in invasive fungal sinusitis
Fungal sinusitis
Figure 5-19-20
Acute invasive
Immunesuppressed
Acute
Pain, fever, local invasion
May be fulminant, rapidly progress
Often treated surgically local resection, orbital
exenteration
High mortality
Allergic fungal
Clinically well
Aspirin intolerance
Chronic
Presents with nasal obstruction
Txed with endoscopic polyp resection
High rate of recurrence
Benign Sinus Lesions
Antro-choanal polyp
Mucocele
Fibrous dysplasia
Osteoma
Juvenile nasopharyngeal angiofibroma (JNA)
Inverted papilloma
Coronal graphic of left antrochoanal polyp.

Antrochoanal polyp [Figure 5-19-20]
Benign maxillary polyp

Extends from maxillary antrum through ostium into nasal cavity
When large may extend to nasopharynx
Entire lesion must be removed to avoid recurrence
Neuroradiology
1243
1245
Paranasal Sinuses
Mucocele [Figure 5-19-21]
Airless, expanded sinus

Sinus walls thinned, may appear dehiscent
Frontal, ethmoid, maxillary, sphenoid in order of
frequency
CT/MR signal intensity variable, depending on age of
secretions
Check sinus ostium for obstructing lesion
Figure 5-19-21
Fibrous Dysplasia [Figure 5-19-22]
Medullary bone replaced by fibroosseous tissue

T1 sagittal (top) and T2 axial (bottom) images
Presents < 30 yrs
show expanded, airless right frontal sinus
Facial asymmetry, esp. cheek
mucocele
Most common maxilla & mandible
Obstruction of sinus ostium results in mucocele, especially
Figure 5-19-22
ethmoid
Fibroosseous Lesions - Fibrous Dysplasia
Fibroosseous
Therefore, often heterogeneous
CT ground glass
MR complex appearance
Mixed increased & decreased SI
Enhances robustly often leads to misinterpretation as tumor
Benign Sinus Tumors - Fibroosseous - Osteoma

[Figure 5-19-23]
Benign proliferation of mature bone

Frontal & ethmoid sinus most common location
Usually small & incidental
May obstruct sinus ostium with sinusitis or mucocele formation
CT may be very dense, or more fibrous
MR decreased SI if primarily osseous
Figure 5-19-23
T1 sagittal (top) and T2 axial

(bottom) images show expanded,
airless right frontal sinus mucocele
Left ethmoid infundibulum osteoma
Juvenile Nasopharyngeal Angiofibroma (JNA)
Benign but aggressive vascular mass

Exclusively in males, adolescents
Presenting sxs depend on location
Nasal obstruction
Minor or major epistaxis
Facial asymmetry/deformity
Proptosis
Serous otitis media
Headache
Paranasal Sinuses
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1246
Neuroradiology
JNA
Arises in posterior nasal cavity (choana)

Involves nasal cavity, nasopharynx, masticator space, sphenoid
sinus, cavernous sinus
Blood supply
ECA - int. max & asc. pharyngeal arteries
ICA supply usually implies intracranial extension
Both ipsi and contralateral ECA/ICA supply
Figure 5-19-24
JNA - Imaging [Figure 5-19-24]
Nasal cavity & nasopharyngeal mass

Expansion of pterygopalatine fossa
Anterior bowing of posterior max. sinus wall
CECT & MR with gado robust enhancement
Left JNA expands PPF, displaces
posterior maxillary sinus wall
JNA - Imaging [Figure 5-19-25]
Heterogeneous on T1 & T2 sequences with flow voids

Axial & cor best
Check for sphenoid sinus, cavernous sinus invasion
T2 images best map entire lesion
Benign sinus lesions - Inverted Papilloma
Epithelial tumor of mucosa

Endophytic growth pattern
Benign appearing mass in nasal cavity/middle meatus
Associated with SCCa 10%-20% of time
Bone remodeling without destruction
On MR
Enhancement
Convoluted cerebriform pattern
Figure 5-19-25
Malignant Sinus Lesions
Sinusitis mimic
Adults, M > F
Usually advanced when detected
Early small lesions clinically attributed to inflammatory sinus
disease
Maxillary, ethmoid most common
SCCa 80%-90%
Malignant Sinus Lesions
Squamous cell ca (most common)

Glandular origin
Olfactory neuroblastoma
Sinonasal undifferentiated ca
Melanoma
Lymphoma
Figure 5-19-26
COMMON IMAGING FINDINGS
JNA shows robust enhancement and

intracranial extension
Bone destruction
Local extension/invasion
Intracranial extension
Most important imaging goal extension of

disease [Figure 5-19-26]
Graphics show patterns of spread of sinus
malignancy
Neuroradiology
1245
1247
Paranasal Sinuses
SCCa Staging critical findings
Figure 5-19-27
Primary site
Size
Bone - maxillary or orbit walls, skull base
Local - cheek, nasal cavity, nasopharynx, orbit
SCCa Staging (maxillary)
T1 Antral mucosa w/o bone involvement or erosion

T2 - Hard palate or sinus walls
T3 Cheek, orbital walls, pterygoid plates, ethmoids
T4 - Skull base (cribriform plateor sphenoid sinus), frontal sinus,
nasopharynx, orbital apex
Squamous cell carcinoma [Figure 5-19-27]
Low SI left nasal cavity SCCa, with

obstructed secretions
Most arise in max sinus or nasal cavity

M > F, adults > 50
Usually advanced at detection
Obstructed sinus secretions may make imaging
appearance complex
Figure 5-19-28
Malignant sinus - SCCa [Figure 5-19-28]
CT bone thinning or destruction

MR T2 best differentiates tumor from benign
secretions
Tumor - decreased SI
Secretions - increased SI
Malignancies of Glandular Origin
10% of all sinus malignancies

Typical local extension & bony erosion
May be higher SI on T2
Adenocarcinoma
Adenoid cystic ca
Propensity for perineural spread
Mucoepidermoid ca
Subtle erosion of planum sphenoidale (top) from

nasal cavity & sphenoid sinus SCCA (bottom)
Figure 5-19-29
Adenoid cystic ca [Figure 5-19-29]
Sinus mass with osseous erosion

Perineural spread
Widened foramen or canal
Enlarged enhancing nerve
Obliteration of fat at skull base foramen
T1 MR shows maxillary mass with hard palate low

SI on left
Perineural spread along vidian nerve
Esthesioneuroblastoma AKA Olfactory

Neuroblastoma
Tumor of neural crest origin

Accounts for 2% of sinonasal malignancies
Arises in nasal cavity near cribriform plate
Age range 3 to elderly
Nasal cavity mass, erosion of cribriform plate, often
with intracranial extension
Peripheral intracranial tumoral cysts
Figure 5-19-30
Esthesioneuroblastoma [Figure 5-19-30]
Intracranial extension from

esthesioneuroblastoma
Paranasal Sinuses
1246
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Neuroradiology
Summary
Development
Anatomy, especially for endoscopic sinus surgery (ESS)
Infection
Acute
Chronic
Complications
Neoplasms
Benign, including tumor-like lesions
Malignant
Neuroradiology
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1249
Paranasal Sinuses
The Sella and Parasellar Region

Recommended Imaging Techniques [Figures 5-20-1 and 5-20-2]
MR Imaging
Multiplanar:
Sagittal & Coronal
Small FOV 16-18
3mm
T1WI, T2WI
Post T1WI +/- FS
Dynamic enhanced
Dynamic Imaging
Microadenomas
3 4 slices
T1 FSE, Turbo SE
Image continuously after contrast (10s)
Increases sensitivity
Normal sella, sphenoid sinus, pituitary gland,

infundibulum, and suprasellar region on graphic
and post-gado. T1-WI
Pituitary: Normal Anatomy
Figure 5-20-1
Anterior Lobe
Lateral
PRL (10%-30%)
GH (50%)
Midline
ACTH (10%-30%)
TSH (5%)
FSH/LH (10%)
Location of adenomas parallels the distribution
Posterior Lobe
Infundibulum
Pituicytes (glial)
Axons
Vasopressin (ADH)
Oxytocin
Figure 5-20-2
Normal adult pituitary gland on sag T1-WI and cor

T2-WI
Figure 5-20-3
Pituitary: Normal Anatomy
Posterior Lobe
Posterior Pituitary Bright Spot (PPBS)
High SI on T1
Doesnt suppress on fat sat
Parasellar Region: Normal Anatomy [Figure 5-20-3]
Parasellar Structures
Cavernous Sinus
Cranial Nerves
III, IV, V1, V2, VI
Cavernous ICA
Optic Chiasm
Hypothalamus
Sphenoid Sinus
Sella and Parasellar Region
Normal coronal graphic through the

sella, including the pituitary gland,
suprasellar cistern, infundibulum, and
cavernous sinuses
1248
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Neuroradiology
Parasellar Region: Normal Anatomy
Figure 5-20-4
Parasellar Structures
Optic Chiasm
Hypothalamus
Tuber cinereum
Mamillary bodies
Sphenoid Sinus

[Figure 5-20-4]
Bony Structures
Planum sphenoidale
Tuberculum sellae
Sella turcica
Dorsum sellae

Sella and Parasellar Pathology
Differential Diagnoses
Intrasellar
Suprasellar
Infundibular
Skull base bony structures around the sella,

including the planum sphenoidale, tuberculum
sellae, sella turcica, and dorsum sellae
Intrasellar Pathology
Nonneoplastic Lesions
Hyperplasia (physiologic, end organ failure)
Cysts (RCC, pars intermedia cyst)
Lymphocytic hypophysitis
Primary Neoplasms
Pituitary adenoma (Most common)
Craniopharyngioma (Only 5% purely intrasellar)
Meningioma (Purely intrasellar rare)
Pituitary carcinoma (Extremely rare)
Metastasis (1%)
Pituitary Neoplasms
Adenoma
Prolactinoma 30%
Null cell 25%
GH 20%
ACTH 10%
FSH/LH 10%
PRL-GH 5%
Mixed, TSH 1%-5%
Incidental pituitary lesions are common (17%)
Figure 5-20-5
Graphic and post-gado T1-WI of small right

microadenoma
Sella: Pathology [Figure 5-20-5]
Pituitary Microadenoma
10 mm or less
10%-20% of autopsies
Micro >>> Macro
Convex margin
Stalk deviation
Sella floor thin
Neuroradiology
1249
1251
Figure 5-20-6
Sella: Pathology
Dynamic Imaging
Increases sensitivity (10% seen only on dynamic
MR)
Enhances slower than normal gland
Physiologic Hypertrophy
Maximum normal height

Pituitary hypertrophy in a patient with
6 mm infants and children
hypothyroidism
8 mm males, postmenopausal females
10 mm young women (convex superiorly)
12 mm late pregnancy, postpartum females (convex superiorly)
Abnormal hypertrophy
End-organ failure (esp. hypothyroid)
Figure 5-20-7
Neuroendocrine tumor (rare)
Pituitary Gland Hypertrophy [Figure 5-20-6]

Sella: Rathke Cleft Cyst
Clinical
Intrasellar 40%
Suprasellar extent 60%
3mm 3cm
Most incidental
Symptomatic
Pituitary dysfunction
Visual change, HA
Intrasellar Rathke cleft cyst, hyperintense on T1

non-contrast MR, with small intra-cyst nodule seen
on T2-WI
Rathke Cleft Cyst: CT
75% hypodense
25% iso/hyperdense
Ca++ rare
May be difficult to differentiate from other benign cysts or craniopharyngiomas
Rathke Cleft Cyst: MR
Imaging Features
Signal varies - cyst content
50%-60% T1 hyperintense
30%-40% follow CSF
75% intracystic nodule
+/- rim enhancement
Rathke Cleft Cyst [Figure 5-20-7]

During pregnancy or shortly after delivery

F >>> M
Pituitary insufficiency
H/A & visual changes
Amenorrhea or inability to lactate
Diffuse enlargement of adenohypophysis
May mimic hyperplasia or adenoma
1250
1252
Neuroradiology
Lymphocytic hypophysitis - Pathology
Diffuse infiltration of the adenohypophysis by lymphocytes & plasma cells

? Autoimmune
Infundibuloneurohypophysitis
Affects infundibulum & neurohypophysis
Thickened pituitary stalk
Diabetes insipidus
Other Intrasellar Masses Uncommon
Craniopharyngioma (5% intrasellar)

Metastasis (1% of sellar masses)
Aneurysm (medially-projecting from cavernous ICA)
Meningioma (rare purely intrasellar)
Suprasellar Masses: Five Most Common
75% of all sellar/parasellar masses

Pituitary macroadenoma (35%-50%)
Approximately 10% each
Meningioma
Aneurysm
Craniopharyngioma
Astrocytoma (hypothalamic-chiasmatic)
Figure 5-20-8
Suprasellar Differential Diagnosis
Adult Lesions
Pituitary Macroadenoma
Meningioma
Aneurysm
Pediatric Lesions
Craniopharyngioma
Chiasmatic / hypothalamic Glioma
Hypothalamic Hamartoma
Pituitary Macroadenoma [Figure 5-20-8]
Clinical / Pathologic
Most common suprasellar mass (50%)
10% of intracranial tumors
Snowman shape
Compressive symptoms
Rare in prepubescent children, adolescent males
Sella and suprasellar macroadenoma

with narrowing where lesion extends
through the diaphragma sellae
Suprasellar: Pathology
Macroadenoma
> 10mm
Enlarged sella turcica
Sellar/suprasellar
MR test of choice
Robust enhancement
? Cav. sinus invasion, mass effect on chiasm
Pituitary Adenoma
Prolactinoma
30% of adenomas
Female >> Males
Galactorrhea
Amenorrhea
Serum PRL > 150ng/mL
If > 1000ng/mL, cavernous sinus invasion
Neuroradiology
1251
1253
Pituitary Macroadenoma: CT
CECT
Enlarged sella turcica
Moderate to strong enhancement
May be heterogeneous (cysts, hemorrhage)
Figure 5-20-9
Pituitary Macroadenoma: MR [Figure 5-20-9]

Imaging Features
Isointense GM: T1, T2WI
May have hemorrhage, cystic components
Figure-eight, snowman
Robust but heterogeneous enhancement
Determining cavernous sinus invasion difficult
Pituitary Macroadenoma: MR
Cavernous Sinus Invasion
More aggressive
Cant be resected
> 2/3 surrounds ICA
ICA venous sulcus compartment filled
Invasive Pituitary Macroadenoma [Figure 5-20-10]
Pituitary macroadenoma with right

cavernous sinus invasion
May extend inferiorly into sphenoid sinus & skull base

Must differentiate from skull base primary tumor
Will involve bony sella turcica & pituitary gland
Figure 5-20-10
Invasive macroadenoma
Pituitary Apoplexy
1.
2.
Hemorrhage into tumor

Pituitary gland infarction
Acute onset
Headache, visual changes, vomiting
Usually hemorrhagic, may be non-hem
May be life threatening
Figure 5-20-11
Pituitary Apoplexy [Figure 5-20-11]

Suprasellar: Pathology
Meningioma
2nd most common (adults)
15% of meningiomas
Tuberculum sellae
Clinoid processes
Cavernous sinus
Look for pituitary gland distinct from mass
Sag images helpful
Pituitary apoplexy can be hemorrhagic or not
1252
1254
Neuroradiology
Suprasellar: Meningioma [Figures 5-20-12 and 5-20-13]
Figure 5-20-12
Suprasellar: Aneurysm
Third most common lesion in adults

Noncalcified suprasellar mass
Must differentiate from other suprasellar masses
Macroadenoma
Meningioma
Suprasellar: Aneurysm - CT
Noncalcified central suprasellar mass

Can be difficult to distinguish from adenoma,
meningioma
Suprasellar meningioma on T2 and post-contrast

T1 coronal images
Parasellar: Aneurysm - MRI [Figure 5-20-14]
Flow void or complex mass separate from pituitary

Phase artifact
Figure 5-20-13
Figure 5-20-14
Suprasellar meningioma with typical extension

along planum sphenoidale
Figure 5-20-15
Left parasellar ICA aneurysm, with

typical flow void
Suprasellar: Aneurysm
Suprasellar Mass: Adult [Figure 5-20-15]
Invasive macroadenoma vs. meningioma
Macroadenoma
Pituitary is mass
Enhancement
Meningioma
Pit separate
Marked C+
Dural tail
Aneurysm
Pit separate
Flow void
Complex SI
Figure 5-20-16
Suprasellar: Craniopharyngioma [Figure 5-20-16]
Clinical
Most common suprasellar mass in children
5-15 yrs
50-60 yrs
Visual changes
Endocrine dysfunction
Mass effect
H/A, N, V, papilledema
Neuroradiology
Graphic of craniopharyngioma,
depicting complex sellar and
suprasellar mass
1253
1255
Suprasellar: Craniopharyngioma
Figure 5-20-17
Pathology
Adamantinomatous
Classic
Crank-case oil in cysts
Papillary (Adults)
70% suprasellar with small sellar component
5% purely intrasellar
Craniopharyngioma: CT
NECT scan
Adamantinomatous
90% Ca++ (rim)
90% Cystic
May enlarge sella
Papillary type
50% Ca++
Majority solid
CECT scan
90% enhance
Solid
Nodular
Rim
Complex multiloculated sellar and suprasellar

craniopharyngioma
Craniopharyngioma: MR
Variable signal
Often heterogeneous
Ca++ difficult to detect
Nodular & rim enhancement
Occasionally optic tract hyperintensity on T2WI mass effect
Craniopharyngioma: MR [Figure 5-20-17]

Chiasmatic-hypothalamic glioma
Clinical
Second most common suprasellar mass in children
Often large at presentation
H/A, visual, endocrine abnormalities common
M=F
15%-30% have NF-I
Chiasmatic-hypothalamic glioma
Pathology
30% of all pilocytic astrocytomas occur in chiasm or hypothalamus
75% Pilocytic astrocytoma
25% Low-grade fibrillary
Long-term survival (90% at 5 yrs, 75% at 10 yrs)
Figure 5-20-18
Chiasmatic-hypothalamic glioma - MR
Variable signal
Iso-, hypointense on T1WI
Spread along optic tracts common
Chiasmatic-hypothalamic glioma [Figure 5-20-18]

T2 and post contrast T1 axial images show a nonenhancing hypothalamic/chiasmic glioma
1254
1256
Neuroradiology
Hypothalamic Hamartoma [Figure 5-20-19]
Figure 5-20-19
Clinical
Precocious puberty
Usually < 2yrs
Gelastic seizures
M>F
Pallister-Hall
Facial anomalies
Polydactyly
Imperforate anus
Graphic and post contrast T1 sagittal image

through hypothalamic hamartoma
Pathology
Hamartoma of tuber cinereum
Congenital nonneoplastic heterotopia
Between infundibular stalk, mamillary bodies
Figure 5-20-20
Hypothalamic Hamartoma : MR
Signal follows GM
Isointense on T1WI
May be slightly T2 hyperintense
Pedunculated or sessile
May project into 3rd ventricle
Do not enhance
Comparison of suprasellar pediatric lesions
Suprasellar Mass: Child [Figure 5-20-20]
Cranio
Complex mass
90% cystic
90% calcified
Astrocytoma
Chiasm/Hypoth
T2 hyperintense
Variable C+
Hamartoma
Hypothalamus
GM signal
No C+
Infundibulum Differential Diagnosis
Figure 5-20-21
Lesions
Germinoma
Sarcoid
Lymphoma, Metastasis
Rare Lesions
Hypophysitis
Pituicytoma
Infundibular: Germinoma [Figure 5-20-21]
Clinical
Suprasellar region is 2nd most common site
M = F suprasellar
90% present < 20 yrs
Endocrine dysfunction
Diabetes insipidus
Panhypopituitarism
Very radiosensitive
Up to 90% 10 survival
Neuroradiology
Graphic of infundibular lesion

1255
1257
Germinoma
Pathology
Pineal most common
Pineal + suprasellar 10%
Similar to seminoma
2/3 of germ cell tumors are germinoma
Germinoma: Imaging
CT & MR
Combined lesion typical but may affect only infundibular stalk
May be hyperdense (CT)
Isointense T1WI
Hyper- to isointense T2WI
Enhances homogeneously
CSF dissemination common
Germinoma: MR
Figure 5-20-22
Clinical
First decade
M>F
Diabetes insipidus
High signal of neurohypophysis is commonly
absent
Thickening of stalk
Formerly Histiocytosis X
Langerhans Cell Histiocytosis [Figure 5-20-22]

Sarcoid
Clinical
Chronic, multisystem, inflammatory disease
Noncaseating granulomas
Neurologic findings 5%
Diabetes insipidus or hormone deficiency
Steroid responsive
Coronal and sagittal images show typical stalk
thickening and enhancement of LCH
Sarcoid
Lymphoma [Figure 5-20-23]
Clinical
NHL (B-cell)
90% supratentorial
Pituitary gland, hypothalamus, stalk
6th - 7th decade
AIDS: 4th decade
Figure 5-20-23
Lymphoma
Imaging
Pituitary gland, hypothalamus, stalk
Hyperdense on CT
T1 Iso- to hypointense
T2 hypointense
Homogeneous enhancement
Lymphoma
1256
1258
Neuroradiology
Metastasis
1% of sellar/parasellar masses
Usually occurs with known primary
Can involve third ventricle, hypothalamus, infundibular stalk
May be both supra-, intrasellar
Figure 5-20-24
Metastasis: Pituitary Gland

Metastasis:Infundibulum
Infundibular Mass: Adult
Sarcoid
Systemic dz
Thickened stalk
Enhancement
Lymphoma
+/- Systemic dz
Stalk or gland
Enhancement
Differential diagnosis of infundibular mass in a child.
Infundibular Mass: Child [Figure 5-20-24]
LCH
Thickened stalk
Bright spot gone
Enhancement
Germinoma
Stalk +/- pineal
T2 hyperintense
CSF spread
Meningitis
Meningeal
Diffuse
Enhancement
Presentation Summary
Intrasellar Mass
Microadenoma, Rathke cleft cyst
Suprasellar Mass
Craniopharyngioma, Macroadenoma, Meningioma, Aneurysm
Infundibular Lesion
Germinoma, LCH
Granulomatous disease, LH
References
1.
2.
3.
4.
5.
6.
7.
Bonneville JF, Cattin A, Racle A, et al: Dynamic CT of the laterosellar extradural venous spaces. AJNR 1989; 10:
535-542
Cottier J-P, Destrieux C, Brunereau L, Bertrand P, Moreau L, Jan M, Herbreteau D. Cavernous sinus invasion by
pituitary adenoma: MR imaging. Radiology 2000; 215:463-469
Elster AD, Chen MYM, Williams DW III, et al: Pituitary gland: MR imaging of physiologic hypertrophy in
adolescence. Radiology 1990;174: 681-685
Elster AD, Sanders TG, Vines FS, et al: Size and shape of the pituitary gland during pregnancy and post partum:
measurement with MR imaging. Radiology 1991; 181 :531-535
Elster AD. Modern imaging of the pituitary. Radiology 1993; 187: 1-14
FitzPatrick M, Tartaglino LM, Hollander MD, Zimmerman RA, Flanders AE. Imaging of sellar and parasellar
pathology. Radiol Clin North Am 1999;37:101-121
Sato N, Tanaka S, Tateno M, Ohya N, Takata K, Endo K. Origin of posterior pituitary high intensity on T1weighted magnetic resonance imaging: immunohistochemical, electron microscopic, and magnetic resonance
studies of posterior pituitary lobe of dehydrated rabbits. Invest Radiol 1995; 30:567-571
Neuroradiology
1257
1259
Congenital Spinal Anomalies

Figure 5-21-1
Spinal Dysraphism [Figure 5-21-1]
Defect of closure of neural tube

For defects of primary neurulation
Involving tubulation of neural plate, separation
from ectoderm, disjunction of superficial from
neural ectoderm
Myelomeningocele [Figures 5-21-2 and 5-21-3]
Failure of neurulation & placode elevation from

expansion of SAS
Placode protrudes through osseous & cutaneous
defect
Most LS, also more proximal, normal appearing
distal cord
Neural tube development. Neural plate and neural
> = 4 sites initiating neurulation, site that fails
crest
derive from midline ectoderm, notochord and
determines defect location
somites arise from midline mesenchyme
Figure 5-21-2
Figure 5-21-3
Axial schematic of
lumbar MMC with
placode forming dorsal
wall of expanded CSF
space
Sagittal MR (22 weeks

gestation) showing posterior
osseous defect and neural
tissue traversing expanded
CSF space
1258
1260
Neuroradiology
Myelocele (Myeloschisis) [Figure 5-21-4]
Figure 5-21-4
Placode of OSD in plane with back

Less common, embryologically similar
Clinical signs & function similar
HemiMMC/Hemimyelocele
Canal split, only one hemicord affected

Rare, crucial to recognize as septum can tether cord --> decline
in function after repair
Present with markedly asymmetrical neurological abnormalities
Chiari II Malformation [Figure 5-21-5]
Absence raises suspicion that MMC really terminal

myelocystocele or lipoMMC
From poor distention rhombencephalic vesicle, herniation CSF
leakage
Not assoc with CSD, may develop in TM
Axial lumbar myelocele with placode

at level of skin defect
Figure 5-21-5
CSD with subcutaneous mass
Lipoma with dorsal defect (lipoMMC/lipomyelocele(schisis))

Terminal myelocystocele
Meningocele
Cervical myelocystoceles & meningoceles extremely rare
Lipoma with Dorsal Defect
Premature disjunction of cutaneous ectoderm from neuroectoderm

allows mesenchyme to contact inner portion of neural tube
As tube tries to close, mesenchyme --> fat, interferes with neurulation
Lipomas contain ectodermal, mesodermal, endodermal elements =
teratomas
Grows in proportion to overall adipose, rarely with AVM, retether
vs MMC
Lipomyelomeningocele [Figure 5-21-6]
Lipoma outside canal, expanded SAS

Placode deformed, with rotation toward lipoma & protrusion of
meninges contralaterally
Nerve roots deformed, short on side of lipoma (tether cord),
elongated on side of meninges
Sagittal MR (22 weeks

gestation) with small posterior
fossa, hindbrain herniation,
loss of supratentoral CSF
spaces
Lipomyelocele (lipomyeloschesis) [Figure 5-21-7]
Lipoma traverses defect to attach to placode within or along edge of canal
Figure 5-21-6
Figure 5-21-7
Axial lumbar lipomyelomeningocele

with asymmetrical placode/lipoma
interface outside canal
Neuroradiology
Axial lumbar lipomyelocele with

placode/lipoma interface along edge
of canal
1259
1261
Meningocele [Figure 5-21-8]
Meningeal-lined CSF sac protruding through defect

Cord does not enter sac, may be assoc with hypertrophy of filum
or cord tethering
? From CSF pulsations
Lateral assoc with NF 1, also post-trauma, connective tissue
disorders
Figure 5-21-8
Terminal Myelocystocele [Figure 5-21-9]
Large, skin covered lumbosacral mass

Dilatation of terminal ventricle
Herniates through SB
High assoc with caudal cell mass anomalies (GU, lower GI, abd
wall)
Cb herniation late
Incontinent, extremely poor LE function
Figure 5-21-9
Thoracic meningocele
Figure 5-21-10
Schematic of terminal
myelocystocele. The expanded CSF
spaces are separate from the
markedly dilated terminal ventricle.
CSD w/o Subcutaneous Mass
Simple Dysraphic States

Posterior spina bifida
Intradural, intramedullary, filum terminale lipoma
Persistent terminal ventricle
Cutaneous stigmata: dimple, hemangioma, hair
4.8% nl neonates (74% simple dimple, No SB)
Atypical dimples = high risk
Larger than 5mm
> 2.5cm cephalad to anus
Hemangiomas, hairy patches, tails
Posterior Spina Bifida
Most basic, commonly encountered

L5 or S1, in isolation or with CSD when cord tethered
Posterior arch of L5 can remain unfused until 5-6 yrs
Intraspinal Lipoma [Figure 5-21-10]
Mesodermal cells contact primitive ependyma

LS, any level
Usually intradural, rarely entirely intramedullary
Intradural, extramedullary lipoma

1260
1262
Neuroradiology
Fibrolipomatous Infiltration of Filum [Figure 5-21-11]
Figure 5-21-11
Anomaly of secondary neurulation, from totipotential caudal cell

mass
Axial T1 most sens
?Small amount of fat anatomical variant vs any fat or
thickening (>2mm) = tether
Tight Filum Terminale [Figure 5-21-12]
Impaired retrogressive diff --> short, hypertrophic filum

Conus low, assoc with SB, scoliosis, dermal sinus
Axial T2 for detection
Figure 5-21-12
Fibrous thickening of filum terminale seen as

hypointense on axial T2
Fat deposition within filum terminale
Figure 5-21-13
Persistent Terminal Ventricle [Figures 5-21-13 and

5-21-14]
Incomplete regression of TV of secondary

neurulation, continuity with central canal ? small
cavity
PTV vs terminal myelocystocele (--> severe
manifestation from inability of CSF to escape)
Common, transient finding until 5 yrs
Identical to CSF, in conus or conus-filum transition
Anatomic variant, no clinical sig, but > 4-5mm can -> pain & neuro signs
Complex Dysraphic States
Disruption during gastrulation --> notochordal

derangement
Dorsal enteric fistula
Neurenteric cysts
Split cord malformation
Dorsal dermal sinus
Caudal regression syndrome
Segmental spinal dysgenesis
Development of distal spine from caudal cell mass

via retrogressive differentiation
Figure 5-21-14
Gastrulation [Figure 5-21-15]
Cells migrate towards primitive streak, through primitive groove --> endoderm
& mesoderm
Prospective notochordal cells in cranial margin of Hensen node become
notochordal process
Dorsal Enteric Fistula
Most multifaceted, failure of notochordal integration

Persistence of neurenteric canal --> cleft from bowel to dorsal skin surface,
Terminal ventricle on
through vertebral column & spinal canal
axial ultrasound through
Usually lumbar
conus medullaris
High assoc with other CNS & non-CNS anomalies (renal, GI, CDH, pulmonary)
Neuroradiology
1261
1263
Neurenteric Cyst [Figure 5-21-16]
From partial regression of neurenteric canal

Lined by secretory epith, contents iso to CSF or proteinaceous
Intraspinal, ventral to T cord, anywhere
Assoc with dysplastic vert > GI, resp anom
Present late teens, compressive signs & sx
Figure 5-21-15
Split Cord Malformation [Figure 5-21-17]
Diastematomyelia (splitting) & diplomyelia (duplication), not

radiologically distinguishable
Type I: less common, osseous septum dividing two dural tubes
& hemicords, assoc vertebral anomalies
Type II: more common, hemicords in single dural tube
fibrous septum
Each hemicord contains a central canal, dorsal horn & ventral
horn, each --> 1 nerve root
Can be asymmetrical, smaller easily missed
Septum --> tethering, ?SCM at CTJ under-recognized
Migration of prospective notochordal
Despite lack of s/sx, ~ 75% abnl voiding
cells during gastrulation
Cutaneous stigmata (Type I), F > M
Present: scoliosis, ULE weak, wasting, tether
Figure 5-21-16
8%-45% of OSD, separate from site of non-neurulation --> rarity
of hemiMMC
Dorsal Dermal Sinus [Figures 5-21-18 and 5-21-19]
Epith-lined tube from dorsal skin to cord/coverings

Focal nondisjunction neuroectoderm & cutaneous ectoderm
Risk for bacterial infxn
Epithelium secretes squamous debris, can exude cheesy material
P/w mass or recurrent infxn
10% intraspinal dermoid
Figure 5-21-18
Thoracic osseous anomalies seen

with neurenteric cyst
Figure 5-21-19
Figure 5-21-17

Scoliosis and hairy patches indicating

underlying split cord malformation

1262
1264
Neuroradiology
Caudal Regression Syndrome [Figure 5-21-20]
Spectrum from coccygeal/LS hypogenesis --> sirenomelia

Infants of diabetic mothers, 1 in 7500 live births
Assoc with:
OEIS (omphalocele, exstrophy, imperforate anus, spinal
anomalies)
VACTERL (vertebral, renal, cardiac, limb anomalies with
anorectal atresia & TEF)
Currarino triad (sacral hypogenesis, anorectal malformations,
presacral teratoma or meningocele)
Level determines type & severity
Type I: < =S1, even mid-T, cord terminates high, blunted tip &
deformation of cauda common, ant & post separation of roots
Type II: > = S2, less severe, distal-most conus absent,
tethered by tight filum, lipoma, or CSD with subQ mass
Mild CRS: only tip of conus absent, cord not tethered, may be
missed
Figure 5-21-20
Segmental Spinal Dysgenesis
Focal segment of lumbar or thoracic spine agenetic-markedly

hypogenetic, cord segmentally disrupted, distal canal unaffected
Distal cord large, focal kyphosis --> early presentation
Anomalous lower extremities, incontinence
? Within CRS, morphology depends on level of notochordal
disruption
Distal --> CRS, Proximal --> SSD
Frequency of CRS (11:1), indicates higher degree of
susceptibility of the caudal cell mass to derangement
Blunted conus and absent distal

sacrum/coccyx of caudal regression
syndrome
References
1.
2.
3.
4.
Barkovich AJ. Pediatric Neuroimaging. 4th Ed. Lippincott, Williams & Wilkins, Philadelphia 2000.
Dias MS, Partington M. Embryology of myelomeningocele and anencephaly. Neurosurg Focus 2004; 16:E1.
Ellison D, Love S, Chimelli L, Harding BN, Lowe J, Vinters HV. Neuropathology: A Reference Text of CNS
Pathology. 1st ed. Mosby International Ltd, London 1998.
Tortori-Donati P, Rossi A, Cama A. Spinal dysraphism: a review of neuroradiological features with embryological
correlations and proposal for a new classification. Neuroradiology 2000; 42:471-491.
Neuroradiology
1263
1265
Imaging of the Suprahyoid Neck:

Superficial, Parapaharyngeal and Carotid Spaces
Wendy R. K. Smoker MS, MD, FACR
Figure 5-22-1
Cervical Fascia [Figure 5-6-1]
Superficial Cervical Fascia

Fat-filled layer of connective tissue that completely surrounds
the neck and permits the skin to glide easily over deeper
structures
Deep Cervical Fascia
Superficial Layer (Investing Fascia)
Middle Layer (Visceral or Pre-tracheal Fascia)
Deep Layer (Perivertebral Fascia)
Fascial Spaces of the Suprahyoid Neck
Superficial Space
Parapharyngeal Space
Carotid Space
Masticator Space
Parotid Space
Pharyngeal Mucosal Space
Retropharyngeal/Danger Space
Perivertebral Space
Posterior Cervical Space
Superficial Space-Contents
Sternocleidomastoid muscle
Trapezius muscle
Platysma muscle
Lymph nodes
Blood vessels/EJV
Hair follicles
Fat
Hemangioma
Figure 5-22-2
Superficial Space-Pathology
Hair follicles
Sebacceous cyst
Blood vessels:
EJV thrombosis
Hemangiomas/vascular malformations
Lymph nodes
Reactive/suppurative adenopathy;
Nodal metastases
Fat
Lipoma/liposarcoma
Pseudomass
Fibromatosis coli
Hemangiomas [Figures 5-22-1 and 5-22-2]
Most common head and neck TUMOR of infancy and

childhood
Rarely present at birth but manifest in early infancy,
grow slowly, and involute by adolescence
80% are isolated lesions
Females > males
Large facial hemangioma: Isointense on T1WI,
Isointense on T1WI, hyperintense on T2WI
hyperintense on T2WI, and intense enhancement
+Enhancement
Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces
1266
Neuroradiology
Lymphatic Malformations [Figures 5-22-3 and 5-22-4]
Figure 5-22-3
Arise from sequestrations of the primitive embryonic

yolk sac
Classified according to lymphatic vessel size
(capillary, cavernous, cystic) Largest is the cystic
variety, previously termed cystic hygroma.
Typically multiseptated
Fluid-fluid levels within the lesions are almost
pathognomonic
Madelungs Disease
Almost exclusively a disease of middle aged

alcoholic males
50 years of age
Appearance of lesions is preceded by 10 years of
heavy drinking
Non encapsulated fatty masses
SIGHT DIAGNOSIS
A large lymphatic malformation with multiple

septations
Liposarcoma
Slowly enlarging, painless, non-ulcerated mass

Middle-aged onset
Most arise de novo; frequently arise from the stroma rather than the
submucosal or subcutaneous fat
WHO classification recognizes 5 categories of
liposarcomas:
Well differentiated (adipocytic, sclerosing, and
inflammatory subtypes)
Dedifferentiated
Myxoid
Round cell
Pleomorphic
Figure 5-22-4
Fibromatosis Coli [Figure 5-22-5]

(Stenocleidomastoid Tumor of Infancy)
Diffuse SCM enlargement

Most common type of congenital torticollis
Fibrocollagenous infiltration-Cause?????
Typical course:
Not detected at birth
Palpable neck mass at 2-4 weeks of age
Increases in size for a few weeks (growth phase)
Most recede spontaneously at 4-8 months of age
This is a LEAVE ALONE lesion
Parapharyngeal Space (PPS)
2 year-old child with a large, mixed, venolymphatic malformation
Figure 5-22-5
[Figure 5-22-6]
Is an in-between space lying between other

fascially-defined spaces. Not fascially-defined itself.
Few lesions actually arise within this space but
typically originate from surrounding spaces and
produce characteristic encroachment on the PPS fat.
Can thereby define the space of origin.
The parapharyngeal space can be considered as
consisting of two compartments:
Prestyloid = Parapharyngeal Space (PPS)
Retrostyloid = Carotid Space (CS)
Fibromatosis coli
Neuroradiology
1267
Parapharyngeal Space - Contents
Figure 5-22-6
Fat
Branches of the mandibular nerve (V3)
Internal maxillary artery branches
Ascending pharyngeal artery
Pharyngeal venous plexus
Ectopic salivary gland tissue
Parapharyngeal Space - Pathology

[Figures 5-22-7 to 5-22-11]
Pseudomass
Asymmetric pterygoid plexus of veins
Congenital/Vascular
Atypical second BCC, hemangioma,
The normal in-between location of the PPS,
lymphangioma, aneurysm
colored in the diagram. T1W MR images
Inflammatory
optimally demonstrate the symmetric, fat Cellulitis/abscess
filled PPS spaces.
Benign Tumor
Pleomorphic adenoma from ectopic salivary
gland rests, neurogenic tumor, lipoma
Malignant Tumor
MECa and ACCa from ectopic salivary gland rests, direct spread from
malignancies in surrounding spaces, liposarcoma, distant mets (rare)
Figure 5-22-7
Figure 5-22-9
Small PPS hemangioma (arrows) demonstrates calcification on

the CT image. The lesion is iso-intense on T1WI,
hyperintense on T2WI, and exhibits intense enhancement
Figure 5-22-8
Small PPS
lymphangioma
(arrows) is isointense on T1WIs
and demonstrates a
fluid-fluid level on
the T2WI
PPS odontogenic abscess (arrows) in

a 17 year-old male, status-post
left third molar removal 5 days
ago, now with facial pain and
swelling
1268
Neuroradiology
Figure 5-22-10
Figure 5-22-11
Pleomorphic adenoma, well-centered within the PPS, is

clearly separated from the deep lobe of the parotid
gland (arrows)
Predominantly PPS lipoma. Since the PPS is not

fascially-defined, intrinsic pathology is free to
extend along fascial planes, as can be seen
with this lipoma
Carotid Space - Contents
Internal carotid artery

Cranial nerves (IX-XII)
Sympathetic Chain
Internal jugular vein
Deep cervical (internal jugular) lymph node chain
Carotid Space - Pathology
Pseudomass
Ectatic CCA or ICA, asymmetric IJV (can mimic a vascular tumor)
Congenital
Second branchial cleft cysts
Inflammatory
Cellulitis/abscess, adenopathy
Vascular Lesions
IJV thrombosis or thrombophlebitis, ICA thrombosis, aneurysm, or
dissection
Benign Tumor
Paragangliomas (jugular, vagal, carotid body), nerve sheath tumors,
meningioma (from jugular foramen)
Malignant Tumor
SCCa nodal metastases, direct invasion by SCCa, NHL,
other nodal mets
Figure 5-22-12
Second Branchial Cleft Cyst [Figure 5-22-12]
The most common of the branchial cleft cysts

66%-75% in children
Classic location at, or just caudal to, the angle of mandible (but
may present in a variety of locations):
Posterior to the submandibular gland
Anterior to the SCM
Lateral to the carotid space
Occasionally see a beak with the cyst pointing medially
between the ECA and ICA (track)
Often enlarge with URIs and become painful if infected
ICA Dissection with Pseudoaneurysm [Figure 5-22-13]
Neuroradiology
1269
Classic displacements produced by

second branchial cleft cysts:
Anterior displacement of the
submandibular gland (SMG),
medial displacement of the
carotid space (CS) structures,
and posterior displacement of the
sternocleidomastoid muscle
(SCM)
Paragangliomas
Highly vascular tumors arising from non-chromaffin

cells of neural crest origin
Usually asymptomatic from endocrine standpoint but
rare catecholamine-secreting lesions do occur
No resemblance to true glomus tumors found in skin
and superficial soft tissues so term glomus tumor
should be avoided
Familial and non-familial patterns exist with
synchronous lesions seen in 5-8% of non-familial
cases and up to 25% of familial cases
Highly vascular Salt and Pepper appearance of
larger lesions on MR suggestive in correct locations
Both regional and distant (lung, liver) metastatic
disease are reported in 10-15% of cases.
Etiology is unclear-probably due to hypoxic stimuli
Four common locations:
Middle ear cavity-tympanicum paragangliomas
Jugular foramen-jugular paragangliomas
High carotid space-vagal paraganliomas
Carotid bifurcation-carotid body paragangliomas
Jugular Paragangliomas [Figure 5-22-14]
Figure 5-22-13
Hemorrhage in the wall of the vessel is bestdemonstrated on non-contrast T1WI (arrow).

The angiogram demonstrates the dissection
to be maximal just below the base of the skull
and shows a pseudoaneurysm at the level of
approximately C1 (arrow)
Arise in adventitia of IJV, from Arnolds nerve (IX) and

Jacobsons nerve (X)
Represent the most common tumor found in the jugular foramen
Permeative erosive changes with amputation of the jugular spine
demonstrated on CT
Multicentric in 5% of sporadic cases and up to 25%
in familial cases
Sx: Pulsatile tinnitus; IX-XI cranial neuropathy +/- XII
Malignant with mets in approximately 3%
Figure 5-22-15

Figure 5-22-14
High carotid space meningioma (arrows) is isointense on T1WI, mixed intensity on T2WI,
and markedly enhances. Calcification
demonstrated on CT mitigates against
consideration of a paraganglioma
A right jugular paraganglioma extends inferiorly into the

high carotid space (arrows). Prominent flow-voids
are seen on the T1WIs. The lesion erodes the
jugular tubercle and fills the hypoglossal canal, the
normal counterparts labeled on the left (arrows).
Note late chronic denervation atrophy of the right
hemitongue
1270
Neuroradiology
Vagale Paragangliomas [Figure 5-22-16]
Figure 5-22-16
Arise from paraganglia located around nodose

ganglion, the more caudal of the two vagal ganglia
Situated just below skull base, lower than typical
jugulare lesions and higher than typical carotid body
tumors
Usually lie entirely within carotid space (post-styloid
parapharyngeal space)
As vagus nerve lies dorsal to ICA, these tumors
usually displace ICA anteriorly
Approximately 10% incidence of malignancy
Figure 5-22-17
Large vagal paraganglioma
displaces the ICA
anteriorly with the ECA
(arrows). Large flow
voids are seen. The
MRA optimally
demonstrates the
anterior vascular
displacements (arrows)
Enhancing lesion in the high carotid space, which

displaces the ICA anteriorly (arrows), suggests
a vagal paraganglioma. However,
identification of associated calcification and
sclerosis (arrows) of the jugular tubercle, and
lack of destruction, makes the diagnosis of
meningioma
Neuroradiology
1271
Figure 5-22-18
Carotid space vagal
schwannoma
displaces the
ICA anteriorly
(arrows). The
lesion is very
homogeneous
and exhibits no
flow voids
Figure 5-22-19
Carotid Body Paragangliomas
[Figures 5-22-19 and 5-22-20]
Arise from paraganglia located in the crotch of the

carotid bifucation-most common location
Pathognomonic finding is splaying of the ECA and
ICA and filling the bifucation
Multiple in 5%-14% of sporadic cases and up to 33%
in familial cases
Sx: Only 8% of these lesions are large enough to
present as carotid space mass; may have X and/or
XII neuropathy
Malignant in 10%-15% of cases
Classic displacements associated with carotid

body paraganglioma with splaying of the ECA
and ICA (arrows)
Figure 5-22-20
Patient with large ipsilateral

vagal and jugular
paragangliomas
1272
Neuroradiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
Ablin DS, et al. Ultrasound and MR imaging of fibromatosis colli (sternomastoid tumor of infancy). Pediatr
Radiol. 28(4):230-233, 1998.
Ahuja A, et al. Madelung disease: Distribution of cervical fat and preoperative findings at sonography. MR, and
CT. AJNR Am J Neuroradiol. 19(4):707-710, 1998.
Alkadhi H, et al. Evaluation of topography and vascularization of cervical paragangliomas by magnetic resonance
imaging and color duplex sonography. Neuroradiology. 44(1):83-90, 2002.
Bancroft LW, et al. Imaging characteristics of spindle cell lipoma. AJR Am J Roentgenol. 181(5):1251-1254,
2003.
Bousson V, et al. Dissections of the internal carotid artery: Three-dimensional time-of-flight MR angiography and
MR imaging features. AJR Am J Roentgenol. 173(1):139-143, 1999.
Eldevik OP, et al. Imaging findings in schwannomas of the jugular foramen. AJNR Am J Neuroradiol.
21(6):1139-1144, 2000.
Fruin ME, et al. The carotid space in the suprahyoid neck. Seminars Ultrasound CT MR 11:504-510, 1990.
Gilbert MR, et al. Meningioma of the jugular foramen: Glomus jugulare mimic and surgical challenge.
Laryngoscope. 114(1):25-32, 2004.
Gilmer-Hill HS, et al. Neurogenic tumors of the cervical vagus nerve: Report of four cases and review of the
literature. Neurosurgery. 46(6):1498-1503, 2000.
Harnsberger et al. Diagnostic Imaging: Head and Neck. Amirsys Publishers, 2005.
Heis HA, et al. Carotid body tumors. Int Surg. 88(4):226-230, 2003.
Koeller KK, et al. Congenital cystic masses of the neck: Radiologic-pathologic correlation. RadioGraphics.
19(1):121-146, 1999.
Macdonald AJ, et al. Primary jugular foramen meningiomas: Imaging appearance and differentiating features.
AJR Am J Roentgenol. 182(2):373-377, 2004.
Mafee MF, et al. Glomus faciale, glomus jugulare, glomus tympanicum, glomus vagale, carotid body tumors, and
simulating lesions: Role of MR Imaging. Radiol Clin North Am. 38(5):1059-1076, 2000.
Nadgir RN, et al. Simultaneous bilateral internal carotid and vertebral artery dissection following chiropractic
manipulation: Case report and review of the literature. Neuroradiology. 45(5):311-314, 2003.
Noujaim SE, et al. Paraganglioma of the temporal bone: Role of magnetic resonance imaging versus computed
tomography. Top Magn Reson Imaging. 11(2):108-122, 2000.
Rao AB, et al. From the archives of the AFIP. Paragangliomas of the head and neck: Radiologic-pathologic
correlation. Armed Forces Institute of Pathology. RadioGraphics. 19(6):1605-1632, 1999.
Sharma S, et al. Fibromatosis colli in infants: A cytologic study of eight cases. Acta Cytol. 47(3):359-362, 2003.
Snitzer EL, et al. Magnetic resonance imaging appearance of fibromatosis colli. Magn Reson Imaging.
15(7):869-871, 1997.
Neuroradiology
1273

Masticator and Parotid Spaces
Figure 5-22-21
Masticator Space - Contents

[Figure 5-22-21]
Muscles of Mastication
Lateral and medial pterygoid muscles
Masseter muscle
Temporalis muscle
Inferior alveolar nerve (branch of V3)
Inferior alveolar artery and vein
Ramus and posterior body of the mandible
Masticator Space
Normal Anatomy Coronal Plane
[Figure 5-22-22]
Figure 5-22-22
Normal anatomy of the masticator space. Axial images
best demonstrate muscles within this space. The coronal
and parasagittal images demonstrate the mandibular
division of the trigeminal nerve (V3) (arrow) and are best to
assess for perineural tumor
Figure 5-22-23
The fascia attaches to the skull base

MEDIAL to the foramen ovale putting
the foramen within this fascial
compartment. The cortical margins of
the foramen ovale are arrowed. V3 is
well-visualized traversing the foramen
There is volume loss and

complete fatty replacement
of the muscles innervated by
V3, including those
innervated by the mylohyoid
branch (mylohyoid and
anterior digastric muscles).
The responsible
meningioma is indicated by
the arrow
Suprahyoid Neck: Masticator and Parotid Spaces
1274
Masticator Space - Pathology
Figure 5-22-24
[Figures 5-22-23 to 5-22-32]
Pseudotumors
Denervation atrophy patterns
Benign masseteric hypertrophy
Accessory parotid tissue
Congenital Lesions
Hemangiomas/Lymphangiomas
Vascular
Aneurysm
Inflammatory/Infection
Odontogenic abscess, with or without, osteo is most common
Benign masseteric hypertrophy
Benign Neoplasms
Note benign-appearing enlargement
Lipomas
of the right masseter muscle. Not
Neurogenic tumors
infrequently, this is perceived as a
Aggressive fibromatosis (Desmoid)
parotid mass
Hemangiopericytomas
Malignant Neoplasms
Malignant schwannomas
Figure 5-22-26
Chondrosarcoma
Osteogenic sarcoma
SCCa spread from contiguous spaces
Osseous mets to mandible
(Long Parotid Tails and) Accessory Parotid

Tissue
[Figure 5-22-25]
Encountered in approximately 20% of the population

May be unilateral or bilateral
Has separate ductal system draining into Stensens
duct
Neoplastic involvement is uncommon, but when it
does occur, 50% of lesions are malignant (as
oppossed to main parotid gland in which majority of
tumors are benign)
Figure 5-22-25
Masseter hemangioma (arrows): Isointense on

T1WI, hyperintense on T2WI and exhibits
enhancement
Figure 5-22-27
The right parotid tail is covering the entirety of the

masseter muscle (arrows). Patient complained of a
right cheek mass
Severe masticator space infection spreading up
along the temporalis muscle (arrows on coronal
image) with medial pyerygoid muscle abscess
(arrows on axial images). The elderly male had
suffered a fall, complained of very severe
headaches, and was suspected of having a SDH.
PMH indicated he had a dental procedure 2
months prior to his fall!
Neuroradiology
1275
Masticator Space Infections
Figure 5-22-28
[Figure 5-22-27]
Most common cause is seeding from infected tooth following dental

manipulation
In patients s/p zygomatic arch wiring for treatment of facial fractures, evaluate
the suprazygomatic MS
Check bone windows for mandibular osteomyelitis
Check all spaces for pus pockets
Generally, one drain is necessary for each fascial space involved
Benign Neoplasms
[Figure 5-22-28]
Neurogenic Tumors
Aggressive Fibromatosis (Extraabdominal Desmoid)
Lipomas
Aggressive Fibromatosis [Figures 5-22-29]
Extra-abdominal desmoid tumor of fibrous origin

More aggressive than their abdominal counterparts-infiltrate muscles and
encase vessels and nerves
High recurrence rate after incomplete resection
Isointense to muscle on T1WI
Variable T2 signal depending on amount of fibrous tissue and collagen
Enhance after contrast
Figure 5-22-29
Massive enlargement of
the right foramen ovale
(arrows) produced by a
large V3 schwannoma
traversing the foramen
Aggressive fibromatosis. CT demonstrates loss of the fat

planes within the left masticator space and bowing of the
posterolateral wall of the maxillary sinus (arrow). The lesion
(predominantly replacing the lateral pyerygoid muscle) is
isointense on T1WI, very hypointense on T2WI (reflecting
the very fibrous content) and demonstrates mild
enhancement (arrows)
1276
Neuroradiology
Malignant Neoplasms
[Figures 5-22-30 to 5-22-32]
Malignant schwannoma
Non-Hodgkin lymphoma
Chondrosarcoma
Osteogenic sarcoma
SCCa spread from contiguous spaces
Metastases (usually mandibular)
Figure 5-22-30
Non-Hodgkin lymphoma. There is loss of the fat planes within the masticator space, including
enlargement and infiltration of the temporalis muscle in the suprazygomatic masticator space
(arrow). The right pterygopalatine fossa is enlarged indicating V2 perineural extension.
Involvement of the extraconal orbit and extra-axial middle cranial fossa are evident. Involvement
of the lateral pterygoid and temporalis muscles are evident (arrows) and extension along V2 and
V3 is noted (open arrows)
Figure 5-22-32
Figure 5-22-31
T1 WI
Chondrosarcoma - This lesion replaces fat at the

entrance to the inferior alveolar canal, widens the
foramen, and replaces the normal fatty marrow
(colored arrows). The normal fat at the entrance
to the inferior alveolar canal on the left is indicated
by the white arrows
Neuroradiology
Mandibular breast metastasis There is almost complete destruction

of the right mandible in this 42 yearold woman presenting with trismus.
This was the INITIAL manifestation
of her breast carcinoma
1277
Parotid Space - Contents
Figure 5-22-33
[Figures 5-22-33 and 5-22-34]
Parotid gland and duct (Stensons duct) coursing through buccal

space
Facial nerve (VII): Creates a surgical plane that divides parotid into
superficial and deep lobes; Not usually identifiable on either CT or
MR; Courses immediately lateral to retromandibular vein
Retromandibular vein (lateral to ECA)
External carotid artery
Intraparotid lymph nodes: 20-30 nodes; first order drainage for adj
scalp, EAC, and deep face
Parotid Space - Pathology [Figure 5-22-36]
Pseudomass
Stylomandibular tunnel - Superficial
Long parotid tails, accessory parotid glands, parotid agenesis
parotid space pathology may push
Congenital
through and enlarge the
First branchial cleft cyst; hemangiomas; lymphangiomas
stylomandibular
tunnel (double
Inflammatory / Infection
headed
arrow)
and
compress the
Abscess or cellulitis; benign lymphoepithelial lesions; reactive
PPS
fat
from
a
posterolateral
adenopathy; sialoliths; sialectasis (including autoimmune)
direction (shaded arrow)
Benign Neoplasm
Pleomorphic adenoma; Warthins tumor; oncocytoma; lipoma;
Figure 5-22-34
VII neurogenic tumor
Malignant Neoplasm
Mucoepidermoid CA; adenoid cystic CA; NHL; acinic cell CA;
malignant myxoid tumor; metastases (SCCa, melanoma, NHL)
1st Branchial Cleft Cysts [Figure 5-22-35]
Account for 8% of BCCs

Typical History: Middle aged female with h/o multiple parotid
abscesses unresponsive to drainage and antibiotics (Otorrhea if
cyst connects to bony-cartilaginous junction of EAC)
If there is an external sinus, it is typically found in the skin at angle
of mandible
Will image as cyst superficial to, within, or deep to parotid gland.
Normal anatomy: Dots=parotid
Wall thickness varies with degree of inflammation
glands;
Parotid ducts (short arrows)
CT is preferred to MR as it better defines cystic nature of lesion in
traverse
the buccal space fat and
some cases
pierce the buccinator muscles (long
arrows) to enter the vestibule of the
Benign Lymphoepithelial Lesions [Figure 5-22-37]
oral cavity opposite the second
Old term of benign lymphoepithelial cysts replaced as both solid
maxillary molar
and cystic lesions occur
Typical history: Bilateral parotid swelling associated with cervical adenopathy,

usually in a patient seropositive for HIV virus (parotid lesions may occur prior
to seroconversion and may be the initial presentation)
Imaging features: Bilateral parotid enlargement associated with intragladular
cystic and solid lesions. May see associated cervical adenopathy
Figure 5-22-35
A well-circumscribed, first
branchial cleft cyst involves the
parotid tail (arrows). Other cystic
lesions cannot be differentiated
1278
Neuroradiology
Figure 5-22-36
Figure 5-22-37
A large hemangioma (arrows) replaces much of

the left parotid gland, isointense on T1WI,
hyperintense on T2WI, and exhibiting marked
enhancement
Lymphoepithelial lesions - There are multiple

cystic and solid lesions in the parotid glands
bilaterally in this 27 year-old HIV positive
Sjogrens Syndrome [Figure 5-22-38]
Clinical triad:
Enlarged salivary glands with xerostomia
Enlarged lacrimal glands with keratoconjunctivitis
sicca
Connective tissue disease (RA most common)
Increased risk of developing a lymphoma, often
aggressive biologically
Sialography: Punctate, globular, cavitary, and
destructive lesions can all be seen
CT/MR: Enlarged glands with honeycomb
appearance; Some cysts may be quite large and
indistinguishable from LEL of AIDS by imaging alone
Figure 5-22-38
Pleomorphic Adenoma [Figures 5-22-39 and 5-22-40]
Most common benign parotid tumor (80%); if left

Sjogrens syndrome - The non-contrast CT scan
untreated, est. 25% will undergo malignant
demonstrates cystic enlargement of the parotid
degeneration
glands with specks of calcification identified,
Path: Mixture of epithelial and myoepithelial cells
suggestive
of an infectious process. The MR
Typical patient: >50 years of age with slow-growing
images
reveal
multiple, various-sized cysts in the
lump in the cheek
superficial
and deep lobes of both glands
Sharply marginated; round, oval, or lobulated
CT: Variable enhancement, infrequent dystrophic
calcification, and internal pockets of low density when large due to mucoid
Figure 5-22-39
matrix
MR: Very hyperintense on T2WI; may
have internal pockets of greater
hyperintensity if mucoid matrix
Classic superficial lobe pleomorphic adenoma

Neuroradiology
1279
Figure 5-22-40
Figure 5-22-41
Large deep lobe
pleomorphic adenoma
herniates through and
widens the right
stylomandibular tunnel
(arrows)
Warthin Tumor:
Warthins Tumor
Large cystic/solid right parotid superficial mass in a
65
year-old man. The heterogeneity would suggest
(Papillary Cystadenoma Lymphomatosum)
a lesion other than a pleomorphic adenoma
[Figure 5-22-41]
Second most common benign tumor of the parotid space

80% male, greater than 50 years of age
Usually slow-growing mass in region of parotid tail
Arise from ectopic salivary gland rests within
intraparotid lymph nodes
Limited to PAROTID GLAND ONLY
Bilateral in 10%-15% of patients
Imaging: Well-circumscribed, usually 3-4 cm
Complex mixture of solid and cystic components
Appears more complex than typical PA
Figure 5-22-42
Oncocytoma
Occurs exclusively in adults over 50 years of age; No

sex predilection; about 1% of parotid tumors
Oncocytes are large cells with granular eosinophilic
cytoplasm that may be found in groups, normally
Multiple facial nerve neurofibromas (arrows) in a
within the parotid gland.
patient with NF2. Also note the ipsilateral V2
An ONCOCYTOMA describes a solid tumor
neurofibroma in the right retromaxillary fat, bowing
composed entirely of oncocytes
the posterolateral maxillary wall
Imaging features are essentially identical to those of
a pleomorphic adenoma
May be multiple
Figure 5-22-43
Malignant Tumors
The smaller the salivary gland, the higher

the chance a mass is malignant
Sublingual mass-70% malignancy
Submandibular mass-60% malignancy
Parotid mass-20% malignancy
Mucoepidermoid Carcinoma-more than 80%
occur in parotid glands; most common
malignant salivary gland tumor in most
series
Adenoid Cystic Carcinoma-2%-6% of parotid
tumors
Acinic Cell Carcinoma-10%-30% of parotid
tumors
Low-grade superficial parotid lobe mucoepidermoid
Adenocarcinoma-rare in major salivary
carcinoma.
The lesion is somewhat complex and exhibits
glands
indistinct margins laterally (arrows) leading away from the
diagnosis of a benign neoplasm
1280
Neuroradiology
Mucoepidermoid Carcinoma [Figure 5-22-43]
Most common malignant lesion in parotid gland

Most common between 35 and 60 years of age but
may be found at any age and is most common
malignant tumor in persons under 20 years of age.
Slight predilection for women
Clinical: Rock hard mass with associated pain and
itching over the course of the facial nerve.
Imaging features depend on grade:
Low grade: Benign appearance; cannot
distinguish from pleomophic adenoma
Higher grade: Infiltrating, indistinct margins; look
for PNT along VII!!!
Low to intermediate signal on T1 and T2WI
Figure 5-22-44
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
VII Perineural Tumor - This patient has recurrent

adenoid cystic carcinoma of the parotid gland.
Curtin HD. Detection of perineural spread: Fat
Note perineural extension of tumor along the facial
suppression versus no fat suppression. AJNR Am J
nerve (VII) up through the stylomastoid foramen
Neuroradiol. 25(1):1-3, 2004.
(arrow on coronal) to extend to the geniculate
Go JL, et al. The trigeminal nerve. Semin Ultrasound CT
ganglion (arrow on axial)
MR. 22(6):502-520, 2001.
Harnsberger et al. Diagnostic Imaging: Head and Neck.
Amirsys Publishers, 2005.
Holliday RA, et al. Benign lymphoepithelial parotid cysts and hyperplastic cervical adenopathy in AIDS risk
patients: A new CT appearance. Radiology. 168:439-441, 1998.
Izumi M, et al. MR imaging of the parotid gland in Sjogrens syndrome: A proposal for new diagnostic criteria.
AJR. 166:1483-1487, 1996.
Marsot-Dupuch K, et al. Mandibular nerve: MR versus CT about 10 proved unusual tumors. Neuroradiology.
32:492-496, 1990.
Palacios E, et al. Benign asymmetric hypertrophy of the masticator muscles. Near Nose Throat J. 79(12):915,
2000.
Russo CP, et al. MR appearance of trigeminal and hypoglossal motor denervation. AJNR Am J Neuroradiol.
18(7):1375-1383, 1997.
Shah GV. MR imaging of salivary glands. Magn Reson Imaging Clin N Am. 19(4):631-662, 2002.
Triglia JM, et al. First branchial cleft anomalies: A study of 39 cases and a review of the literature. Arch
Otolaryngol Head Neck Surg. 124(3):291-295, 1998.
Tryhus MR, et al. The normal and diseased masticator space. Semin Ultrasound CT MR. 11:476-485, 1990.
Williams LS, et al. MR imaging of the trigeminal ganglion, nerve, and the perineural vascular plexus: Normal
appearance and variants with correlation to cadaver specimens. AJNR Am J Neuroradiol. 24(7):1317-1323, 2003.
Yonetsu K, et al. Deep facial infections of odontogenic origin: CT assessment of pathways of space involvement.
AJNR. 19:123-128, 1998.
Neuroradiology
1281

Pharyngeal Mucosal Space and Oral Cavity
Pharyngeal Mucosal Space
Figure 5-22-45
[Figures 5-22-45 and 5-22-46]
Extends from skull base to hyoid bone and

includes the nasopharynx, oropharynx, and
hypopharynx
The mucosa lining the upper aerodigestive tract
Pharyngeal Mucosal Space - Contents
Mucosa
Waldeyers ring of lymphatic tissue
Minor salivary glands (esp. in soft palate)
Superior and middle pharyngeal constrictor
muscles
Cartilaginous (distal) end of eustachian tube
(torus tubarius) (nasopharynx)
Levator palatini muscle (nasopharynx)
Pharyngeal Mucosal Space - Pathology
Pseudomass
Congenital
Tornwaldt cyst
Infection / Inflammatory
Post-inflammatory cysts (retention cysts);
cellulitis/abscess
Extension of the nasopharynx
Benign Neoplasms
Pleomorphic adenoma
Malignant Neoplasms
NPSCCa; non-Hodgkin lymphoma; minor salivary gland neoplasms
(mucoepidermoid Ca, adenoid cystic Ca)
Figure 5-22-46
A) Arrows indicate cartilaginous ends of eustachian tubes;

B) short arrows=medial pterygoid muscles; long arrows=PPS fat;
C) long arrows on PPS fat; short arrows indicate air in lateral pharyngeal recesses
Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity
1282
Neuroradiology
Nasopharyngeal Carcinoma (NPSCCa) [Figures 5-22-47 and 5-22-48]
Centered in lateral pharyngeal recess

Invasion of levator palatini results in eustachian tube
dysfunction and serous otitis media with CHL (check
mastoids!)
Perivascular spread (via foramen lacerum) and
perineural extension (mainly V3) are common so
skull base must be carefully assessed
Nodal metastases present in 90% at presentation
(retropharyngeal, level II, and level V-first)
Distant metastases-uncommon at presentation
(<10%) (bone, lung, and liver)
Strong relationship with Epstein-Barr virus (EBV)
Figure 5-22-47
PMS Non-Hodgkin Lymphoma (NHL)

[Figure 5-22-49]
Tends to remain localized and grow slowly

NHL in H&N from nasopharyngeal lymphoid tissue in
CT shows NPSCCa of left lateral recess (arrows)
35% of cases (versus palatine tonsil lymphoma in
with ipsilateral foramen ovale enlargement (arrow).
50% and lingual tonsil lymphoma in 15%)
MR also demonstrates the mass (arrow) with
50% have associated adenopathy at presentation
enhancing perineural tumor extending through the
20% may have GI tract NHL involvement
foramen (arrow)
AIDS and Sjogrens Syndrome predispose
Usually >50 years; M:F = 1.5:1
Figure 5-22-48
Bulky mass filling nasopharynx
T1WI-isointense, T2WI-hyperintense, enhance
48 year-old male
with bilateral IX-XII
palsies
Figure 5-22-49
Very large
NPSCCA replaces
the entire
basiocciput (distal
clivus), occipital
condyles, jugular
tubercles, and
portions of the C1
lateral masses
(seen on the
coronal images).
Coronal images
also demonstrate
extensive bilateral
adenopathy
This homogeneous, bulky, NHL mass fills the

nasopharynx but exhibits no infiltrtion of adjacent
spaces and no skull base involvement
Neuroradiology
1283
Oropharynx
Figure 5-22-50
[Figure 5-22-50]
Oral PMS Anatomy

Oro - PMS Pathology
[Figure 5-22-51]
Congenital
Thyroglossal duct cyst; lingual thyroid
Infection / Inflammatory
Post-inflammatory cysts (retention
cysts); cellulitis/abscess (tonsillar)
Benign Neoplasms
Pleomorphic adenoma
Malignant Neoplasms
SCCa (base of tongue and faucial
pillars); non-Hodgkin lymphoma; minor
salivary gland malignancies (especially
soft palate)
Pseudomass
Lymphoid hyperplasia (lingual tonsil)
Extension of the oropharynx
Thyroglossal Duct Cysts

[Figure 5-22-52]
Most common non-odontogenic extracranial head and neck cyst

20% are suprahyoid in location
Well-circumscribed
2-4 cm
Occasionally septated
Capsular enhancement
Smooth margins
DDx: submental node
Figure 5-22-51
Figure 5-22-52
Oropharyngeal Mucosal Space Anatomy

A) Arrows indicate tonsillar pillars; B) Long arrows
indicate tonsillar pillars; short arrows indicate fat in
PPS; C) Long arrows indicate tonsillar pillars;
short arrow indicates soft palate; D) Arrows
indicate regions of glossotonsillar sulci
Foramen cecum thyroglossal duct cyst (arrows).

1284
Neuroradiology
Lingual Thyroid Gland
Figure 5-22-53
[Figure 5-22-53]
Failure of thyroid descent from foramen cecum

Accounts for 90% of ectopic thyroid
High female predominance (7:1)
Only functioning thyroid tissue in 70-80%
CT: Hyperdense with avid enhancement
MR: Iso-to hyperintense to muscle on both T1- and
T2WI with strong enhancement
Look in lower neck to confirm lack of gland in normal
location
Subject to typical thyroid pathology
Figure 5-22-54
Lingual thyroid gland. Note absence of thyroid in
normal location in lower neck
Figure 5-22-55
Bilateral tonsillar abscesses
Figure 5-22-56
Right tonsillar SCCa with an

ipsilateral level 2A node
Large base of tongue SCCa

Neuroradiology
1285
Oral Cavity Normal Anatomy
Figure 5-22-57
[Figures 5-22-57 to 5-22-59]
Figure 5-22-58
The location of the oral cavity.

The oral cavity is separated from the
oropharynx by the circumvallate papillae,
the soft palate complex, and the anterior
tonsillar pillars
Figure 5-22-59
T1 WI
A: Sagittal MR.
Black arrows=intrinsic muscles of the
tongue;
white arrows=genioglossus muscles;
dotted arrows=geniohyoid muscles
T1 WI
B. Coronal MR.
Vertical white arrows=sublingual
spaces;
horizontal black/white
arrows=submandibular spaces;
vertical black/white arrows=platysma
muscles
1286
Neuroradiology
Oral Cavity Pathology
Figure 5-22-60
[Figure 5-22-60]
Congenital Lesions
Hemangiomas/vascular
malformations; dermoids/epidermoids
Inflammatory Lesions
Cellulitis/abscesses; sialoliths;
ranulas
Benign Neoplasms
Pleomorphic adenomas; aggressive
fibromatosis
Malignant Neoplasms
SCCa (floor of mouth, oral tongue);
minor salivary gland neoplasms
(SMG, SLG)
Dermoid Cysts
[Figure 5-22-61]
Refers to dermoid. epidermoid, and

teratoid lesions
Most uncommon of the congenital lesions Multiple oral cavity venous malformations in a child with Blue
Rubber Bleb Nevus Syndrome
Sublingual and submandibular locations
Low density/intensity, unilocular, well-circumscribed
Figure 5-22-61
Cyst wall enhances with contrast
Individual fat globules=dermoid
In absence of fat, cannot DDx epidermoid from
dermoid
Ranulas
[Figures 5-22-62 and 5-22-63]
Mucoceles/mucous retention cysts of the floor of the

mouth
Secondary to trauma or obstruction of sublingual
gland/ducts
Thin-walled; unilocular; non-enhancing
Two varieties:
Simple ranula-in SLS (true)
Plunging ranula-in SMS (pseudocyst)
Dermoid cysts in 2 patients with classic bag of
marbles appearance
Figure 5-22-62
Figure 5-22-63
Simple ranula in a 30 year-old

female with obstructive sleep apnea
Neuroradiology
Plunging ranulas in 3 different patients

1287
Pleomorphic Adenomas
Figure 5-22-64
[Figure 5-22-64]
Most common benign glandular tumor--majority in

parotid gland
8% in submandibular gland; 0.5% in sublingual gland
Well demarcated; homogeneous when small
Heterogeneous (cystic changes necrosis,
hemorrhage)
Hypo-isointense on T1WI
Homo/heterogeneous enhancement
Exostoses
[Figure 5-22-65]
Dense cortical bone w/ or w/o cancellous bone

Incidental unless they preclude proper denture fitting
Occasionally very large and interfere with swallowing
Torus palatinus
Torus mandibularis
interna / externa
Torus maxillaris
interna / externa
Sublingual gland pleomorphic adenoma
Figure 5-22-65
Figure 5-22-66
This T3N2b oral tongue SCCa (double arrows)

invades the ipsilateral mylohyoid muscle (dots) to
extend to the floor of the mouth. An ipsilateral
node is indicated by the single arrow
Arrows indicate torus palatinus,

bilateral mandibular tori interna, and
bilateral maxillary externa tori
1288
Neuroradiology
Figure 5-22-67
Left floor of mouth SCCa (arrows)

extends to block the submandibular
gland ducts bilaterally (arrows)
References
1.
2.
3.
4.
5.
6.
7.
7.
8.
Fischbein NJ, et al. Clinical utility of positron emission tomography with 18F-fluorodeoxyglucose in detecting
residual/recurrent squamous cell carcinoma of the head and neck. AJNR Am J Neuroradiol. 19(7):1189-1196,
1998.
Harnsberger et al. Diagnostic Imaging: Head and Neck. Amirsys Publishers, 2005.
King AD, et al. In vivo proton MR spectroscopy of primary and nodal nasopharyngeal carcinoma. AJNR AM J
Neuroradiol. 25(3):484-490, 2004.
Mukherji SK, et al. Squamous cell carcinoma of the oropharynx and oral cavity: How imaging makes a
difference. Semin Ultrasound CT. 19:463-475, 1998.
Roh JL, et al. Nasopharyngeal carcinoma with skull base invasion: A necessity of staging subdivision. Am J
Otolaryngol. 25(1):26-32, 2004.
Sigal R, et al. CT and MR imaging of squamous cell carcinoma of the tongue and floor of the mouth.
RadioGraphics. 16:787-810, 1996.
Smoker WRK, et al. Computed tomography of the nasopharynx and related spaces. Seminars Ultrasound CT MR.
7:107-130, 1986.
Smoker WRK. The Oral Cavity in Head and Neck Imaging (4th ed) Som and Curtin, eds. Mosby Year Book
Publishers. pp 1377-1464, 2002.
Weber AL, et al. Malignant tumors of the oral cavity and oropharynx: Clinical, pathologic, and radiologic
evaluation. Neuroimaging Clin N Am. 13(3):443-464, 2003.
Neuroradiology
1289
Spine: Degenerative Disease and

Infections
Figure 5-23-1
Low Back Pain Annual Costs
250,000 operations/year
18-56 billion dollars/year
85% of costs are due to recurrent or chronic disability
Degenerative Disc Disease

Lumbar Nomenclature [Figures 5-23-1 and 5-23-2]
Normal
Bulge (symmetric, asymmetric)
Annular tear/fissure
Herniation (focal or broad-based)
Protrusion
Extrusion
Extrusion with free fragment
Annular Tear/Fissure [Figure 5-23-3]
Typically seen with degenerated discs but even seen in 25% of

patients < 20 years of age
Disc protrusions/extrusions often associated
Primary failure of the annulus all layers involved
Present with back/radicular pain
MR: 1-2 mm band of increased T2 signal (High intensity zone-HIZ)
linear area of contrast enhancement (96%)
not as sensitive as discography
Figure 5-23-3
Disc bulges
Upper: Symmetric
Lower: Asymmetric (>50% of
circumference)
Figure 5-23-2
Multilevel disc bulges

Note high intensity zone on
these T2WIs (arrows)
Spine: Degenerative Disease and Infections
1288
1290
Neuroradiology
Disc herniations and types
[Figures 5-23-4 and 5-23-5]
Figure 5-23-4
Figure 5-23-5
Focal: <25% of
circumference
Broad-based:
> 25% but < 50% of
circumference
Protrusion
Extrusion
Protrusion
Extrusion
Extrusion with migration
L4-5 Disc Extrusion with Migration [Figure 5-23-6]
Degenerative changes typically

induce secondary changes in the
adjacent vertebral bodies
(degenerative discovertebral
changes) (Modic Changes)
Figure 5-23-6
This extruded disc retains a connection to the parent L4-5 disc

as it migrates down behind the L5 vertebral body (arrows)
Type I
Vascularized Marrow (edema)
Low T1WI
High T2WI
Type II
Proliferation of endplate fatty marrow
High T1WI
High T2WI
Type III
Neuroradiology
plus sequestered fragment
Low T1WI
Dense bone devoid of marrow (sclerosis) Low T2WI
1289
1291
Lumbar Spinal Canal and Foraminal Stenosis
Figure 5-23-7
Lumbar Facet Arthropathy [Figures 5-23-7 and 5-23-8]

Figure 5-23-8
Multilevel degenerative disease with severe right L1-2 foraminal

stenosis from a combination of disc bulge, facet arthropathy,
and ligamentum flavum buckling
Facet Joint Synovial Cysts [Figure 5-23-9]
Note the nerve root exits

Intraspinal juxta-articular synovial cysts are uncommon; associated with facet
superiorly within the
arthopathy
neural foramen, just
Most in lumbar spine, especially L4-5
under the pedicle
Slight female preponderance; mean age 58 years
(arrow). Note the normal
Origin: DJD? Trauma??
keyhole configuration
No specific symptom history; waxing and waning symptoms as these increase of the neural foramen
and decrease in size
CT: Cystic lesion w/ or w/o calcified rim adjacent to degenerated facet
MR: Iso- or slightly hyperintense on SE sequences
Figure 5-23-9
DDx: Ganglion cyst (does not communicate with joint)
Failed Back Surgery Syndrome

(FBSS) [Figure 5-23-10]
10%-40% of patients
Intractable pain with variable incapacitation
Differential considerations include:
Recurrent/residual disc herniation
Post-op infection
Second level disease
Facet disease
Arachnoiditis
Neuritis
Epidural fibrosis (Scar)
Miscellaneous
Synovial cyst produces

compression on the
thecal sac from a
posterolateral location
(arrows). Compression
of the nerve roots is best
appreciated on the MR
myelogram (right image)
1290
1292
Neuroradiology
FBSS: Type I Arachnoiditis [Figure 5-23-11]
Figure 5-23-10
Figure 5-23-11
FBSS: Recurrent Disc. A large recurrent disc

(arrows) demonstrates mild enhancement
but the surrounding epidural fibrosis
enhances to a much greater degree
Type I arachnoiditis. Nerve roots are
clumped centrally within the thecal sac
(arrows)
FBSS: Type II Arachnoiditis [Figure 5-23-12]

Figure 5-23-12
Type II arachnoiditis. Nerve roots are

plastered to the margins of the thecal sac
producing an empty appearance (large
arrows). The more proximal nerve roots are
seen as distinct entities (small arrows)
Cervical Spinal Canal Stenosis

Cervical Spinal Canal Stenosis [Figures 5-23-13 and 5-23-14]
Figure 5-23-13
Congenital cervical spinal canal stenosis

may be produced by either pedicle or lamina
hypoplasia. The underlying dimensions of
the canal should be assessed on every study
as even mild degenerative disease can be
significant in the face of underlying
congenital stenosis
Neuroradiology
1291
1293
Ossification of the Posterior

Longitudinal Ligament (OPLL)
Figure 5-23-14
C2
[Figures 5-23-15 and 5-23-16]
Begins with calcification followed by

frank ossification of the posterior
longitudinal ligament in upper C-spine
and progresses into upper T-spine
Can see on plain films in 0.12% of
asymptomatic North Americans and
20%-30% of symptomatic patients
Most easily seen on CT
On MR, thick band of decreased signal
on T1 and T2WI with mass effect on
thecal sac and cord
Associated with DISH
Figure 5-23-15
C4-5
C5-6
Acquired cervical spinal canal stenosis. Disc disease, coupled

with ligamentum flavum buckling, produces severe canal
stenosis at multiple levels (dark arrows) with associated
pathologic signal in the spinal cord (white arrows), most severe
at C5-6
T1WI
OPLL and DISH

Thick hypointense signal is identified
between the spinal cord and posterior
vertebral bodies on MR (arrows). CT
confirms the diagnosis of OPLL severely
compromising the spinal canal diameter.
DISH is present at lower levels
Figure 5-23-16
Ossification of Ligamentum Flavum

Marked thecal sac and cord compression
produced by OLF
1292
1294
Neuroradiology
Infections
Figure 5-23-17
Pyogenic Osteomyelitis
[Figures 5-23-17]
Ill-defined T1-hypointense vertebral

marrow with loss of adjacent endplate
definition on both sides of infected disc;
hyperintense on T2WI; enhances
Lumbar-48%; Thoracic-35%; Cervical6.5%
Paraspinal and/or epidural involvement
in 75%
S aureus most common; E coli if gram
negative; salmonella in sickle cell
Source: GU, GI, lung, cardiac,
cutaneous/mucous-seeds vascularized
subchondral bone
Disc first site of involvement in children
(vascularity)
Bimodal: Pediatric patients and 6th-70th
decade; also IV drug abusers and those
with HIV
Pain, tenderness, and fever
Pyogenic osteomyelitis. Note paraspinal and epidural

involvement (arrows).
Figure 5-23-18
Post Gd
Tuberculous Osteomyelitis
[Figure 5-23-18]
AKA: Potts disease

Typical: Gibbus vertebrae with relatively
intact disc and paraspinal abscess
Abscesses dissect over considerable
length
Mid-thoracic or thoracolumbar most
common
Inoculum in anterior vertebral body;
spreads under anterior longitudinal
ligament; spares discs due to absence
of proteolytic enzymes
M=F; presents in 50s; fever infrequent
Concomitant pulmonary TB in 10%
T2 WI
Tuberculous osteomyelitis. Note gibbus deformity and

extension under the anterior longitudinal ligament (arrows)
Epidural Phlegmon / Abscess

[Figures 5-23-19 and 5-23-20]
S aureus most common (57-73%), then

TB (25%)
Predisposing conditions: IV drug users,
immunocompromised states, DM, CRF,
alcoholism
Anterior from adjacent discitis or
osteomyelitis
Posterior from GU, GI, lung, cardiac
Direct inoculation from penetrating
trauma, surgery.
Peaks in 50s and 70s
Lumbar EA may mimic herniated disc
Figure 5-23-19
Epidural Phlegmon. A definite abscess cavity is not defined

but abnormal enhancing soft tissue fills the posterior epidural
space (arrows)
Neuroradiology
1293
1295
Miscellaneous
Figure 5-23-20
Brachial Plexus Traction Injury

[Figure 5-23-21]
AKA: Traumatic meningocele

Avulsion of root(s) of brachial plexus
invariably from traumatic injuries:
(Adults-ATVs, motorcycles; infants:
complicated deliveries)
Location critical to planning and
prognosis:
Pre-gang (central to dorsal root
ganglion)-worse prognosis
Post-gang (peripheral to dorsal
root ganglion)-better prognosis
Demonstration of rootless
meningoceles are diagnostic
Idiopathic Transdural Cord

Herniation
Epidural and paraspinal abscesses.

The paraspinal abscess is confirmed on the DWIs.
The etiology was a septic right facet joint
[Figure 5-23-22]
Typical presentation is Brown-Sequard

Syndrome
Clinical deficits tend to be progressive
unless treated
Surgical reduction of the herniated cord
can lead to improvement in the motor
deficit
Typical patient is an adult, affected
location is the upper and mid-thoracic
level (T2-T7)
??Cause??
Cong weakness of ventral dura?
Damage by disc hernitation?
Abnormal adhesions of cord to
dura?
Large root sleeve diverticulum?
Interruption of the usually smooth
ventral margin of the cord over a short
segment (pulled anteriorly)
Primary differential: Is cord is being
displaced by a posterior mass
(arachnoid cyst?) or is cord tethered
ventrally?
Figure 5-23-21
Brachial Plexus Traction Injury

(8 year-old boy s/p ATV accident with dead right arm).
Multiple traumatic meningoceles are demonstrated (arrows)
Figure 5-23-22
Idiopathic transdural spinal cord

herniation (arrows)
1294
1296
Neuroradiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
Akman S, et al. Magnetic resonance imaging of tuberculous spondylitis. Orthopedics. 26(1):69-73, 2003.
Ben Hamouda K, et al. Thoracic myelopathy caused by ossification of the ligamentum flavum: A report of 18
cases. J Neurosurg. 99(2 Suppl):157-161, 2003.
Boos N, et al. Classification of age-related changes in lumbar intervertebral discs: 2002 Volvo Award in basic
science. Spine. 27(23):2631-2644, 2002.
Carragee EJ. The clinical use of magnetic resonance imaging in pyogenic vertebral osteomyelitis. Spine.
22(7):780-785, 1997.
Cinotti G, et al. Stenosis of lumbar intervertebral foramen: Anatomic study on predisposing factors. Spine.
27(3):223-229, 2002.
Consensus statement on nomenclature and classification of lumbar disc pathology by NASS, ASSR, and ASNR.
2001.
Dix JE, et al. Spontaneous thoracic spinal cord herniation through an anterior dural defect. AJNR Am J
Neuroradiol. 19(7):1345-1348, 1998.
Doi K, et al. Cervical nerve root avulsion in brachial plexus injuries: Magnetic resonance imaging classification
and comparison with myelography and computerized tomography myelography. J Neurosurg. 96(3 Suppl):277284, 2002.
Eastwood JD, et al. Diffusion-weighted imaging in a patient with vertebral and epidural abscesses. AJNR Am J
Neuroradiol. 23(3):496-498, 2002.
Fassett DR, et al. Spinal epidural lipomatosis: A review of its causes and recommendations for treatment.
Neurosurg Focus. 16(4):Article 11, 2004.
Geers C, et al. Polygonal deformation of the dural sac in lumbar epidural lipomatosis: Anatomic explanation by
the presence of meningovertebral ligaments. AJNR Am J Neuroradiol. 24(7):1276-1282, 2003.
Modic MT, et al. Degenerative disk disease: Assessment of changes in vertebral body marrow with MR
imaging. Radiology. 166(1 Pt 1):193-199, 1988.
Munter FM, et al. Serial MR imaging of annular tears in lumbar intervertebral disks. AJNR Am J Neuroradiol.
23(7):1105-1109, 2002.
Ross JS, et al. Assessment of extradural degenerative disease with Gd-DTPA-enhanced MR imaging:
Correlation with surgical and pathologic findings. AJNR Am J Neuroradiol. 10(6):1243-1249, 1989.
Ross JS, et al. Association between peridural scar and recurrent radicular pain after lumbar discectomy:
Magnetic resonance evaluation. Neurosurgery. 38:855-861, 1996.
Ross JS, et al. MR imaging of lumbar arachnoiditis. AJR. 1987;149:1025-1032.
Sasaoka R, et al. Idiopathic spinal cord herniation in the thoracic spine as a cause of intractable leg pain: Case
report and review of the literature. J Spinal Disord Tech. 16(3):288-294, 2003.
Van Goethem JW, et al. Review article: MRI of the postoperative lumbar spine. Neuroradiology. 44(9):723239, 2002.
Wang MY, et al. Intradural spinal arachnoid cysts in adults. Surg Neurol. 60(1):49-55; discussion 55-56, 2003.
Watters MR, et al. Transdural spinal cord herniation: Imaging and clinical spectra. AJNR Am J Neuroradiol.
19(7):1337-1344, 1998.
Neuroradiology
1295
1297
Spinal Tumors, Cysts, and Mimics

First Things First!! - Localize the Lesion!! [Figure 5-24-1]
Figure 5-24-1
Extradural
Extramedullary
Intramedullary
Intradual
Localizing a spinal lesion to the appropriate compartment (extradural, extramedullary-intradural, or
intramedullary) allows a tailored differential diagnosis
Common Intramedullary Lesions
Non-neoplastic:
Acute trauma (contusion, edema)
Syringohydromyelia
Syrinx-cavity in cord NOT lined by ependyma
Hydromyelia-dilatation of the central canal lined by ependyma
Demyelinating disease (MS, ADEM)
Neoplastic
Ependymoma
Astrocytoma
THERE IS NO SPECIFIC IMAGING PATTERN THAT RELIABLY

PERMITS DIFFERENTIATION BETWEEN EPENDYMOMAS AND
ASTROCYTOMAS
Uncommon
Non-neoplastic Lesions
Acute cord ischemia/stroke
Myelitis (Post viral ADEM, etc)
Neoplastic Lesions
Hemangioblastoma
Astrocytoma (anaplastic and GBMs))
Rare
Non-neoplastic Lesions
Vascular lesions (cavernomas, AVM, etc)
Infections (sarcoid, TB, Lyme disease)
Neoplastic Lesions
Metastases
Lipoma
Subependymoma
Oligodendroglioma
Ganglioma
Paraganglioma
1296
1298
Neuroradiology
Ependymomas [Figure 5-24-2]
Figure 5-24-2
Most common intramedullary tumor in

adults- 60%
Mean age 43 years; females
predominate (slightly)
Arise from ependymal cells lining
central canal
Slow-growing; canal expansion is
typical
56% cervical; 28% thoracic; 16%
lumbar
75% are isointense on T1WI; 100%
hyperintense on T2WI
Cystic degeneration and hemorrhage
are common
Hemosiderin deposition-common at
periphery
Heterogeneous enhancement in 65%
Ependymoma - Note lesion isointensity on T1WIs and

relatively homogeneous enhancement on the Post-Gd images.
This lesion exhibits unusual hypointensity on the T2WI, most

likely due to the prominent amount of hemosiderin noted on
histology. Also note the associated areas of cystic
Astrocytomas [Figure 5-24-3]
Second most common IM tumor overall; degeneration, indicated by the arrows. Significant cord edema
most common tumor in children
in best appreciated as marked increased signal intensity on the
Cervical=thoracic; M=F; mean age 21
T2WI
years
Figure 5-24-3
Typically pilocytic and diffuse fibrillary
types (low grade); anaplastic
astrocytomas and GBMs are rare
May extend to involve the entire cord
Cyst formation is common; Syrinxabove or below tumor
Iso- to hypointense on T1WI;
Hemorrhage is LESS COMMON than
for ependymomas
Virtually 100% enhance
Hemangioblastoma [Figure 5-24-4]
Uncommon; 1%-5% of cord tumors

Peak age between 30 and 40 years
One third associated with VHL
syndrome
85% intramedullary or combined
intramedullary/ extramedullaryintradural
50% thoracic; 40% cervical; 80% are
solitary
Isointense on T1WI; hyperintense on
T2WI; strong enhancement; may see
flow voids, etc.
On angio, highly vascular mass; dense
prolonged tumor stain, prominent
draining veins
Neuroradiology
Ependymoma - Lesion demonstrates isointensity on T1WIs,

hyperintensity on T2WIs, and manifests only very faint
enhancement on post-Gd images (somewhat unusual). A large
signal void is present on the T2WIs, possibly representing an
area of hemosiderin/calcification. Significant edema is also
best appreciated on the T2WIs
1297
1299
Intramedullary Metastases [Figure 5-24-5]
Figure 5-24-4
Intramedullary metastases are rare

1%-3% of all intramedullary tumors
No specific imaging characteristics to
clearly distinguish from other
intramedullary lesions Breast and lung
most common
Also lymphoma, leukemia, and
malignant melanoma
Non-Neoplastic Intramedullary
Pathology
Cavernoma
[Figure 5-24-6]
Acute Disseminated
Encephalomyelitis [Figure 5-24-7]
Figure 5-24-5
Classic appearance of an intramedullary hemangioblastoma

with T1 isointensity, T2 hyperintensity, and marked
enhancement with numerous flow-voids, best seen on the T2
and post-Gd images (arrows)
Figure 5-24-6
Classic cavernoma appearance with central slight

T1 hyperintensity and heterogeneous T2
hyperintensity (popcorn appearance). Note the rim
of hemosiderin which blooms on the T2WI. The
finding of multiple areas of intracranial
hemosiderin on the gradient echo (GRE) image
further solidifies the diagnosis.
(Courtesy M Modic)
Figure 5-24-7
Intramedullary Metastasis (79 year-old female with

lung carcinoma and brain metastases) - Note subtle
enlargement of the spinal cord without significant
signal change on the T1WI, hypointensity of the
lesions on the T2WI, and lesion enhancement. The
smaller lesion (arrow) would be almost impossible to
detect without contrast
1298
1300
Acute Disseminated Encephalomyelitis (15 yearold male with headache, lethargy, and nuchal
rigidity) - Note significant cord expansion with
slight T1 hypointensity, marked T2 hyperintensity,
and very patchy enhancement. This boy had a
URI two weeks prior to onset of symptoms
Neuroradiology
Multiple Sclerosis
[Figure 5-24-8]
Extramedullary-Intradural Lesions
Common
Uncommon
Neurogenic Neoplasms
Neurofibromas
Schwannomas
Meningiomas
Myxopapillary
Ependymomas
Arachnoiditis
Lipomas
Arachnoid Cysts
Epidermoids/Dermoids
Drop metastases
AVM
Infection
Paragangliomas
Figure 5-24-8
Nerve Sheath Tumors
Most common extramedullary-intradural tumors

(70%-75%). 15% extradural; 15% dumbell
Schwannomas slightly more common than
neurofibromas
Neurofibromas: M=F; 20-30 years. No true capsule
Localized, diffuse, or plexiform
Schwannomas: M=F; 30-60 years. Encapsulated
40% cystic changes; 10% hemorrhage; target
sign
Neural foraminal enlargement; pedicle thinning
Isointense on T1WI; Hyperintense on T2WI; Enhance
Multiple in neurofibromatosis
Neurofibroma
[Figures 5-24-9 to 5-24-11]
vs.
Neurofibroma
Schwann cells
Fibroblasts
Acellular material
Infiltrating
Resect parent nerve
Multiple Sclerosis - Note absence of significant

spinal cord expansion. The MS plaques are not
Schwannoma
appreciated on the T1WI, only faintly suspected on
the T2WI, but very well seen on the STIR
sequence.
Faint enhancement of one, probably
Schwannoma
more active, plaque can be seen on the post
Schwann cell neoplasm
contrast sequence (arrow)
Secondary vascular
Figure 5-24-9
changes
Mostly cellular
Encapsulated
Nerve sparing surgery
Figure 5-24-10
T1WI
T2WI
Post Gd
Schwannoma at the level of the conus. Sagittal

images nicely demonstrate the classic
subarachnoid cap at the inferior margin of the
lesion with widening of the subarachnoid space.
Note hypointensity on the T1WI and hyperintensity
on the T2WI. The post-contrast images
demonstrate a nice target sign. The lesion
occupies most of the spinal canal and significantly
compresses the spinal cord, best seen on the axial
post contrast image (arrow)
Neuroradiology
Classic dumbbell neurofibroma at C2-3

demonstrates isointensity on T1WIs,
hyperintensity on the T2WI and manifests
very significant enhancement
on the coronal post-Gd image
1299
1301
Meningiomas
[Figures 5-24-12 and 5-24-13]
Second most common spinal tumor (25%)

90% intradural; 10% extradural or dumbell
Females > males at 4:1; Primarily 5th-6th decade
Thoracic (80%) > Cervical (16%) > Lumbar (4%)
More often anterior in cervical region
Below C7, more common posterior to cord
85% intradural; usually single unless NF 2
Iso on T1WI; iso or hyperintense on T2WI;
hypointense if Ca++
Marked homogeneous enhancement
Figure 5-24-11
Figure 5-24-12
A large schwannoma is seen arising from the S1
nerve root (arrows), isointense on T1WI,
heterogeneously hypointense on T2WI, and
primarily peripheral enhancement following
contrast
Figure 5-24-13
Meningioma (C Spine-Ventral) - The sagittal

images clearly localize this lesion as
extramedullary-intradural by demonstrating a nice
subarachnoid cap (widening of the subarachnoid
space-arrows). The lesion severely compresses
the spinal cord, best appreciated on the axial T2WI
image (outlined). The lesion is essentially
isointense to cord on T1 and T2WIs
Myxopapillary Ependymoma
[Figure 5-24-14]
Meningioma (T Spine-Dorsal) - Note lesion

27%-30% of all ependymomas; 90% of filum tumors
isointensity
on T1 and T2WIs and homogeneous
Genetically different from intracranial ependymomas
enhancement.
The subarachnoid cap is best
Occur exclusively in conus and filum terminale-from
appreciated
at
the
superior margin of the tumor on
ependymal cells in filum
the T2WI
Males:females = 2:1; peak between 30 and 40 years
Figure 5-24-14
Slow growing and may fill entire lumbar spinal canal
Vertebral scalloping; canal enlargement
Highly vascularhemorrhage is common
Iso on T1WI; hyper on T2WI; hypointense margin if
hemosiderin
Intense enhancement in 100%
Other Extramedullary-Intradural
Neoplastic Lesions
A large myxopapillary ependymoma fills and

expands the lumbar spinal canal, scalloping the
lower vertebrae. Note isointensity on the T1WI,
hyperintensity on theT2WI and marked
enhancement. The small superior component
(arrow) was suspected on the T2WI but only
confirmed on the post-contrast image
1300
1302
Neuroradiology
Hemangioblastoma
Lipomas
Figure 5-24-15
[Figure 5-24-15]
[Figure 5-24-16]
Originate from fat cells in subpial region

Vertebral and dermal anomalies are NOT associated
with these lipomas
25% are diagnosed within the first 5 years of life
No sex predilection
Excessive weight gain and pregnancy may
predispose
All Post-Gd
Figure 5-24-16
Small hemangioblastoma in a patient with von
Hipple Lindau syndrome. The enlarged feeding
vessels (arrows) are well-demonstrated and may
initially suggest a diagnosis of AVF
Figure 5-24-17
Large cervical lipoma has caused marked

widening of the spinal canal and severe spinal
cord compression. The lesion manifests fat signal
intensity on the T1 and T2WIs. The intraoperative
photograph shows fat bulging through the dura
Type III Meningeal Cysts (Arachnoid Cysts)

[Figure 5-24-17]
Intradural arachnoid cysts are rare

Unclear etiology: ?congenital; ?hemorrhage;
?inflammation
Thoracic spine is most common location
80% arise near septum posticum and located
posterior to cord
Most communicate with subarachnoid space
CTM: compressed cord displaced anteriorly
MR: signal intensity of CSF so may not be able to
identify unless cord is displaced/deformed
Dermoid Cysts [Figure 5-24-18]
Congenital (100%)
Symptomatic before age 20; M=F
80% in lumbosacral or cauda
Hypointense areas-? Water content from sweat gland
secretions
Fat hyperintensity on T1WI
A large dorsal arachnoid cyst has produced
May cause chemical meningitis if rupture with
marked
expansion of the spinal canal with severe
cholesteol crystals discharged into CSF
pedicle thinning and cord compression. The
compressed spinal cord is outlined on the softtissue windows.
The lesion exhibits fluid signal intensity on the T1
and T2WIs. Note severe cord compression
(arrows)
Neuroradiology
1301
1303
Figure 5-24-18
Figure 5-24-19
T1WI
Implantation Epidermoid Cyst (7 year-old female-had LP as an

infant) - The lesion is slightly hyperintense on the T1WI, very
hyperintense on the T2WI (such that the margins cannot be
separated from the surrounding CSF), and does not enhance.
A tract from the prior LP is not identified
Lumbar dermoid cyst. The
complex nature of the lesion with
identification of fat signal (high)
on the T1WI should suggest the
diagnosis.
(Courtesy M Modic)
Epidermoid Cysts
Figure 5-24-20
[Figure 5-24-19]
Less than 1% of spinal tumors

Congenital-60%; Aquired-40%
Upper thoracic-17%; lower thoracic26%; lumbosacral-22%; cauda equina35%
Strong association with lumbar puncture
in neonatal period (implantation
epidermoid)
Iso or slightly hyperintense on T1WI;
hyper on T2WI
Mild rim enhancement
DWI can DDx from arachnoid cysts
and other lesions
Drop Metastases
Drop Metastases (25 year-old woman with breast CA and

radicular symptoms) - Numerous enhancing lesions on the
cauda equina are identified
[Figures 5-24-20 and 5-24-21]
Figure 5-24-21
CNS Primary Tumors

Astrocytomas
Medulloblastomas
Pineal cell tumors
Ependymomas
Germ cell tumors
Non-CNS Primary Tumors
Breast
Lung
Lymphoma
Melanoma
Pituitary
Diffuse Mantle Cell Lymphoma.

Note marrow signal in the thoracic spine is
diffusely abnormal (darker that that of the
intervertebral discs). There is infiltration of
virtually all of the nerve roots of the cauda
equina such that they are not visualized as separate entities on the sagittal image. Enlargement of
individual roots is best appreciated on the axial images
1302
1304
Neuroradiology
Extradural Lesions
Common
Uncommon
Epidural Metastases
Herniated discs, etc
Degenerative lesions (osteophytes,
ligament infolding)
Lymphoma
Infection (discitis, etc)
Epidural Hematoma
Epidural Abscess
Arachnoid cysts
Lipomas
Primary vertebral tumors-benign
Primary vertebral tumors-malignant
Pagets disease
Epidural hematoma
Epidural lipomatosis
Extramedullary hematopoesis
Angiolipomas
[Figure 5-24-22]
Epidural Lipomatosis
[Figures 5-24-23 and 5-24-24]
Most often associated with chronic steroid use (exogenous or endogenous

Cushings syndrome)
Also associated with rapid weight gain and obesity
Thoracic spine most common
Myelopathic symptoms predominate
Figure 5-24-22
Figure 5-24-23
Three year-old girl with 2 day h/o neck pain and

UE/LE weakness - A large epidural hematoma
(from C2 to T5) is producing severe spinal cord
compression. A fluid-fluid level is indicated by the
arrows. (Courtesy A Gean)
Figure 5-24-24
Epidural Lipomatosis - Severe

accumulation of dorsal epidural fat in
the thoracic region is producing
severe cord compression
Epidural lipomatosis is producing severe
compression of the cauda equina in the lumbar
spine
Neuroradiology
1305
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
Arslanoglu A, et al. MR imaging characteristics of pilomyxoid astrocytomas. AJNR Am J Neuroradiol.

24(4):1906-1908, 2003.
Chu BC, et al. MR findings in spinal hemangioblastoma: Correlation with symptoms and with angiographic and
surgical findings. AJNR Am J Neuroradiol. 22(1):206-217, 2001.
Cohen-Gadol AA, et al. Spinal meningiomas in patients younger than 50 years of age: A 21-year experience. J
Neurosurg. 98(3 Suppl):258-263, 2003.
Conti P, et al. Spinal neurinomas: Retrospective analysis and long-term outcome of 179 consecutively operated
cases and review of the literature. Surg Neurol. 61(1):34-43; discussion 44, 2004.
Fujiwara F, et al. Intradural spinal lipomas not associated with spinal dysraphysm: A report of four cases.
Neurosurgery. 37(6):1212-1215, 1995.
Garg RK. Acute disseminated encephalomyelitis. Postgrad Med J. 79(927):11-17, 2003.
Hickman SJ, et al. Imaging of the spine in multiple sclerosis. Neuroimaging Clin N Am. 10(4):689-704, viii,
2000.
Khong PL, et al. Childhood acute disseminated encephalomyelitis: The role of brain and spinal cord MRI.
Pediatr Radiol. 32(1):59-66, 2002.
Kikuchi K, et al. The utility of diffusion-weighted imaging with navigator-echo technique for the diagnosis of
spinal epidermoid cysts. AJNR Am J Neuroradiol. 21(6):1164-1166, 2000.
Koeller KK, et al. Neoplasms of the spinal cord and filum terminale: Radiologic-pathologic correlation.
RadioGraphics. 20:1721-1749, 2000.
Murphey MD, et al. Imaging of musculoskeletal neurogenic tumors: Radiologic-pathologic correlation.
RadioGraphics. 19:1253-1280, 1999.
Potgieter S, et al. Epidermoid tumours associated with lumbar punctures performed in early neonatal life. Dev
Med Child Neurol. 40(4):266-269, 1998.
Simon JH. Brain and spinal cord atrophy in multiple sclerosis. Neuroimaging Clin N Am. 10(4):753-770,
2000.
Sun B, et al. MRI features of intramedullary spinal cord ependymomas. J Neuroimaging. 13(4):346-351, 2003.
Thakkar SD, et al. Spinal tumours in neurofibromatosis type I: An MRI study of frequency, multiplicity and
variety. Neuroradiology. 41(9):625-629, 1999.
Wanebo JE, et al. The natural history of hemangioblastomas of the central nervous system in patients with von
Hippel-Lindau disease. J Neurosurg. 98(1):82-94, 2003.
Wang MY, et al. Intradural spinal arachnoid cysts in adults. Surg Neurol. 60(1):49-55; discussion 55-56, 2003.
Yamada CY, et al. Myxopapillary ependymoma of the filum terminale. AJR Am J Roentgenol. 168(2):366,
1997.
1304
1306
Neuroradiology
Congenital Abnormalities of the Brain

Figure 5-25-1
Timing of Events
Disorders of Dorsal Induction

(3-4 weeks)
Disorders of Ventral Induction
(5-10 weeks)
Disorders of Neuronal Proliferation
(2-5 months)
Disorders of Dorsal Induction

(Insult at 3-4 weeks)
Cranioschesis
Anencephaly
Cephaloceles
Chiari Malformations
Chiari I
Chiari II (Arnold Chiari Malformation)
Chiari III
Chiari IV (Cerebellar hypoplasia)
Anencephaly
Symmetric absence of calvaria, cerebral hemispheres,

diencephalon
Replaced by flat amorphous vascular- neural mass
Facial structures and orbits present
Most common CNS malf (1:1000)
Invariably fatal (aborted/stillborn)
Implications for future pregnancies:
1 in 50 (2%) risk in next child
if 2 prior pg with CNS mal, risk is 1 in 10)
Cephaloceles
Skull base (enchondral bone)

Failure of neural tube closure or failure of ossification centers
to unite
Large cephalocele containing brain,
Calvarium (membraneous bone)
ventricles, and midline fat. Note
Defective bone induction, pressure erosion, or failure of neural absence of the corpus callosum and
tube closure
an open-lip schizencephalic cleft
Occipital
(Arrow)
80%-90%; F>M; assoc with neural tube defects; most
common in Caucasian NAs and Europeans
Frontal
Sincipital
Nasofrontal
Nasoethmoidal-Most common in SE Asia; M>F
Nasoorbital
Basal
Transethmoidal, transsphenoidal, sphenomaxillary, sphenoorbital
Frontal Lipoencephalocystocele [Figure 5-25-1]
Neuroradiology
1305
1307
Chiari I Malformation
Figure 5-25-2
Probably due to occipital bone dysplasia and small posterior

fossa
Caudal displacement of pegged cerebellar tonsils into upper
cervical spinal canal
Associated with:
Syringohydromyelia (25%-50%)
CVJ anomalies in up to 50%
Associated with many brainstem/lower cranial nerve sxs
(hearing loss, vertigo, abnormal gag/swallowing, etc.)
Chiari I Malformation [Figure 5-25-2]
Tonsillar ectopia
number of millimeters the tonsillar tips extend below the
foramen magnum (basion to opisthion)
< 3mm Normal
3-5mm Low-lying
>5 mm is quoted to be 100% specific and 92% sensitive
for Chiari I
Difference with age
tonsils regress with age
between 5-15 years, even 6mm may be OK if asymptomatic
Up to 50% have osseous CVJ anomalies
Basiocciput hypoplasia (short clivus)
Platybasia
Atlanto-occipital non-segmentation
Non-segmentation of C2-C3
Klippel-Feil deformity
Chiari I Malformation. Note

protrusion of cerebellar tonsils
(arrow) below the plane of the
foramen magnum (dotted line) and
cervical syringohydromyelic cavity.
The fourth ventricle (dot) is normal in
location and configuration
Chiari II Malformation - (Arnold Chiari Malformation)
Downward displacement of the cerebellar tonsils, inferior cerebellar vermis,

fourth ventricle, and medulla into the upper cervical spinal canal
Very commonly associated with a lumbar myelomeningocele
Chiari II Malformation - Imaging findings
Figure 5-25-3
[Figures 5-25-3 to 5-25-5]
Luckenschadel skull-universal at birth

Typically associated with Chiari II
An ossification disturbance of the membraneous skull
Is NOT the result of hydrocephalus
Disappears by approximately 6 months of age
Macrocephaly with colpocephaly
Large massa intermedia
Falx and tentorium hypoplasias
Beaking of the tectum
Triple peak appearance of pons/medulla
Low torcula (shallow posterior fossa)
Syringohydromyelia (50%)
Luckenschadel Skull-plain and CT

1306
1308
Neuroradiology
Figure 5-25-4
Figure 5-25-5
A
Chiari II Malformation-sagittal.
Note tectal beaking,
caudal displacement of cerebellar tonsils to C4, slit-like,
caudally-elongated fourth ventricle,
vertical straight sinus (small posterior fossa),
and concavity of the clivus
Chiari III Malformation
[Figure 5-25-6]
Herniation of cerebellum +/- brainstem, fourth ventricle, and upper

cord into a low occipital or high cervical encephalocele
Figure 5-25-6
Chiari III Malformation
Chiari IV Malformation
[Figure 5-25-7]
A severe cerebellar hypoplasia with essentially a CSF-filled

posterior fossa
Very small brainstempons most severely affected
Chiari IV Malformation
Figure 5-25-7
C
Chiari II Malformation-axial. Note
tectal beaking in A (arrow); triplepeak appearance in B (arrows); and
tentorial hypoplasia in C (arrows)
Neuroradiology
1307
1309
Disorders of Ventral Induction

(Insult at 5-10 weeks)
Figure 5-25-8
Holoprosencephaly
Alobar, semilobar, lobar
Septo-optic dysplasia (DeMorsiers syndrome)
Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome)
Posterior fossa malformations
Dandy Walker malformation and variants
Jouberts syndrome
Rhombencephalosynapsis
Alobar Holoprosencephaly [Figure 5-25-8]
Alobar holoprosencephaly
Most severe form-no diverticulation

Midline facial deformities (cycloplegia, cebocephaly, hypertelorism,
hypotelorism, cleft anomalies)
Microcephaly with monoventricle (absent septum pellucidum)
DDx: Hydranencephaly
Horseshoe-shaped forebrain
Fusion of basal ganglia and thalami
Absence of: Corpus callosum, falx/interhemispheric fissure,
olfactory tracts and bulbs
Azygos anterior cerebral artery
Figure 5-25-9
Alobar Holoprosencephaly - DDx: Hydranencephaly

[Figure 5-25-9]
CSF replaces cerebrum supplied by the internal carotid arteries

bilaterally (ICA occlusions occurring between 3 and 6 months in
utero)
Cerebellum, brainstem, and thalami are not involved as they are
supplied by the posterior circulation
Associated with: TORCH infections, maternal syphillis,
abdominal trauma, radiation
Semilobar Holoprosencephaly [Figure 5-25-10]
Small brain with monoventricle (absent septum pellucidum)

Falx may be present
Fusion of basal ganglia and thalami
Corpus callosum may be absent or incomplete
Some rudimentary sulcation may be present
Olfactory bulbs and tracts are absent
Azygos anterior cerebral artery
Hydranencephaly.
Note normal, non-fused thalami and
presence of falx anteriorly which
distinguish this from
alobar holoproencephaly
Lobar Holoprosencephaly [Figure 5-25-11]
LEAST severe form

Normal size brain
Monoventricle (absence of the septum pellucidum)
Usually no facial deformities
Lack of cleavage is usually subtle and frontal
CORONAL IMAGING IS BEST TO DETECT
Septo-Optic Dysplasia (DeMorsiers Syndrome) [Figure 5-25-12]
Partial or complete absence of the septum pellucidum

Hypothalamic / pituitary axis abnormalities in 66%
Absent fornix and corpus callosum dysgenesis
Schizencephaly in approximately 50%
Diminutive optic nerves and chiasm
1308
1310
Neuroradiology
Figure 5-25-10
Figure 5-25-11
Lobar holoprosencephaly
Figure 5-25-12
Semilobar holoprosencephaly
Septo-optic dysplasia
Neuroradiology
1309
1311
Cerebral Hemiatrophy
(Dyke-Davidoff-Masson Syndrome) [Figure 5-25-13]
Figure 5-25-13
Clinical: Hemiparesis, mental retardation, seizures

Small cerebral hemisphere from in utero ischemia or infarction
after infection or trauma
Compensatory ipsilateral:
Lateral ventricular enlargement
Calvarial thickening
Enlarged paranasal sinuses
Enlarged mastoid air cells
Posterior Fossa Malformations
Dandy-Walker Complex
Dandy-Walker malformation
Dandy-Walker variant
Mega cisterna magna
Jouberts syndrome
Dandy-Walker Malformation [Figure 5-25-14]
Hypoplasia of the cerebellar hemispheres, hypoplasia or aplasia

of the inferior cerebellar vermis, and marked enlargement of the
fourth ventricle
Etiology unclear but probably NOT failure of development of
fourth ventricular foranima
Prominent imaging features include:
Enlarged posterior fossamacrocephaly
Torcular-lambdoid inversion
Absence of the falx cerebelli
Hydrocephalus (91% at diagnosis)
Other brain anomalies are common
66% have associated anomalies including corpus callosal
hypogenesis(30%)
Polymicrogyria/ heterotopia(5%-10%),
Occipital cephaloceles (16%), syringohydromyelia
Association with various syndromes Fetal Alcohol, TORCH,
Aicardi , Klippel Feil..
Figure 5-25-14
Cerebral hemiatrophy.
Small left cerebral hemisphere. Note
left mastoid air cells and frontal sinus
are larger than right counterparts.
The left middle cranial fossa is also
diminutive
Note hydrocephalus,
torcular-lambdoid inversion
with large posterior fossa
cyst, and severe vermian
hypoplasia
1310
1312
Neuroradiology
Jouberts Syndrome [Figure 5-25-15]
Figure 5-25-15
Rare syndrome of vermian aplasia with

brainstem hypoplasia
Autosomal recessive inheritance
Variable symptoms: Ataxia, mental
retardation, pendular nystagmus,.
Molar-tooth sign from prominent superior
cerebellar peduncles
Bat-wing shape to fourth ventricle
Jouberts Syndrome.
Note vermian
hypoplasia and the
classic molar tooth
appearance of the
brainstem, best
appreciated on the
axial images
(Courtesy M Castillo)
Rhombencephalosynapsis [Figure 5-25-16]
Congenital fusion of cerebellar hemispheres,

dentate nuclei, and superior cerebellar
peduncles with vermian agenesis
Keyhole configuration of fourth ventricle
Absent septum pellucidum +/- SOD or
holoprosencephaly
Often hydrocephalus (aqueduct stenosis)
Clinical: Variable depending upon other
anomalies
Figure 5-25-16

(Insult at 2-4 months)
Microcephaly (usually intrauterine ischemia)

Generalized Megalencephaly
Unilateral Megalencephaly (hemimegalencephaly)
Hydranencephaly
Neurocutaneous Disorders
Aqueduct Anomalies (stenosis, etc.)
Unilateral Megalencephaly
(Hemimegalencephaly) [Figure 5-25-17]
Clinical: Intractable siezures, mental retardation, hemiplegia,

developmental delay
Cerebral hemisphere may appear so anomalous as to appear
unrecognizable
Association with Linear Sebaceous Nevus syndrome
Multiple migration and sulcation anomalies
Polymicrogyria/Agyria
Grey matter heterotopias
Figure 5-25-17
2 year-old male with ataxia and
developmental delay
Hemimegalencephaly. Note enlargement of the right hemisphere with

diffuse cortical thickening in a pachygyric appearance with abnormal
sulcation. The ipsilateral lateral ventricle is markedly enlarged
Neuroradiology
1311
1313

(Insult at 2-5 months)
Figure 5-25-18
Lissencephaly (agyria, pachygyria)

Non-lissencephalic cortical dysplasias (microgyria and
polymicrogyria)
Schizencephaly
Type I (closed-lip); Type II (open-lip)
Grey matter heterotopias
Callosal abnormalities
Complete/partial agenesis
Pericallosal lipomas
Lissencephaly [Figure 5-25-18]
Smooth Brain
Interruption during last phase of migration (11-26 weeks)
Usually severe disabilities, developmental delay, seizures
Lissencephaly Type I (Classic)
Lissencephaly Type II (Cobblestone)
Lissencephaly Type I
Arrested neuronal migration

Also termed agyria-pachygyria complex
Thick gray and thin white matter
Agyria: Parietal-occipital
Pachygyria (incomplete lissencephaly): frontal and
temporal
Figure 8 or hourglass configuration
Assoc with Miller-Dieker syndrome
Large deletion gene on chromosome 17p13.3
Lissencephaly Type II
Classic lissencephaly
Also termed Cobblestone lissencephaly

Due to neuronal overmigration
Associations include:
Walker-Warburg Syndrome
Fukuyama congenital muscular dystrophy
Figure 5-25-19
Non-Lissencephalic Cortical Dysplasias

Microgyria/Polymicrogyria
Innumerable, small cerebral convolutions with thickened cortex

and abnormal cortical histology
Cortex around Sylvian fissure commonly involved
DDx: Stenogyria (packed gyri) associated with the Chiari II
malformation
Typically associated with other migrational disorders
Schizencephaly (Cleft Brain) Type I

(Closed-Lip (Fused)) [Figure 5-25-19]
Cleft with fusion of opposing grey matter layers (no intervening

CSF) extending from cortex to ventricle
Fusion of ventricular ependyma and pia covering the brain (pialependymal seam)
Ventricular diverticulum (tit) at base of cleft is very useful for
identification of closed cleft
May have near-normal mentation +/- seizures and spasticity;
bilateral=worse prognosis
35%-50% have septo-optic dysplasia
Closed-lip schizencephaly. Note gray matter lining the cleft and a

ventricular tit at base of the seam (arrow). Patient also has septooptic dysplasianote absence of the septum pellucidum
1312
1314
Neuroradiology
Schizencephaly Type II
(Open-Lip (Separated)) [Figure 5-25-20]
Figure 5-25-20
CSF interposed between grey matter lining the cleft

IF NO GREY MATTER INTERPOSED, CONSIDER
PORENCEPHALY
Usually severe mental retardation, seizures, hypotonia, spasticity,
blindness, inability to walk or speak
Absent septum pellucidum (80%-90%)
Genetic counseling is required as there is a 5%-20% incidence of
brain abnormalities in siblings
Grey Matter Heterotopias [Figures 5-25-21 to 5-25-23]
Arrest of migrating neurons between ventricle and pial surface of

the brain (looks like GM on all imaging sequences)
Seizures and mental retardation are common
Associated with schizencephaly, callosal agenesis, agyria,
hemimegalencephaly
Three varieties
Nodular/periventricular-subependymal (nodules of GM indent
lateral ventricles; irregular ventricular walls)
Focal or diffuse subcortical (clumps of GM within the WM)
Band (circumferential and symmetric)
Most severe form with worst prognosis
Open-lip schizencephaly in two
Neurons fail to reach their destination.
different patients. Note absence of
Confluent band of gray matter between lateral ventricle
septum pellucidum in the patient with
and cortex separated from both by layer of white matter
bilateral schizencephaly
Figure 5-25-21
Figure 5-25-23
Nodular/periven
tricular
heterotopia
note signal
intensity of the
periventicular
nodules is
identical to that
of cortical grey
matter
Figure 5-25-22
A
Neuroradiology
Band heterotopia with the

classic double cortex sign
Bilateral subcortical nodular heterotopia (A and C-arrows) with complete callosal

dysgenesis noted in B. Also note closed-lip schizencephaly in A (open arrow)
C
1313
1315
Callosal Dysgenesis [Figures 5-25-24 and 5-25-25]
Corpus callosum forms anterior to posterior:

Genu ---> Body ---> Splenium --->Rostrum
Complete Agenesis
Clinical: Siezures, developmental delay, microcephaly
Imaging:
Absent corpus callosum
Elevated third ventricle
Separated lateral ventricles
Colpocephaly
Partial Dysgenesis
Acquired: Anterior CC is affected
Developmental: Posterior CC is affected
Lipomas
Associated with ACC in 40%
Located in interhemispheric fissure
Often encase the pericallosal arteries
Figure 5-25-24
Figure 5-25-25
B
A
B
Partial callosal dysgenesis: (A) The
splenium is absent. (B) Note gyri
separating the occipital horns in a
position normally occupied by the
splenium on the axial image
Callosal dysgenesis (complete). Note radially arranged gyri
converging toward the third ventricle in A; parallel, separated lateral
venticles in B; Probst bundles (arrows); vertical orientation to
hippocampi in C; and colpocephaly in D
1314
1316
D
Neuroradiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
Altman NR, Naidich TP, Braffman BH. Posteri or fossa malformations. AJNR Am J Neuroradiol 13:691-724,
1992
Barkovich AJ, Chung SH, Norman D. MR of neuronal migration anomalies. AJNR Am J Neuroradiol 8:10091017, 1987
Barkovich AJ, Chung SH. Unilateral megalencephaly: Correlation of MR imaging and pathologic characteristics.
AJNR Am J Neuroradiol 11:523-531, 1990
Barkovich AJ, Jackson DE, Jr., Boyer RS. Band heterotopias: A newly recognized neuronal migration anomaly.
Barkovich AJ, Kjos BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cystlike
malformations based on the results of multiplanar MR imaging. AJNR Am J Neuroradiol 10:997-988, 1989
Barkovich AJ, Kjos BO. Grey matter heterotopias: MR characteristics and correlation with developmental and
neurological manifestations. Radiology 182:493-499, 1992
Barkovich AJ, Kjos BO. Non-lissencephalic cortical dysplasia: Correlation of imaging findings with clinical
deficits. AJNR Am J Neuroradiol 13:95-103, 1992
Barkovich AJ, Kjos BO. Schizencephaly: Correlation of clinical findings with MR characteristics. AJNR Am J
Neuroradiol 13:85-94, 1992
Barkovich AJ, Norman D. MR imaging of schizencephaly. AJNR Am J Neuroradiol 9:297-302, 1988
Barkovich AJ, Norman D. Anomalies of the corpus callosum. AJNR Am J Neuroradiol 9:493-501, 1988
Barkovich AJ. Subcortical heterotopia: A distinct clinicoradiologic entity. AJNR Am J Neuroradiol 17:1315-1322,
1996
Byrd S, Osborn R, Bohan T, Naidich T. The CT and MR evaluation of migration disorders of the brain, II:
Scizencephaly, heterotopia, and polymicrogyria. Pediatr Radiol 19:219-222, 1989
Catilo M. Bouldin TW, Scatliff JH, Suzuki K. Radiologic-pathologic correlation: Alobar holoprosencephaly.
Fitz CR. Holoprosencephaly and related entities. Neuroradiol 25:225-238, 1983
Naidich TP, Altman NR, Braffman BH, McLone DG, Zimmerman RA. Cephaloceles and related malformations.
Osenbach RK, Menezes AH. Diagnosis and management of the Dandy-Walker malformation: 30 years of
experience. Pediatr Neurosurg 18:179-189, 1992
Truit CL, Barkovich AJ, Shanahan R, Marlado TV. MR imaging of rhombencephalosynapsis: Report of three cases
and review of the literature. AJNR Am J Neuroradiol 12:957-965, 1991
Neuroradiology
1315
1317
Neuroradiology Seminar 1:
Discussion of Unknown Cases
History
6-year-old girl with 10 days of vomiting
Pediatric Posterior Fossa Tumors
Medulloblastoma (PNET):1/3
Juvenile pilocytic astrocytoma (JPA): 1/3
Brain stem glioma: 1/6
Ependymoma: 1/6
Medulloblastoma or JPA?
Use non-contrast CT
Medulloblastoma: hyperdense
JPA: iso- or hypodense
Most reliable imaging feature to distinguish between these tumors
Medulloblastoma
Most common (?) childhood brain tumor

Childhood: 75% < 15 y/o, 50% < 10 y/o
Peak 4-8 y/o; second peak 15-35 y/o
Vermis into fourth ventricle
Cysts, calcification, hemorrhage rare
CT: 90-95% homogeneous, slightly hyperdense on NCCT, uniform
enhancement
Medulloblastoma
MR
Hypointense on T1WI
Cerebellopontine angle involvement rare
CSF spread: 20-25% at time of diagnosis
Check spine post-gad after brain MRI
Neuroradiology Seminar 1
1318
Neuroradiology
History
46-year-old male with marked short-term memory loss and bizarre behavior
over 9 month period
Sellar Masses: SATCHMO
Sellar tumor, Sarcoid

Aneurysm, Arachnoid cyst
Teratoma
Craniopharyngioma
Hypothalamic glioma, Histiocytosis, Hamartoma of tuber cinereum
Meningioma
Optic glioma
Craniopharyngioma
Arises from squamous epithelial remnants

50% <20 y/o; second peak: middle age
Location
70%: both intrasellar and suprasellar
20%: intrasellar only
10%: purely suprasellar
Craniopharyngioma
CT: typical = cystic with enhancing rim

Partially calcified enhancing mural nodule
MR: hyper or hypointense on T1WI; hyperintense on T2WI
Appearance does not correlate well with chemical composition of contents
Enhancement of rim: more common than in Rathkes cleft cyst
History
30-year-old woman (12 weeks pregnant) with headaches, nausea, and

vomiting. Her obstetrician saw papilledema on fundoscopic exam and asked
for an imaging study.
Neuroradiology
1319
Hemangioblastoma
Young, middle-aged adults

10-20% in von Hippel-Lindau (often multiple)
Cerebellar hemispheres: most common
Cervical spinal cord: #2 location
Cystic with mural nodule: 60%
Solid 40%
Calcification very rare
Nodule enhances intensely
History
62-year-old female with increasingly severe headaches
Pineal Region Masses
Germ Cell Tumors (60%)

Pineal parenchymal tumors (14%)
Pineocytoma
Pineoblastoma
Others
Pineal cyst, arachnoid cyst, lipoma
Vein of Galen AV fistula
Glioma
Meningioma
Most common primary non-glial intracranial neoplasm (16% of all brain

tumors)
Females > males (2:1)
Multiple 6-9%
Rare in children unless neurofibromatosis
Arise from meningothelial arachnoid villi and possibly dural fibroblasts or pial
cells
History
35 year-old African-American male with ataxia and headaches
1320
Neuroradiology
Germinoma
Lymphoma
Optic nerve glioma
Metastasis
Tuberculosis
Sarcoidosis
Sarcoidosis
Etiology: unknown
Worldwide prevalence
United States: more common in African-Americans and women
Peak age: 20-40 years old
Multi-organ disease
CNS: 5% of cases
Sarcoidosis Clinical
Adenopathy
Skin rash
Ocular abnormalities
Elevated angiotensin converting enzyme
Diagnosis: biopsy of skin or nodes
Sarcoidosis Imaging
4 forms
Parenchymal mass
Periventricular
Leptomeningeal
Mixed
Enhances intensely
Combination of parenchymal and leptomeningeal enhancement: clue to
diagnosis
Sarcoidosis Imaging
Hyperintense on T2-weighted images

Parenchymal: may mimic glioma
Dural: may mimic meningioma
Hydrocephalus
Lesions diminish with steroid therapy
Neuroradiology
1321
Neuroradiology Seminar 2:
Discussion of Unknown Cases
History
11-year-old male with sudden onset of headache and vomiting at school,

followed 5 days later by onset of left hemiplegia and weakness
Ring-enhancing Masses
MAGICAL DR
Metastasis
Abscess
Infarct (subacute)
Contusion
AIDS:
Toxoplasmosis
Lymphoma (usually immunocompromised)
Demyelinating disease
Resolving hematoma, Radiation necrosis
Most common primary CNS neoplasm overall

12-15% of all primary tumors
50% of all astrocytomas
Most common supratentorial neoplasm in adults
Peak age: 45-70 years old; children: 10%
Shorter clinical duration (usually less than 3 months)
Heterogeneous hemispheric mass with abundant vasogenic edema

Most develop from pre-existing astrocytomas
Subcortical white matter: frontal-temporal predilection
1322
Neuroradiology
WHO Grade IV
Mitotic activity
Pleomorphism
Hemorrhage: common
Endothelial proliferation and/or necrosis
Subarachnoid seeding: 2-5%
Heterogeneous mass
Necrosis and hemorrhage common
Calcification: rare
Enhancement: >90%
Ring-enhancement: central necrosis
Butterfly pattern: corpus callosum extension
History
70-year-old male with recurrent basal cell carcinoma and squamous cell
carcinoma. Had radiation therapy 18 months prior to this study.
Radiation Necrosis vs. Tumor
Conventional MR: difficult

Advanced imaging
MR spectroscopy: lactate, acetate, and succinate peaks without choline
peak
DWI: restricted water diffusion
PET/SPECT: hypometabolic
History
42-year-old male with 2-day history of left upper extremity and shoulder
weakness.
Neuroradiology
1323
Lymphoma
Increasing prevalence in immunocompromised and immunocompetent

populations
Dismal prognosis: some survivors at 4 years from time of diagnosis
B-cell lymphoma: perivascular spaces
Periventricular or leptomeningeal
Primary: brain parenchyma
Secondary: dura
Lymphoma
Non-contrast CT: hyperdense mass

Long TR images: hypointense
Enhancement virtually always but may be heterogeneous
Lymphoma in AIDS
Necrosis more common than in immunocompetent hosts

CT: hypodense
T2WI: hyperintense
Ring enhancement
Lymphoma vs. Toxoplasmosis
Anti-toxoplasma therapy for 3 weeks

Smaller: toxo
No change: probable lymphoma
PET / SPECT-Thallium
Lymphoma: hypermetabolic
History
41-year-old male.
History withheld.
Contusion
Rough inner table of skull

Anterior cranial fossa (frontal)
Middle cranial fossa (temporal)
Bowl of Jell-O model
Spectrum: contusion -> hematoma
Perivascular space -> parenchyma
Variable clinical disability
1324
Neuroradiology
History
7-year-old female with optic neuritis
Multiple Sclerosis
Clinical diagnosis
Etiology remains unknown
Cooler climate predilection
Children: very rare especially before puberty
Optic neuritis
Retrobulbar pain, central loss of vision, Marcus-Gunn pupil
Strong affinity for females and MS
Multiple Sclerosis: MR
T1WI: Hypointense
T2WI: Hyperintense
Frequently shows lesions that are clinically unsuspected
Active plaques enhance
Chronic plaques: no enhancement
MR often shows more disease than predicted clinically
Neuroradiology
1325
Pediatric Radiology
Childhood Urinary Tract Infection

Ellen Chung, LTC, MC
Urinary Tract Infection
Most common disorder of the urinary tract in children

Second most common infection in children
More common in girls after first 3-6 mo short urethra
Diagnosis bag vs. clean-catch vs. catheter
Bag specimen only valuable if negative
UTI
E. coli responsible for vast majority of cases

High recurrence rate
Infants and young children are less likely to have specific symptoms
Lower versus upper tract infection fever, systemic illness
Imaging Studies
Ultrasound
Infant and young child kidneys versus adult
Growth
Fetal lobulation
Increased cortical echogenicity <1 yo
Hyopechoic pyramids
Hydronephrosis vs. splaying of renal sinus fat
Renal size
Scarring, cortical thinning
Anomalies
Bladder filling-defects, diverticula, wall thickening, PVR
Lateral position of ureteral orifice
Ultrasound: Limitations
US is NOT a screening exam

Less sensitive than VCUG/RNC for diagnosis of VUR
Less sensitive than CECT and DMSA for acute pyelonephritis
Less sensitive than DMSA for renal scar
Figure 6-1-1
Renal agenesis
Renal ectopia
Simple
Crossed
Renal fusion
Horseshoe
Lump or cake
Renal Agenesis [Figure 6-1-1]
1 in 1000 live births

Ureter and ipsilateral hemitrigone are absent
Medial positioning of colonic flexure
Look in pelvis for ectopic kidney
If observed kidney is large, it is the only functioning kidney
Medial positioning of the hepatic
flexure, which almost touches the
spine, indicating absence of right
kidney from right renal fossa.
Differential includes renal agenesis,
renal ectopia, or nephrectomy
Pediatric Radiology
1327
1329
Renal Agenesis [Figures 6-1-2 and 6-1-3]
Figure 6-1-2
Ipsilateral adrenal is elongated and may

mimic kidney on US in newborn
25% have associated anomalies
Mullerian duct duplication
ipsilateral obstruction common
Seminal vesicle cyst ipsilateral
Renal Ectopia and Fusion
Failure to separate into two blastemas or

In the absence of the kidney, the adrenal gland (arrow) is
to migrate from pelvis in utero
elongated, and may be mistaken for a small kidney. The
Most common ectopia is pelvic
finding is bilateral in this patient, who has associated
Most common type of fusion is
pulmonary hypoplasia with bilateral pneumothorax. Bilateral
renal agenesis is incompatible with life
horseshoe
Anomalous kidney is often small,
dysmorphic and malrotated
Figure 6-1-4
Increased risk of trauma, stones, infection, renovascular
hypertension and possibly tumors
Horseshoe Kidney [Figure 6-1-4]
1 in 400-600 live births

Higher incidence in Turner syndrome
Low position with abnormal axis
Usually malrotated
Midline parenchymal isthmus well seen on US in young children
Multiple renal arteries and veins
Ureters cross isthmus
Associated with UPJO
Figure 6-1-3
Two cervices (arrows) and two widely separated

uterine horns (arrow heads) associated with right
renal agenesis
Renal Ectopia [Figures 6-1-5 and 6-1-6]
Most are small and dysmorphic

Collecting system is superficial and renal sinus echo complex is
absent or eccentric
May be mistaken for a mass
Blood supply from iliac arteries
1328
1330
Ultrasound of the kidneys shows illdefined lower poles on the

longitudinal images and a midline
parenchymal connection anterior to
the aorta on transverse imaging.
The midline parenchymal isthmus is
well seen in babies and toddlers with
horseshoe kidney. The kidneys are
abnormally echogenic in this patient
with renal dysfunction
Pediatric Radiology
Imaging Studies
Figure 6-1-5
VCUG
Gold standard exam for
reflux and urethral
abnormalities
Versus
cystosonography
Patient and parental
preparation
Male voiding image
Early-filling and oblique Empty left renal fossa with pelvic kidney behind the bladder and directly in
front of the spine. Note the abnormally small size, somewhat dysmorphic
views
appearance, and the eccentric renal sinus echo complex
Cyclic study in young
infants - more sensitive
Figure 6-1-6
Indications for VCUG
Febrile UTI
Abnormal ultrasound
Patient < 6 yo and
First UTI male or recurrent UTI female
First degree relative with VUR (RNC)
Solitary functioning kidney
Any patient with neurogenic bladder
Follow-up of patient with VUR (RNC)
Post-op to confirm success
Imaging Studies
Radionuclide cystogram
Lower radiation dose
Female gonads
Continuous imaging
Lack of spatial resolution
Ureteral insertion
Male urethra
Grade I VUR
Crossed fused renal ectopia on

excretory urogram. Note orthotopic
insertions of left and right ureters
Imaging Studies
Renal cortical scintigraphy

DMSA or glucoheptonate
More sensitive than ultrasound for renal scarring and pyelonephritis
Indications
Recurrent breakthrough infections
Suspected acute pyelonephritis but equivocal laboratory or imaging
findings
Imaging Studies
CT
Disadvantage of ionizing radiation and need for intravenous contrast
compared with US
More sensitive than US for acute pyelonephritis
Imaging Studies
MR
Gd-enhanced MR may be more sensitive than renal scintigraphy in acute
pyelo
Sensitive for renal scarring
Less available
Costly
Need for sedation
Pediatric Radiology
1329
1331
Imaging Studies
Figure 6-1-7
Diuretic Renography
Obstructive versus nonobstructive
dilatation when VCUG shows no
reflux
Differential function
VUR
Vesicoureteric Reflux
Primary abnormality related to length

and angle of submucosal course of
distal ureter
Versus secondary bladder outlet
obstruction, neurogenic bladder,
bladder diverticulum
Up to 50% of children with UTI
Internationally standardized VCUG reflux grading system.

Illustration by Heike Blum, MFA
Figure 6-1-8
Vesicoureteric Reflux
Normal US in 75%
Hydronephrosis especially if changing
Mild pelviectasis does not predict VUR
Renal scar, cortical thinning, or lack of growth
Urothelial thickening
VCUG Reflux Grading System [Figure 6-1-7]

What to Look for in Addition to VUR
Ureteral insertion
Bladder filling defect
Axis of collecting system
Coexisting obstruction
Intrarenal reflux
VCUG showing ureter inserting into a Hutch

diverticulum. This finding is an indication for
surgery
Abnormal Ureteral Insertion
Into or near diverticulum secondary rather than primary VUR

Ectopic insertion too close to bladder neck
Bladder Diverticula [Figure 6-1-8]
Usually secondary to urethral obstruction and neurogenic bladder

Congenital diverticula also occur usually solitary
Herniation of urothelium through defect in muscular
wall of bladder
Hutch diverticulum at UVJ, associated with VUR
Figure 6-1-9
Ectopic Ureter [Figure 6-1-9]
Lower than normal

3-4 x more frequent in females usually upper pole
of duplex system
Girls may present with lifelong, day and night
incontinence
Left image from a VCUG showing an ectopic right
In males, the ectopic ureter inserts above the
ureter inserting into the urethra below the external
sphincter in this girl with lifelong day and night
external sphincter
incontinence. Right image shows the ectopic
The associated kidney may be dysplastic or atrophic,
ureter
is an upper pole ureter of a duplex system,
especially upper pole of a duplex
which is usually the case in girls with ectopic
ureter; however, this is a very unusual image,
because ectopic upper pole ureters do not reflux.
This image was obtained because the catheter
preferentially selected the upper pole ureter rather
than the bladder when it was placed in the urethra
1330
1332
Pediatric Radiology
Ureterocele [Figure 6-1-10]
Dilation of the distal ureter, usually caused by

stenosis of the UVJ, with invagination into the bladder
Ectopic vs simple
In females, usually ectopic
Can be so large as to cause obstruction of bladder
outlet or contralateral UVJ
Cobra-head or spring onion appearance
With bladder filling, can evert into distal ureter and
mimic a diverticulum
Figure 6-1-10
Abnormal Axis of Intrarenal Collecting

System [Figure 6-1-11]
Normally a line drawn through the upper- and lowermost calcyces points to the opposite shoulder.
Causes of abnormal axis
Duplicated collecting system
Malrotation with or without ectopia/fusion
anomaly
Mass, especially neuroblastoma
Ureteropelvic duplication [Figures 6-1-12 and 6-1-13]
Common 1 in 160
Spectrum from bifid pelvis to complete duplication
50% bilateral
Complete duplication associated with increased
incidence of UTI, VUR, scarring and obstruction
Upper pole ureter is ectopic and may be obstructed
Lower pole moiety may have VUR or UPJ obstruction
Everting ureterocele. On upper left early filling

view, the ureterocele is easily identified as a
round filling defect. It becomes progressively
smaller the increased bladder filling until, in the
lower right image, it everts into the distal ureter,
mimicking a Hutch diverticulum
Figure 6-1-11
Figure 6-1-12
The kidney on the left demonstrates a normal

axis, directed toward the opposite shoulder. The
kidney on the right shows the axis directed toward
the ipsilateral shoulder. This is the drooping lily
sign of a duplex kidney
Figure 6-1-13
There is grade II VUR on the right

and a straight renal axis due to
duplication. There is grade V VUR on
the left and a drooping lily sign. Note
the filling defect in the left side of the
bladder. This is due to the urine-filled
ureterocele of the obstructed upper
pole. The course of the lower pole
ureter is unusual because it is
intertwined with the dilated upper pole
ureter. The upper pole ureter is not
seen on the VCUG, because it is
obstructed and does not reflux
Pediatric Radiology
The dilated upper pole ureter and its ureterocele

are seen on ultrasound
1331
1333
Coexisting Obstruction [Figure 6-1-14]
Not associated with each other, but both common

The presence of contrast in upper tract due to reflux
allows one to diagnose the obstruction
3 signs
Hesitation
Dilution
Delayed drainage
Figure 6-1-14
Intrarenal Reflux
Important factor in the pathogenesis of renal scarring

More commonly occurs at the poles
Indication for surgery
Vesicoureteric Reflux [Figures 6-1-15 to 6-1-17]
Relationship between VUR and renal scarring controversy

Can reflux of sterile urine cause scarring?
Does asymptomatic bacteriuria require tx?
3 Conditions are necessary for renal scarring to
occur
UTI
VUR
Intrarenal reflux
VCUG showing the 3 signs of VUR with coexisting
obstruction. First, the obstruction works in both
directions, so the contrast hesitates to go past the
obstruction. Second, behind the obstruction is a
lot of trapped, unopacified urine. When the
contrast gets past the obstruction, it is diluted by
the unopacified urine. Third, there is delay in the
drainage of the contrast that got past the
obstruction
Figure 6-1-15
Figure 6-1-16
Illustration and gross specimen of the simple

papilla. In the simple, unfused papilla with its
pyramidal shape, the collecting ducts empty onto
the papillary surface at an oblique angle. As the
surrounding calyx becomes distended with urine,
these slit-like openings tend to close off
Figure 6-1-17
In the compound papilla, there is distortion of the

surface, which is greatest where there is the most
fusion. As shown in this diagram and gross
specimen, at these flatter, distorted surfaces, the
openings of the collecting ducts are more rounded.
When the calyx gets full, these cannot close, and
urine in the calyx can flow retrograde, which is
intrarenal reflux
The blush of renal parenchyma seen in the VCUG

of this patient with VUR shows the typical brush
or fan like configuration of the papillary ducts and
collecting tubules. This is macroscopic intrarenal
reflux
1332
1334
Pediatric Radiology
Acute Pyeloneprhitis
Figure 6-1-18
May be hematogenous, but usually an ascending

infection
Barriers to infection
Perineal resistance which is overcome by
Vaginal reflux, labial adhesions
Uncircumcised male
Frequent voiding
Functioning UVJ
Papillary/calyceal structure resistance to
intrarenal reflux
Acute Pyeloneprhitis - Clinical
Infants usually present with fever, but may present

with nonspecific symptoms
Older children present with fever, flank pain but may
call it abdominal pain
Pathologic appearance of acute pyelonephritis.
If straight forward clinical picture, no imaging needed The gross image on the left shows that the surface
of the involved parenchyma is pale compared to
in acute setting
the surrounding normal parenchyma. Note the
sharp demarcation between normal and abnormal.
Acute Pyelonephritis - Pathology [Figure 6-1-18] There is a patchy or lobar distribution. On the cut
Gross Path full thickness, loss of C-M
specimen on the right, the medial upper pole is
normal. In the lateral upper pole and mid portion,
differentiation, enlargement, sharp demarcation,
the infection involves a full-thickness wedge from
urothelial thickening
the papilla to the surface of the kidney. The tissue
Histo tubulointerstitial nephritis
is expanded. There is disruption of the
corticomedullary differentiation and a striated
Acute Pyelonephritis - Imaging [Figure 6-1-19]]
appearance
If fails to respond to therapy, US vs. CT to evaluate
for complication
CT/US/Nuc - triangular, peripheral focus of decreased flow, decreased corticomedullary differentiation
Diffuse or focal enlargement can appear mass-like
VCUG indicated may perform while hospitalized
Figure 6-1-19
US demonstrating focal enlargement with decrease flow mimicking a mass. The more
sensitive enhanced CT shows more diffuse triangular and striated areas of decreased
enhancement typical of acute pyelonephritis
Complicated Upper Tract Infection
Renal or perinephric abscess

Pyonephrosis must be treated urgently
Reflux Nephropathy
Renal scarring chronic pyelonephritis post-infectious nephropathy

Related to bacterial infection, VUR, and intrarenal reflux
Usually at poles especially upper
Scarring can be prevented or limited if early diagnosis of upper tract infection
Pediatric Radiology
1333
1335
Reflux Nephropathy [Figure 6-1-20]
Destruction centered in medulla

Full thickness - indentation overlying dilated calyx (versus fetal
lobulation)
Echogenic focal scar
Small kidney, no growth
Compensatory hypertrophy between scars can appear masslike
Almost all cases of severe scarring have VUR
Figure 6-1-20
Vesicoureteric Reflux - Treatment
Medical with annual follow-up US, RNC and urine culture

Surgical reimplantation
Endoscopic subureteric injection
Surgery is considered for
Failure to resolve or worsening
Scarring or growth failure
High grade VUR
Intrarenal reflux
Frequent breakthrough infections
Upper pole cortical thinning due to

reflux nephropathy. Note that the
upper pole calyx extends almost to
Neurogenic Bladder
the interface between the kidney and
Failure of detrusor muscle and internal and external sphincters to
the liver
function in concert to hold and release urine
Emptying or filling phase dysfunction
Causes spinal dysraphysm, caudal regression, paraplegia, presacral
teratoma, anterior sacral meningocele
Suspect in patients with recurrent UTI and constipation
Lower motor neuron lesion large, smooth-walled bladder
Upper motor neuron lesion small, trabeculated bladder
Secondary VUR
Neurogenic Bladder - Imaging
Trabeculated, thick-walled or large and atonic

Taller than wide
Funnel-shaped bladder neck
Large post-void residual
Look for spinal dysraphism
Bladder Augmentation
Used to treat small, noncompliant bladders

If small bowel is used, gut signature and peristalsis are seen on US
Complication of bladder rupture
Alternative - autologous bladder cells grown in tissue culture
Antenatal Pelvicaliectasis
Prevalence of prenatal sonography has changed the natural history of some

causes of neonatal hydronephrosis
Common causes
VUR
UPJO
Obstructed upper pole of duplex system
PUV
Antenatal Pelvicaliectasis - Work-Up
Perform postnatal ultrasound after DOL 4 or 5

VCUG if mod-severe hydro
In male with bilateral severe hydro, perform VCUG before discharge
Repeat at 6 weeks if normal or mild
If VCUG negative, perform diuretic renography or excretory urogram
1334
1336
Pediatric Radiology
Posterior Urethral Valves
Most common cause of urethral obstruction in the male infant

Usually present in infancy (first UTI male) or prenatally diagnosed
Less commonly delayed presentation with failure to thrive,
incontinence, or hypertension
Figure 6-1-21
Posterior Urethral Valves - Imaging [Figures 6-1-21 and 6-1-22]
3 types
Type 1 - folds attach below the veru montanum
Type 2 folds attach above the veru
Type 3 diaphragm with central opening
Trabeculated or thick-walled bladder
Dilated posterior urethra perineal window US
Bilateral hydronephrosis not a constant finding but bilateral
hydro in a male infant is PUV until proven otherwise
Posterior Urethral Valves [Figure 6-1-23]
In utero obstruction causes renal dysplasia (dysgenesis)

Prognosis related to degree of renal dysplasia
Factors that protect one or both kidneys
Large bladder
Bladder or calyceal diverticula
Unilateral VUR or no VUR (50%)
Urinary ascites
No VUR valve bladder
Figure 6-1-22
Figure 6-1-23
Same patient showing posterior

urethral valve and dilated posterior
urethra
Ultrasound of a patient with PUV showing

irregular, thickened bladder wall, patulous UVJ,
and hydronephrosis. Note also the cysts and poor
corticomedullary differentiation due to associated
renal dysplasia
Prune Belly Syndrome
Trabeculated bladder in 1 month-old

male with bilateral prenatal
hydronephrosis. Note the irregularity
of the wall despite the bladder being
full
AKA Eagle-Barrett or Triad syndrome

Triad
Hypoplastic or absent abdominal wall musculature
Cryptorchidism
Urinary tract anomalies
Almost exclusively males
Prune Belly Syndrome 2 Types
Severe
Complete urethral obstruction (bladder like PUV)
Renal dysplasia and pulmonary hypoplasia
Associated anomalies of GI tract, genital tract, CHD
Death in first year of life
Pediatric Radiology
1337
1335
Figure 6-1-24
Mild-Moderate
Functional abnormality of bladder emptying
floppy, dilated bladder
Urachal remnant
Mild to markedly dilated renal pelvis and ureters
Ureters mimic small intestine
+/- pulmonary hypoplasia
Long-term survival
Prune Belly Syndrome [Figures 6-1-24 and 6-1-25]
Dilated ureter, bladder and urethra with patchy foci of

Young infant with Eagle-Barrett
deficient musculature
Syndrome. Note dilated flaccid
bladder, reflux into tortuous ureters
Other findings dilated posterior urethra,
megalourethra, urethral diverticula, dilated prostatic with the appearance of small bowel
loops, urachal remnant at dome of
utricle
bladder and dilated posterior urethra
without valve
Other Causes of Hydronephrosis
Ureteropelvic Junction Obstruction
Most common cause of upper tract obstruction

Previously presented with flank pain, mass, UTI or hematuria with
mild trauma
Now commonly prenatally diagnosed
Dilated pelvis and calices no dilated ureter
Ddx: extrarenal pelvis
Figure 6-1-25
UPJO
Associations
Increased risk of abnormality of contralateral kidney most
common is UPJO
Renal dysplasia
VUR
UVJ obstruction
Lower pole moiety of duplex kidney
Horseshoe kidney
UPJO - Treatment
Mild mod obstruction is followed and treated if it worsens

Severe obstruction in young children is treated with
dysmembered pyeloplasty
In adults and adolescents alternative treatment is endopyelotomy
Often remain dilated after repair
Figure 6-1-26
UPJO [Figures 6-1-26 and 6-1-27]
Radiograph of same patient showing

flaccid abdominal wall musculature.
Also the infant is intubated with small
lungs, bell-shaped thorax and medial
right pneumothorax due to associated
pulmonary hypoplasia
Intrinsic versus extrinsic

Intrinsic fibrosis, stricture, valve/fold, etc
Extrinsic crossing vessel
Intermittent symptoms and findings
Dietls crisis
Prenatally diagnosed 10-15% extrinsic
Diagnosed due to symptoms - 50% extrinsic
CT of patient with UPJO obtained when the patient
presented with severe abdominal pain and
peritoneal signs following a MVC. Note the dilated
pelvis and calyces, and the urine in the
retroperitoneum due to UPJ rupture. Note also the
large crossing vein that was found to be the cause
of the UPJO at surgery. Prior to this accident the
patient previously complained of chronic
abdominal pain and was treated for lactose
intolerance and had an appendectomy for a
normal appendix
1336
1338
Pediatric Radiology
Primary Megaureter [Figure 6-1-28]
Functional obstruction analogous to Hirschprung

disease of the GI tract
Short aperistaltic segment of distal ureter
Proximal ureter dilates aperistaltic segment fixed
and narrowed
More common in boys and on the left
Predisposed to UTI and stone formation
Surgically repaired only if severe or symptomatic
Figure 6-1-27
Congenital Megacalyces
Nonobstructive enlargement of calyces and

hypoplasia of the medullary pyramids
Benign nonprogressive condition
Can coexist with megaureter
Occasional stone formation, hematuria
Complications of endopyelotomy performed in a

patient with UPJO due to crossing vessel. The
upper left image shows the origin of the left main
Large bladder without obstruction
Large ureteral orifices with free reflux and voiding into renal artery. The lower right image shows the
origin of an accessory renal artery to the lower
markedly dilated ureters
pole, which on the lower left image is seen to
cross the stent in the ureter (arrow). This artery
Summary
was cut in the procedure causing the perirenal
hematoma seen well in the lower two images.
VUR may be primary or secondary to bladder outlet
obstruction/ neurogenic bladder or abnormal ureteral Note also the infarction of the anterior lower pole
in the upper right image, due to spasm of the cut
insertion
artery
Primary VUR is familial and resolves by age 6, but
secondary VUR requires surgical intervention
In primary VUR the VCUG appears normal except for reflux
Figure 6-1-28
US is insensitive and is not a screening exam
On US, look for scarring and lack of
growth
RNC best for sibling screen, girls, and
follow-up
VCUG best for symptomatic patients
and boys
Surgical options are surgical
reimplantation or subureteric injection
Bilateral hydro in an infant male is due
to PUV until proven otherwise
Not all boys with valves have reflux and
hydronephrosis
In utero obstruction causes renal
dysplasia
UPJO is associated with abnormality of
Primary megaureter. Excretory urogram shows dilation of the
other kidney
left ureter proximal to a short, fixed, relatively narrow segment
UPJO diagnosed in utero or on
of ureter near the UVJ
screening US is caused by intrinsic
abnormality in 85% of patients
UPJO diagnosed due to symptoms is caused by extrinsic compression
(crossing vessel) in 50% of patients
Extrinsic compression causes transient sx and findings
Endopyelotomy is contraindicated in patients with crossing vessels
Congenital Megacystis-Megaureter
Pediatric Radiology
1337
1339
References
Texts
1. Kirks DR, ed. Practical Pediatric Imaging, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998.
2. Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002.
3. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams & Wilkins,
2004.
Journal Articles
1. American Academy of Pediatrics Committee on Quality Improvement Subcomittee on Urinary Tract Infection. Practice
parameter: the diagnosis, treatment and evaluation of the initial urinary tract infection in febrile infant and young
children. Pediatrics 1999;103:842-852.
2. Berrocal T, Gaya F, Arjonilla A. Vesicoureteral reflux: diagnosis and grading with echo-enhanced cystosonography
versus voiding cystourethrography. Radiology 2001;221:359-365.
3. Blane CE, DiPietro MA, Strouse PJ, et al. Pediatric renal pelvic fullness: an ultrasonographic dilemma. J Urol
2003;170:201-203.
4. Blane CE, DiPietro MA, Zerin JM, et al. Renal sonography is not a reliable screening examination for vesicoureteral
reflux. J Urol 1993;150:752-755.
5. Brown T, Mandell J, Lebowitz RL. Neonatal hydronephrosis in the era of sonography. AJR Am J Roentgenol
1987;148:959-963
6. Daneman A, Alton DJ. Radiographic manifestations of renal anomalies. Radiol Clin North Am 1991;29:351-363.
7. Davey MS, Zerin JM, Reilly C, et al. Mild renal pelvic dilation is not predictive of vesicoureteral reflux in children.
Pediatr Radiol 1997;27:908-911.
8. Donnelly LF, Gylys-Morin VM, Wacksman J. Unilateral vesicoureteral reflux: association with protected renal function
in patients with posterior urethral valves. AJR Am J Roentgenol 1997;168:823-836.
9. Eggli KD, Eggli D. Color Doppler sonography in pyelonephritis. Pediatr Radiol 1992;22:422-425.
10. Elder JS, Peters CA, Arant BS Jr, et al. Pediatric vesicoureteral reflux guidelines panel summary report of primary
vesicoureteral reflux in children. J Urol 1997;157:1846-1851
11. Fernbach SK, Feinstein KA, Schmidt MB. Pediatric voiding cystourethrography: a pictoral guide. RadioGraphics
2000;20:155-168.
12. Gross GW, Lebowitz RL. Infection does not cause reflux. AJR Am J Roentgenol 1981;137:929.
13. Hoffer FA, Lebowitz RL. Intermittent hydronephrosis: a unique feature of ureteropelvic junction obstruction caused
by a crossing renal vessel. Radiology 1985;156:655-658.
14. Lavocat MP, Granjon D, Allard D, et al. Imaging of pyelonephritis. Pediatr Radiol 1997;27:159-165.
15. Lebowitz RL, Blickman JG. The coexistence of ureteropelvic junction obstruction and reflux AJR Am J Roentgenol
1983;140:231-238.
16. Lebowitz RL, Olbing H, Parkkulainen KV, et al. International system of radiographic grading of vesicoureteral reflux.
International Reflux Study in Children. Pediatr Radiol 1985;15:105-109.
17. Lonergan GJ, Pennington DJ, Morrison JC, et al. Childhood pyelonephritis: comparison of Gadolinium- enhanced
MR imaging and renal cortical scintigraphy for diagnosis. Radiology 1998; 207:377-384.
18. Mentzel JJ, Vogt S, Patzer L, et al. Contrast enhanced sonography of vesicoureterorenal reflux in children: preliminary
results. AJR Am J Roentgenol 1999;173:737-740.
19. Orellana P, Baquedano P, Rangarajan V. Relationship between acute pyelonephritis, renal scarring and vesicoureteral
reflux. Results of a coordinated research project. Pediatr Nephrol 2004;19:1122-1126.
20. Paltiel HJ, Mulkern RV, Perez-Atayde A. Effect of chronic low-pressure sterile vesicoureteric reflux on renal growth
and function in a porcine model: a radiologic and pathologic study. Radiology 2000;217:507-515.
21. Paltiel HJ, Rupich RC, Kiruluta HG. Enhanced detection of vesicoureteral reflux in infants and children with use of
cyclic voiding cystourethrography. Radiology 1992;184:753-755.
22. Rooks VJ, Lebowitz RL. Extrinsic ureteropelvic junction obstruction from a crossing renal vessel: demography and
imaging. Pediatr Radiol 2001;31:120-124.
23. Sargent MA. What is the normal prevalence of VUR? Pediatr Radiol 2000; 30:87-593.
24. Van den Abbeele AD, Treves ST, Lebowitz RL, et al. Vesicoureteral reflux in asymptomatic siblings of patients with
known reflux: radionuclide cystography. Pediatrics 1997;79:147-153.
25. Walsh G, Dubbins PA. Antenatal renal pelvis dilatation: a predictor of vesicoureteral reflux? AJR Am J Roentgenol
1996;167:887-890.
1338
1340
Pediatric Radiology
Neonatal GI Tract Obstruction

Figure 6-2-1
Objectives
To become familiar with the diagnosis of the major

causes of proximal and distal GI obstruction in the
neonatal period (first 24 to 48 hours)
To develop an approach to the evaluation of the
obstructed neonate including a method of performing
diagnostic and therapeutic enema
Neonatal Intestinal Obstruction - General

Principles
Any obstruction distal to the ampulla of Vater causes

biliary emesis
Perforation may be present without free air
Loop immediately proximal to atresia often
disproportionately dilated
Start evaluation with plain radiographs
Small and large bowel cannot be differentiated on
plain film
Neonatal Intestinal Obstruction
High
Proximal to mid ileum
Few dilated loops
UGI or no further imaging
Low
Distal ileum, colon
Many dilated loops
Contrast enema
5 types of esophageal atresia/distal fistula
ESOPHAGUS
Figure 6-2-2
Esophageal Atresia
Error in differentiation of the foregut into trachea and esophagus

Spectrum from esophagotrachea to H-type fistula without
esophageal atresia
Presentation feeding intolerance, regurgitation, choking,
aspiration, increased oral secretions, symptoms in first 24h of life
in 85 - 95%
Half have other anomalies - VACTERL
Esophageal Atresia [Figures 6-2-1 and 6-2-2]
Junction of upper and middle 1/3s

Fistula 0.5 to 1.0 cm above carina in 89%
5 types
Length of the gap - longest without fistula
Atresia with distal fistula is most common
H-type present later due to chronic aspiration, actually Nshaped
Most common type is esophageal

atresia with distal fistula
Pediatric Radiology
1339
1341
Esophageal Atresia - Imaging [Figures 6-2-3 and 6-2-4]
Esophageal Atresia - Imaging
Figure 6-2-3
Prenatal
Polyhydramnios
+/- no stomach bubble
+/- dilated fluid-filled proximal pouch
Neonatal
Anterior tracheal displacement on lateral CXR
Distal bowel gas (85%)
Gasless (8%)
Dilated air-filled pouch
OG tube coiled in pouch
UGI usually not indicated use air
Cross-sectional imaging: 3D CT, virtual

bronchoscopy
Document side of arch for surgical planning
Ddx: pharyngeal perforation pneumomediastinum,
pleural effusion
EA/distal TEF. AP radiograph shows dilated, airfilled proximal pouch. Lateral view shows NG
coiling in pouch and anterior displacement of the
trachea
Esophageal Atresia Post-op
Complications
Anastomotic leak
Anastomotic stricture
Recurrent fistula
Reflux esophagitis/stricture
Expected findings
Disordered motility below anastomosis
GER
Tracheomalacia
Figure 6-2-4
VACTERL
Non-random association of anomalies

No one patient has all
High perinatal mortality > 60%
46% of patients with TEF
Recurrence risk (offspring) 2-3%
Recurrence risk (siblings)
TEF or EA < 1%
Other VACTERL lesions 1.2%
Ddx: Pharyngeal Perforation
Traumatic delivery
Traumatic intubation
Nasogastric tube placement
STOMACH
Gastric Atresia/Antral Web
Very rare
Atresia presents near birth
Web is usually perforated so present in childhood with recurrent
nonbilious emesis
Esophagram showing H-type fistula

with filling of tracheobronchial tree
Microgastria
Rare
Isolated vs. associated anomalies, especially asplenia
Small tubular midline stomach and dilated distal esophagus
Duodenal bulb may also dilate
1340
1342
Pediatric Radiology
DUODENUM
Figure 6-2-5
Normal Rotation of Midgut Loop [Figure 6-2-5]
GI tract straight short tube

Midgut divided by SMA
270 degrees counterclockwise rotation 6th week
Normal mesentery is broad based
Malrotation/Malfixation - Pathogenesis
All types referred to as malrotation

Malfixation - short root of mesentery predisposes to midgut volvulus
Ladd bands
Attempts to secure the bowel
Most common site is from high medial cecum across 2nd-3rd portion of
duodenum to porta hepatis
May cause obstruction
Figure 6-2-6
Normal intestinal rotation

and fixation showing
long root of the
mesentery (line)
Figure 6-2-7
Illustrations showing malrotation and midgut volvulus (center)
Midgut Volvulus - Presentation
Prenatal
Necrosis of bowel and multiple atresias
First month of life most patients
Bilious emesis, occasional bloody stool
Older child
Chronic recurrent abdominal pain, failure to thrive, diarrhea,
malabsorption
Volvulus can occur at any age
Most common location of

Ladd band
Figure 6-2-8
Midgut Volvulus - Pathology [Figure 6-2-8]
Volvulus impedes venous and lymphatic return leading to bowel

wall edema
If prolonged, arterial obstruction and bowel infarction
Malrotation Plain Radiograph [Figure 6-2-9]
Evaluation of emesis/obstruction begins with plain film

Classic - partial obstruction of duodenum 2nd -3rd portion -->
malro until proved otherwise
May mimic gastric outlet obstruction
Ileus or distal SBO
Gasless abdomen
Normal
Chronic midgut volvulus
Pediatric Radiology
1341
1343
Malrotation UGI
Figure 6-2-9
Duodenal jejunal junction diagnostic

Normal position on AP is on or to the left of the left pedicle of L1
Normal position on lateral is posterior/retroperitoneal
AP must be perfectly positioned
Malrotation UGI [Figures 6-2-10 and 6-2-11]
Jejunum in RUQ
DJJ may be displaced by distended bowel, masses and enlarged
organs
If DJJ is equivocal, empty stomach with NG tube or complete
small bowel follow-through
Figure 6-2-10
Plain radiograph of a newborn with

bilious emesis. High small bowel
obstruction is noted, with all air-filled
loops on the right
Normal position of the DJJ on frontal view (left

image) on or to the left of the left pedicle of L1.
Lateral view from an UGI (right image) showing
the normal retroperitoneal location of the
duodenum
Figure 6-2-11
Midgut Volvulus UGI [Figure 6-2-12]
Duodenal obstruction
Beak
Corkscrew appearance of duodenum and jejunum
Malrotation Contrast Enema
No longer part of work-up

Cecum and DJJ rotate independently
Cecum normal in 20% of patients with malrotation so enema is no
longer used in the work-up
High mobile cecum is a common normal variant 15%
Entire colon may be to left of midline in malrotation
More often cecum is high and medial
Malrotation. Duodenal jejunal junction
low and to the right of midline
Figure 6-2-12
Lateral view showing obstruction of

the 2nd 3rd portion of the
duodenum and corkscrew
appearance of the jejunum diagnostic
of malrotation
1342
1344
Pediatric Radiology
Malrotation Ultrasound/CT [Figures 6-2-13 and 6-2-14]
Dilated proximal bowel, wall thickening, ascites

Inversion of SMA/SMV relationship1
Normally the SMV is to the right of the SMA
33% surgically proven malrotation have normal relationship
8 of 9 with SMA/SMV inversion had malrotation
Below the portal confluence, look for the swirl sign
Figure 6-2-13
1Zerin JM, DiPietro MA. Superior mesenteric vascular anatomy as
ultrasound in patients with surgically proved malrotation of the midgut.

Radiology 1992;
183:693-4
Ultrasound images showing inversion
of the SMV/SMA relationship. The
Figure 6-2-14
SMV is usually larger and the SMA is
usually surrounded by echogenic fat,
but he identity of each vessel must
be confirmed with Doppler or by
connecting SMV to the portal vein
Midgut volvulus in 4 yo with acute

onset of emesis. Inversion of
SMA/SMV relationship (lower image)
and swirl sign (upper image) of
volvulus. SMV (arrow) is to the left of
the SMA
Malrotation - Treatment
Ladd procedure
Reduce midgut volvulus
Lyse bands
Place in orientation of nonrotation all small bowel on the right and all
colon on the left
Inversion appendectomy
95% have no recurrence
Malrotation Associated Congenital Anomalies
Omphalocele
Gastroschisis
Diaphragmatic hernia
Bowel atresia/stenosis
Heterotaxy not surgically repaired
Duodenal Atresia/Stenosis/Web
Atresia much more common than stenosis

3-6 weeks gestation failure of canalization of solid tube of foregut
Annular pancreas in 20%
May have preduodenal portal vein
Pediatric Radiology
1343
1345
Duodenal Atresia - Clinical
30% have Downs syndrome

Associated with other atresias, biliary anomalies, CHD,
VACTERL
Almost always just distal to ampulla of Vater --> bilious emesis
Figure 6-2-15
Duodenal Atresia - Imaging [Figures 6-2-15 to 6-2-18]
Polyhydramnios
Double bubble
Windsock deformity
How do we know it is not midgut volvulus? Dilation of duodenal
bulb indicates it is a chronic condition
Double bubble is diagnostic, but if obstruction is not complete,
an UGI is indicated
Figure 6-2-16
Double bubble is diagnostic of duodenal

atresia. vein
Figure 6-2-17
Prenatal ultrasound for

polyhydramnios shows fluid-filled
double bubble. This finding should
prompt chromosomal analysis for
possible Trisomy 21
Figure 6-2-18
Annular pancreas. Upper GI shows

circumferential narrowing of second
portion of duodenum
Caution: Incomplete obstruction mandates an

UGI. Double bubble appearance with distal gas
caused by midgut volvulus with beak appearance
on upper GI
1344
1346
Pediatric Radiology
JEJUNUM
Figure 6-2-19
Jejunal Atresia and Stenosis
Atresia more common than stenosis

Ischemic injury to developing gut
Primary vascular accident more common
Secondary to mechanical obstruction, e.g. in utero volvulus
Jejunal Atresia - Presentation
Bilious emesis first hours of life

Distended epigastrium with scaphoid lower abdomen
+/- failure to pass meconium
Intraoperative photograph
demonstrating jejunal atresia
Atresia/Stenosis - Pathology [Figure 6-2-19]
Most common sites are proximal jejunum and distal ileum

20% multiple
5 types
Two types inherited
Apple-peel (Christmas tree)
Syndrome of multiple intestinal atresias and intraluminal calcification
Figure 6-2-20
Jejunal Atresia - Imaging [Figure 6-2-20]
Triple bubble
Dilated loop of bowel proximal to atresia is disproportionately
dilated with bulbous end
Proximal bowel may be fluid-filled and mimic a mass
UGI is rarely indicated
Surgeon may request BE to evaluate for additional distal atresia
Proximal Neonatal Intestinal Obstruction - Ddx
Esophageal atresia
Gastric atresia/web
Malrotation/midgut volvulus
Duodenal atresia
Jejunal atresia
All surgical UGI only to determine who needs to go emergently
Low Intestinal Obstruction
Plain film
Multiple dilated loops of bowel
Contrast enema
Dilute ionic water-soluble contrast (cystography)
Low osmolar nonionic contrast
Microcolon
Unused colon of small caliber, <1 cm
ILEUM
Triple bubble sign of jejunal atresia.

Upper GI is not necessary
Figure 6-2-21
Ileal Atresia [Figure 6-2-21]
Primary vascular accident or secondary to

mechanical obstruction (in utero volvulus)
Plain film - low obstruction
Contrast enema
Microcolon
Abrupt cut off of contrast column at atresia
No filling of dilated ileum
Plain radiograph (left) shows a large number of

dilated tubular loops of bowel. Contrast enema
(right) reveals a microcolon
Pediatric Radiology
1345
1347
Meconium Peritonitis [Figures 6-2-22 and 6-2-23]
Figure 6-2-23
In utero bowel perforation with leakage of meconium

Linear, clumps or at periphery of pseudocyst, or generalized
Scrotal
Perforation may have sealed
Intraluminal severe stasis, anorectal or cloacal
malformation
Figure 6-2-22
Newborn with scrotal swelling due to

calcifications in both scrotal sacs from
meconium peritonitis
Figure 6-2-24
Generalized meconium peritonitis.

Note calcifications around liver and
spleen. Perforation was due to ileal
atresia
Meconium Ileus [Figure 6-2-24]
Inspissated abnormal meconium in the distal ileum

and colon
Almost all have cystic fibrosis - presenting feature of
CF in 5-20%
50% complicated - volvulus, perforation, atresia or
peritonitis
Meconium Ileus Plain Film [Figure 6-2-25]
Meconium ileus. Illustration of pellets of

inspissated meconium causing distal small bowel
obstruction. Intraoperative photograph shows
thick, viscous meconium in dilated small bowel
with microcolon distal to the obstruction
Classic
Distal obstruction
Bubbly appearance in RLQ,
Variation in caliber of loops
Paucity of air-fluid levels
Complicated
Peritoneal calcifications
Bowel wall edema
+/- free air
Figure 6-2-25
Meconium Ileus - Contrast Enema [Figure 6-2-26]
Microcolon (smallest)
Distal 10 - 30 cm of ileum small in caliber but still larger than
colon
Multiple round filling defects
Contrast eventually fills dilated ileum proximal to obstruction
Meconium ileus. Supine radiograph
shows many dilated, unfolded loops
of bowel and classic soap-bubble
lucencies in the right lower quadrant
(arrow)
1346
1348
Pediatric Radiology
Meconium Ileus - Treatment
1968 Noblett nonoperative therapy - Gastrograffin enema

Treatment enema - half-strength Gastrograffin or full urographic
contrast
1-2 enemas per day - several attempts
50-80% success rate
<2% perforation rate
Hydrostatic reduction failure =>complicated meconium ileus =>
surgical intervention
Figure 6-2-26
Berdon Syndrome
Megacystis-microcolon-intestinal hypoperistalsis syndrome

Functional small bowel obstruction
Malrotation common
Transient microcolon and dilated SB loops
Large unobstructed bladder, also dilated ureters and pelvicalyceal
system
4:1 F:M
Associated GU and cardiac anomalies
Poor prognosis for long term survival
COLON/RECTUM
Colonic Atresia
Rare (stenosis even rarer)

Diaphragm, web, fibrous cord, gap
Intrauterine vascular accident
Plain film - disproportionately dilated, bulbous loop of proximal
colon
Contrast enema - microcolon distal to atresia
Meconium ileus. Contrast enema

shows a microcolon and pellet-like
filling defects in the distal small bowel
Figure 6-2-27
Functional Immaturity of the Colon
1956 Clatworthy - meconium plug syndrome

1974 Davis -small left colon syndrome
1975 Lequesne and Reilly small left colon could occur with or
without meconium plug
1977 Berdon - "functional immaturity above are overlapping
entities in a spectrum of functional neonatal intestinal obstruction
Functional Immaturity of the Colon
Abnormal intestinal motility in the left colon

May have a meconium plug effect rather than cause of
obstruction
Risk factors
Infant of diabetic mother
Mother treated with magnesium sulfate
Functional immaturity of the colon.

Contrast enema showing abrupt
transition to small caliber left colon at
the splenic flexure
Figure 6-2-28
Functional Immaturity - Contrast Enema

[Figures 6-2-27 and 6-2-28]
Narrow descending and rectosigmoid colon with abrupt transition

to distended colon at splenic flexure
Passage of plug classic but not common
Passage of lots of meconium - nonspecific
Clinical improvement after enema in hours or days
Meconium plug
Pediatric Radiology
1347
1349
Differentiation from Hirschprung Disease

Functional
Immaturity
Location of
Always at splenic
transition zone
flexure
Quality of transition Abrupt
zone
Caliber of left colon Small
Hirschprung
Disease
Uncommon at
splenic flexure
Gradual
Distensibility of
rectum
Non distensible
Distensible
Normal
Figure 6-2-29
Hirschprung Disease
Functional obstruction of colon due to absence of

intramural ganglion cells of myenteric plexus
Failure of distal intestine to relax
Etiology - ? arrest of craniocaudal migration of
neuroblasts in the distal colon in 12th week
1 in 5000 live births
Hirschsprung Disease - Presentation
80% present in 1st 6 weeks of life

Term newborns
Boys:Girls 3-4:1 for short segment
Equal sex distribution for total colonic
1/3 develop NEC-like enterocolitis in first month of
life
May present with neonatal appendicitis
Hirschprung - Location
Hirschprung disease. Gross specimen in center

shows transition zone with narrow distal sigmoid
and rectum. Histology specimen from aganglionic
segment (left) shows hypertrophied neural
bundles. Histology specimen from proximal colon
shows normal ganglion cells
Ultrashort segment we dont see

Short segment rectosigmoid (73%)
Intermediate - long segment
Descending colon (14%)
Proximal colon (10%)
Total colonic familial (1-3%)
Figure 6-2-30
Hirschprung - Pathology [Figure 6-2-29]
Superficial suction biopsy

Absent ganglion cells
Hypertrophied submucosal nerve bundles
Hirschsprung Plain Films [Figure 6-2-30]
Distal obstruction
Paucity of rectal gas
Prone cross table lateral may show transition zone
Pneumatosis of bowel proximal to aganglionic segment possible
5% present with pneumoperitoneum usually total colonic
Hirschsprung Contrast Enema [Figure 6-2-31]
Hirschprung disease. Plain

No balloon catheters in the rectum
radiograph shows distal bowel
Lateral view best
obstruction and paucity of gas in
rectum
Only enough contrast to diagnose
Low rectosigmoid index (normally > 1)
Irregular contractions of aganglionic segment
Early evacuation film may help
24 hour delayed film retention and lack of movement to the left, but not specific
Initial enema may be normal in neonates
1348
1350
Pediatric Radiology
Total Colonic Aganglionosis [Figure 6-2-32]
Figure 6-2-31
Microcolon
Normal caliber colon
Dilated colon
Small bowel transition zone
Family history
3%-5% Down syndrome

Esophageal dysmotility syndromes
Malrotation
Ileal and colonic atresia
Neurocristopathies
Neuroblastoma
Ondine curse central hypoventilation and congenital
neuroblastoma
Imperforate Anus
Hirschprung disease. Lateral image

from contrast enema showing
abnormally low recto sigmoid ratio
and saw tooth contractions in
aganglionic segment
Anorectal malformation abnormal separation of GU tract from hindgut

High vs. low - levator sling development
determines surgical approach and
prognosis
Low fistula to perineum
High fistula to
Boys post urethra, calcified
meconium, air in bladder
Girls vagina or vestibule
Figure 6-2-32
Imperforate Anus [Figure 6-2-33]
VACTERL
GU
L-S spine
Dysraphism
Tethered cord
Currarino triad imperforate anus,
sacral defect, presacral mass
Post-op incontinence => MRI
Twin boys with total aganglionosis, twin (A) manifesting a

microcolon; twin (B) with normal colon caliber & small bowel
transition zone (arrow)
Figure 6-2-33
Neonatal Low Intestinal Obstruction Ddx
Ileal atresia
Meconium ileus
Colonic atresia
Functional immaturity of the colon
Hirschprung disease
Imperforate anus
Neonatal Bowel Obstruction Always remember, and

please dont ever forget . . .
Start with plain film

All complete high obstructions are surgical. Usually no further
imaging required.
UGI for incomplete high obstruction or normal film
Contrast enema for low obstruction
Differentiates surgical from medical causes
Therapeutic in medical cases
Sacral defect associated with history

of imperforate anus
Pediatric Radiology
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References
1.
2.
3.
4.
5.
6.
Buonomo C. Neonatal Gastrointestinal Emergencies. Radiologic Clinics of North America 1997;35: 845-864
Cohen MD. Choosing Contrast Media for the Evaluation of the Gastrointestinal Tract of Neonates and Infants.
Radiology 1987;162:447-56
Kao SC, et al. Nonoperative treatment of simple meconium ileus: a survey of the Society for Pediatric Radiology.
Pediatr Radiol 1995;25: 97-100
Kirks DR, et al. Practical Pediatric Imaging. 1998
Kirks DR. Emergency Pediatric Radiology. American Roentgen Ray Society. 95th Annual Meeting April, 1995
Long FR, Kramer SS, Markowitz RI, Taylor GE. Radiographic patterns of intestinal malrotation in children.
RadioGraphics 1996;16:547-556.
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Pediatric Radiology
Acute GI Disorders of Infants and Children

Necrotizing Enterocolitis Pathogenesis
Hypoxemia ischemic bowel bacterial invasion

Feeding plays a role hyperosmolar, early
Risk factors
Prematurity
Term with CHD
Hirschprung disease
UAC
Figure 6-3-1
NEC Clinical Features [Figure 6-3-1]
Onset 3-6 DOL

Abdominal distension
Vomiting
Metabolic acidosis
Temperature lability
Hypotension
Apnea/bradycardia
Necrotizing Enterocolitis Pathology [Figure 6-3-2]
Ileum and colon most common

Coagulative and hemorrhagic necrosis
Dilated, friable bowel
Submucosal and subserosal gas bubbles
Complications perforation, sepsis, stricture
Distended and discolored abdomen

in a neonate with severe NEC
NEC Radiographic Findings [Figure 6-3-3]
Normal intestinal gas pattern of a neonate uniform polygonal lucencies

throughout the abdomen
NEC nonspecific plain film findings
Distended loops
Sentinel loop sign
Wall edema
Ascites
Figure 6-3-2
Figure 6-3-3
Gross specimen of ileum showing

submucosal and subserosal
pneumatosis in NEC
Normal neonatal bowel gas pattern

Pediatric Radiology
1353
NEC Radiographic Findings
Figure 6-3-4
[Figures 6-3-4 to 6-3-6]
NEC Specific plain film findings

Pneumatosis intestinalis
Subserosal curvilinear
Submucosal bubbly, looks like stool
Portal venous gas
Indication for surgical intervention
Free air
Figure 6-3-5
NEC with diffuse pneumatosis and
pneumoperitoneum below the liver tip on left
lateral decubitus view (right image).
Figure 6-3-6
Branching portal venous air
NEC Other Imaging [Figure 6-3-7]
US
Thickened bowel wall
Mural gas
Portal venous gas - mobile
Contrast enema
Contraindicated acutely
Late strictures - 20%
Free air around the falciform ligament

(arrow). The American football sign
STOMACH
Hypertrophic Pyloric Stenosis
Acquired hypertrophy of antropyloric circular muscle

Etiology unknown
Common 1 in 500 live births in the US
Males more often affected 5:1
First born
Family history 5%
Figure 6-3-7
HPS Clinical Features
Present at 2-9 weeks of age

Rare after 3 mo
Uncommon in preemies
Progressive nonbilious projectile vomiting
Dehydration
Failure to thrive
HPS Pathology [Figures 6-3-8 and 6-3-9]
Neurons supplying the circular muscle layer lack nitric oxide

synthetase activity
Circular muscle layer undergoes hypertrophy and elongation
Colonic stricture due to prior NEC
HPS Imaging [Figures 6-3-10 and 6-3-11]
Plain film
+/- dilated stomach with little distal gas
Gastric hyperperistalsis
Ultrasound diagnostic
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Pediatric Radiology
Long pyloric channel (>17 mm) with thick muscular wall (> 3 mm)
Measure sonolucent part of one wall
May have fluid/debris filled stomach
No passage of fluid through pylorus
Figure 6-3-8
Figure 6-3-9
Normal pylorus on left and hypertrophic pyloric

stenosis on right
Figure 6-3-11
Gross specimen of hypertrophic
pyloric stenosis
Figure 6-3-10
AP radiograph shows markedly

dilated stomach with deep
indentations due to hyperperistalsis
and little distal bowel gas
HPS Imaging
Upper image shows hypertrophic

pyloric stenosis with thickened
sonolucent muscle and marked
elongation compared to normal (lower
image)
Figure 6-3-12
Ultrasound
Borderline measurements follow-up in 1 or 2 days
Pyloric US is only good for HPS - if US negative, look for
other causes of vomiting
SMA/SMV inversion
Hydronephrosis
Pitfalls: overdistended stomach, coapted antrum
HPS Imaging [Figure 6-3-12]
UGI
No longer used unless post-op with persistent symptoms
Multiple signs string, double string, beak, tit, shoulder and
mushroom
Ddx: pylorospasm, antral gastritis
UGI in HPS showing

string sign (A), beak sign
(B), shoulder sign (C),
and tit sign (D)
Pediatric Radiology
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HPS Treatment
Figure 6-3-13
Hydration and electrolyte replacement

Pyloromyotomy U.S.
Nonoperative treatment U.K. and Scandinavia
Gastrostomy Tubes [Figure 6-3-13]
Usually in neurologically impaired child

Use water-soluble contrast, not too hyperosmolar
Trouble shooting
Tube not in track or not in stomach
Obstruction due to balloon migration into gastric
outlet
GJ tubes in babies may cause
intussusception/curtain-rodding
Bezoar [Figure 6-3-14]
How to do a G-tube check. Frontal view suggests

the tube is properly positioned, but lateral view
tangential to the tube track, shows the tube is in
the track and not in the stomach
Lactobezoar too concentrated formula

Trichobezoar young and emotionally disturbed children
Can embolize
Can be treated endoscopically unless embolic
Figure 6-3-14
SMALL BOWEL
Duodenal Hematoma
Etiology blunt abdominal trauma

Handlebar
Seat belt
Inflicted trauma
Associated injury to other organs, especially
pancreas
Usually present with vomiting, pain, less commonly
mass, jaundice
Embolic trichobezoar mimicking malrotation as

emboli connected to gastric bezoar by hair that
straightened the bowel
Duodenal Hematoma Imaging
US - mixed echogenicity mass which becomes more hypoechoic as it liquefies

UGI
Intramural mass causes curved impression on duodenum
Widened, separated folds stack of coins
NAT-Visceral Injury
Seen at all ages

Usually blunt
Delay in seeking treatment
20%-50% mortality
Proximal SB hematoma, distal SB perforation
Differential of Intramural Hemorrhage
Blunt abdominal trauma

Coagulation disorder
Hemophilia
Blood dyscrasia with pancytopenia
Henoch-Schonlein purpura
Ischemia
Henoch-Schnlein Purpura
Idiopathic anaphylactoid reaction with diffuse vasculitis

In the small bowel it causes intramural hemorrhage
Jejunum most frequently involved
Enteroenteric intussusception common
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Pediatric Radiology
HSP Clinical Features
Figure 6-3-15
Characteristic purpuric rash of LEs and buttocks

Abdominal pain may precede rash by days
Arthritis
Nephritis
Vasculitis
Acute scrotum
HSP - Pathology [Figure 6-3-16]
Gross focal mucosal hemorrhage, edema, and

Ultrasound and CT of HSP showing markedly
thickened wall and echogenic/enhancing
erosions
thickened
mucosa of the dilated small bowel
Histo - acute leukoclastic vasculitis of small vessels
in the submucosa or deep lamina propria
Ddx: SLE, enterohemorrhagic strains of E. Coli (0157:H7 which causes
hemolytic uremic syndrome)
HSP Imaging Findings [Figures 6-3-15 and 6-3-16]
Thickening of SB wall
Focal areas of dilatation alternating with stenosis
Separation of bowel loops
Submucosal masses
Enteroenteric intussusception
Figure 6-3-16
Inguinal Hernia
Most common cause of intestinal obstruction in

young infants
Usually a clinical diagnosis
Incarceration or strangulation can cause bowel
obstruction
Most male - 90%
Bowel, fat, fluid, ovaries can herniate
Gross images showing the dilated markedly

thickened small bowel and classic purpuric rash
Inguinal Hernia - Imaging [Figure 6-3-17]
Plain film look for air in scrotum or thickened inguinal fold

Ultrasound
Bowel or fat in the inguinal canal or scrotum
Color Doppler to evaluate for flow to incarcerated bowel
Figure 6-3-17
Intussusception - Clinical [Figure 6-3-18]
90% ileocolic or ileoileocolic

90% due to lymphoid hyperplasia seasonal (winter and spring)
6 mo to 3.5 years (peak 5-9 mo)
Outside that age range consider lead points
Colicky pain, vomiting, bloody stools, lethargy, palpable mass
RLQ
10% recurrence rate
Figure 6-3-18
Young infant with distal bowel

obstruction with air over the right
inguinal canal
Ileocolic (left) and ileoileocolic (right)

intussusception
Pediatric Radiology
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Intussusception - Pathology
Figure 6-3-19
Invagination of one segment of bowel into another

Edema, congestion, coagulative and hemorrhagic necrosis
Prominent Peyer patches
Intussusception - Pathology
8-10% pathologic lead points

Meckel diverticulum if younger
Lymphoma if older
Polyp
Appendix
Henoch-Schonlein purpura
Intussusception - Imaging [Figures 6-3-19 to 6-3-21]
Plain film
Left lateral decubitus films
No air in cecum or filling defect
Air crescent sign
SBO possible especially in infant
US graded compression
Pseudokidney/donut/target sign
Doppler flow to wall viable
Ascites nonspecific
Intussusception. Crescent of air

surrounds intussusceptum in the
transverse colon
Figure 6-3-20
Intussusception Reduction
Contraindications
Free air/peritoneal signs
Septic shock
Hx >24 hours
Preparation
Surgical consult capable surgeon present
IV antibiotics
Someone to monitor patient
16-G Angiocath to treat tension
pneumoperitoneum
US in 10 mo with intussusception showing donut

(left) and pseudokidney signs. Echogenic material
within the intussuscepiens is mesenteric fat
Intussusception Reduction
Figure 6-3-21
Air up to 120 mm Hg1 or

Water soluble contrast
3 attempts, 3 minutes each
Largest tip possible
Squeeze buttocks when at IC valve
If losing air, apply forward pressure to tip
Endpoint is reduction of soft tissue mass AND rush
of air into the SB
1Shiels WE, Maves CK, Hedlund G, Kirks DR. Air enema
for diagnosis and reduction of intussusception: clinical

experience and pressure correlates. Radiology
1991;181:169-172
CT of colonic intussusception due to polyp. Note

fat and vessels inside the intussuscepiens
Intussusception Reduction [Figure 6-3-22]
Air slightly more effective than water soluble contrast

Perforations with air are smaller1
Perforations usually occur near IC valve and are associated with necrotic
bowel
Perforation is probably the uncovering of a perforation that was already there
If concerned for recurrence, go straight to enema
1Shiels WE, Kirks DR, Keller GL, et al. Colonic perforation by air and liquid
enemas: comparison study in young pigs. AJR 1993;160:931-935.
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Pediatric Radiology
Meckel Diverticulum
Omphalomesenteric/vitelline duct remnant

Most common congenital anomaly of the GI tract
2% of population
45% present under age 2y
Within 2 ft of IC valve
2% complications intussusception, obstruction,
hemorrhage, diverticulitis, perforation
Figure 6-3-22
Meckel Diverticulum - Presentation
Most commonly become symptomatic in first years of

life
Bleeding due to ulceration caused by secretion of
acid by ectopic gastric mucosa
Obstruction due to inverted Meckel diverticulum
serving as a lead point for intussusception
Vitelline band can serve as a fulcrum for volvulus
Meckel Diverticulum - Pathology [Figure 6-3-23]
1-5 cm long
Therapeutic air contrast enema in older child with
Antimesenteric border
intussusception due to lymphoma. Watch as
Most discovered incidentally are lined by small bowel
mass moves from hepatic flexure to cecum. The
epithelium
procedure is not successful until the mass is
Those that present with symptoms are more likely to completely reduced and air rushes into the ileum
contain ectopic gastric mucosa - 15-25%
as shown in the lower right image of another
patient
Can be giant
Meckel Diverticulum - Imaging [Figures 6-3-24 and 6-3-25]
Plain film
May be visible if giant mottled air collection
May be filled with air or entherolith
Ultrasound
Gut signature resembles normal bowel
When inflamed, mimics appendicitis
Tc99mPertechnetate scan positive if contains gastric mucosa
Figure 6-3-23
Figure 6-3-24
Meckel diverticulum
Figure 6-3-25
Lethargic 4 mo with small bowel obstruction.

Ultrasound shows fluid-filled lead point due to
Meckel diverticulum
Meckel diverticulum on small bowel

follow-through
Pediatric Radiology
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Figure 6-3-26
Figure 6-3-27
Longitudinal (left) and transverse images showing

intussusception with large fluid-filled lead point, a
duplication cyst
Intraoperative image showing ileocolic

intussusception and sectioned resected specimen
showing duplication cyst lead point
Enteric Duplication Cyst
Developmental tubular or cystic structures adjacent to the GI tract

Share wall and blood supply with adjacent bowel
Usually round and do not communicate with bowel lumen
Occasionally tubular, communicating with GI tract at one end and blind-ending at the other
Duplication Clinical Features
Thoracic respiratory symptoms, incidental finding

Abdominal obstruction, mass, pain, GI bleeding, incidental on prenatal ultrasound
Duplication Cyst - Location
Ileum - 40%
Thorax (posterior mediastinum) - 20
Jejunum - 10
Stomach - 10
Colon - 10
Multiple - 5
Duplication Cyst - Pathology [Figure 6-3-27]
Often share muscularis layer with adjacent bowel (intramural)

Mesenteric border
Filled with mucoid material
Histologically, recapitulate normal GI tract
May contain ectopic gastric mucosa (20%) or pancreatic tissue
Duplication Cyst - Imaging [Figures 6-3-26 and 6-3-28]
Plain film as soft tissue mass if large

Ultrasound
Preferred study
Rim sign gut signature in the wall
Peristalsis
May contain debris
Figure 6-3-28
Crohn Disease
present in childhood
Abdominal pain, diarrhea, hematochezia, weight loss
CT for abscess, UGI/SBFT for diagnosis
Ddx: TB, yersinia, pseudomembranous colitis,
lymphoma
Appendicitis
Children more often have atypical presentation

Children have a higher rate of negative laparotomy
and of perforation than adults
Rare in infants
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Duplication cyst (longitudinal and transverse)

showing round fluid-filled cavity surrounded by a
wall with gut signature. Note echogenic rim of
mucosa
Pediatric Radiology
Appendicitis
Appendicolith 10-15%
US blind-ending, uncompressible, > 6mm
CT ? IV, oral, rectal contrast young children have no fat
Pitfalls
Perforated appendix may be decompressed
Tip appendicitis
COLON/RECTUM
Neutropenic Enterocolitis
AKA typhlitis
Necrotizing enterocolitis often affecting the right colon in patients with neutropenia
Pathologically similar to NEC in premature infants and pseudomembranous colitis
Transmural risk of perforation
Neutropenic Enterocolitis - Clinical
Usually leukemic children on chemotherapy, but also those with aplastic

anemia or immunosuppression for organ transplantation, and
AIDS
Fever, nausea, vomiting, diarrhea, abdominal tenderness
Mortality has decreased from 80% to less than 20% due to early
recognition and treatment
Figure 6-3-29
Neutropenic Enterocolitis - Imaging
All imaging findings are nonspecific clinical setting most helpful

Plain films
No gas in RLQ
Dilated ascending colon
Pneumatosis
Ultrasound
Thickened bowel wall and thickened mucosa
Dilated, fluid-filled loops
No ascites
Neutropenic Enterocolitis - Imaging [Figure 6-3-29]
Contrast enema contraindicated

CT
Transmural wall thickening
Infiltration of the surrounding fat
Can exclude other causes of RLQ pain
Ddx: pseudomembranous colitis, leukemic infiltration, intramural
hemorrhage, ischemic colitis
Neutropenic enterocolitis. KUB shows

lack of bowel gas on the right and
markedly thickened haustra in the
right transverse colon. Note also the
dual lumen catheter overlying the
abdomen, a clue to the underlying
diagnosis of leukemia
References
Texts
1. Donnelly LF. Fundementals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001.
2. Kuhn JP, Slovis TL, Haller JO, eds. Caffeys Pediatric Diagnostic Imaging. Philadelphia: Mosby, 2004
3. Stringer DA, Babyn PS, eds. Pediatric Gastrointestinal Imaging and Intervention. Hamilton: B.C. Decker Inc.,
2000
4. Stocker JT, Dehner LP, eds. Pediatric Pathology. Philadelphia: Lippincott Williams & Wilkins, 2002
5. Swischuk LE. Imaging of the Newborn, Infant, and Young Child. Philadelphia: Lippincott, 2004
Journal Articles
1. Berrocal T, Lamas M, Gutieerez J, et al. Congenital anomalies of the small intestine, colon and rectum.
RadioGraphics 1999:19:1219-1236
2. Blumhagen JD, Maclin L, Krauter D, et al. Sonographic diagnosis of hypertrophic pyloric stenosis. AJR
1988;150:1367-1370
3. Buonomo C. Neonatal Gastrointestinal Emergencies. Radiology Clinics of North America 1997; 35: 845-864
Pediatric Radiology
1359
1361
4.
5.
6.
7.
8.
9.
Kleinman PK, Brill PW, Winchester P. Resolving duodenal-jejunal hematoma in abused children. Radiology
1986;160:747-750.
OKeeffe FN, Stansberry SD, Swischuk LE, Hayden CK, Jr. Antropyloric muscle thickness at US in infants: what
is normal? Radiology 1991;178:827-830
Merritt CR, Goldsmith JP, Sharp MJ. Sonographic detection of portal venous gas in infants with necrotizing
enterocolitis. AJR 1984;143:1059-1062.
Segal SR, Sherman NH, Rosenberg HK, et al. Ultrasonographic features of gastrointestinal duplications. J
ultrasound Med 1994; 13:863-870.
Sivit CJ, Taylor GA, Newman KD, et al. Safety-belt injuries in children with lap-belt ecchymosis: CT findings in
61 patients. AJR 1991;157:111-114.
Sloas MM, Flynn PM, Kaste SC, Patrick CC. Typhlitis in children with cancer: a 30 year experience. Clin Infect
Dis 1993;17:484-90
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Pediatric Radiology
Diseases Affecting the Pediatric Airway

Figure 6-4-2
Figure 6-4-1
Normal AP views of the airway. The left image

shows the vocal cords open and the right image
shows the vocal cords coapted (arrows). Note
also the false cords (block arrows). Between the
true and false cords is the laryngeal ventricle. The
true cords create shoulders on the subglottic
trachea resembling those of a wine bottle
Normal sagittal MR of an adult and lateral soft

tissue neck radiograph of a child showing normal
upper airway structures.
1.Adenoids (none in adult)
2. Vallecula
3. Epiglottis
4. Aryepiglottic folds
5. Subglottic trachea
6. Retropharyngeal soft tissues
Figure 6-4-3
ACUTE UPPER AIRWAY OBSTRUCTION

Viral Croup
Laryngotracheobronchitis
Characteristic barking, brassy cough w/
inspiratory stridor
Most frequent cause of
stridor 6 mo - 3 yo
Self-limited disease-7
days
Parainfluenza
Subglottic edema 5-10
mm below cords
Less than 1% need
intubation
Figure 6-4-4
Viral croup. Note the

hypopharyngeal overdistension and
subglottic tracheal narrowing and
indistinctness (block arrow). The
most important finding on this film is
the normal pinkie-like epiglottis
(arrow)
Viral croup, AP view, showing

subglottic narrowing and loss of the
normal shoulders of the subglottic
trachea
Pediatric Radiology
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Diseases Affecting The Pediatric Airway
Figure 6-4-5
Epiglottitis [Figure 6-4-5]
Life-threatening supraglottic inflammatory disease

Clinically appear toxic
Typically 3-6 yo
Haemophilus influenzae, Group A strep
Do not make uncomfortable
Edema of epiglottis and aryepiglottic folds
Epiglottitis DDX
Caustic ingestion or thermal injury

Angioneurotic edema
Radiation
Sarcoidosis
Hemorrhage
Abscess
Epithelial cyst
Omega epiglottis
Retropharyngeal Cellulitis [Figures 6-4-6
Epiglottitis with epiglottis shaped like

a thumb, thickened aryepiglottic
folds, and loss of the normal
concavity of the aryepiglottic folds
(arrow)
to 6-4-8]
Most common in children between 6 to 12 months

Fever, stiff or wry neck, dysphagia
Straightening or reversal of normal cervical lordosis
Thickening of the prevertebral soft tissues
May cause airway compression, vasospasm or venous thrombosis
(Lemierre syndrome)
May extend into mediastinum or rupture into airway
Figure 6-4-7
Figure 6-4-6
AP and lateral films demonstrate a normal

epiglottis and aeryepiglottic folds with thickened
prevertebral soft tissues and reversal of the normal
lordosis of the cervical spine
Figure 6-4-8
Adapted from Rivera and Young,

unpublished material, 1992. A (solid
line) represents the thickness of the
prevertebral soft tissues anterior to
the intervertebral disc space C2-3. B
(dashed line) represents the AP
diameter of the base of C2
Retropharyngeal cellulitis. Contrast enhanced

axial CT shows marked prevertebral soft tissue
swelling and ill defined hypodensity without ring
enhancement
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Pediatric Radiology
Figure 6-4-9
Retropharyngeal Cellulitis - DDX
Adenopathy
Leukemia/lymphoma
Kawasaki
Langerhans cell histocytosis
Metastatic disease
Edema
SVC syndrome
Angioneurotic edema
Parapharyngeal Abscess [Figure 6-4-9]
Plain film cannot distinguish between cellulitis and abscess

CT skull base to aortic arch
Low density center surrounded by an enhancing rim characteristic
of an abscess
Drainable pus vs. focal cellulitis delayed imaging helps
Axial CT of the neck demonstrates a
Ddx: necrotic nodes
low density ring enhancing lesion in
the left parapharyngeal space
Membranous Tracheitis
consistent with an abscess
AKA Membranous croup, bacterial tracheitis
Uncommon, life-threatening
Exudative plaques form along tracheal walls like those seen in diphtheria
Irregular tracheal wall, asymmetric subglottic narrowing, loss of definition of
the wall
ACUTE LOWER AIRWAY OBSTRUCTION

Reactive Airways Disease
Very common with increasing incidence

Obstruction due to bronchospasm
May have other associated allergic disorders
Precipitating factors
Infections, especially RSV
Weather changes
GER
Aspirin or NSAIDs
Reactive Airways Disease
Chest radiograph normal to hyperinflated with mild peribronchial cuffing and

parahilar increased markings
Complications
Atelectasis
Air trapping
Pneumonia
Air block phenomena
Aspirated Foreign Body Clinical
Choking
Loss of phonation
Cough
Wheezing
Asymmetric or decreased breath sounds
Hemoptysis
Recurrent or persistent pneumonia
Pediatric Radiology
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Aspirated Foreign Body Radiology [Figure 6-4-10]
Figure 6-4-10
Maintain a high index of suspicion in crawling infants

and toddlers
If unwitnessed, may present with chronic symptoms
suggesting pneumonia
The vast majority are not radiopaque
Most commonly lodge in the bronchus
If initial radiographs are normal (up to 1/3 of
patients), do additional views to look for air trapping
lack of change of volume of the affected lung
18 month old with normal PA and lateral chest
Atelectasis and air trapping
radiographs but high clinical suspicion of aspirated
Suspect in children <3 yo with pneumonia that fails
foreign body. Right lung fails to collapse with
to clear after 2 weeks or with air block phenomena
dependent positioning due to tortilla chip in the
right mainstem bronchus
Aspirated Foreign Body
Treatment bronchoscopic removal

Complications
Pneumothorax
Pneumomediastinum
Chronic/recurrent pneumonia
Bronchiectasis
Figure 6-4-11
Hydrocarbon aspiration
Hydrocarbons furniture polish, gasoline, kerosene, lighter fluid

Aspirated due to low viscosity and surface tension
Severe chemical pneumonitis with destruction of surfactant
Radiographic abnormalities develop within 24h
Pneumatoceles may develop
Upper esophageal foreign body
Can be a cause of stridor

Accompanied by dysphagia, but this symptom may go unnoticed
Coins in esophagus are seen en face on PA chest, whereas coins
Axial CT in bone window showing
in trachea are seen in tangent on PA chest due to posterior gap in right bony choanal atresia (arrow).
the cartilage rings of the trachea
Note the thickening of the posterior
UGI helpful for radiolucent esophageal foreign bodies. Start with
midline vomer and medial deviation
of the lateral wall of the nasal cavity
water soluble contrast.
CHRONIC CONDITIONS AFFECTING THE AIRWAY

Nasal Cavity: Paranasal Sinuses
Figure 6-4-12
Choanal Atresia [Figure 6-4-11]
Bony or membranous septum at posterior nasal septum

If bilateral, will present early as neonates are obligate nasal
breathers
CT with very thin cuts following nasal suction +/- topical
decongestants
90% are bony
Thickening of the vomer and medial bowing of the lateral wall
of the nasal cavity
Air-fluid level
Axial CT showing piriform aperture
stenosis (arrows). Note the hour-glass
configuration of the nasal cavity
CHARGE Association
Coloboma
Heart defect
Atresia of the choana
Retardation of growth and developmental delay
Genital hypoplasia
Ear deformities and deafness
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Pediatric Radiology
Pyriform Aperture stenosis [Figure 6-4-12]
Figure 6-4-13
AKA inlet stenosis

Cyanosis with feeds
Width less than 11 mm considered abnormal
Also well evaluated with CT
Associated with abnormalities of pituitary gland and
dentition and craniofacial syndromes
Congenital Midline Nasal Mass
Frontoethmoidal cephalocele
Nasal glial heterotopia
Nasal dermoid
Nasal dermoid (arrow).Note midline bony efect,

seen best on CT
Congenital Midline Nasal Mass
CT shows bony defect 1- 1.5 mm w/ sagittal reformats

MR shows intracranial extension and associated brain abnormalities
Cephalocele dysgenesis of CC,interhem cyst/lipoma, cortical
dysplasia, craniofacial dysraphism
Dermoid intracranial dermoid/epidermoid
Nasal glial heterotopia no intracranial extension
Figure 6-4-14
Nasal Dermoid
Midline, usually round and cystic

May have hypertelorism or nasal pit
May occur anywhere along dermal sinus tract
Tract may communicate with the cranial contents
Dermoid bony defect [Figure 6-4-13]

Cephalocele [Figure 6-4-14]
Meninges or meninges and brain herniate into the nasal cavity

through a defect in the cribiform plate or an open suture
Anterior cephaloceles sincipital (roof of nose) or basal (skull
base)
Sincipital nasofrontal, nasoethmoidal, or naso-orbital
Nasal obstruction, rhinorrhea, epistaxis
Sagittal T2-weighted MRI showing

sphenoethmoidal encephalocele
with herniation of meninges and
optic chiasm
Nasal Glial Heterotopia
AKA nasoglioma
Related to cephalocele
No communication with intracranial contents
Juvenile Angiofibroma
Highly vascular, locally invasive, histologically benign

Adolescent boys w/ nasal obstruction, sinusitis and epistaxis
Originates in sphenopalatine foramen, nasopharynx or posterior nasal cavity
Spreads early into pterygopalatine fossa,
infratemporal fossa, middle cranial fossa, orbit or
sphenoid sinus
Figure 6-4-15
Juvenile Angiofibroma [Figure 6-4-15]
CT - sharply marginated mass with homogeneous

enhancement; anterior bowing of the posterior wall of
the maxillary sinus
MR - iso- to hypointense to muscle on T1 and iso- to
hyperintense on T2
Juvenile angiofibroma. CT on left shows anterior
MR differentiates tumor from secretions in obstructed bowing of posterior wall of maxillary sinus due to
sinus
mass in pterygopalatine fissure. T1-weighted
Prominent tumor vessels may be seen as flow voids contrast enhanced MR shows enhancing mass in
left nasal cavity and ethmoid air cells with
internal maxillary artery
nonenhancing trapped secretions in the maxillary
sinus
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Sinus Mass Differential
Figure 6-4-16
Fungal sinus infection

Mucocele
Rhabdomyosarcoma
Esthesioneuroblastoma
Lymphoma
Melanoma
Esthesioneuroblastoma [Figure 6-4-16]
AKA olfactory neuroblastoma

Very rare tumor originating from neural crest cells in
the olfactory groove
Olfactory neuroblastoma
Extensive destruction
Extends intracranially
Rhabdomyosarcoma
3rd most common childhood primary malignancy of

the head and neck
Esthesioneuroblastoma on CT and coronal Gd40% arise in the head and neck
enhanced T1W MRI showing mass in nasal cavity
Orbit and nasopharynx most common
with invasion of the right frontal lobe with
Also paranasal sinuses and middle ear
surrounding edema
Usually embryonal cell subtype
May spread intracranially via skull base foramina
CT-iso to brain and enhances uniformly
MR- T1 iso to muscle, T2 hyperintense and heterogeneous
MR shows intracranial extension through fissures and foramina well
CT shows bony erosion well
Enhancement may make extracranial portions of the tumor less conspicuous
on MR
Fat saturate post-gadolinium sequences of orbits and face
Nasal polyps
Uncommon in children except those with cystic fibrosis

Can also be associated with recurrent infection and allergies
In CF can be so large and chronic as to widen the nasal passages
Antrochoanal Polyp [Figure 6-4-17]
Polyp originating in the maxillary sinus

Protrudes through an opening into the nasopharynx
Unilateral nasal congestion is the most common symptom
May present as large mass in oropharynx
Ipsilateral maxillary sinus is opacified
Incomplete resection associated with high recurrence rate
Palatine Tonsil and Adenoid Enlargement
Adenoids are lymphoid tissue in the posterior nasopharynx

Absent at birth
Absence after 6 months suggests immune deficiency
May cause snoring, hypoxemia, respiratory acidosis,
pulmonary hypertension
May obstruct Eustachian tubes
Adenoidal pillow normally smooth
Physiologic enlargement maximum age 3-5 years
Significant enlargement obliterates nasopharyngeal
airway
Mononucleosis (EBV,CMV) rare <3, mean 14 yo
Figure 6-4-17
Antrochoanal polyp extending from right maxillary

sinus into nasopharynx
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Oral Cavity Hypopharynx
Figure 6-4-18
Macroglossia
Downs syndrome
Hypothyroidism
Beckwith-Wiedemann syndrome
Storage diseases
Mandibular Hypoplasia
Hemifacial microsomia unilateral

Ipsilateral TMJ hypoplasia
Congenital ear defects
First arch syndromes
Pierre Robin sequence
Goldenhar syndrome
Weyer mandibulofacial dysostosis
Treacher Collins mandibulofacial dysostosis
Trisomies
Airway obstruction by normal sized tongue
Inflammatory disease of the salivary glands
Left image is an IV contrast-enhanced CT

showing left parotitis. Compared to the normal
right parotid gland, the left is enlarged, enhancing
and has small hypodense foci consistent with
intragland abscesses. The image on the right
shows a large calcification in the duct of the right
submandibular gland
[Figure 6-4-18]
Acute infection is viral or bacterial

Bacterial sialadenitis is most common in parotid gland
Bacterial infection usually due to decrease in flow of
secretions
Predisposing conditions include sialolithiasis
Can occur in dehydrated newborns
On CT the affected gland is enlarged and
hyperdense
Intragland abscess may be seen
Most calcified duct stones are visible on noncontrast
CT
US can be used to evaluate salivary glands
Obstruction of a sublingual duct or accessory duct is
a ranula
Figure 6-4-19
Inflammatory disease of the salivary glands
Chronic disease
Recurrent bacterial infection
Autoimmune disease Sjogren syndrome
Lymphoepithelial cysts in HIV disease
Hypopharyngeal cyst
Epiglottic or aryepiglottic folds

Retention cysts or lymphatic malformations
Inspiratory stridor or choking during feeding
Present in infancy or early childhood
Treated with marsupialization
Vallecular cyst (arrow). Bright on T2WI (top) and

nonenhancing on post-Gd T1WI (bottom)
Vallecular Cyst [Figure 6-4-19]
Caused by obstruction of submucosal mucous and serous glands between the

epiglottis and base of tongue
If large can cause SOB, hoarseness, dysphagia, failure to thrive, acute airway
obstruction
Tongue Base Mass
Hemangioma
Enlarged lingual tonsils
Thyroglossal duct cyst
Lingual thyroid
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Lingual Thyroid [Figure 6-4-20]
Document orthotopic thyroid whenever evaluating a

near midline tongue-base or neck mass
Ectopic thyroid most commonly near foramen cecum
Figure 6-4-20
Subglottic Trachea Neck

Childhood Neck Neoplasms
Neuroblastoma
Neurofibroma/schwannoma
Lymphoma
Hemangioma
Lymphatic malformation/lymphangioma
Teratoma
Subglottic Hemangioma
Rare cause of stridor in infants, but most common

subglottic soft tissue mass causing upper airway
obstruction
Noisy breathing at 6-12 months
50% have cutaneous hemangiomas
Asymmetric narrowing of subglottic trachea
Ddx: subglottic cyst, papilloma, cervical ectopic
thymic tissue, tracheal granuloma
Hemangioma
Benign vascular tumor of infancy high flow lesion

Most common tumor of infancy
Usually absent at birth and appears in first few
months
Early proliferative phase, later involutional phase
May involve skin and have characteristic appearance
Lingual thyroid. Left CT image shows

homogeneous intensely enhancing mass at base
of tongue. Lower right CT image shows no thyroid
gland in the base of the neck. Upper right image
is a pertechnetate scan showing no uptake above
the sternal notch and most uptake at the base of
tongue
Figure 6-4-21
Hemangioma [Figure 6-4-21]
May occur in the liver and cause high output heart

failure
Very well marginated, intensely enhancing with large
feeding vessels
If it does not look like a hemangioma, it is not a
hemangioma
Only require treatment if affect airway or vision or
cause intractable heart failure
Hemangioma on contrast enhanced T1-weighted

MR image in the posterior neck. The mass very
well defined and homogeneous except for the
multiple vascular flow voids. Compare to well
marginated, lobulated surface of the resected
tumor
Hemangioma
PHACE syndrome
Posterior fossa abnormalities
Hemangioma of the face
Arterial abnormalities
Coarc/cardiac defects
Eye abnormalities
Kassabach-Merritt syndrome
Thrombocytopenia and consumptive
coagulopathy associated with vascular tumor
Figure 6-4-22
Congenital teratoma of anterior neck. MR

and CT show complex appearance
characteristic of a mature teratoma with
cystic and solid components. Note
deviation of the trachea
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Pediatric Radiology
Teratoma [Figure 6-4-22]
Arise from pleuripotential cells

20% malignant
Neck large anterior mass in neonate, respiratory distress and dysphagia
Heterogeneous cyst, calcification, fat
Lymphoma
Common malignant tumor in the neck

Both Hodgkin's and NHL
NHL more frequently involves extranodal sites, including tonsils and adenoids
Immunodeficiency predisposes
Enlarged lymph nodes and conglomerate masses of nodes
On ultrasound the nodes may be sonolucent
Lymphoma
On CT, isointense to muscle

On MR, isointense to muscle on T1WI and hyperintense on T2WI
Burkitt lymphoma mass originating in jaw
Neuroblastoma
Primary or metastatic in neck

Neck or thoracic primary has better prognosis than intra-abdominal primary
US may show increased blood flow
May involve skull or extend intracranially
Fibromatosis Colli [Figure 6-4-23]
Figure 6-4-23
Focal thickening or mass of sternocleidomastoid muscle

associated with torticollis
Noted at or shortly after birth
Histologically atrophy and partial replacement of muscle with
fibrous tissue
US preferred shows continuity of mass with SCM
Resolves with physiotherapy
Larynx and Trachea - Intrathoracic Airways

Laryngomalacia
Most common laryngeal abnormality of the neonate

Early inspiratory stridor
Worsens at rest - unusual
Laryngeal collapse during inspiration with hypopharyngeal
overdistension seen on airway fluoroscopy
Usually resolves by age 1 year
US of sternocleidomastoid muscle
showing fusiform enlargement
characteristic of fibromatosis colli
Laryngotracheal cleft
AKA persistent esophagotrachea

Extreme form of failure of separation of trachea from foregut
Spectrum from posterior laryngeal cleft to common tube
May have abnormal cry or mutism
Symptoms mimic esophageal atresia
Esophagram shows massive aspiration
Tracheomalacia
Collapse of the trachea with expiration

Delayed development of cartilage
Focal or generalized
Recurrent infections and stridor
Compressed in AP diameter
Associated with esophageal atresia and vascular ring
Also common in Downs syndrome
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Tracheal Stenosis [Figure 6-4-24]
Acquired traumatic intubation

Congenital
Subglottic smooth, circumferential narrowing
looks like croup
Intact cartilage rings
Associated with pulmonary sling, TE fistula,
pulmonary atresia or hypoplasia
Biphasic stridor
Figure 6-4-24
Tracheobronchomegaly
AKA Mounier-Kuhn
Dilation of airways in inspiration
Due to congenital deficiency of elastic tissue
3rd-5th decade
Dilated trachea and central bronchi with diverticulosis
of trachea
Perihilar bullae
Tracheal bronchus [Figure 6-4-25]
RUL bronchus arises directly from trachea

1% of the population
May supply whole RUL or a supernumerary segment
Persistent RUL pneumonia, atelectasis or air trapping
Associated with TEF, tracheal stenosis, pulmonary
sling, Down syndrome
Bronchogram showing long segment tracheal

stenosis in a patient status post surgical
correction of pulmonary sling. Note that the
trachea is of smaller caliber than either mainstem
bronchus
Figure 6-4-25
Bronchial Atresia [Figure 6-4-26]
Lobar or segmental luminal fibrosis of bronchus

Recurrent infections, dyspnea or asx
UL and RML most frequent
Involved lung is fluid then air-filled with air trapping
Tubular soft tissue density trapped mucus just distal
to atresia
Laryngeal- Tracheopapillomatosis
Tracheal bronchus. Postmortem bronchogram

Most common laryngeal tumors in infancy
and specimen showing small bronchus to
Human papilloma virus implicated
supernumerary right upper lobe arising directly
2/3 pts less than 4 yo
from the trachea
Dx made on endoscopy with nodules on vocal cords
Figure 6-4-26
Transbronchial spread < 5%, related to surgical procedures
Pulmonary solid lesions with cavitation
Poor prognosis with pulmonary involvement
Bronchial atresia with abrupt cut off

of bronchus dilated and filled with a
mucous plug (arrow). Note also the
adjacent air trapping
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References
Texts
1. Ball WS Jr. Pediatric Neuroradiology. Philadelphia: Lippincott-Raven, 1997.
2. Barkovich AJ. Pediatric Neuroimaging. 3rd ed. Philadelphia: Lippincott-Raven, 2000.
3. Donnelly LF. Fundamentals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001.
4. Kirks DR, ed. Practical Pediatric Imaging. 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998.
Journal Articles
1. Capitanio MA and Kirkpatrick JA. Upper respiratory tract obstruction in infants and children. Radiol Clin North
Am 1968;6:265
2. Chinwuba C, Wallman J and Strand R. nasal obstruction: CT assessment. Radiology 1986;159:503
3. Dunbar JS. Upper respiratory tract obstruction in infants and children. AJR Am J Roentgenol 1970;109:227-246.
4. John SD, Swischuk LE. Stridor and upper airway obstruction in infants and children. RadioGraphics 1992;12:625643.
5. Panicek DM, et al. The continuum of pulmonary developmental abnormalities. RadioGraphics 1987;7:747.
Pediatric Radiology
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Vascular Rings and Slings

Figure 6-5-1
Vascular Rings
Double aortic arch

Right arch with aberrant LSCA
Pulmonary sling
Left arch with aberrant RSCA
Anomalous innominate artery
Descending aorta-carina compression
Respiratory symptoms tight complete rings

Stridor
Recurrent respiratory difficulties
Apnea
Feeding difficulties
Choking with feeds
Failure to thrive
Solid food dysphagia
Early in fetal development the

primitive aorta develops as a ventral
tube which separates into two ventral
aortae. Two dorsal aortae fuse to form
a single vessel supplying the lower
body. As the pharyngeal pouches
Evaluation of Stridor/Dysphagia
develop at the rostrum of the embryo,
PA and lateral chest and high kV airway films determine side of so too do paired pharyngeal arterial
arch
arches, numbering 6 (but no 5th arch
in humans) between the ventral and
Esophagram with airway fluoroscopy
dorsal aortae. These arches go on to
MRI/MRA or CTA if vascular ring suspected
fuse, partially regress and fully
Otherwise, CT
regress to form the pulmonary
arteries and the mature left-sided
Embryology of the Normal Left Aortic Arch
aortic arch and its branches
[Figures 6-5-1 to 6-5-4]
Arches 1 and 2 regress

Arch 3 --> common and proximal internal carotid arteries
Arch 4
Right --> regresses (portion of right subclavian)
Left --> persists as LEFT AORTIC ARCH
Arch 5 rudimentary in humans
Arch 6 pulmonary arteries and ducti arteriosi
Right regresses
Left becomes ligamentum arteriosum
Dorsal aortae
Right regresses part of RSCA
Left becomes descending aorta
Figure 6-5-2
In the development of the normal left

aortic arch, the gap or complete
regression occurs in the 8th segment
of the right dorsal aorta. The normal
arch has 3 branches the
brachiocephalic (innominate), the left
common carotid, and the left
subclavian arteries
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Pediatric Radiology
Figure 6-5-3
Edwards postulated the existence of a double arch with
bilateral ducti arteriosi in the embryo. This double arch can be
represented by this ring. The T in the center represents the
pulmonary arteries. The black lines connecting the main
pulmonary arteries to the aorta represent the bilateral ducti
arteriosi. The anterior center of the ring (dotted circle)
represents the ventral or ascending aorta branching into
bilateral arches which join to form the descending aorta in the
posterior center of the ring (dotted circle). Each arch gives rise
to its own carotid and subclavian artery. The anterior solid
circles represent the bilateral carotid arteries, and the posterior
solid circles are the bilateral subclavian arteries. The portion of
the ring between the carotid and subclavian arteries is formed
from the 4th pharyngeal arch. The portion of the ring dorsal to
the subclavian artery represents the contribution of the 8th
segment of the dorsal aorta. These are the most common
sites of gaps or complete regression of a portion of the ring
that give rise to the different types of aortic arches
Figure 6-5-4
Figure 6-5-5
Diagram representing the normal regression

of the 8th segment of the right dorsal aorta
and right ductus arteriosus in development of
the normal left arch. The right subclavian
artery is separated from the descending
aorta and arises in common with the right
common carotid artery from the ascending
aorta
In the development of the aberrant right

subclavian artery, there is early obliteration of the
right 4th arch, and the 8th segment of the right
dorsal aorta persists, so the right subclavian artery
maintains its connection to the descending aorta,
becoming the last branch from the aortic arch
(arrow). Thus, there are four, rather than the
normal three, branches of the aortic arch and
there is no brachiocephalic (innominate) artery.
The descending aorta is on the left, so the
aberrant RSCA then crosses behind the
esophagus to get to the right side
Aberrant RSCA [Figures 6-5-5 to 6-5-8]
Asymptomatic in children normal variant

Due to early complete obliteration of right 4th arch
and persistence of 8th segment of the right dorsal aorta
Left arch
RSCA originates distal to LSCA
Posterior impression on the esophagus
Figure 6-5-6
The gap or obliteration

occurs in the right 4th arch.
The aortic arch is on the
left
Pediatric Radiology
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Figure 6-5-7
Figure 6-5-8
When the RSCA crosses from left to right a

posterior impression is created on the esophagus,
as seen on the lateral view (left image). In the AP
view (right image), a normal left aortic arch is
seen as well as an oblique impression on the
contrast-filled esophagus extending from the left
arch to the right shoulder
The oblique course of the aberrant

right subclavian artery creates a
sloped contour of the aortic knob
(arrow) that may be visible in older
children and adults
Determining Side of Arch [Figures 6-5-9 and 6-5-10]
Figure 6-5-10
Tracheal deviation, buckling or impression the most reliable sign

indicating the side of the aortic arch
Asymmetric density of pedicles
Descending aorta can be on right or left with right arch
Figure 6-5-9
PA chest radiograph showing rightsided impression on the trachea due

to right aortic arch
Normal tracheal buckling away from

the left arch on an expiratory chest
radiograph (arrow)
Right Arch
Branching patterns
Aberrant LSCA
Mirror image
Isolated LSCA congenital subclavian steal
High association with congenital heart disease
Mirror Image Right Arch [Figures 6-5-11 and 6-5-12]
Association with CHD 98%

25% of patients with tetralogy of Fallot have mirror image right arch
35% of patients with truncus arteriosus have mirror image right arch
90% of patients with mirror image right arch have tet
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Pediatric Radiology
Figure 6-5-11
Figure 6-5-12
Patient with right-sided arch and

descending aorta and enlarged heart
with upturned apex, a classic plain
film diagnosis of Tetralogy of Fallot
Figure 6-5-13
Diagram showing the mirror-image
branching right arch from the front.
The ductus is on the left (arrow) so
this is not a ring. A rare variant with
ductus arteriosus from proximal
descending aorta coursing behind
esophagus to left pulmonary artery,
the so-called retro esophageal
ductus, is a true ring
Aberrant LSCA [Figure 6-5-13]
Mirror image of aberrant RSCA

Left ductus arteriosus completes the ring
Symptomatic patients typically have a a tight ductus
or large diverticulum of Kommerell (dilation of origin
of aberrant artery)
This type needs to be distinguished from double arch
with MRI or angiography
Association with congenital heart disease 5-12%
Dysphagia lusoria
Posterior impression on the esophagus
Right-sided impression on the trachea
Ddx: double arch
Right arch with aberrant LSCA and left

ductus [Figure 6-5-14]
Diagram showing development of right arch

aberrant left subclavian artery which is the mirror
image of left arch aberrant right subclavian artery
(shown on the left). The right image shows early
obliteration of the left 4th pharyngeal arch,
separating the left subclavian artery from the left
common carotid artery. Persistence of the 8th
segment of the left dorsal aorta maintains
continuity of the left subclavian artery with the
descending aorta. The left ductus completes the
ring
Figure 6-5-14
This diagram shows the right arch with aberrant

left subclavian artery viewed from anterior. Note
the four branches of the aortic arch, the last of
which is the left subclavian artery. The left
ductus (arrow) completes the ring, so that the
trachea and esophagus are completely
surrounded by aorta and pulmonary arteries
Pediatric Radiology
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Right arch aberrant LSCA with posterior impression

on esophagus [Figures 6-5-15 to 6-5-18]
Figure 6-5-16
Figure 6-5-15
CT of same patient showing right

arch and posterior course of the
aberrant left subclavian artery (arrow)
Esophagram shows posterior impression on the esophagus at
the level of the aortic arch. When the patient is in the AP
projection the right arch is identified. If there were a left arch,
this patient would have an asymptomatic normal variant. With
the right arch, the diagnosis is a complete vascular ring
Cervical aortic arch
Arch above the clavicle not a specific

arch anomaly
May have pulsatile mass in
supraclavicular fossa
80% are right arches
Half are symptomatic rings
Most common variant - right arch that
descends on the right then crosses to
left behind esophagus and gives off left
subclavian artery and left ductus
Figure 6-5-18
Double Aortic Arch

[Figures 6-5-19 to 6-5-21]
Persistence of both left and right fourth

arches
Arteriogram showing a
Most common symptomatic vascular
right arch with aberrant
ring
LSCA and a
Right is usually larger, higher and
diverticulum of
posterior
Kommerell (arrow), or
enlargement of the
Anterior and bilateral lateral
origin of the aberrant
impressions on the trachea
subclavian artery
Posterior and bilateral lateral
impressions on esophagus
Treatment is to ligate the nondominant arch
This sagittal MR image in a 4 month

old with recurrent respiratory
infections, shows the aberrant
subclavian artery in cross section
posterior to the trachea (arrow).
Normally there is no large artery
behind the trachea
Figure 6-5-19
Figure 6-5-20
Esophagram in same
patient. The double arch
causes bilateral lateral
impressions on the
esophagus as seen on
the AP view (left image).
On the lateral view (right
image), there is a
prominent posterior
impression caused by
the joining arches. Thus,
there are 3 impressions
on the esophagus
Figure 6-5-17
PA chest radiograph in a patient with

double aortic arch, showing a higher
larger right sided aortic impression
on the trachea and a smaller, lower
left sided impression (arrow). The
left impression is often difficult to
discern, so double aortic arch is in
the differential of right aortic arch
seen on plain film
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Pediatric Radiology
Pulmonary Sling [Figures 6-5-22 and 6-5-23]
Figure 6-5-21
AKA anomalous pulmonary artery

Left PA originates from right
Anterior impression on esophagus at level of carina
Posterior impression on trachea
Ductus passes from origin of RPA to aorta forming a complete
ring around the trachea only
Compression of bronchus intermedius by anomalous artery
Associated tracheal abnormalities
Tracheomalacia
Complete tracheal rings
T-shaped trachea
Other associated anomalies abnormal pulmonary lobation,
bronchus suis, CHD
Figure 6-5-22
3-D MRA of a double aortic arch,

viewed from posterior
Figure 6-5-23
Left image is a lateral view from an esophagram

showing posterior impression on the trachea and
anterior impression on the esophagus at the level
of the hila. Black blood MRI image shows the left
pulmonary artery originating from the right and
coursing behind the trachea to get to the left lung
Illustration of pulmonary sling viewed

from anterior with ascending aorta cut
away to show the left pulmonary
artery (arrow) originating from the
right, then passing between the
trachea and esophagus to get to the
left side
Innominate Artery Compression Syndrome
Normally the innominate artery passes in front of the trachea just below the
thoracic inlet
In infants it arises more to the left than in adults and there is also thymus in
this region, so it may cause symptomatic compression of the trachea
Increased incidence of symptomatic compression in patients with dilated
esophagus
Compression decreases with advancing age
Midline Aorta Carina Compression Syndrome
Midline course of descending aorta or abnormal position of the carina allows

aorta to compress carina or mainstem bronchus
Other CV Abnormalities That Can Compress the Airway

Congenital Heart Disease
Tetralogy of Fallot with absent pulmonary valve

Large left to right shunts
Massive cardiomegaly
Pediatric Radiology
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Absent Pulmonary Valve Syndrome [Figure 6-5-24]
Variant of Tetralogy of Fallot

Severe pulmonic regurgitation
Aneurysmal dilatation of proximal left and right
pulmonary arteries
Compression of adjacent bronchi
Figure 6-5-24
PA and lateral chest radiographs in patient

Tetralogy of Fallot with absent pulmonary valves.
Note the massive enlargement of the pulmonary
arteries, hyperinflation of the lungs due to bilateral
bronchial compression and associated right aortic
arch
References
Texts
1. Donnelly LF. Fundamentals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001.
2. Kirks DR, ed. Practical Pediatric Imaging. 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998.
3. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams &
Wilkins, 2004.
Journal Articles
1. Berdon WE and Baker DH. Vascular anomalies and the infant lung: rings, slings and other things. Semin
Roentgenol 1972;7:39-63.
2. Berdon WE. Rings, slings and other things: vascular compression of the infant trachea updated from the
midcentury to the millenniumthe legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD. Radiology
2000;216:624-632.
3. Bisset GS III et. Al. Vascular rings: MR imaging. AJR Am J Roentgenol 1987;149:251
4. Donnelly LF, Bisset GS 3rd , McDermott B. Anomalous midline location of the descending aorta: a cause of
compression of the carina and left mainstem bronchus in infants. AJR AM J Roentgenology 1995; 164:705-707.
5. Donnelly LF, Strife JL, Bisset GS III. The spectrum of extrinsic lower airway compression in children: MR
imaging. AJR Am J Roentgenol 1997;168:59-62
6. Kussman BD, Geva T, McGowan FX. Cardiovascular causes of airway compression. Paediatr Anaesth 2004;14:6072.
7. Newman R, Meza MP, Tobin RB, et al. Left pulmonary artery sling: diagnosis and delineation of associated
tracheobronchial anomalies with MR. Pediatr Radiol 1996;26:661-668
8. Pickhardt PJ, Siegel MJ, Gutierrez FR. Vascular rings in symptomatic children: frequency of chest radiographic
findings. Radiology 1997;205:581-582
9. Shuford WH, Sybers RG, Edwards FK. The three types of right aortic arch. AJR 1970;109:67-74
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Cystic Renal Disease of Childhood

Terminology
Cyst
Polycystic kidney disease ARPKD and ADPKD only
Multicystic kidney
Old Classification1
Type I, infantile polycystic kidney disease

Type II, multicystic dysplastic kidney
Type III, adult polycystic kidney
Type IV, cortical cysts associated with massive hydronephrosis
1 Osthanondh V, Potter EL. Pathogenesis of polycystic kidneys: historical survey.
Arch Pathol 1964;77:459
Genetically-based Classification2
Nongenetic
Multicystic dysplasia
Multilocular cyst (tumor)
Simple cyst/calyceal diverticulum
Medullary sponge kidney
Acquired cystic disease
Genetic
Autosomal recessive polycystic kidneys
Autosomal dominant polycystic kidneys
Juvenile nephronophthisis (AR)-medullary cystic disease (AD) complex
Cysts associated with multiple malformation syndromes
2Glassberg KI, Stephens FC, Lebowitz RL, et al. Renal dysgenesis and cystic
disease of the kidney: a report of the Committee on Terminology, Nomenclature

and Classification, Section on Urology, American Academy of Pediatrics. J Urol
1987, Oct; 138:1085
Simple Renal Cyst
Uncommon in children
Usually solitary
Found with increasing frequency due to US screening in patients with UTI
Arise in renal cortex
Do not communicate with collecting system
Simple Renal Cyst
Observed on PNUS screen for malformation syndrome

US criteria for simple cyst no further imaging unless recurrent symptoms of
infection
Otherwise CT to exclude tumor
No treatment unless symptomatic or obstructing collecting system
Calyceal Diverticulum
Cyst that communicates with collecting system

Need contrast study to distinguish from cyst
Urine stasis leads to infection and stone formation
Look for stone in tic on US, KUB, or non-con CT
Delayed images show contrast-filling of the cyst and a neck
Treatment surgical ablation if symptomatic
Pediatric Radiology
1381
Medullary Sponge Kidney
Figure 6-6-1
Congenital focal dilation of collecting tubules usually presenting in

adulthood
Associated with stones or infection but may be found incidentally
Usually bilateral
Medullary nephrocalcinosis radially aligned
Streaky linear densities in involved pyramids
US may show calcifications before plain film
Acquired Renal Cysts
AIDS
Hemo- and peritoneal dialysis
Increase in number and size with length time on dialysis
Complications intracyst or subcapsular or perinephric
hemorrhage
Multicystic Dysplastic Kidney
Most common form of cystic renal disease in infants and children

One of the most common causes of renal mass in first week of
life
Rarely bilateral
Due to early severe in utero obstruction
Extreme end of spectrum of UPJO
Classic MCKD gross specimen

showing nonreniform shape, cysts of
multiple sizes and no identifiable
renal parenchyma
Risk of abnormality of contralateral kidney 20-50% - UPJO, VUR

Negligible renal function
Nodular blastemal elements in 3-5% - risk of Wilms tumor
MCDK - Presentation
Figure 6-6-2
PNUS
Neonate with abdominal mass
Incidentally in older child mimics agenesis
MCDK Gross Pathology [Figures 6-6-1 to 6-6-3]
Macrocysts of variable size

Randomly distributed
Cysts do not communicate
Hydronephrotic variant large central cyst
Rarely segmental upper pole of duplex or lower crossed fused
ectopic
No identifiable normal parenchyma
Associated atresia of ureter or infundibulopelvis
Hydronephrotic variant of MCDK with

reniform shape and patent central
pelvis
Figure 6-6-3
Stillborn baby with bilateral MCDK.

Plain radiograph shows small, bellshaped thorax with airless lungs and
bulging flanks. Autopsy specimen
(viewed from posterior) reveals bilateral
enlarged kidneys which are much
larger than the hypoplastic lung
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Pediatric Radiology
MCDK - Histopathology [Figure 6-6-4]
Histologic hallmark presence of immature dysplastic-appearing tubules

surrounded by collarettes of PAS-staining condensed mesenchyme
Cysts of varying size formed by dilated, dysplastic tubules
Cysts can occur in any part of the nephron
High nuclear to cytoplasmic ratio dysplasia
Figure 6-6-4
MCDK - Imaging [Figures 6-6-5 and 6-6-6]
Large mass with cysts of varying sizes scattered throughout

Cysts do not communicate
Hydronephrotic type large central cyst but no identifiable parenchyma
No identifiable cortex or medulla
Nonreniform shape
Nuclear renogram no significant excretion
VCUG 25% VUR
Figure 6-6-5
Photomicrograph of
dysplastic kidney showing
primitive ducts surrounded
by mesenchymal collarettes
Figure 6-6-6
Prenatal ultrasound showing right

MCDK
MCDK - DDx
Multilocular cystic kidney

A tumor
Cysts within the intervening septa
Severe hydronephrosis
UPJO
Nuclear renogram
Contralateral kidney also affected
Renal ultrasound showing normal right kidney and

left MCDK. Note noncommunicating cysts and
lack of visible normal renal parenchyma
MCDK Course and Prognosis

Natural history of a true MCDK is to resolve

Formerly these were all removed due to rare reports of nephroblastoma1
Now followed to resolution
If they do not resolve, surgical removal is indicated to prevent
complications of infection and neoplasm
Figure 6-6-7
1Strife JL, Souza AS, Kirks DR, Strife CF, Gelfand MJ, Wacksman J.
Multicystic dysplastic kidney in children: US follow-up. Radiology.

1993 Mar;186(3):785-8.
Autosomal Recessive Polycystic Kidney Disease

(ARPKD)
Kidneys and liver ectasia and fibrosis

Kidneys ectasia of the collecting tubules
Delayed CT diagnosis of left MCDK
Liver biliary duct ectasia and periportal fibrosis
which has regressed to a partially
calcified nubbin
Latter develops in early childhood
Degrees of renal and liver involvement are inversely proportional and
determine age of presentation and prognosis
Pediatric Radiology
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ARPKD Spectrum of Presentation

Blyth and Ockenden Clinical Classification1
Figure 6-6-8
[Figures 6-6-9 and 6-6-10]
Perinatal 90% of tubules involved, bilateral nephromegaly,

Potter syndrome, death in first week
Neonatal 60%, present in first month, death by 1 year
Infantile 25%, present at 3-6 mo
Juvenile 10%, presentation in first decade
Portal hypertension
Incidental finding on US
Presentation in second decade with renal failure
1Blyth H, Ockenden BG. Polycystic disease of kidney and liver
presenting in childhood. J Med Genet. 1971 Sep;8(3):257-84.
CT showing Wilms tumor which arose

in a left MCDK
Figure 6-6-9
Figure 6-6-10
ARPKD Autopsy
ARPKD Pathology - Kidneys [Figure 6-6-11]
Large kidneys
Cysts are dilated collecting tubules predominantly in the medulla
Dilated (1-2mm) tubules arranged in a fan-shape
Cortex relatively spared
No dysplasia
On cut section, cortex and medulla unrecognizable
Few coalescent macrocysts
ARPKD Pathology- Liver [Figures 6-6-12 and 6-6-13]
All associated with congenital hepatic fibrosis = ductal plate

Gross image showing Potter facies
malformation
Dilation of interlobular bile ducts associated with a variable
amount of portal fibrosis (Caroli syndrome)
All portal areas are expanded and contain dilated ducts at the periphery with
blood vessels in the middle
Sinusoidal portal hypertension
ARPKD Plain Film
Bilateral flank masses in newborns

Small, bell-shaped thorax
Pneumothorax
Older children may have slightly enlarged kidneys,
hepatosplenomegaly, and/or ascites
Figure 6-6-11
ARPKD - Ultrasound [Figure 6-6-14]
Large kidneys with increased echogenicity in the

medulla due to multiple accoustic interfaces of
dilated, ectatic ducts
May mimic nephrocalcinosis
Compressed, spared cortex may form relatively dark
rim
Poor delineation of cortex, medulla, sinus
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ARPKD. From left to right cut gross specimen,

photomicrograph and diagram show dilated
collecting tubules in the medulla
Pediatric Radiology
Figure 6-6-12
Formation of the intrahepatic biliary radicals
begins with a single layer of primitive ductal plate
surrounding the portal vein. Some insult can
cause congenital hepatic fibrosis by promoting
fibroblast proliferation.
Figure 6-6-13
Figure 6-6-14
Gross specimen showing ectatic

biliary ducts of Caroli disease
ARPKD - Ultrasound [Figure 6-6-15]
High frequency linear transducer may resolve tubular structures

in fan-like array or tiny cysts
Occasional macrocysts
Those who present as children have milder renal findings
normal or mild nephromegaly, +/- increased echogenicity of
medulla, loss of corticomedullary differentiation
Ultrasound - Liver [Figure 6-6-16]
ARPKD. Coronal ultrasound shows

both kidneys to be markedly enlarged
and echogenic centrally with a
relatively sonolucent rim of
compressed cortex
Increased echogenicity +/- ductal ectasia in older children

Portal radicals surrounded by bile ducts
Splenomegaly, varices, and ascites may also be seen in older children
Figure 6-6-16
Figure 6-6-15
12 yo diagnosed in infancy with ARPKD, now

preop for liver transplant. Ultrasound on left shows
markedly dilated biliary ducts with hepatic artery
and portal vein branches in the center, creating a
target appearance. CT shows same target
appearance of biliary ducts as well as cysts in the
renal medulla and splenomegaly due to portal
hypertension
ARPKD. High frequency linear transducer

ultrasound images show echogenic, markedly
enlarged kidneys in which tiny cysts can be
resolved
Pediatric Radiology
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ARPKD - Other Imaging [Figures 6-6-17 to 6-6-19]
Imaging beyond US is rarely necessary

Spoke-wheel or striated appearance of nephrogram
Prolonged nephrographic phase
Calyces compressed, separated and distorted
In older children, mild tubular ectasia similar to medullary sponge kidney
MR - few cortical cysts in about half of patients
Figure 6-6-18
Figure 6-6-17
ARPKD. CT of infant showing

markedly enlarged kidneys with
striated delayed nephrograms due to
compression of parenchyma by
radially oriented dilated fluid-filled
collecting ducts. The kidneys are of
density similar to water due to the
unopacified urine in the dilated ducts.
Note rim of enhancing cortex (arrow).
Also note left kidney hypodense
macrocyst (block arrow)
Excretory urogram of ARPKD
showing bilateral massively enlarged
kidneys with striated nephrograms
and distorted collecting systems
ARPKD - Prognosis
Infantile poor prognosis due to renal insufficiency and

pulmonary hypoplasia
Outcome in childhood is better than previously thought
Early recognition and management are important
Figure 6-6-19
Large Echogenic Kidneys in Neonate
Glomerulocystic disease
ADPKD
Glomerulocystic Disease
Rare, sporadic or heritable (AD)

May be found in some patients with malformative syndromes
Occasionally found in children with family history of ADPKD
Glomerulocystic Disease Clinical Features
Present early with renal failure and palpable abdominal masses

Renal function normal but deteriorates with age
ARPKD with biliary duct ectasia. CT
of the kidneys shows delay of transit
of contrast into dilated, urine-filled
collecting tubule
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Pediatric Radiology
Glomerulocystic Disease Pathology [Figure 6-6-20]
Figure 6-6-20
Cystic dilation of Bowman capsule and proximal

convoluted tubule
Periportal fibrosis, bile duct hyperplasia and hepatic
cysts may also be found
Glomerulocystic Disease Ultrasound
Echogenic normal-sized to enlarged kidneys

Poor corticomedullary differentiation
Tiny cysts may be seen in cortex (vs. ARPKD)
Autosomal Dominant Polycystic Kidney

Disease (ADPKD)
Much more common than AR

Three genetic loci PKD1-3
Family screening routine
Spontaneous mutations frequent
Cysts become larger and more numerous with age
Autosomal Dominant Polycystic Kidney

Disease (ADPKD)
Glomerulocystic disease in a patient with

Zellweger or cerebrohepatorenal syndrome. Gross
and low magnification images show small cortical
cysts
Cysts not usually seen in 1st and 2nd decades but can be seen in neonates
screened for positive family history
Affects multiple organs kidneys, liver, pancreas, spleen, seminal vesicles,
ovaries
Association with occult intracranial aneurysms screen in adulthood
ADPKD - Presentation
Present in 4th-5th decade with hypertension and renal failure

Does not present in childhood but may be found incidentally or secondary to
flank pain due to bleeding into cyst
Rarely presents in infancy minimal cysts in enlarged, echogenic kidneys
ADPKD - Pathology
Enlarged but reniform kidneys

Cysts of varying size scattered throughout the kidney (cortex and medulla)
Usually bilateral but may be asymmetric or even unilateral
Abnormality of the ampullary and interstitial portions of the collecting tubules
and nephrons
Hepatic fibrosis is rare
ADPKD - Ultrasound [Figure 6-6-21]
Figure 6-6-21
Infantile presentation small, spherical cysts on high resolution

US (vs. fan-like, tubular appearance in AR)
Older children - cysts of varying size in cortex and medulla
Can be unilateral at presentation
Normal size or slightly enlarged
Look for cysts in liver and pancreas
ADPKD - Prognosis
Most develop renal failure in 4th-5th decade

Presentation in infancy more severe renal cystic disease, more
hypertension, more rapid progression to renal failure than adult
relatives
Medullary Cystic Disease Complex
Juvenile nephronophthisis AR, presents in first decade

Medullary cystic disease AD, presents in 3rd decade
Polydypsia, polyuria, salt wasting, severe anemia
Progressive renal failure
Growth retardation
Pediatric Radiology
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Neonatal ADPKD. Kidneys are

echogenic but high resolution
ultrasound helps to distinguish small
cysts (arrows) (vs. radially aligned,
tubular cysts in ARPKD)
Medullary Cystic Disease Complex Pathology

[Figure 6-6-22]
Figure 6-6-22
<1.5 cm cysts that increase in size and number with age

Cysts in medulla or subcortical region -70% of patients
Secondary glomerulosclerosis small, fibrotic kidneys
Associated retinitis pigmentosa, hepatic fibrosis, skeletal defects
and CNS abnormalities in familial juvenile form
Medullary Cystic Disease Complex Imaging

[Figure 6-6-23]
Small echogenic kidneys

Multiple small medullary or corticomedullary cysts may not be
present when very young
Cysts Associated with Syndromes
Zellweger - cerebrohepatorenal syndrome AR, lethal

peroxisomal deficiency
Meckel-Gruber AR, posterior encephalocele, polydactyly, cystic
kidneys, congenital hepatic fibrosis
Beckwith-Wiedemann syndrome macroglossia, visceromegaly
and omphalocele, Wilms tumor, also medullary cysts in 13-19%
Cysts Associated with Syndromes
Turner
Downs
Orofaciodigital
Jeune, short rib polydactyly
TS cortical cysts 33-50%
VHL multifocal cystic adenocarcinomas in 45% after 3rd decade
Medullary cystic disease. Sectioned

gross specimen shows cysts in the
medulla and corticomedullary
junction
Figure 6-6-23
Summary
ARPKD and MCKD are most common in perinatal period

Classic MCDK is managed conservatively
Renal dysplasia is caused by in utero obstruction
VCUG indicated in MCDK to exclude contralateral VUR (solitary
functioning kidney)
ARPKD is a spectrum of renal and hepatic disease
ARPKD and ADPKD can both be diagnosed in infants as well as
adolescents/adults
References
12 yo girl with medullary cystic

disease
Texts
1. Kuhn JP, Slovis TL, Haller JO, eds. Caffey's Pediatric Diagnostic Imaging, 10th Ed. Philadelphia: Mosby, 2004.
2. Hartman DS. Renal cystic disease. AFIP Atlas of Radiologic-Pathologic Correlation. Fascicle I. Philadelphia: WB
Saunders; 1989:1-5.
3. Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002.
4. Stocker T, Dehner L, ed. Pediatric Pathology, 2nd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002.
5. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams &
Wilkins, 2004.
Journals
1. Blane CE, Barr M, DiPietro MA, Sedman AB, Bloom DA.
2. Blickman JG, Bramson RT, Herrin JT. Autosomal recessive polycystic kidney disease: long-term sonographic
findings in patients surviving the neonatal period. AJR Am J Roentgenol. 1995 May;164(5):1247-50.
3. Corrales JG, Elder JS. Segmental multicystic kidney and ipsilateral duplication anomalies. J Urol. 1996
Apr;155(4):1398-401.
4. Diard F, Le Dosseur P, Cadier L, Calabet A, Bondonny JM. Multicystic dysplasia in the upper component of the
complete duplex kidney. Pediatr Radiol. 1984;14(5):310-3.
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1388
Pediatric Radiology
5.
6.
7.
8.
9.
Evans WP, Sumner TE, Lorentz WB Jr, Resnick MI. Association of crossed fused renal ectopia and multicystic
kidney. J Urol. 1979 Dec;122(6):821-2.
Narchi H. Risk of Wilms' tumour with multicystic kidney disease: a systematic review. Arch Dis Child. 2005
Feb;90(2):147-9.
Oddone M, Marino C, Sergi C, Occhi M, Negri F, Kotitza Z, et al. Wilms' tumor arising in a multicystic kidney.
Pediatr Radiol. 1994;24(4):236-8.
Renal obstructive dysplasia: ultrasound diagnosis and therapeutic implications. Pediatr Radiol. 1991;21(4):274-7.
Traubici J, Daneman A. High-resolution renal sonography in children with autosomal recessive polycystic kidney
disease. AJR Am J Roentgenol. 2005 May;184(5):1630-3.
Pediatric Radiology
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Pediatric Renal Tumors:

Infancy & Young Children
Objectives
Describe a variety of renal masses in infants and children

Recognize the imaging features of these masses
Understand unique clinical and pathologic features of these tumors
Clues to Assessing Renal Tumors
Imaging:
Dominant tissue composition
Soft tissue
Fluid (cystic)
Fat
Other clues:
Patient age
Pattern of metastases
Soft Tissue Masses
< 5 years of age

Nephroblastomatosis
Rhabdoid tumor
Clear cell sarcoma
Ossifying renal tumor of infancy
Mesoblastic nephroma
> 5 years of age
Renal cell cancer
Lymphoma
Wilms Tumor: Epidemiology
85% of renal masses

6%-7% of all childhood cancers
Approximately 500 cases/year
6/1,000,000 children
Mean age at diagnosis = 3 yrs
90% < 7 yrs
Sex (M:F) equal
Risk Factors
Race: Afro-Americans > Caucasians > Asians

Familial predisposition (1%)
Autosomal dominant
Aniridia
Deletion of tumor suppressor genes on short arm of chromosome 11
11p13 locus (WT1 gene)
11p15 locus (WT2 gene)
Risk Factors: Congenital Syndromes
WAGR syndrome (Wilms tumor, aniridia, genital abnormalities, retardation)

(WT1 gene)
Beckwith-Wiedemann syndrome & hemihypertrophy (WT2 gene)
Drash syndrome (nephritis & male pseudohermaphrodism) WT 1 gene
Trisomy 18
Renal Tumors
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Pediatric Radiology
Wilms Tumor: Gross Features [Figure 6-7-1]
Figure 6-7-1
Large cortical mass

(400 to 500 gm)
Hemorrhage & necrosis (90%)
Pseudocapsule
Spares collecting system
Calcification & fat <10%
Favorable Histology (85%) [Figure 6-7-2]
Triphasic composition
Metanephric blastema
Immature stroma
Tubular elements
Good prognosis if triphasic
Unfavorable Histology (15%) [Figure 6-7-3]
Anaplastic changes
Nuclear enlargement
> 3x size
Hyperchromatic nuclei
Atypical mitotic figures
Implies poor prognosis & resistance to conventional therapy
Wilms tumor, gross pathology
Figure 6-7-2
Figure 6-7-3
Wilms tumor. Atypical histology showing anaplastic nuclear

changes
Wilms tumor. Classic

triphasic histology
showing immature
blastema,
tubules/glomeruli, and
stroma
Wilms Tumor: Clinical Features
Mean age: 3-36 mos (range, 6 mos-4 yr)

Symptoms
Mass: 90%
Pain, fever, hematuria: 30%
Hypertension: 75% - 90%
Aniridia, hemihypertrophy
Budd-Chiari syndrome
Wilms Tumor: US Findings
Well-defined margins
Solid, intrarenal mass
Tumor matrix
Homogeneous: 50%
Heterogeneous : 50%
IVC thrombus (5%-10%)
Relatively avascular
Pediatric Radiology
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Renal Tumors
Wilms Tumor
Figure 6-7-4
Wilms Tumor [Figure 6-7-4]
Wilms Tumor: Caval Thrombus US

Wilms Tumor
US
CT
Sensitivity (%)
100
100
Wilms tumor, ultrasound. Two gray-scale sonograms showing

well-defined, echogenic mass (M) in lower pole of right
kidney. Doppler sonogram (right image) showing flow in
surrounding parenchyma. Tumor is avascular
Accuracy (%)
25
> 95
US performed to confirm presence of a mass and its location

CT to determine tumor extent
Figure 6-7-5
Wilms Tumor
Contrast enhanced CT
low density, intrarenal mass
rim of compressed parenchyma
Little enhancement
Central necrosis 75%
May contain fat or calcifications
Wilms Tumor
Exophytic mass, pseudocapsule
Wilms tumor, CT. Transverse and coronal CT

reformation showing large mass extending
exophytically from lower pole of right kidney
Wilms Tumor
Calcified Wilms Tumor (< 5%) [Figure 6-7-6]
Figure 6-7-6
Role of CT
To confirm presence of tumor and assess tumor extent

Important diagnostic questions:
Tumor thrombus (5%-10%)
Contralateral tumor (5%-10%)
Hepatic or lung metastases
Wilms Tumor: Venous Extension
Renal or caval extension: 5-10% of cases

Often clinically silent
Calcified Wilms tumor
May prolapse through tricuspid valve
Tumor thrombus in heart or hepatic segment of IVC alters surgical approach
Cardiopulmonary bypass
Renal Tumors
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Pediatric Radiology
Wilms Tumor with IVC Extension [Figure 6-7-7]
Figure 6-7-7
Tumor Thrombus
Tumor Thrombus - Bilateral Wilms Tumors
[Figure 6-7-8]
Bilateral Wilms Tumor
5%-10% of patients at diagnosis (synchronous

disease)
Younger mean age: 27 mos
Dominant renal mass & small contralateral tumor or
bilateral large masses
Wilms Tumor: Bilateral Tumors
Wilms tumor with inferior vena caval extension of

tumor
Wilms Tumor
Figure 6-7-8
Wilms Tumor: Metastases
About 10% have metastases at

diagnosis
Lung: 85%-90% of all mets
Liver: 10%-15%
Plain radiographs have FN rate of 729% when CT positive
CT is study of choice for distant
staging
Bilateral Wilms tumor with caval invasion (arrow)
Wilms Tumor: Magnetic Resonance Imaging
Dark on T1-weighted images

Bright on fat-suppressed images
Contrast enhances
Tumor thrombus seen as a luminal
defect
Figure 6-7-9
Wilms Tumor: MRI

Wilms Tumor Staging: NWTS-5
I. Limited to kidney, completely excised

II. Extracapsular extension, but
completely removed
III. Residual tumor confined to
abdomen
IV. Hematogenous mets
V. Synchronous bilateral tumors
Wilms Tumor: Treatment
En bloc resection of affected kidney

Wilms tumor, lung metastases
Excisional biopsy of nodes and lung
nodules
Postop chemotherapy
Preoperative chemotherapy for invasive disease or bilateral Wilms, then
surgery
Pediatric Radiology
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Renal Tumors
4-Year Relapse Free Survival
Stage I: 91%
Stage II: 88%
Stage III: 79%
Stage IV: 78%
Stage V: 70%
Screening High Risk Patients (BWS, WAGR syndromes)
US every 3 months until age 6 or 7

Hypothesis is that tumor will be detected at a lower stage
Risk of developing Wilms tumor increases if nephromegaly at birth
Figure 6-7-10
Other Soft Tissue Tumors - Nephroblastomatosis
Defined as "the presence of nephrogenic rests or nephrogenic

blastema beyond 36 weeks gestation
Important because it is a precursor to Wilms tumor
Nephrogenic Rests: Location
2 types by location
Perilobar
Peripheral cortex or columns of Bertin
Intralobar
Deep cortex
Greater risk of Wilms tumor
Nephrogenic Rests [Figure 6-7-10]

Nephroblastomatosis: Imaging
Findings vary with burden

Small lesions may be inapparent
Larger lesions
Multifocal cortical nodules & masses
Nephromegaly with confluent solid peripheral rind
Nephroblastomatosis: Imaging
Nephrogenic rests. Pathology. Small

cortical rests in peripheral cortex
(arrowheads) and larger Wilms
tumor in deep cortex
US: Hypo-, iso-, or hyperechoic masses or diffuse renal

enlargement
CT: Poorly enhancing low attenuation confluent subcortical rind or peripheral
nodules
MRI: Low signal on T1, iso- or slightly increased signal on T2
Diffuse Nephroblastomatosis [Figure 6-7-11]
Figure 6-7-11
Diffuse Nephroblastomatosis
Clue is peripheral tissue rind
Diffuse Nephroblastomatosis
Diffuse nephroblastomatosis. Confluent peripheral soft tissue

tumor rind, which may involve both cortex and medulla or
the subcapsular space
Renal Tumors
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Pediatric Radiology
Nephroblastomatosis:Cortical Nodules [Figure 6-7-12]
Figure 6-7-12
Focal nephroblastomatosis. Longitudinal sonogram and contrast-enhanced CT and MR

showing small peripheral nodules. The peripheral distribution is the clue to the diagnosis
Nephroblastomatosis: Cortical Nodule
Figure 6-7-13
Nephroblastomatosis: Treatment
Controversial
Chemotherapy in some centers
Close imaging surveillance for enlarging masses in others
Renal sparing surgery
Rhabdoid Tumor of Kidney
2% of childhood renal neoplasms

Arises from renal medulla
Mean age 16 months (90% cases < 3yrs)
Synchronous CNS lesions (10%)
Metastases
Primary neuroectodermal tumor, typically posterior fossa
Rhabdoid Tumor: Pathology [Figure 6-7-13]
Pathology
Large mass (<300 gm)
Infiltrating
Histology
Mononuclear cells
Eccentric nuclei and eosinophilic cytoplasm
Rhabdoid Tumor of Kidney: Radiologic features
Heterogeneous soft tissue mass

Calcification: 66%
Peripheral low density crescent: 70%
Characteristic but non-diagnostic
Rhabdoid tumor. Gross pathology

showing large intrarenal mass
arising in medulla and infiltrating
parenchyma. Histology,
mononuclear cells with eccentric
nuclei
Rhabdoid Tumor
Clue: peripheral low density collection
Figure 6-7-14
Bilateral Rhabdoid Tumors

[Figure 6-7-14]
Rhabdoid tumor. Two CT scans showing a

large necrotic mass in the left kidney
with a peripheral low density crescent
(arrow). Also noted is a solid mass in
the right kidney and multiple hepatic
metastases
Pediatric Radiology
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Renal Tumors
Rhabdoid Tumor with PNET
Figure 6-7-15
Rhabdoid Tumor: Outcome
Poor prognosis
Highly aggressive tumor
mets 80% at diagnosis
lung, liver, brain, nodes
20% survival @ 18 months
Clear Cell Sarcoma (Bone metastasizing renal tumor of

childhood)
4% of pediatric renal neoplasms

Peak incidence 2nd year of life
Arises from medulla
Aggressive tumor
Mets to bone (40%-75%), lung, nodes
Clear cell sarcoma.

Solid mass with
infiltrating margins
Clear Cell Sarcoma: Pathology [Figure 6-7-15]
Pathology
Solid, white surface
Infiltrating margins
Histology
Clear cytoplasm
Vascular stroma
Figure 6-7-16
Clear Cell Sarcoma: Imaging Features [Figure 6-7-16]
Heterogeneous intrarenal mass

Cystic changes (70%)
dilated tubules
mucoid substance
Indistinct margins
Bone metastases
Clear Cell Sarcoma

Clear Cell Sarcoma
Clear cell sarcoma. Large infiltrating

mass in the left kidney with cystic
areas
Cystic Change
Clear Cell Sarcoma: Outcome
Treatment is nephrectomy & chemotherapy

Survival rates 60%-70%
Figure 6-7-17
5% of all renal tumors

Diagnosis in neonatal period
mean age, 2 months
Incidental mass
May be diagnosed in utero
Other findings
hypertension (renin)
hypercalcemia (parathormone)
Mesoblastic Nephroma: Path [Figure 6-7-17]
Firm, rubbery, yellow-gray

Unencapsulated
Hemorrhage & necrosis uncommon
Renal Tumors
Mesoblastic nephroma, gross

pathology. Solid rubbery mass
with minimal hemorrhage. The
tumor replaces most of the kidney
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Pediatric Radiology
Mesoblastic Nephroma: Histology
Figure 6-7-18
Classic pattern: mature spindle cells

Cellular pattern: increased mitoses,
potentially more aggressive
May entrap glomeruli & tubules
Mesoblastic Nephroma: Imaging
Homogeneous mass
Cystic changes rare
Well circumscribed
Mimics Wilms tumor

Clue: patient age
Mesoblastic Nephroma [Figure 6-7-18]

Mesoblastic Nephroma: Therapy
and Prognosis
Most behave in benign fashion

Cured by nephrectomy
Metastases & recurrence very rare
Associated with atypical cellular
histology
Overall prognosis excellent
Mesoblastic nephroma. Longitudinal sonogram and CT

showing soft tissue mass replacing renal parenchyma.
Histologic specimen showing mature spindle cells. Gross
specimen showing classic solid tumor without necrosis or
hemorrhage
Figure 6-7-19
Ossifying Renal Tumor of Infancy
Very rare!! < 20 cases reported

Age range 6 days-14 months
Most patients < 4 months
Small, 2-3 cm diameter
Typically present with hematuria
Cancer 1980; 45: 609-612
Ossifying Renal Tumor [Figure 6-7-19]
Arises in medulla
Ill-defined margins
Extends into collecting system
Obstructs collecting system
Plump, ovoid spindle cells with associated osteoid & bone

production
Ossifying renal tumor, gross

pathology. Medullary mass
involving collecting system with
associated hydronephrosis
Ossifying Renal Tumor of Infancy: Imaging Features
Central solid mass causing hydronephrosis

Calcification (ossification) 80%
May mimic a staghorn calculus
Figure 6-7-20

[Figure 6-7-20]
Ossifying renal tumor of infancy. Noncontrast scan (left panel) showing

soft tissue mass with calcification.
Enhanced CT scan (right panel)
showing a central tumor (T) with
associated hydronephrosis
Pediatric Radiology
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Renal Tumors
Ossifying Renal Tumor of Infancy:Treatment & Prognosis
Managed surgically
Apparently benign
No reported cases of malignant spread or recurrence to date
Cystic Masses
< 5 years of age

> 5 years of age
Simple renal cysts (rare)
Figure 6-7-21
Multilocular Cystic Renal Tumor: Clinical Findings
Biphasic age distribution

Boys 3 months to 2 years
Women > 40 years
Usually asymptomatic mass
Pain & hematuria from ureteral prolapse of cyst
Multilocular Cystic Renal Tumor [Figure 6-7-21]
Composed of cysts & septa

Encapsulated
Mean diameter 7 to 10 cm
Multilocular cystic renal tumor. Gross

pathology showing multiple fluidfilled locules with surrounding
septations
Multilocular Cystic Renal Tumor Histologic Features
Two grossly identical but histologically distinct lesions

Cystic nephroma (CN)
Cystic partially differentiated nephroblastoma (CPDN)
Figure 6-7-22
Cystic nephroma (CN) [Figure 6-7-22]
Peak age, 18 months

Cysts 2 to 5mm
Mature renal elements in septa
No blastema
Cystic Partially Differentiated Nephroblastoma

(CPDN) [Figure 6-7-23]
Mean age, 12 months

Cysts, 2 to 3 mm
Immature elements in septa
Usually blastema
Multilocular Cystic Renal Tumors:Imaging
Cystic nephroma, histology. Fibrous

septa with differentiated tubules
in septa
Figure 6-7-23
Cystic, fluid-filled mass

Water density, signal intensity
Variable thickness septations
Septations enhance, but not fluid contents
Multilocular Cystic Renal Tumor (CN)
Septa enhance
Multilocular Cystic Renal Tumor

Cystic partiallly differentiated
nephroblastoma, histology.
Immature blastemal cells in septa
Renal Tumors
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Pediatric Radiology
Multilocular Cystic Renal Tumor [Figure 6-7-24]
Figure 6-7-24
Multilocular Cystic Renal Tumor:

Therapy & Prognosis
Both cystic and partially differentiated

forms usually cured by complete
resection
Partially differentiated tumor has
potential for aggressive behavior &
recurrence
warrants follow-up imaging
Multilocular Cystic Renal Tumor:


Non-functioning
Entire kidney involved
Conservative treatment, not surgery
Usually found in neonates
Multilocular cystic renal tumor. CT and gross path (left panel)

showing multicystic mass with septations. T1-weighted
(middle panel) and gadolinium contrast enhanced image
(right panel) also showing a multicystic mass
Figure 6-7-25

[Figure 6-7-25]
Multicystic Dysplastic Kidney -CT

Fat-containing
Masses:Angiomyolipoma
Any age, but more often after 5 years

Associated with tuberous sclerosis
80% of tuberous sclerosis patients have
angiomyolipomas
Imaging:
Bilateral fatty, renal masses
Solitary lesions, very rare
Multicystic dysplastic kidney. Gross path and longitudinal

sonogram showing multiple cysts of variable size with no
normal renal tissue
Figure 6-7-26
Angiomyolipoma: Imaging [Figure 6-7-26]

Tumors of Older Children - Renal Cell Cancer
< 2% of all renal cell neoplasms

Mean age, 9 years
Clinical findings
flank pain, hematuria, mass
Imaging findings:
solid renal mass
average diameter, 4 cm
Renal Cell Cancer: Pathology - Classic Clear Cell

Cancer
Non-specific mass with hemorrhage and necrosis

Tumor cells with clear cytoplasm, arranged in nests
Angiomyolipomas, tuberous sclerosis.

Transverse sonogram showing highly
echogenic kidney. CT, multiple fatty
tissue masses. The presence of fat is
the clue to the diagnosis
Pediatric Radiology
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Renal Tumors
Renal Cell Cancer
Figure 6-7-27
Clue: small lesion, older pt age
Renal Cell Cancer [Figure 6-7-27]

Renal Cell Cancer: Outcome
Metastases at diagnosis: 30%

75% lung
other sites- lymph nodes, bone,
liver
Prognosis
regional spread: 75% survival
vascular invasion or metastases: 0
to 10% survival
Renal cell cancer, 10 year old boy. Two contrast-enhanced CT

scans showing a solid intrarenal mass with associated
perinephric hemorrhage
Lymphoma
Secondary involvement from direct extension or hematogenous spread

Non-Hodgkin >> Hodgkin disease
Occurs late in course of disease
Figure 6-7-28
Lymphoma: Imaging Patterns
Multiple or solitary nodules (80%)

Diffuse infiltration
Direct invasion
Perinephric involvement
Renal Lymphoma: Nodules

[Figure 6-7-28]
Summary: Lesions you need to

know
< 5 years of age

Nephroblastomatosis
Rhabdoid tumor
Clear cell sarcoma
Ossifying renal tumor of infancy
> 5 years of age
Renal cell cancer
Lymphoma
Lymphoma, multiple appearances. Multiple nodules, top left

image. Solitary nodule, lower left image. Perinephric
tumor, top right image. Diffuse infiltration, bottom right
image.
References
1.
2.
3.
4.
5.
6.
7.
8.
Siegel MJ. Urinary Tract. In: Siegel MJ, ed. Pediatric Sonography, 3rd ed. Lippincott Williams & Wilkins.
Philadelphia. 2002; 385-473.
Siegel MJ. The Kidney. In: Siegel MJ, ed. Pediatric Body CT. Philadelphia, Lippincott Williams & Wilkins,
1999; 226-252.
Siegel MJ. MRI of the pediatric abdomen. MRI Clin North Am 1995; 3:161-182.
Geller E, Smergel E, Lowry P. Renal neoplasms of childhood. Rad Clin North Am 1997; 35:1391-1413.
Green DM, Coppes MJ, Breslow NE, et al. Wilms tumor. In: Pizzo PA, Poplack DG, (eds). Principles and Practice
of Pediatric Oncology, 3rd ed. New York. Lippincott-Raven. 1997; 733-759.
Lowe LH, Isuani BH, Heller RM, et al. Pediatric renal masses: Wilms tumor and beyond. RadioGraphics 2000;
20:1585-1603.
Navoy JE, Royal SA, Vaid YN, Mroczek-Musulman EC. Wilms tumor: unusual manifestations. Pediatr Radiol
1995; 25:S76-S
DeBaun MR, Siegel MJ, Choyke PL. Nephromegaly in infancy and early childhood: a risk factor for Wilms
tumor in Beckwith-Wiedemann syndrome. J Pediatr 1998; 132:401-404.
Renal Tumors
1398
1400
Pediatric Radiology
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
Lonergan GJ, Martinez-Leon MI, Agrons GA, Montemarano H, Suarez ES. Nephrogenic rest,
nephroblastomatosis, and associated lesions of the kidney. Radiographics 1998; 18:947-968.
Rohrschneider WK, Weirich A, Rieden K, Darge K, Troger J, Graf N. US, CT, and MR imaging characteristics of
nephroblastomatosis. Pediatr Radiol 1998; 28:435-443
Agrons GA, Kingsman KD, Wagner BJ, Sotelo-Avila C. Rhabdoid tumor of the kidney in children: a comparative
study of 21 cases. AJR 1997; 168:447-45
Chung CJ, Lorenzo R, Rayder S, Schemankewitz E, Guy CD, Cutting J, Munden M. Rhabdoid tumors of the
kidney in children: CT Findings. AJR 1995; 164:697-700.
Kabala JE, Shield J, Duncan A. Renal cell carcinoma in childhood. Pediatr Radiol 1992; 22:203-205.
Davidson AJ, Choyke PL, Hartman DS, Davis CJ, Jr. Renal medullary carcinoma associated with sickle cell trait:
radiology findings. Radiology 1995; 195:83-85
Chepuri NB, Strouse PJ, Yanik GA. CT of renal lymphoma in children. AJR 2003; 180:419-431.
Hugosson C, Mahr MA, Sabbah R. Primary unilateral renal lymphoblastic lymphoma. Pediatr Radiol 1997;
27:23-25
Wooten SL, Rowen SJ, Griscom NT. Congenital mesoblastic nephroma. RadioGraphics 1991; 11:719-721
Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologicpathologic correlation. RadioGraphics 1995; 16:653-669.
Hopkins JK, Giles HW, Wyatt-Ashmead J, Bigler SA. Cystic nephroma. Radiographics 2004; 24:589-593
Pediatric Radiology
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Renal Tumors
Pediatric Adrenal Masses

Objectives
Discuss differential diagnoses of adrenal masses in neonates and children

Describe imaging features of common adrenal masses in a pediatric
population
Describe pitfalls in diagnosis
Adrenal Masses of Childhood: Differential Considerations
Neoplastic
Medullary tumors
Adrenocortical neoplasms
Metastases
Non-neoplastic
Hemorrhage
Congenital hyperplasia
Storage disorders
Figure 6-8-1
Adrenal Medullary Tumors
Neoplastic tumors
Neuroblastic tumors
Neuroblastoma
Ganglioneuroma
Pheochromocytoma
Neuroblastic Tumors: Histogenesis
Arise from neural crest tissue

Involve adrenal medulla or anywhere in sympathetic
chain
Spectrum of differentiation and biologic behavior
Neuroblastic Tumors: Spectrum of

Histology
Spectrum of neuroblastic tumors, histology.

Neuroblastoma (NB) (left panel), characterized
by immature small blue cells.
Ganglioneuroblastoma (GNB) (middle panel)
containing neuroblasts and mature cells
(gangliocytes). Ganglioneuroma (GN) (right
panel) composed of mature gangliocytes and
mature stroma
Neuroblastoma (NB): immature cells

Small round blue cells
Ganglioneuroblastoma (GNB): both neuroblasts & mature cells (gangliocytes)
Ganglioneuroma (GN): mature gangliocytes & mature stroma
Neuroblastic Tumors: Histology [Figure 6-8-1]

Neuroblastic Tumors: Spectrum of Catecholamine Production
Less mature tumors more active than mature tumors

90% of all tumors produce catecholamines
Vanillylmandelic acid (VMA) - metabolite of epinephrine & norepinephrine
Homovanillic acid (HVA) - metabolite of dopamine
Vasoactive intestinal peptide (VIP) - elaborated by ganglion cells
Neuroblastoma: Epidemiology
Most common extracranial solid neoplasm of childhood

2nd most common abdominal malignancy (after Wilms tumor)
10% of pediatric cancers
500-525 new cases/yr in the US
Mean age ~ 2 yrs.
75% < 5 yrs.
Adrenal Masses
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Pediatric Radiology
Neuroblastoma: Symptoms
Figure 6-8-2
Symptoms are age dependent

Infants < 6 mos:
Skin nodules
blueberry muffin syndrome
Hepatomegaly (liver mets)
Pepper syndrome
Respiratory compromise
from massive liver
Abdominal mass is small & may not be palpable
Blueberry Muffin Syndrome

Neuroblastoma: Symptoms
Infant > 6 months

Palpable abdominal mass
75% have systemic symptoms
bone pain
hepatomegaly
paraplegia
opsoclonus, ataxia
diarrhea (VIP production)
Distribution of neuroblastoma
Neuroblastoma: Location [Figure 6-8-2]
Abdomen: 75%
Thorax: 20%
Neck: 1%-5%
Pelvis: 2%-3%
Unknown primary: 1%
Present with mets
Figure 6-8-3
Neuroblastoma: Pathology [Figure 6-8-3]
Mean size 6-8 cm

Hemorrhage and necrosis common
Path-deep red to gray-white to tan appearance
Neuroblastoma: Histology
Small blue cell tumors with occasional cluster of cells arranged in

rosettes
Neuroblastoma: Genetic Associations
N-myc oncogene
Located on distal end of chromosome 2p
Multiple copies (n-myc amplification) associated with
aggressive tumor behavior
Deletion of short arm of chromosome p1
More aggressive tumor behavior
Neuroblastoma, gross pathology.

Large solid extrarenal mass, not
encapsulated
Role of Imaging
Identification of primary tumor

Determination of extent of local disease
Detection of distant metastases
Abdominal Neuroblastoma: Spectrum of Imaging Features

Neuroblastoma: Approach to Diagnosis
Screening abdominal US (if palpable mass)

Chest X-ray
CT &/or MRI following abnormal US or X-ray
Pediatric Radiology
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Adrenal Masses
Figure 6-8-4
MIBG
Bone marrow aspirate/biopsy
Neuroblastoma: Imaging Spectrum
Typical findings
Infants (> 6 mos) & children
Predominantly solid mass
Atypical findings
Neonates
Predominantly cystic
Neuroblastoma: Typical US Findings
Extrarenal mass
Irregular margins
Heterogeneous
90%
Homogeneous
10%
Mixed pattern reflects high cellularity, dystrophic calcification and
necrosis
Neuroblastoma: US [Figure 6-8-4]
Typical appearance infants & children

Predominantly solid mass
Neuroblastoma: Typical CT Findings
Neuroblastoma. Typical appearance

in infants and children. Solid
extrarenal mass with small cystic
areas and/or calcification
Extrarenal mass
Smooth or irregular margins
Density less than adjacent tissues
No definable capsule
Midline extension
Calcifications 85%
Figure 6-8-5
25% of cases ipsilateral

No midline extension
75% of cases-midline extension
Neuroblastoma: Atypical Findings
Neonates
Predominantly cystic tumor
degenerative change or microcysts
Appearance non-specific, mimics
hematoma
Diagnosis requires evidence of
metastatic disease, + VMA analysis or
serial US
Neuroblastoma. Two CT images showing a well-defined, right

adrenal mass containing calcification and displacing the
kidney inferiorly. The mass is localized to the right
abdomen and does not cross the midline
Figure 6-8-6
Neuroblastoma. Three CT scans showing a large left adrenal

mass with irregular margins and areas of necrosis. The
tumor crosses the midline, encases vessels, and displaces
the left kidney laterally
Adrenal Masses
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Pediatric Radiology
Neonatal Neuroblastoma [Figure 6-8-7]
Figure 6-8-7
Neuroblastoma: MRI
T1-weighted images:
low signal intensity (black)
Fat-suppressed images:
high signal intensity (bright)
Contrast enhances
Neuroblastoma: MRI [Figure 6-8-8]

Local Extension: Diagnostic
Questions
Midline extension (> 30%) (see Fig. 6)

Vascular encasement (>30%)
Liver metastases (5-10%)
Intraspinal extension (15%)
Midline Extension: Vascular

Encasement [Figure 6-8-9]
Neonatal neuroblastoma. Predominantly cystic mass arising in

the right adrenal gland, reflecting necrosis, hemorrhage or
intrinsic cystic changes
Spinal Invasion [Figure 6-8-10]

Cervicothoracic Neuroblastoma: Imaging Features
Figure 6-8-10
Figure 6-8-8
Neuroblastoma. T1-weighted axial image (left panel) showing a low

signal intensity mass with high signal intensity areas representing
hemorrhage. T2-weighted image (middle panel) showing a high
signal intensity mass. Coronal gadolinium-enhanced image (left
panel) showing an enhancing suprarenal mass
Figure 6-8-9
Neuroblastoma. Transverse CT
scan (top panel) and sagittal
STIR (bottom panel) images
showing intraspinal tumor
extension (arrows)
Neuroblastoma. T2-weighted axial (left panel) and coronal
STIR images (right panel) showing large left adrenal mass,
which crosses the midline and encases the aorta, renal
vessels and superior mesenteric artery
Pediatric Radiology
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Adrenal Masses
Thoracic Neuroblastoma [Figure 6-8-11]
Figure 6-8-11
Thoracic Neuroblastoma
Posterior mediastinum
Fusiform shape
Soft tissue density
Calcifications (50%)
Extends over several interspaces
Osseous erosions (rib/vertebra)
Thoracic Neuroblastoma [Figure 6-8-12]

Thoracic neuroblastoma. Posterior mediastinal
mass
Figure 6-8-12
Figure 6-8-13
Pelvic neuroblastoma. Transverse and sagittal CT

scans show a large presacral pelvic mass (M)
which invades the spinal canal (arrows)
Thoracic neuroblastoma. Transverse, coronal and

sagittal CT images showing a right paraspinal
mass with a fusiform shape extending over
several vertebral body levels
Thoracic Neuroblastoma: MRI - Intraspinal Extension

Pelvic Neuroblastoma [Figure 6-8-13]
Neuroblastoma Metastases: Age Dependent Pattern
< 6 months
Liver (usually diffuse)
Skin
Bone marrow
> 6 months
Cortical bone & bone marrow
Liver (solitary or diffuse)
Lymph nodes
Figure 6-8-14
Hepatic Metastases (5%-10%)

[Figure 6-8-14]
Infant with diffuse liver mets

Pepper syndrome
Neuroblastoma. Transverse hepatic sonogram showing diffuse
parenchymal heterogeneity. Transverse CT showing
diffuse hepatic metastases and a small right primary tumor
(M).
Adrenal Masses
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Pediatric Radiology
Hepatic Metastases
Distant Metastases: Skeleton Imaging Studies (sensitivity)
Bone scintigraphy: 90%

Metaiodobenzylguanidine (MIBG)
I-123 MIBG: 95%
In-111 pentetreotide
Total body MRI: > 95%
FDG PET: 100%
X-ray: 35%-70%
Figure 6-8-15
Sites for Skeletal Metastases
Metaphyses of long bones

Calvarium-dura
Ribs & vertebral bodies
Flat bones
I.e., the sites of red marrow
Neuroblastoma, bone metastases. Plain

radiographs (left panel) showing lytic
metaphyseal lesions. Bone scintigraphy (right
panel) showing increased radionuclide activity
in metaphyseal ends of the long bones
Neuroblastoma: Radionuclide Imaging

Sensitivity
Primary tumor
35-90%
Skeletal metastases
Radionuclide 90%
X-ray
35-70%
lytic or permeative
Metastases are usually asymmetric and metaphyseal in location
Skeletal Metastases: Bone Scintigraphy [Figure 6-8-15]

Split Suture Sign - Dural Metastases
MIBG Scintigraphy [Figure 6-8-16]
Figure 6-8-16
Norepinephrine analogue
I-123 MIBG
Sens: 80% to 95%
Sens >> bone scan
Skeletal Metastases
MRI
FDG-PET
Staging
Neuroblastoma. MIBG scintigraphy. Increased activity in right

adrenal mass and in skeleton
Stage 1:
Localized tumor confined to area of
origin; complete excision
Stage 2A:
Unilateral tumor with incomplete excision; ipsilateral nodes negative
microscopically.
Stage 2B:
Unilateral tumor complete or incomplete excision; positive ipsilateral nodes
Stage 3:
Tumor across midline; or unilateral tumor with contralateral node
involvement; or midline tumor with bilateral lymph node involvement
Stage 4:
Spread to distant lymph nodes, bone, bone marrow, liver, or other organs
Stage 4S:
Unilateral primary tumor with spread limited to liver, skin and/or bone
marrow.
Limited to infants < 1 year of age.
Pediatric Radiology
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Adrenal Masses
Neuroblastoma: Stage Distribution [Figure 6-8-17]
Figure 6-8-17
Neuroblastoma: Treatment
Resectable disease: surgery

Unresectable disease:
chemotherapy
Radiation therapy for tumor that does not regress with
chemotherapy
Neuroblastoma: Overall Survival
Stage 1: 94%
Stage 2: 90%
Stage 3: 64%
Stage 4: 24%
Stage 4S: 75%
Distribution of stages of neuroblastoma.

Majority of patients have advanced
disease at time of diagnosis
Favorable Outcome: Associated Factors
Low stage
Young patient age (< 1 yr)
No N-myc amplification
No chromosome 1p
Stroma-rich
Neuroblastoma: Outcome- 3-year survival
Stage & age of patient at diagnosis most important predictors of outcome

Stages 1, 2, & 4S: 75%-90%
Children < 1 year
Stage 3 disease: 80%-90%
Stage 4 disease: 60%-75%
Children > 1 year
Stage 3: 50%
Stage 4: 15%
Figure 6-8-18
Other neuroblastic tumors

Ganglioneuroma
Pheochromocytoma
Adrenocortical tumors
Metastases
Other neuroblastic tumors. Ganglioneuroblastoma (left panel).

Ganglioneuroma (right panel). The appearance overlaps
that of neuroblastoma
Other Neuroblastic Tumors [Figure 6-8-18]
Neuroblasts & ganglion cells
Ganglioneuroma
Mature ganglion cells
Neurofibroma
Pheochromocytoma
Mean age: 11 yr, (range 6-18 yr)

10% malignant
Bilateral 20%: associated with:
MEN-IIA & IIB
von Hippel-Lindau syndrome
von Recklinghausen syndrome (NF1)
Clinical
Paroxysmal hypertension, headaches, diaphoresis
Adrenal Masses
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Pediatric Radiology
Pheochromocytoma: Locations
Figure 6-8-19
97% abdominopelvic
Adrenal (90%)
Paraganglioma (10%)
Organ of Zuckerkandl
Retroperitoneum
Pelvis
Thorax (rare site)
Pheochromocytoma: Gross Path [Figure 6-8-19]
Rounded solid mass

3-5 cm
Hemorrhage &necrosis common
Rarely purely cystic
Pheochromocytoma: Imaging
Cystic changes, hemorrhage common

US: Solid circumscribed mass with variable heterogeneity
CT: Heterogenous mass (when large), enhances
MR imaging: high signal T2-WT image
MIBG avid
Pheochromocytoma [Figure 6-8-20]
Pheochromoctyoma. Gross
pathology showing a round
cystic mass with hemorrhagic
contents
Adrenocortical Tumors
Rare in children
Adrenal carcinoma most common
Mean patient age: 9 years
Hormonally active producing virilization,
less often Cushing syndrome
Adenomas rare
Figure 6-8-20
Adrenocortical Cancer: Path &

Imaging [Figure 6-8-21]
Large solid mass (mean, 6 cm)

Well circumscribed
Lobulated surface
Cystic areas of hemorrhage and
necrosis common
Calcification 20%
Mets to liver, lung, bone
Adrenocortical Cancer [Figure 6-8-22]
Pheochromocytoma. Longitudinal sonogram showing an

echogenic suprarenal mass (M). Coronal T2-weighted
image showing a high signal intensity mass (M) with
central necrosis
Figure 6-8-21
Adrenocrotical cancer. Cut section

shows a solid mass with areas of
hemorrhage and necrosis
Pediatric Radiology
Figure 6-8-22
Adrenal cancer. Longitudinal sonogram (left panel) showing a

solid adrenal mass. Coronal T1-weighted image (middle
panel) showing a large, left suprarenal mass with necrosis.
T2-weighted image (right panel) showing a high-signal
intensity mass with areas of necrosis
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Adrenal Masses
Adrenal Metastases
Figure 6-8-23
Rare in children
Lymphomatous involvement may occur
Nonspecific uniform solid adrenal mass
Adrenal Masses of Childhood: Differential

Considerations
Neoplastic
Medullary tumors
Adrenocortical neoplasms
Metastases
Non-neoplasticneonatal diseases
Hemorrhage
Storage disorders
First you need to know------US of Normal Adrenal

[Figure 6-8-23]
Echogenic medulla
Hypoechoic cortex
Inverted V or Y shape
Mean length, 15 mm
Mean width, 3 mm
Adrenal Hemorrhage
Most common neonatal adrenal mass

Result of passive venous engorgement during delivery
Predisposing conditions:
birth trauma, hypoxia, IDM, coagulopathy, renal vein
thrombosis, sepsis
Does not cause adrenal insufficiency
R > L (3-4:1), may be bilateral
Normal adrenal gland. Upper panel:

V shaped adrenal gland. Lower
panel Y-shaped adrenal gland.
Note: hypoechoic cortex,
echogenic medulla
Figure 6-8-24
Adrenal Hematoma
Clinical findings
Abdominal mass
Anemia
Jaundice
Adrenal Hematoma: US
Appearance varies with age of

hematoma
Day1-2: echogenic or complex
mass (fibrin,debris)
Day 3 to 2 wks: complex
(liquefaction)
Later: cystic (hypo- or anechoic)
Shrinkage within 1 to 2 weeks
May calcify as early as 1 week
Associated caval thrombus
Adrenal Hematoma
[Figures 6-8-24 and 6-8-25]
Adrenal Masses
Adrenal hematoma. Longitudinal sonograms on day 1 (left

upper panel), on day 12 (left lower panel), at 6 weeks
(right upper panel) and at 2 months (right lower panel)
showing changes in echogenicity and near complete
resolution of a right adrenal hematoma
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Pediatric Radiology
Differential Diagnosis: Neuroblastoma
Figure 6-8-25
Neuroblastoma does not liquefy

(but may spontaneously regress)
Neuroblastoma makes catecholamine
byproducts
US surveillance to document resolution
of hemorrhage (may take 6 to 8 weeks)
Adrenal Hematoma
Calcified hematomas
Congenital Adrenal Hyperplasia
Adrenal hematoma. Left panel. Day 3, complex suprarenal

mass. Right panel, day 10, nearly complete involution
Autosomal recessive enzymatic

deficiency
21-hydroxylase deficiency most common (>90%)
Androgen overproduction
virilism in girls
premature masculization in boys
advanced somatic development in both sexes
Aldosterone underproduction
salt wasting crisis
Figure 6-8-26
Congenital Adrenal Hyperplasia: Pathology [Figure 6-8-26]
Bilateral adrenal enlargement with cerebriform appearance

Mean length > 20 mm
Mean width > 4 mm
Congenital Adrenal Hyperplasia: Imaging
Adrenal enlargement
Wavy contour
Cerebriform appearance
Woman Disease
Congenital adrenal hyperplasia.

Gross section showing large
bilateral adrenal glands with
cerebriform pattern
Figure 6-8-27
Rare inborn lysosomal acid lipase deficiency

Cholesterol esters accumulate in all
organs
Presents in infancy, death in 1 year
Woman Disease: Imaging
Marked adrenomegaly
Preserved adreniform contour
Punctate or coarse calcifications
Hepatosplenomegaly
Wolman Disease [Figure 6-8-27]

Adrenal Tumor-Neonate:
Hemorrhage or Neuroblastoma?
Enlarged adrenal gland?

Both
Cystic and/or solid?
Both
Complete involution?
Hemorrhage
Liquefies?
Hemorrhage
Pediatric Radiology
Wolman disease. Plain radiographs (upper panels) showing

enlarged, calcified adrenal glands. Longitudinal sonogram
(left lower panel) showing a suprarenal mass with marked
shadowing. CT (right lower panel) showing bilateral
calcified adrenal glands.
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Adrenal Masses
Adrenal Tumor-Neonate: Hyperplasia or Wolman Disease
Enlarged adrenal gland?

Both
Solid?
Both
Cerebriform pattern?
Hyperplasia
Calcifies?
Wolman
Hormonally active?
Hyperplasia
Figure 6-8-28
The Spectrum: Neonate

[Figure 6-8-28]
Adrenal Tumor-Infant & Older Child:

Neuroblastoma, Cancer, Pheo
Solid mass?
All
Cystic changes?
All
Bone mets?
Neuroblastoma
Virilization?
Adrenal cancer
Paroxysmal hypertension
Pheochromocytoma
Neonatal adrenal lesions. Neuroblastoma (upper left panel).

Wolman (lower left panel). Hemorrhage (upper right
panel). Congenital adrenal hyperplasia (lower right panel)
Spectrum of Adrenal Lesions: US - Infant & Older Child

[Figure 6-8-29]
Spectrum of Adrenal Lesions: CT - Infant & Older Child

[Figure 6-8-30]
Figure 6-8-29
The Need to Know Adrenal

Masses
Neoplastic
Medullary tumors
Neuroblastic
Pheochromocytoma
Adrenocortical cancer
Non-neoplastic
Hemorrhage
Wolman disease
Adrenal lesions infant and older children, ultrasound.

Neuroblastoma (left panel). Pheochromoctyoma (middle
panel). Cancer (right panel)
Figure 6-8-30
Adrenal lesions infant and older children, CT. Neuroblastoma

(left panel). Pheochromoctyoma (middle panel). Cancer
(right panel)
Adrenal Masses
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Pediatric Radiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
Siegel MJ. Adrenal glands, pancreas and other retroperitoneal structures. In: Siegel MJ (ed), Pediatric Sonography,
3rd. Philadelphia. Lippincott Williams & Wilkins. 2002; 475-527.
Siegel MJ. Adrenal glands, pancreas and other retroperitoneal structures. In: Siegel MJ (ed), Pediatric Body CT.
Philadelphia. Lippincott-Williams & Wilkins. 1999; 253-286.
Westra SJ, Zaninovic AC, Hall TR, Kangarloo H, Boechat MI. Imaging of the adrenal gland in children.
RadioGraphics 1994; 14:1323-1340.
Abramson SJ. Adrenal neoplasms in children. Radiol Clin North Am 1997; 35:1415-1453.
Brodeur GM, Maris JM. Neuroblastoma. In: Devita VT, Hellman S, Rosenberg SA, eds. Cancer Principles and
Practice of Oncology. Lippincott Williams & Wilkins. Philadelphia. 2001; 895-933.
Lonnergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma and ganglioneuroma:
radiologic-pathologic correlation. Radiographics 2002; 22:911-934.
Berdon W, Ruzal-Shapiro C, Abramson S. The diagnosis of abdominal neuroblastoma: Relative roles of
ultrasonography, CT and MR. Urol Radiol 1992; 14:252-262
Cassady C, Winter WD. Bilateral cystic neuroblastoma: imaging features and differential diagnoses. Pediatr
Radiol 1997; 27:758-759.
Teoh SK, Whitman GJ, Chew FS. Neonatal neuroblastoma. AJR 1997; 168:54.
Meyer JA, Harty MP, Khademian Z. Imaging of neuroblastoma and Wilms tumor. Magn Reson Imaging Clin
2002; 10:275-302.
Siegel MJ. MR imaging of pediatric abdominal neoplasms. MRI Clin North Am 2000; 8:837-851
Gelfand M J. Meta-iodobenzylguanidine in children. Semin Nucl Med 1993; 23: 231-242
Shulkin BL, Wieland DM, Baro ME, et al. PET hydroxyephedrine imaging of neuroblastoma. J Nuc Med 1996;
37:16-21.
Argons GA, Lonergan GJ, Dickey GD, Perez-Monte JE. Adrenocortical neoplasms in children: radiologicpathologic correlation. Radiographics1999; 19:989-1008.
Riberio J, Ribeiro RC, Fletcher BD. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol 2000;
30:45-51.
Sivit CJ, Hung W, Taylor GA, Catena LM, Brown-Jones C, Kushner DC. Sonography in neonatal congenital
adrenal hyperplasia. AJR 1991; 156:141143.
zmen MN, Aygn N, Kili I, Kuran L, Yalin B, Besim A. Wolmans disease: ultrasonographic and computed
tomographic findings. Pediatr Radiol 1992; 22:541542.
Pediatric Radiology
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Adrenal Masses
Pediatric Pelvic Masses

Figure 6-9-1
Objectives
Review normal anatomy

Discuss causes of pelvic masses
Describe imaging features of common and some uncommon
pelvic lesions
To recognize the abnormal, one must first know the

normal
J. Caffey
Normal Maturation: Growth
Ovarian volume
Uterine volume
Age vs. Ovarian Volume
Neonate (1day-3 mos)

Infant (4-12 mos)
Early childhood (2-8 yrs)
Late childhood (9-14 yrs)
Pubertal
1.1 cm3
0.7 cm3
0.8-1.1 cm3
2.2-4.2 cm3
9.8 cm3
Ovarian Maturation: Morphology
Prepubertal ovary
Usually homogeneous
But may be heterogeneous due to presence of primordial
follicles (< 1cm)
Pubertal ovary
Usually heterogeneous
Reflects presence of primordial & functional follicles (1- 3cm)
Ovarian Morphology: Need to Know
Presence of cysts is normal in infants & children

Do not confuse for pathology
Figure 6-9-2
Normal Maturation
Normal prepubertal ovary. Two 2year-old girls. Left panel: The ovary
is relatively homogeneous. Right
panel: Longitudinal sonogram
showing multiple small follicles,
measuring less than 9 mm in
diameter. B = bladder
10 follicles stimulated each cycle

1 becomes dominant
Grows to 15 to 30 mm
Normal Prepubertal Ovary: US

[Figure 6-9-1]
Normal Pubertal Ovary: US

[Figure 6-9-2]
Normal pubertal ovaries. Left panel: Longitudinal sonogram on

day 10 of the menstrual cycle shows multiple follicles less than
10 mm in diameter (calibers=right ovary). Right panel:
Longitudinal sonogram on day 20 of the menstrual cycle shows
a dominant follicle (arrow), measuring 18 mm in length. This
likely represents a corpus luteum cyst
Pediatric Pelvis Masses
1412
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Pediatric Radiology
Normal Ovary: CT and MR [Figure 6-9-3]
Figure 6-9-3
Uterine Morphology: Maturation
Prepubertal uterus
tubular shape
fundus equal to cervix
endometrial canal seen in neonates,
not in infants and children
Pubertal uterus
Normal ovaries. Left panel: CT scan of a 6 year old girl. The
fundus elongates and thickens
ovaries
are not well seen. Middle panel: CT scan of a 13-year fundus larger than cervix
old
girl
shows homogeneous right ovary (arrow) and a small
endometrial canal again present
developing follicle, measuring 2.5 cm diameter, in the left ovary.
Right panel. Fat-saturated T2 weighted MR of an adolescent
Age vs. Uterine AP Diameter
girl showing multiple high signal intensity follicles in both
Neonate (1day-3 mos)
2.1 cm
ovaries (arrowheads)
Infant (4-12 mos)
0.8 cm
Early childhood (2-8 yrs)

Late childhood (9-14 yrs)
Pubertal
0.7 cm
1.4 cm
1.6-3 cm
Figure 6-9-4
Normal Uterus: US [Figure 6-9-4]

Normal Uterus: CT and MR
[Figure 6-9-5]
Pelvic Mass Lesions
Anterior Pelvis
Ovarian
Bladder and lower genital tract
Posterior Pelvis (Presacral)
Neurogenic tumors
Teratomas
Pelvic Masses: Imaging Approach
Normal uterine morphology. Left panel: Neonatal uterus.

Longitudinal sonogram shows a tubular uterus (arrowheads)
with prominence of the uterine fundus and a thin, hyperechoic
endometrial stripe. Middle panel: A 2-year-old girl. The uterus
is small and tubular with no differentiation between fundus and
cervix and no recognizable endometrial stripe. Right panel.
Sonogram showing a pear-shaped uterus with a fundus that is
larger than the cervix. The endometrial stripe (calipers) is again
visualized and varies in thickness with the phase of the
menstrual cycle
US is screening examination of choice

for most clinically suspected masses
CT is study of choice for evaluation of gynecologic, bladder and prostate
lesions shown on sonography
MRI usually reserved for presacral masses
Ovarian Masses
Ovarian Tumors: First Key Point
Figure 6-9-5
Cystic
Follicular cysts
Paraovarian cysts
Cystic neoplasms
Teratoma, cystadenoma
Solid
Malignant germ cell tumors
Sex cord-stromal tumors
Epithelial tumors are extremely rare in
the 1st two decades
Put them low on the list
THINK SIMPLE CYSTS, GERM CELL
TUMORS, OR STROMAL TUMORS
Pediatric Radiology
Normal uterus. Left panel: CT scan of a 5-year-old girl. The

uterus (arrow) is seen as a small oval soft tissue structure.
Middle panel: Normal pubertal uterus. CT scan of a 15-yearold girl shows an oval uterine fundus that demonstrates zonal
differentiation-- higher attenuation myometrium and
endometrium and the lower attenuation endometrial canal.
Right panel: T2-weighted image from a 12-year-old girl shows
normal zonal anatomy--endometrial complex (e), junctional
zone (arrow), and outer myometrium (m)
1413
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Follicular Cysts
Figure 6-9-6
Most common ovarian mass

Over stimulated follicle
Imaging findings
fluid-filled lesion
unilocular
thin-walled
avascular
3 to 8 cm
Functional Ovarian Cyst: US

[Figure 6-9-6]
Functional Ovarian Cyst CT/MR

[Figure 6-9-7]
Hemorrhagic Ovarian Cyst: US
Functional ovarian cysts, US. Left panel: Sonogram showing a

6-cm anechoic cyst (C) with imperceptible walls and sound
transmission. Right panel: Color Doppler sonogram of another
patient with an ovarian cyst (C). The cystic contents show no
color signal. Minimal flow is noted in the adjacent parenchyma
US Findings
Complex mass (>85%)
Septations, fluid-debris level
Hyperechoic mass (<15%) (early)
Acoustic transmission
Doppler: avascular
Painful masses
Figure 6-9-7
Hemorrhagic Ovarian Cysts:

Spectrum of US Features
[Figure 6-9-8]
Hemorrhagic Cysts: CT/ MRI

[Figure 6-9-9]
CT
High density
fluid-fluid level
MR
Mixed signal
fluid-fluid level
Functional ovarian cysts, CT and MR. CT (left panel) showing

a 6 cm low attenuation cyst (C) arising from the right ovary
displacing the bladder (B) to the left. Fat-saturated T2weighted image (right panel) showing a high signal intensity,
right ovarian cyst, measuring 5 cm in diameter. The normal
zonal anatomy of the uterus--endometrial complex, inner
junctional zone, and outer myometrium-- is noted
Figure 6-9-8
Figure 6-9-9
Hemorrhagic ovarian cysts. Different adolescent patients. Left

panel: Early hemorrhage. A hyperechoic cyst (arrow) with
acoustic enhancement. Ut = uterus. Middle panel: Late
hemorrhage. A complex cyst (C), measuring 5 cm in diameter,
with internal echoes and septations. Right panel: A complex
mass (arrows) with fluid-debris level
Hemorrhagic ovarian cyst, CT/ MRI. Upper image: CT

showing high attenuation right ovarian cyst (arrow). Lower
image: T2-weight fat-saturated MR showing mixed signal
intensity cyst (arrows) with a blood-fluid level (arrowhead). U =
uterus
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Pediatric Radiology
Follicular & Hemorrhagic Cysts: More Points
Most cysts regress within 2 cycles

If the cyst remains for > 3 cycles, likely not functional
Suspect paraovarian cyst or neoplasm
Cysts > 6 cm should be followed
Hemorrhagic Ovarian Cyst: Serial US to document resolution

DDX: Follicular Cysts
Paraovarian cysts
Teratoma
Cystadenoma
Figure 6-9-10
Paraovarian Cysts
Arise in broad ligament or fallopian tube

Imaging:
round or oval
fluid-filled
No cyclic changes
Do not regress
Can make diagnosis if cyst is separate
from ovary
Paraovarian Cysts (Fallopian

Tube)
Paraovarian Cyst [Figure 6-9-10]
Paraovarian cyst. Left panel: Longitudinal sonogram showing

a large cyst adjacent to a normal right ovary (arrow). BL =
bladder. Right panel: Fat-saturated T2-weighted axial MR
image showing a high signal intensity cyst separate from both
ovaries
Cystic Neoplasms: Ovarian Teratoma
Most common ovarian tumor

90% benign
75% asymptomatic
25% pain due to torsion
Bilateral 8% to 15%
Large, mean diameter 15 cm
Usually adolescent girls
Figure 6-9-11
Mature Teratoma: Pathology

[Figure 6-9-11]
Gross
Cystic mass
Foci of fat, Ca++, bone
Histology
Respiratory, GI elements
Sebaceous glands, hair skin
Muscle, cartilage
No malignant elements
Ovarian teratoma, pathology. Cut section showing cystic

teratoma containing small nidus of calcification posteriorly
(arrow). Histologic section showing sebacous glands and skin
Cyst Ovarian Teratoma: Imaging
90% predominantly cystic

>>50% fluid contents
Minimal soft tissue
mural nodule
septations
Foci of fat & Ca++
10% contain only fat & hair contents
Avascular
Pediatric Radiology
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Cystic Ovarian Teratoma: US [Figure 6-9-12]
Figure 6-9-12
Multiple Benign Teratomas: CT

[Figure 6-9-13]
Teratoma
15 yo girl with abdominal
distention
Ovarian Teratoma: MRI
Ovarian Cystadenoma
Epithelial tumor
< 5% neoplasms, benign
Mucinous >> serous
4 to 20 cm diameter
Epithelial linings
Simple (serous type)
Columnar, mucinous (mucinous
type)
Mature teratomas, US. Different patients. Left panel:

Longitudinal sonogram showing a predominantly cystic mass
with a an echogenic mural nodule (dermoid plug) (arrow).
B=bladder; UT=uterus. Right panel: Sonogram showing a
complex cystic mass containing large echogenic mural nodule
anteriorly (arrow) with acoustic shadowing
Figure 6-9-13
Cystadenoma: Imaging [Figure 6-9-14]
Usually large
Cystic
Multilocular with septations
Sometimes unilocular
Particularly serous type
Serous Cystadenoma
Ovarian Malignancies
Germ cell tumors (85%)

Stromal tumors (15%)
Epithelial tumors
Malignant Germ Cell Tumors
10% of germ cell tumors

Immature: neural (brain) tissue
Malignant: frank malignant elements
Predominantly soft tissue (>50%) plus fat and
calcium
Mature ovarian teratomas, CT. Spectrum of

appearances
Malignant Ovarian Tumors: Path [Figure 6-9-15]

Figure 6-9-15
Figure 6-9-14
Mucinous cystadenoma. Longitudinal sonogram

showing a multilocular mass. CT showing a cystic
mass with enhancing septations
Malignant ovarian tumors, pathology. Left panel,

dysgerminoma. Right panel, malignant teratoma.
Malignant tumors typically have lobulated surfaces
and contain predominantly solid elements with
areas of necrosis and hemorrhage
1416
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Pediatric Radiology
Malignant Germ Cell Neoplasms: Clinical & Imaging
Pelvic or abdominal masses

Imaging findings suggestive of
malignancy:
solid or complex mass
> 50% soft tissue elements
thick, irregular walls
Nonspecific findings: > 10 cm size,
Ca++
Metastasize to lymph nodes and liver
(rarely to omentum or mesentery)
Dysgerminoma [Figure 6-9-16]

Malignant Teratoma Clue:
Predominantly Solid Mass
Figure 6-9-16
Dysgerminomas. Left panel: Longitudinal sonogram showing a

large predominantly solid mass with hypoechoic areas
representing necrosis. Middle and right panels. Different
patients. Contrast-enhanced CT scans showing a large mostly
solid mass with cystic components
Figure 6-9-17
Endodermal Sinus Tumor (Yolk Sac Cancer)

Sex Cord-Stromal Tumors (15%)
Prepubertal girls
Granulosa theca cell & Sertoli-Leydig
Hormonally active
Estrogens (Granulosa-Theca)
Androgens (Sertoli-Leydig)
Can be malignant or benign
Spread to peritoneum and liver
Sex cord stromal tumors, pathology. Left panel:

granulosa theca cell tumor. Right panel: SertoliLeydig tumor. Both tumors appear as large,
predominantly solid mass with varying size areas
of necrosis
Sex Cord-Stromal Tumors: Pathology

[Figure 6-9-17]
Figure 6-9-18
Sex Cord-Stromal Tumors: Imaging
Granulosa cell tumors

mixed solid-cystic mass with thick,
irregular septations
Sertoli-Leydig tumors
homogeneous solid mass or mixed
solid-cystic mass
Metastases, although rare, are to
peritoneal surfaces and liver
Granulosa-theca Cell Tumor

[Figure 6-9-18]
Sertoli-Leydig cell tumor

[Figure 6-9-19]
Granulosa-theca cell tumor. 5 year-old girl with breast

development and vaginal bleeding. Transverse sonogram
showing a heterogenous solid right ovarian mass with cystic
areas. CT showing a low attenuation mass with irregular
septations
Figure 6-9-19
Setoli-Leydig cell tumor. Young girl with

virilization. Two transverse CT scans
showing a heterogeneous mass with
solid and cystic (necrotic) areas. The
appearance mimics malignant germ cell
tumors, but the diagnostic clue is
hormonal activity
Pediatric Radiology
1417
1419
Ovarian Cancer
Rare lesion in pediatric population

Epithelial origin
Imaging
Solid mass
Often necrotic
Smaller in size than germ cell tumors
Spreads to mesentery and omentum
Ovarian Cancer (<5% of tumors)

Which Ovarian Tumor?
Cystic with mural nodules?

Teratoma
Cystic with septations?
Cystadenoma
Solid with cystic elements?
Malignant germ cell tumors, stromal tumors
Older age (pubertal)?
Germ cell tumors, Cystadenoma
Younger age?
Stromal tumors
Figure 6-9-20
Uterine Masses
Cystic
hydrocolpos
Solid
rhabdomyosarcoma
Hydrocolpos
Vaginal obstruction
due to stenosis or membrane
Result is pelvic/abdominal mass
Imaging findings
Fluid-filled midline mass
Well defined walls
Internal debris or blood
(hemato- or
hematometrocolpos)
Hydrocolpos-hematocolpos, US. Left panel. Neonate.

Longitudinal sonogram showing dilated fluid-filled vagina (V)
outlining the cervix (C). B = bladder. Right panel.
Hematocolpos. Longitudinal sonogram showing a dilated
vagina (V) with low level central echoes, representing blood.
Uterus (U) is normal
HydrocolposHematocolpos [Figure 6-9-20]

Hydrocolpos: CT & MRI [Figure 6-9-21]
Rhabdomyosarcoma
Most common pelvic malignancy

Bimodal age distribution:
2 to 6 and 14 to 16 years
Embryonal cell type
Sites:
Head/Neck
38%
GU
21%
Extremity
18%
Figure 6-9-21
Hydrocolpos, CT and MR. CT showing a dilated fluid-filled

vagina (V). Axial T2-weighted MRI showing high-signal
intensity fluid in the vaginal canal (V). B = bladder
1418
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Pediatric Radiology
Rhabdomyosarcoma: Female Pelvis
Figure 6-9-22
Arises in vagina
Vaginal bleeding
Imaging findings
Soft tissue mass
Enlarged pelvic nodes
Mets to liver, lung, node and bone
Vaginal Rhabdomyosarcoma [Figure 6-9-22]

Rhabdomyosarcoma: Male Bladder &
Prostate
Bladder
Trigone or bladder base
Polypoid mass
Prostate gland
Solid mass
Deforms base of bladder
Elongates prostatic urethra
Vaginal rhabdomyosarcoma. Sagittal T1-weighted

and fat-suppressed T2-weighted images showing
a soft tissue mass filling the vaginal canal (arrow).
(Case courtesy of Mary E. McCarville, MD,
Memphis, TN)
Prostatic Rhabdomyosarcoma [Figure 6-9-23]

Rhabdomyosarcoma: Prostate
Bladder Rhabdomyosarcoma
Bladder Masses: Differential Diagnosis
Figure 6-9-23
Rhabdomyosarcoma
Pheochromocytoma
Neurofibroma
Transitional cell
Leiomyosarcoma
Which Lower Genital

Tract Tumor?
YOU DONT NEED CLUES

Rhabdomyosarcoma
Solid, infiltrative?
Rhabdomyosarcoma
Presacral Masses
Cystic
benign teratoma
meningocele
Solid
malignant teratoma
neuroblastoma
Prostatic rhabdomyosarcoma. CT scans showing an

enlarged prostate gland (P) The planes between the
mass and right obturator internus muscles are
obliterated. Invasion of pelvic sidewalls confirmed at
surgery
Sacrococcygeal Teratoma
CA++:
Malignancy:
60%
10% newborn
90 > 2 months
Location:
45 % external
45% external & internal
10% presacral
Bony defect: very low frequency
Arise from coccyx
Do not invade spinal canal
Pediatric Radiology
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1421
Sacrococcygeal Teratoma: Imaging
Figure 6-9-24
Benign teratomas
Contain predominantly fluid or fat
Sometimes Ca++
Malignant teratomas
Predominantly solid
Fed by sacrococcygeal and iliac arteries
Benign Sacrococcygeal Teratoma

Benign SC Teratoma [Figure 6-9-24]
Anterior Meningocele
Herniation of spinal contents through a

congenital defect in a vertebral body
Scimitar shaped sacrum
CT/MR
absent sacral elements
tethered cord
Anterior Meningocele
Benign sacrococcygeal teratoma. T1-weighted MR (left panel)

showing a predominantly fat-containing mass with small fluidfilled nodules. Fat-saturated T2-weighted MR (right panel).
The fatty tissue has decreased in signal intensity. Fluid
components are bright. The tumor arises from the coccyx
(arrow)
Presacral Masses
Figure 6-9-25
Cystic
benign teratoma
meningocele
Solid
malignant teratoma
neuroblastoma
Malignant SC Teratoma [Figure 6-9-25]

Which Presacral Tumor?
Cystic Mass, normal spine?

Teratoma
Cystic mass, abnormal spine?
Anterior meningocele
Solid with normal spine
Malignant teratoma
Solid with spinal canal invasion
Neuroblastoma
Malignant sacrococcygeal teratoma. Transverse CT scan (left

panel), T1-weighted MR (middle panel) and T2-weighted MR
(right panel) showing a large presacral mass with a
predominance of soft-tissue components
Figure 6-9-26
Presacral neuroblastoma. CT showing a

soft-tissue mass (M) anterior to the sacrum
and posterior to the bladder (BL). The
tumor has invaded the right obturator fossa
(arrow). Sagittal STIR image showing a
presacral soft-tissue mass (M) extending
into the spinal canal (arrowheads) and
displacing the bladder superiorly. F = foley
catheter
1420
1422
Pediatric Radiology
Lateral Pelvic Masses
Adenopathy
Lymphoma
Neurofibroma
Neurofibromas
Nerve sheath tumors

Often plexiform in the pelvis
multiple or interlacing masses
Affect about 5%-15% of people with NF1
Imaging
Multiple soft tissue masses
Target sign T2 MR
Enlarged sacral foramen
Plexiform Neurofibromatosis
Clue: enlarged sacral foramen
Neurofibromatosis: T2 MR
Lymphadenopathy
Top 10 Pelvic Lesions: What you need to know
Functional ovarian cyst

Ovarian teratoma
Malignant ovarian tumors
Sex cord stromal tumors
Hydrocolpos
Rhabdomyosarcoma
Anterior meninogocele
Presacral neuroblastoma
Neurofibromatosis (NF1)
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
Garel L, Dubois J, Grignon A, Filiatrault D, Van Vliet G. US of the pediatric female pelvis: a clinical perspective.
Radiographics 2001; 21:1393-1407.
Siegel MJ. Female pelvis. In: Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia. Lippincott Wiliams &
Wilkins.
States LJ, Bellah RD. Imaging of the pediatric female pelvis. Semin Roentgenol 1996; 31:312-329.
Boechat IN. MR imaging of the pediatric pelvis. MRI Clin North Am. 1996; 4:679-697.
Rigsby CK, Siegel MJ. CT appearance of pediatric ovaries and uterus. J Comput Assist Tomogr 1994; 18:72-76.
Siegel MJ. Pelvic organs and soft tissues. In: Siegel MJ, ed. Pediatric Body CT. Philadelphia, Lippincott
Williams and Wilkins, 1999, pp 287-311.
Siegel MJ. Magnetic resonance imaging of the adolescent female pelvis. Mag Reson Imaging Clin North Am
2002; 10:303-324.
Baltarowich OH. Female pelvic organ measurements. In: Goldberg BB, Kurtz AB, eds. Atlas of Ultrasound
Measurements. Chicago. Year Book Medical Publishers. 1990; 190-242.
Cohen HL, Eisenberg P, Mandel F, Haller JO. Ovarian cysts are common in premenarchal girls: a sonographic
study of 101 children 2-12 years old. AJR 1992; 159:89-91.
Siegel MJ. Pelvic tumors. Radiol Clin North Am 1997; 35:1455-1475.
Fried AN, Kenney CM III, Stigers KB, Kacki MH, Buckley SL . Benign pelvic masses: sonographic spectrum.
RadioGraphics 1996; 16:321-334
Kim JS, Woo SK, Suh SJ, Morettin LB. Sonographic diagnosis of paraovarian cysts: value of detecting a separate
ipsilateral ovary. AJR 1995; 164:1441-1444
Jabra AA, Fishman EK, Taylor GA. Primary ovarian tumors in the pediatric patient: CT evaluation. Clin Imaging
1993;17:199-203.
Pediatric Radiology
1421
1423
14. Quillin SP, Siegel MJ. CT features of benign and malignant teratomas in children. J Comput Assist Tomogr 1992;
16:722-726.
15. Castleberry RP, Cushing B, Perlman E, Hawkins EP. Germ cell tumors. In: Pizzo PA, Poplack DG, et al.
Principles and Practice of Pediatric Oncology. Philadelphia: Lippincott Raven 1997; 921-945.
16. Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex-cord-stroma and steroid cell tumors of the
ovary. Radiographics 1998; 18:1523-1546.
17. Blask ARN, Sanders RC, Gearhart JP. Obstructed uterovaginal anomalies: demonstration with sonography. Part I
neonates and infants. Radiology 1991; 179:79-83.
18. Blask ARN, Sanders RC, Rock JA. Obstructed uterovaginal anomalies: demonstration with sonography. Part II
teenagers. Radiology 1991; 179:84-88
19. Argons GA, Wagner BJ, Lonergan GJ, Dickey GE, Kaufman MS. Genitourinary rhabdomyosarcoma in children:
20. Fletcher BD, Kaste SC. Magnetic resonance imaging for diagnosis and follow-up of genitourinary, pelvic, and
perineal rhabdomyosarcoma. Urol Radiol 1992; 14:262-272.
21. Argons GA, Wagner BJ, Lonergan GJ, Dickey GE, Kaufman MS. Genitourinary rhabdomyosarcoma in children:
1422
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Pediatric Radiology
Bone Marrow Imaging

Lecture Objectives
Review normal bone marrow anatomy

Discuss MRI marrow techniques
Describe features of normal marrow on MRI
Recognize causes of pathologic marrow on MRI
Why study bone marrow?
We all have it
It shows up on every MRI
It is an important site of pathology
We need to know the normal before we can recognize the abnormal
M. Siegel, Stating the Obvious
Why Study Bone Marrow? It Has Clinical Applications
MRI has become the imaging modality of choice for evaluating marrow
changes.
MRI can help characterize the composition of marrow or marrow process in
question
MRI provides excellent anatomic detail of surrounding structures, boundaries
of disease
PART I: Bone Marrow Constituents

Bone Marrow: 3 Components
Osseous Matrix
Red Marrow
Yellow Marrow
Figure 6-10-1
Marrow Components
Osseous matrix
(aka cancellous or spongy bone)
Provides support for cellular components
Marrow Components
Red Marrow
Cellular, active or myeloid marrow
Composed of red & white blood cells & platelets
Yellow Marrow
Inactive or fatty marrow
Composed of fat cells
Normal marrow constituents.

Histologic section showing
hematopoietic and yellow marrow
The Major Marrow Constituents: Histology [Figure 6-10-1]

Red and Yellow Marrow: Different Chemical Composition
Red
Yellow
Water
40
15
Fat
40
80
Protein 20
MRI appearance of marrow reflects the relative

fractions of red & yellow marrow
Pediatric Radiology
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Bone Marrow Imaging
Bone Marrow Vascularity
Red
Rich sinusoidal vascular supply
Favors metastases, infection
Yellow
Sparse vascular supply
Favors infarction
PART II: MRI Techniques

PART II: Technique
Depends on clinical indication

Dedicated MRI of limited body part is used in the evaluation of localized pain
or to follow a focal lesion
Whole body MRI is used for staging, restaging and surveillance
Basic Pulse Sequences
For imaging pathology, these are your main sequences:

Spin echo T1
STIR or T2 with fat suppression
Whole-body MRI
Technique
Vertex to toes
Coronal plane
Sagittal plane
Table moves 4-6 times
Total imaging time
~ 7-20 minutes
Lauenstein, Radiology 2004;233:139
Whole-body MRI: Results
51 patients. 43 with metastases

Reference standards: CT and bone scan
All brain, lung, liver metastases > 6 mm seen
Small lung metastases missed
did not change therapeutic strategies
Whole-body MR imaging on per-patient basis -- 100% sensitivity & specificity
values
Lauenstein, Radiology 2004;233:139
Part III: MR Features of Normal Marrow

Normal Marrow: T1
This is your Fat Finder sequence

Yellow marrow will be bright due to abundant fat composition
Red marrow contains much less fat, and will be intermediate in signal. (But
enough fat not to be dark!!!!)
Normal Marrow: Fat Suppression
STIR & T2 with fat saturation

Both sequences exhibit T2 weighting, while suppressing fat signal
Red Marrow remains intermediate
Yellow marrow is dark, as fat is suppressed
Normal Marrow Imaging: T2
T2 weighted imaging causes both red and yellow marrow to be intermediate in

intensity, making them look similar
Not a widely used sequence
Bone Marrow Imaging
1424
1426
Pediatric Radiology
Part III: MRI Features of Normal Marrow
Figure 6-10-2
Red Marrow
T1-weighted: dark (> muscle < fat)
STIR/FS: mildly bright (>muscle <
fat)
Yellow Marrow
T1-weighted: bright (= fat)
STIR/FS : black (< muscle)
What About Gadolinium?
Used if lesion is identified on T1 or fat

suppressed images
Increases lesion conspicuity
Worthwhile to remember that normal
adult red & yellow marrow does not
enhance
Red marrow in neonate enhances
Pathologic lesions enhance
Normal red marrow. T1-weighted (left panel) and fat-saturated

T2-weighted (right panel) images. Hematopoietic marrow in
the pelvis and femora exhibits a signal intensity equal to
that of muscle
Figure 6-10-3
Normal Red Marrow Signal: Neonate

[Figure 6-10-2]
In neonate, marrow has minimal lipid content

Red marrow = muscle
Red Marrow Signal: Older Child [Figure 6-10-3]
Red > muscle
Normal Yellow Marrow Signal

[Figure 6-10-4]
Review: Red/Yellow Signal Intensity (T1)
Yellow > red > muscle

Unossified cartilage is equal to muscle
Review: Red/Yellow Signal Intensity (STIR)
Red > muscle > yellow

Unossified cartilage is bright on T2, due to its
watery content
MR Normal Marrow: Variations in

Distribution
Red marrow signal intensity. Red marrow has

signal intensity slightly higher than that of
muscle on T1-WT and STIR images, reflecting
greater fraction of lipids. Yellow epiphyseal
marrow has signal intensity identical to that of
subcutaneous fat on both sequences
Conversion of red to yellow marrow occurs during growth and development

and has a predictable and orderly pattern
You need to know this to avoid mistakes in diagnosis
Figure 6-10-4
Patterns of Marrow Distribution
Neonate: virtually all red marrow

Shortly after birth red-to-yellow marrow conversion
begins
Overall: extremities to axial
In a given bone: epiphysis/apophysis ? diaphysis
--> metaphysis
Marrow Conversion
Appendicular to Axial Conversion
Custer. J Lab Clin Med 1932
Long Bone Conversion
Normal yellow marrow. T1-weighted image (left

panel) and fat-saturated T2-weighted image
(Right panel). Yellow marrow has a signal
intensity similar to subcutaneous fat on both
sequences
Distal to proximal in individual bones

Epiphysis to diaphysis to metaphysis
Pediatric Radiology
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Bone Marrow Imaging
Marrow Distribution [Figure 6-10-5]
Adult distribution by age 25 years

From nearly 100% red marrow at birth to 40% red marrow in adulthood
Figure 6-10-5
Vogler JB III. Radiology 1998; 168:679-693.
Variations in Marrow Distribution
A common EXCEPTION to the rule that red marrow converts to

yellow marrow is that the epiphyses & apophyses completely
convert to yellow marrow once they ossify
Controversial whether they ever contain red marrow
Variations in Marrow Distribution
Another exception is that red marrow can persist in the proximal

humeral and femoral epiphyses of adults
Variations in Red Marrow Distribution
Epiphysis: subchondral, curvilinear focus

Other patterns are likely abnormal
Bone Marrow Variations: Vertebral Marrow [Figure 6-10-6]
Yellow marrow around basivertebral vein common in children

Other patterns common in adults
Islands of fat can mimic metastases
Ricci C. Radiology 1990;177:83-88.
Normal Vertebral Marrow: MRI

Summary Key Points: Criteria for Normal Marrow
Shows expected signal intensities on all image sequences

Shows expected distribution in the skeleton for patient age
Is symmetric
% of fatty marrow increases with age
PART IV: Marrow Pathology
Reconversion
Replacement
Depletion
Vascular mediated lesions
Edema
Ischemia
Adult marrow distribution. Yellow

marrow predominates in the
appendicular skeleton. Red
marrow is found mainly in the
skull, flat bones, spine, proximal
metaphyses of both the humeri
and femora
Marrow Reconversion
Opposite of conversion
Results when increased demand for hematopoiesis
Generally symmetric
Causes:
Chronic anemias (sickle cell, thalassemia)
Increased O2 needs (altitude, athletes, smokers)
Granulocyte colony stimulating factor
Cyanotic heart disease
Figure 6-10-6
Reconversion
Axial skeleton responds first, followed by extremities
Marrow Reconversion
Reverse order from normal conversion process!

Proximal to Distal!!
Bone Marrow Imaging
Vertebral marrow, red-yellow marrow

distribution. Yellow marrow is
located near basivertebral vein in
neonates and young children
(upper right diagram). Other
patterns predominate in
adolescents and adults
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Pediatric Radiology
Marrow Reconversion: Histology [Figure 6-10-7]
Increased red cells

No fat cells to generate signal
Figure 6-10-7
Marrow Reconversion: Sickle cell anemia
T1-WT images: dark signal intensity

Red = or slightly > muscle
STIR/Fat sat T2 images: slightly bright
Reconversion: Sickle Cell Anemia [Figure 6-10-8]

Reconversion: Thalassemia
Granulocyte Colony Stimulating Factor
Stimulates marrow hyperplasia

Focal abnormality (mimics tumor)
Clues to diagnosis
onset 2 to 8 weeks after treatment
increased white blood cell count
affects metaphysis and diaphysis
G-CSF
Marrow Replacement or Infiltration [Figure 6-10-9]
Implantation of cells in marrow that do not normally exist there

Follows red marrow distribution
Causes
Neoplastic (lymphoma, leukemia,
mets)
Inflammatory (osteomyelitis)
Myeloproliferative (fibrosis)
Lipidoses
Marrow reconversion. Histologic

section showing predominance of
red cell elements
Figure 6-10-8
Marrow Replacement
T1 WT: dark
STIR/Fat sat: very bright
Can be diffuse or focal
Predominates in red marrow (axial
skeleton)
Diffuse Replacement Disorders:

Leukemia [Figure 6-10-10]
Marrow reconversion. Two different patients. Coronal T1weighted image of the knees shows diffuse low signal
intensity red marrow in the distal femoral and proximal tibial
metaphyses. Scattered high signal intensity foci represent
islands of yellow marrow and/or infarcts
Figure 6-10-10
Figure 6-10-9
Marrow replacement. The

normal hematopoietic
elements have been
replaced by neoplastic cells,
characteristic of chronic
lymphocytic leukemia
Pediatric Radiology
Marrow replacement by leukemia. Coronal T1-weighted image

(left panel) shows diffusely low signal intensity marrow
throughout the pelvis and femora. Fat-saturated T2weighted image (right panel). Replaced marrow has a
signal intensity much brighter than that of muscle
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Bone Marrow Imaging
Diffuse Replacement Disorders: Metastatic Disease

Focal Replacement: Metastases
Metastases prefer red marrow

Preferred sites:
Vertebrae - 69%
Pelvis - 41%
Femur - 25%
Skull - 14%
Vertebral Metastases: Breast Cancer

Review: Hyperplasia or Tumor?
Distinguishing criteria:
Red marrow: orderly distribution
Tumor: random distribution
Red marrow: usually symmetric bilaterally
Tumor: usually asymmetric
Red marrow: minimally bright on STIR/FS
Tumor: extremely bright
Hyperplasia or Tumor? Fat Suppressed Images
Normal red marrow

SI: = or slightly > muscle
Tumor infiltration
SI: >>>>> muscle
Figure 6-10-11
Pitfall: Compression Fractures
Acute compression due to osteopenia?

Malignant compression fracture?
Malignant Compression Fracture
Abnormal signal intensity

May involve pedicle, posterior element or entire vertebral body
Convex posterior cortex
Epidural mass
Paraspinal soft tissue mass
Marrow enhancement post gadolinium
Pathologic Fracture
Osteoporotic Fracture
Partial body involvement

Usually involves end plate
Signal of spared marrow is normal
Thoracolumbar junction
Clustering of abnormalities
Thin paraspinal soft tissue mass
Improves in 6 to 8 weeks
Compression fractures
Pathologic and Osteoporotic Fractures [Figure 6-10-11]
Variations in Appearance of Metastases
Blastic and fibrotic lesions

Low signal on T1 and fat -suppressed images
Often heterogeneous
Vertebral fractures. Pathologic

fracture (left panel). Gadoliniumenhanced T1-weighted MR
shows abnormal signal intensity
in entire vertebral body, convex
posterior cortex, and small
epidural mass. Osteoporotic
fracture (right panel). T1weighted MR shows partial body
involvement (upper end plates),
normal marrow signal, and
clustering of abnormalities at
thoracolumbar junction
Other Infiltrative Processes with Low T1 and High T2 Signal
Osteomyelitis
Myeloproliferative disorders
Lipidoses
Bone Marrow Imaging
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Pediatric Radiology
Osteomyelitis [Figure 6-10-12]
Infection produces increased cellularity and water content in

marrow (edema)
T1: dark signal
FS: bright signal
Adjacent ST changes
edema
Figure 6-10-12
Vogler JB III. Radiology 1998; 168:679-693
Osteomyelitis [Figure 6-10-13]
T1: DARKER >> muscle. Water, cells replace marrow

FST2: BRIGHTER >>muscle
Figure 6-10-13
Osteomyelitis. Inflammatory cells (I)

have replaced normal marrow
elements. T=supporting
trabeculae
Figure 6-10-14
Osteomyelitis. Coronal T1-weighted image (left
panel) showing low signal intensity area in
distal femoral metaphysis. Fat-saturated T2weighted image (right panel) showing
increased signal intensity
Osteomyelitis: MR
Myelofibrosis [Figure 6-10-14]
Myelofibrosis. Marrow space contains

abundant fibrotic tissue and
sparse red cells
Marrow replacement by fibrosis

Sequela of treated malignancy
Rarer as primary disorder
Results in lowering of signal intensity
Figure 6-10-15
Myelofibrosis [Figure 6-10-15]

Mimics of Fibrosis
Gaucher disease
Marrow replacement by glucocerebroside-laden
cells
Hemosiderin deposition
Marrow replacement by iron
Usually from transfusion therapy
Decreased T1 and T2-weighted signal
Slightly increased signal on STIR
Gaucher Disease
Due to deficiency of the enzyme acid betaglucocerebrosidase, which helps to break down
glucosyl ceramide
Follows distribution of red marrow
Proximal to distal
Pediatric Radiology
Primary myelofibrosis. Coronal T1-weighted (left

panel) and T2-weighted (right panel) MR
images show low signal intensity marrow in
the ilia, femora and vertebral bodies
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Bone Marrow Imaging
Gaucher Disease [Figure 6-10-16]
Figure 6-10-16
Hemosiderin [Figure 6-10-17]
Low SI on all sequences (black)
Marrow Depletion
Fatty Replacement [Figure 6-10-18]
Absence of red marrow

Causes
chemotherapy
radiation therapy
aplastic anemia
Marrow has signal of fat
Radiation & Chemotherapy
Gaucher disease. Decreased signal intensity on T1-weighted

Typical changes
images (left panel) and on STIR (right panel) images
1-2 days: edema
7-14 days: fatty replacement
3-4 wks: regenerating red marrow
May see irreversible changes (fat or fibrosis) with high doses
Figure 6-10-17
Myeloid Depletion: MRI
Signal follows fat

high T1
dark with fat sat
Usually see sharp cut off lines at
radiation port
Andrews. Radiographics 2000
Myeloid Depletion [Figure 6-10-19]
Follows signal intensity of fat
Figure 6-10-19
Hemosiderin deposition. Patient with sickle cell anemia who

received transfusional therapy. T1-weighted (left panel)
and T2-weighted (right panel) MR images showing diffusely
low marrow signal intensity. Scattered high foci area
represent residual fat and/or edema due to infarcts
Figure 6-10-18
Myeloid depletion following radiation treatment. Sagittal

T1-weighted image shows diffuse high signal
intensity fatty marrow in multiple vertebral bodies,
which were included in the radiation port
Bone Marrow Imaging
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1432
Marrow depletion. Marrow elements

have been replaced by fatty
tissue
Pediatric Radiology
Myeloid Depletion
Figure 6-10-20
Pre-treatment
Post-treatment
Vascular Mediated Disorders:

Edema
Results in increased extracellular water

Signal changes are those of water
T1: low signal
STIR/FS: bright signal
Regionally limited
Vascular Mediated Disorders:

Ischemia (infarct)
Marrow edema. Multiple causes. Bone bruise, left panel.

Fracture, middle panel. Transient osteoporosis, right panel
Result of death of yellow & red marrow elements

Signal changes represent balance of cell death and repair
Regionally limited
Subarticular
Likes yellow marrow
Ischemia & Edema: Causes

Not all individually detailed in this lecture
Ischemia
Steroids
Sickle cell
SLE
Gaucher
Pancreatitis
Radiation
Edema
Trauma
Stress facture
Transient osteoporosis
Reflex sympathetic dystrophy
CLUE: These favor yellow marrow
Marrow Edema (STIR): Common Causes [Figure 6-10-20]

Marrow Ischemia: Avascular Necrosis [Figure 6-10-21]
Bone Marrow Disorders
Hyperplasia
T1
STIR/FS
Dark
Intermediate
Replacement Dark
Bright
Fibrosis
Dark
Dark
Depletion
Bright
Dark
Vascular
Mediated
Dark
Bright
Figure 6-10-21
Avascular necrosis.T1-weighted (left panel) and

fat-saturated T2-weighted (right panel) MR
images showing low signal intensity
epiphyseal lesion. Ischemic injuries favor
yellow marrow
Pediatric Radiology
1431
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Bone Marrow Imaging
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
24.
25.
26.
Siegel MJ. MR imaging of paediatric haematologic bone marrow disease. J Hong Kong Coll Radiol 2000; 3:3850.
Steiner RM, Mitchell DG, Rao VM, Schweitzer ME. Magnetic resonance imaging of diffuse bone marrow disease.
Radiol Clin North Am 1993; 31:383-409.
Vande Berg BC, Malghem J, Lecouvet FE, Maldague B. Magnetic resonance imaging of the normal bone marrow.
Skeletal Radiol 1998;27:471-483
Vanel D, Dromain C, Tordivon A. MRI of bone marrow disorders. Eur Radiol 2000; 10:224-229.
Vogler JB III, Murphy WA. Bone marrow imaging. Radiology 1998; 168:679-693.
Eustace S, Tello R, DeCarvalho V, et al. A comparison of whole body turboSTIR MR imaging and planar 99mTcmethylene diphosphonate scintigraphy in the examination of patients with suspected skeletal metastases. AJR
1997; 169:1655-1661.
Lauenstein TC, Goedhe SC, Herborn CU, et al. Three-dimensional volumetric interpolated breath-hold MR
imaging for whole-body tumor staging in less than 15 minutes: a feasibility study. AJR 2002 Aug;179(2):445-9.
Lauenstein TC, Goedhe SC, Herborn CU, et al. Whole-body MR imaging: evaluation of patients for metastases.
Radiology 2004; 233:139-148.
Mazumdar A, Siegel M, Narra V, Luchtman-Jones L. Whole-body fast inversion recovery MR imaging of small
cell neoplasms in pediatric patients: a pilot study. AJR 2002; 179:1261-1266.
OConnell MJ, Hargaden G, Powell T, Eustace SJ. Whole-body short tau inversion recover MR imaging with a
moving table top. AJR 2002; 179: 866-868.
Padhani A, Husband J. Bone. In: Husband JES, Reznek RH, eds. Imaging in Oncology. Isis Medical Media.
Oxford 1998; pgs 765-786.
Kricun ME. Red-yellow marrow conversion: its effect on the location of some solitary bone lesions. Skeletal
Radiol 1985; 14:10-19.
Custer RP, Ahlfeldt FE. Studies of the structure and function of bone marrow: variations in cellularity in various
bones with advancing years of life and their relative response to stimuli. J Lab Clin Med 1932; 17:960-962.
Moore DG, Dawson KL. Red and yellow marrow in the femur: age-related changes in the appearance at MR
imaging. Radiology 1990; 175:219-223.
Ricci C, Cova M, Kang YS et al. Normal age-related patterns of cellular and fatty bone marrow distribution in the
axial skeleton: MR imaging study. Radiology 1990; 177:83-88.
Taccone A, Oddone M, Dell Acqua A, Occhi M, Ciccone MA. MRI road-map of normal age-related bone
marrow. Pediatr Radiol 1995; 25:596-606.
Mirowitz SA. Hematopoietic bone marrow within the proximal humeral epiphysis in normal adults: investigation
with MR imaging. Radiology 1993; 188:689-93.
Fletcher BD, Wall JE, Hanna SL. Effect of hematopoietic growth factors on MR images of bone marrow in
children undergoing chemotherapy. Radiology 1993; 189:745-751.
Ryan SP, Weinberger E, White KS, et al. MR imaging of bone marrow in children with osteosarcoma: effect of
granulocyte colony-stimulating factor. AJR 1995; 165:915-920.
Shellock FG, Morris E, Deutsch AL, et al. Hematopoietic bone marrow hyperplasia: high prevalence on MR
images of the knee in asymptomatic marathon runners. AJR 1992; 335-338.
Baker LL, et al. Benign versus pathologic compression fractures of vertebral bodies: assessment with
conventional spin-echo, chemical shift, and STIR MR imaging. Radiology 1990; 174:495
Blomlie V, Rofstad EK, Skjonsberg A, Tver, Lien HH. Female pelvic bone marrow: serial MR imaging before,
during, and after radiation therapy. Radiology 1995; 194:537-543.
Otake S, Mayr N, Ueda T et al. Radiation-induced changes in the MR signal and contrast enhancement of
lumbosacral vertebrae: do changes occur only inside the radiation therapy field? Radiology 2002; 222:179-183.
Stevens SK, Moore SG, Kaplan I. Early and late radiation changes in the spine: magnetic resonance imaging. AJR
1990; 154:745-750.
Hermann G, Shapiro RS, Abdelwahab IF, Grabowksi G. MR imaging in adults with Gaucher disease type I:
evaluation of marrow involvement and disease activity. Skeletal Radiol 1993; 22:247-251.
Levin TL, Sheth SS, Hurlet A, Comerci SC, Ruzal-Shapiro, Piomelli S, Berdon WE. MR marrow signs of iron
overload in transfusion-dependent patients with sickle cell disease. Pediatr Radiol 1995; 25:614-619.
Bone Marrow Imaging
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Pediatric Radiology
Congenital Lung Malformations

Learning Objectives
Review clinical & pathologic features of common lung anomalies

Describe imaging appearances of the common congenital anomalies
Emphasize imaging differential diagnosis
Congenital Lung Anomalies
Normal vascularity
Lobar emphysema
Cystic adenomatoid malformation
Bronchogenic cyst
Parenchymal agenesis, hypoplasia
Abnormal vascularity
Scimitar syndrome
Sequestration
Arteriovenous malformation
Figure 6-11-1
Congenital Lobar Emphysema
Misnomer; true emphysema not present

Congenital lobar emphysema. Gross section showing an
Infantile lobar emphysema =
overinflated lobe that fails to deflate post resection. Histology
overinflation
showing alveolar overdistention
Progressive lobar air-trapping from
bronchial obstruction;
deficiency of bronchial cartilage
intraluminal web
Onset: 50% 1st week, 90% < 6 months
Neonates--dyspnea, cyanosis, cough
Later-asymptomatic, wheezing
Congenital Lobar Emphysema: Pathologic Features [Figure 6-11-1]
Sponge-like mass, fails to deflate on resection

Compressed normal lung deflates & reexpands
Histo- Alveolar distention 5-10X normal
Figure 6-11-2
Lobar Emphysema: Imaging
Lobar hyperinflation
Atelectatic adjacent lung
Initially opaque if retained lung fluid
Mass effect: mediastinal shift,
attenuated vessels
Lobar predilection:
LUL (50%), RML (24%), RUL (18%)
Lobar Emphysema [Figure 6-11-2]
Neonate with respiratory distress
Companion Case
2-week-old boy with mild dyspnea
Congenital Lobar Emphysema
Congenital lobar emphysema. Chest radiograph showing large

hyperlucent left upper lobe with atelectasis of adjacent lung.
CT showing hyperinflated left upper lobe with attenuated
vascularity
Usually diagnosed in neonates & infants

20% diagnosed in adolescents & adults
Pediatric Radiology
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Lobar Emphysema
Figure 6-11-3
DDX: Swyer-James Syndrome
viral infection in childhood **
Imaging findings
unilateral hyperlucent (low
attenuation) lung
small or normal size **
bronchiectasis **
air-trapping on expiration
**Helps to differentiate from CLE
10-year-old boy, cough
Swyer-James syndrome. Inspiration CT (left panel) and

expiration CT (right panel) showing a low attenuation right lung
with air trapping on expiration. Also noted is bronchiectasis
Swyer-James Syndrome [Figure 6-11-3]

Cystic Adenomatoid Malformation
25% of congenital lung lesions

Result of abnormal proliferation of bronchioles
Normal arterial supply & venous drainage
Communicates with bronchial tree
Figure 6-11-4
CCAM: Clinical
70%-90% symptomatic as neonates

Cyanosis, grunting, tachypnea
Rare in older children & adults
Presents as pneumonia or recurrent infection
May be antenatal diagnosis
CCAM: Histologic Types
Stocker classification
Type I: (50%) Large cyst(s) (> 2 cm)
Type II: (40%) Multiple cysts (< 2 cm)
Type III: (10%) Microcysts on cut-section
Stocker JT. Hum Pathol 1977; 8:155-171
CCAM: Imaging
Air-filled mass, mediastinal shift

Except may be opaque initially if fluid-filled
Type I
Multicystic lesion, with dominant cyst(s)
Air fluid levels
Type II
Heterogeneous, uniform small cysts
Type III
Large, homogeneous, solid
Resembles consolidation
Type I CCAM: Large Cysts [Figure 6-11-4]
Neonate with dyspnea
Cystic adenomatoid malformation,

Type I. Chest radiograph and CT
showing large cystic lesion in the right
middle lobe. Gross pathologic
section also shows a large cystic
mass
1434
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Pediatric Radiology
Type II CCAM: Small Cysts [Figure 6-11-5]
Figure 6-11-5
CCAM [Figure 6-11-6]

Figure 6-11-6
Cystic adenomatoid malformation, Type II. Chest

radiograph showing a lucent area in the left lower
lobe. Two CT scans and gross pathologic section
showing a cystic mass with multiple small cysts
Cystic adenomatoid malformation. Chest

radiograph on day 1 showing an opaque right
upper lobe mass. Follow-up radiographs on days
3 and 7 show a more cystic appearing mass. CT
showing a multicystic mass in the right upper lobe
CCAM Antenatal US Diagnosis
Polyhydramnios
Fetal hydrops
Ascites
Anasarca
Placental edema
Solid or cystic lung mass
High perinatal mortality
Spectrum of CCAM
10% diagnosed in adolescents & adults

Infection common
May mimic infiltrate or mass on imaging studies
Infected CCAM
Adolescent with cough
Figure 6-11-7
Infected CCAM [Figure 6-11-7]

Bronchogenic Cyst
Failure of lung bud to incorporate into

primitive lung
Lung (30%), mediastinum (70%)
Asymptomatic & incidental finding
Symptomatic from mass effect
Infected cystic adenomatoid malformation. Chest radiograph

showing an air-space opacity with a cystic component
superiorly. Transverse CT showing multiple cysts with thick
walls and some air-fluid levels
Pediatric Radiology
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Bronchogenic Cyst: Path [Figure 6-11-8]
Gross
Separate from lung
Round or ovoid
Clear or turbid contents
Histo
Lined by respiratory epithelium
Bronchial glands, cartilage, smooth muscle in wall
Figure 6-11-8
Pulmonary Bronchogenic Cyst: Imaging
Smooth, rounded, unilocular mass

Fluid-filled, usually serous fluid
Sometimes protein or mucin
Air or air-fluid levels if infected
No mediastinal shift
Non-enhancing
Bronchogenic Cyst
7-day old girl, wheezing
Bronchogenic cyst, pathology. Gross

specimen showing a fluid-filled lung
mass. Histologic section showing
respiratory epithelium lining the cyst
wall
Bronchogenic Cyst [Figure 6-11-9]
7- year-old girl with cough
Figure 6-11-9
Figure 6-11-10
Bronchogenic cyst. Transverse and coronal CT

scans showing a well defined, smoothly
marginated mass containing only air in the right
upper lobe
Bronchogenic cyst. Chest radiograph showing a
right paratracheal mass. Transverse CT scans
showing a water attenuation mass with
imperceptible margins in the right paratracheal
area
DDX: Cystic Adenomatoid Malformation
Septations and multiple cysts favor CAM
Review
Lobar emphysema
CCAM
Bronchogenic cyst
Figure 6-11-11
Mediastinal Bronchogenic Cysts
Same characteristics as pulmonary cysts

Round, solitary, air or fluid filled
Mediastinal Bronchogenic Cyst

[Figure 6-11-10]
Bronchogenic Cyst: MRI

Enteric cyst. Transverse CT (left panel) showing a
water attenuation mass in the lower mediastinum.
Gross section (middle image) showing a cystic
mass. Histologic section (right panel) showing
gastrointestinal lining
Differential Diagnosis: Enteric Cysts

[Figure 6-11-11]
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Pediatric Radiology
Enteric Foregut Cyst
Figure 6-11-12
Pulmonary Underdevelopment
Agenesis: Complete absence of lung tissue, artery, & small or

absent bronchus
Hypoplasia: Small lung & bronchus (artery may or may not
develop)
Lung Agenesis [Figure 6-11-12]
Small bronchus
No lung or PA
Companion Case: Lung Agenesis

Arrested Pulmonary Development
Pulmonary hypoplasia
Small lung (hypoplasia)
Small bronchus
Absent or small pulmonary artery
Mediastinal shift to side of hypoplasia
Pulmonary Hypoplasia [Figure 6-11-13]
2-month-old boy, mild dyspnea
Figure 6-11-13
Lung agenesis. Chest radiograph in

a neonate showing an opacified
hemithorax. CT scan showing
absence of the right lung and
pulmonary artery and a small
rudimentary bronchus (arrow)
Pulmonary hypoplasia. Frontal chest radiograph
in a 2 month old boy showing a small right lung
and mediastinal shift to the right. Lateral
radiograph showing a posterior sternal stripe,
representing fibrofatty tissue (arrow)
Figure 6-11-14
Pulmonary Hypoplasia [Figure 6-11-14]

Pulmonary Hypoplasia
Small lung & bronchus No PA
Congenital Anomalies with Abnormal

Vasculature
Hypoplasia with anomalous venous return

Scimitar syndrome
Pulmonary sequestration
Arteriovenous malformation
Hypogenetic Lung Syndrome
Lung hypoplasia with PAPVR

PAPVR into IVC, portal vein/ hepatic vein, or right
atrium
Often asymptomatic and discovered
during evaluation of another anomaly
Pediatric Radiology
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1439
Pulmonary hypoplasia. Transverse chest CT

scans showing a small right lung, main right
bronchus, absent right pulmonary artery and
mediastinal shift to the right
Hypogenetic Lung Syndrome: Imaging Features
Usually right lung

Anomalous pulmonary venous return
Lung hypoplasia
Hypoplasia of Rt pulmonary artery
Dextrocardia
May have abnormal systemic arterial supply to lung
Figure 6-11-15
Hypogenetic Lung Syndrome [Figure 6-11-15]

Pulmonary Sequestration
No normal connection with bronchial tree or

pulmonary arteries
Systemic blood supply
2 types
Intralobar (acquired)
Extralobar (congenital)
ELS
Own pleura
Neonate
90% left
Solid
Syst. arterial supply
Syst.venous drainage
Assoc. anomalies
ILS
Shared pleura
Children & adults
90% left
Cystic or solid
Syst. arterial supply
Pulm. venous drainage
+/- assoc. anomalies
Hypogenetic lung syndrome. Transverse CT scan

showing a small right lung and mediastinal shift
towards the right. Also noted a left aortic arch with
anomalous right subclavian artery. Coronal
multiplanar (middle panel) and 3D volume
rendered image (viewed posteriorly) showing an
anomalous vein (arrow) draining the right lower
lobe
Figure 6-11-16
Sequestration: Vascular Findings
ILS
Arterial supply
Thoracic aorta (70%)
Other-abdominal aorta, intercostal
Venous drainage
Pulmonary (95%), systemic (5%)
ELS
Arterial supply
Abdominal aorta (80%)
Otherthoracic aorta, pulmonary artery
Venous drainage
Systemic (80%), pulmonary (20%)
Intralobar sequestration,
pathology. Cut section shows
abnormal inflamed parenchyma
Intralobar
Extralobar
Figure 6-11-17
Intralobar Sequestration: Path [Figure 6-11-16]
Gross
Edge may abruptly abut normal lung or blend
diffusely
Microscopic
Bronchopneumonia
Extralobar Sequestration: Path [Figure 6-11-17]
Separate from normal lung

Pyramidal, rounded, ovoid
Extralobar sequestration. Gross specimen
Resembles normal lung
resembles
normal lung Histologic section shows
Microscopic
dilated
bronchioles,
alveolar ducts, and alveoli.
Dilated bronchioles, alveoli & subpleural
Also
noted
is
a
well-formed
bronchus near one
lymphatics
edge
of
the
lesion
Well-formed bronchus near edge of lesion (50%)
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Pediatric Radiology
Sequestration: Clinical
Figure 6-11-18
ELS
Often asymptomatic
Dyspnea, cyanosis occasionally (10%)
CHD, diaphragmatic hernia, CCAM
ILS
Symptomatic
Cough, recurrent pneumonia
Sequestration: US Features
Gray-scale
Echogenic mass
Supradiaphragmatic-likely ILS
Subdiaphragmatic (suprarenal)-likely ELS
Doppler US
Feeding artery off aorta
Draining vein usually not identified
Intralobar sequestration. Chest radiograph

showing a left lobe air space opacity. Sonogram
showing a supradiaphragmatic echogenic mass
with a feeding artery in the left lower lobe
Figure 6-11-20
Sequestration: CT Features
Anomalous feeding artery

Draining vein to pulmonary or systemic veins
Parenchymal findings
ILS:
Infiltrate
Abscess
Focal emphysema
ELS
Triangular/round solid mass
Intralobar Sequestration: Infiltrate [Figure 6-11-18]

Intralobar Sequestration: Infiltrate & Emphysema
[Figure 6-11-19]
Figure 6-11-19
Intralobar sequestration. Transverse

CT showing a cystic mass with an
enhancing wall in the left lower lobe,
representing an abscess. 3D
reconstruction (viewed posteriorly)
showing an anomalous artery (arrow)
from the distal thoracic aorta
supplying the sequestered lung
Intralobar sequestration. Transverse CT at lung

windows (left panel) showing a left lower lobe
infiltrate with surrounding emphysema.
Transverse contrast enhanced CT (right panel)
showing an opacified anomalous artery (arrow)
from the distal thoracic aorta supplying the
sequestered lung
Intralobar Sequestration: Drainage via PV to Left Atrium

Intralobar Sequestration Abscess Formation
6-year-old girl, fever and cough
Intralobar sequestration: Abscess Formation [Figure 6-11-20]
Pediatric Radiology
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Extralobar Sequestration: Triangular Mass

[Figure 6-11-21]
Figure 6-11-21
Extralobar Sequestration:
Round Lower Lobe Mass [Figure 6-11-22]
Extralobar Sequestration:
Systemic Drainage
Solid, well-defined LLL mass
Extralobar sequestration. Chest radiograph

showing a triangular mass (arrow) at the left lung
base. Transverse sonogram showing an
heterogeneous mass (arrows) which was below
the left hemidiaphragm
Sequestration: MRI
ELS with CCAM (40%)
Summary
ILS
ELS
Figure 6-11-22
Arteriovenous Malformation
80% Hereditary telangiectasia (OWR)

15% sporadic
5% cardiac surgeries (Glenn or Fontan)
Symptomatic in older patients (cyanosis,
polycythemia, dyspnea)
80%-90% are simple AVMs
single feeding and draining vessel
commonly lower lobes
Extralobar sequestration. Coronal CT reformation
(left panel) and 3D reconstruction (right panel)
showing two anomalous feeding arteries entering
a solid left lower lobe sequestration
Arteriovenous Malformation
Pulmonary AVM
Simple architecture
Simple Pulmonary AVM

Figure 6-11-23
Multiple AVMs [Figure 6-11-23]

Congenital Lung Anomalies
ABNORMAL LUNG - NORMAL VASCULATURE

NORMAL LUNG - ABNORMAL VASCULATURE
Multiple pulmonary arteriovenous malformations.

Two transverse CT scans and 3D reconstructions
showing multiple pulmonary arteriovenous
malformations (arrows)
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Pediatric Radiology
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Siegel MJ. Lung, pleural and chest wall. In: Pediatric Body CT. Philadelphia, Lippincott Williams & Wilkins.
1999;101-.140
Kim WS, Lee KS, Kim IO, et al. Congenital cystic adenomatoid malformation of the lung: CT-pathologic
correlation. AJR 1997; 168:47Rosado-de-Christenson JL, Stocker JT. Congenital cystic adenomatoid malformation. J Comput Assist Tomogr
1989; 13:612-616
Shackelford GD, Siegel MJ. CT appearance of cystic adenomatoid malformation. J Comput Assist Tomogr 1989;
13:612-616.
Felker RE, Tonkin IL. Imaging of pulmonary sequestration. AJR 1990; 154:241-249
Frazier AA, Rosado de Christenson ML, Stocker JT et al. Intralobar sequestration: radiologic-pathologic
correlation. RadioGraphics 1997; 17:725-745.
Ko SF, Ng SH, Lee TY, et al. Noninvasive imaging of bronchopulmonary sequestration. AJR 2000; 175:10051012
Lee E, Siegel MJ, Sierra LM, Foglia RP. Evaluation of angioarchitecture of pulmonary sequestration in pediatric
patients using multidetector CT angiography. AJR 2004; 183:183-188.
Rosado-de-Christenson ML, Frazier AA, Stocker JT, Templeton PA. Extralobar sequestration: radiologicpathologic correlation. From the archives of the AFIP. RadioGraphics 1993; 13:425-441.
Konen E, Raviv-Zilka L, Cohen RA, et al. Congenital pulmonary venolobar syndrome: Spectrum of helical CT
findings with emphasis on computerized reformatting. RadioGraphics 2003; 23:1175-1184
Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. RadioGraphics 1994;
14:349-369.
Remy J, Remy-Jardin M, Giraud F, et al. Angioarchitecture of pulmonary arteriovenous malformations: clinical
utility of three-dimensional helical CT. Radiology 1994; 191:657-664.
Hoffman LV, Kuszyk BS, Mitchell SE, et al. Angioarchitecture of pulmonary arteriovenous malformation:
characterization using volume-rendered 3D CT angiography. Cardiovasc Intervent Radiol 2000; 23: 165-170.
Rotondo A, Scialpi M, Scapati C. Pulmonary arteriovenous malformation: evaluation by MR angiography. AJR
1997; 168: 847-849.
Pediatric Radiology
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Lung Diseases in Neonates

Neonatal Respiratory Distress: Spectrum of Disorders
[Figure 6-12-1]
Surgical Disease
Medical Disease
Figure 6-12-1
Objectives
Discuss lung diseases in preterm and term neonates

Review treatment complications
Understand important differential findings
Neonatal Medical Lung Diseases
WHAT YOU WILL SEE:

Respiratory distress syndrome
Retained lung fluid
Meconium aspiration
Neonatal pneumonia
WHAT YOU MAY SEE:
Chylothorax
Congenital surfactant protein B deficiency
Medical Lung Diseases:

Premature Neonate
Neonatal respiratory distress, spectrum of causes
Premature Births
< 37 weeks
~500,000 preterm deliveries/year
12% of all live births
Lung disease most common problem
CDC National Vital Statistics Reports, Vol. 52, No. 10, Dec 17, 2003
Respiratory Distress Syndrome (RDS)
Same as hyaline membrane disease (HMD) & surfactant deficiency disease

1% of pregnancies, typically prematures
26-34 weeks
Post-term infants of diabetic mothers
More frequent and severe in males
More common in whites than blacks
RDS/HMD: Pathogenesis
Figure 6-12-2
Surfactant deficiency
Leads to alveolar collapse
Increased capillary permeability
Hyaline membrane formation
What are Hyaline Membranes? [Figure 6-12-2]
Line terminal & respiratory bronchioles

Contain
Necrotic alveolar cells
Plasma transudate
Aspirated squames
Fibrin
Hyaline membranes. Eosinophilic

hyaline membranes surrounded by
collapsed air spaces
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Pediatric Radiology
RDS/HMD: Pathogenesis
Mature airspace
Surfactant deficiency
Bronchial collapse & atelectasis
RDS/HMD: Gross Pathology
Firm atelectatic lungs (hepatization)

Alveolar edema
+/- focal hemorrhage
RDS/HMD: Imaging Findings
Small lung volumes

Diffuse bilateral granular opacities
Air bronchograms
Loss of vascular definition
No pleural effusion
Figure 6-12-3
RDS/HMD [Figure 6-12-3]

RDS/HMD: Classic Clinical Approach
Confirm suspected diagnosis with radiographs

Positive pressure ventilator therapy
Increases risk of air leak
Increases risk of BPD
Respiratory distress syndrome, untreated. Low
lung volumes with diffuse bilateral granular
opacities
RDS/HMS: Other Treatment Options
Maternal (in utero) steroid administration

Exogenous surfactant
Exogenous Surfactant Therapy: Clinical Impact
Slow liquid bolus into airway

Desired effect is decreased:
Oxygen requirement
Mortality from RDS
Air leak
Surfactant Therapy:Imaging Findings
Figure 6-12-4
Uniform improvement in granularity at 24-48 hrs (38%)

Asymmetric improvement (35%)
Small cystic lucencies (17%)
No improvement (10%)
Focal hemorrhage or hemorrhagic pulmonary edema
(1%-2%)
Pediatr Radiol 1997; 27:26-31
Surfactant Therapy [Figure 6-12-4]

Complications of Treatment
Related to respirator
Air leaks
Bronchopulmonary dysplasia
Chest radiograph (left image) showing RDS,

pretreatment. Chest radiograph (right image)
showing RDS, 24 hours after surfactant therapy
Air Leak: Pathogenesis
Results from positive pressure ventilation of noncompliant lungs

Rupture at bronchiolar-alveolar junction
Gas dissects into interlobular septa and septal lymphatics (interstitial air)
May enter pleural space, mediastinum, pericardium, peritoneum, venous
system
Pediatric Radiology
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Pulmonary Interstitial Emphysema [Figure 6-12-5]
Figure 6-12-5
Acute Pulmonary Interstitial Emphysema:

Imaging Findings
Tubular & cystic lucencies

Focal or diffuse
Unilateral or bilateral
Pulmonary overexpansion
Mediastinal shift (unilateral PIE)
Small cardiac silhouette (diffuse bilateral PIE)
Pulmonary Interstitial Emphysema [Figure 6-12-6]

RDS/Pulmonary Interstitial Emphysema
Persistent Interstitial Pulmonary Emphysema [Figure 6-12-7]
PIE lasting > 1 week

Associated with pseudocysts
Aggregate thick-walled cysts
Right parahilar location
May resolve completely or may need resection
Mimic solid mass when fluid-filled
Air leak, pulmonary interstitial

emphysema. Gas in
interlobular septa
Williams, et al. AJR 1988; 150:885-887
Persistent PIE: CT Findings
Figure 6-12-6
17 preterm neonates
Hyperexpanded complex cystic
masses developed from typical
PIE (mean, 13 days)
53% underwent resection
Donnelly LF, et al. AJR 2003;

180:1129
Persistent PIE with Air Leak

Pseudocysts [Figure 6-12-8]
Figure 6-12-8
Figure 6-12-7
Acute pulmonary interstitial

emphysema. Chest radiograph
showing hyperinflated lungs with
cystic lucencies bilaterally.
Pathologic section showing interstitial
air
Persistent pulmonary interstitial

emphysema. Chest radiograph
showing cysts in right parahilar
region. CT showing complex
multicystic mass in the right
lower lobe
Persistent interstitial pulmonary

emphysema. Gross specimen.
Mass-like aggregate of thickwalled cysts
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Pediatric Radiology
Air Leak Pseudocysts
Figure 6-12-9
Air Leak: Pneumothorax [Figure 6-12-9]
May be subtle in infants

Collects anteriorly in supine patients
Look for deep sulcus sign and asymmetric lucent
lung
Mediastinal shift with tension pneumothorax
Air Leak: Pneumomediastinum
Most are clinically insignificant

Usually does not dissect into neck because of
persistence of sternopericardial ligament
Thymus elevation (spinnaker sail sign)
Gas beneath heart
Continuous diaphragm sign
Left panel. Two different patients. Chest

radiographs in both patients show a tension
pneumothorax with mediastinal shift and
hyperexpansion of the affected hemithorax. Left
image, also note underlying changes of hyaline
membrane disease. Right image, also note
underlying pulmonary interstitial emphysema
Pneumomediastinum [Figure 6-12-10]

Air Leak: Pneumopericardium [Figure 6-12-11]
Gas delimited superiorly by pericardial reflection about great vessels

No thymic displacement
May require needle compression of tamponade
Figure 6-12-10
Pneumopericardium
Air Leak: Pneumopericardium & PTX
Air Leak: Systemic Gas Embolism
Pathogenesis uncertain
Alveolar-venous fistula?
Lymphatic gas entering right heart?
Virtually 100% fatal
Pneumomediastinum. Elevation of thymic lobes

on frontal radiograph (spinnaker sail sign).
Substernal air seen on lateral radiograph
Bronchopulmonary Dysplasia: Clinical

Diagnosis
> 3 days positive pressure ventilation + 02 during 1st 2 weeks of life

Respiratory distress lasting > 28 days
Require supplemental 02 > 28 days to maintain Pa02 > 50 mm Hg
Characteristic radiologic findings
Figure 6-12-11
Bronchopulmonary Dysplasia: Risk Factors
Stress factors
Barotrauma
Oxygen cytotoxicity (free radicles)
Infection
Pulmonary edema (related to PDA)
Host Factors
Genetics (family history of atopy & asthma)
Endogenous low steroids
Bronchopulmonary Dysplasia:
Basic Pathologic Features
Pneumopericardium. Air extends to

level of great vessels. Also noted is
underlying pulmonary interstitial
emphysema
Tracheal & bronchial injury

Mucosal ulceration & necrosis
Pulmonary arterial injury
Intimal & adventitial thickening
Hypertensive vascular disease
Bronchiolar & alveolar injury
Cell necrosis & septal edema
Pediatric Radiology
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Figure 6-12-12
Reparative phase
Alveolar septal fibrosis
Stocker JT. Hum Pathol 1986; 17:943
Bronchopulmonary Dysplasia [Figure 6-12-12]

Classic Stages of BPD:Imaging Findings
I: 0-4 days granular opacities of RDS

II: 4-10 days increased opacities
III: 10-30 days Bubbly Lungs
IV: > 30 days disordered aeration
All 4 stages rarely observed
Variant in Stages of BPDPrecocious BPD
Bubbly lungs develop at end of 2nd postnatal week

< 1000 gram newborns
Lower respiratory tract infection
Crouse DT et al. Clin Infect Dis 1993; 17: S 122-130
Classic BPD: Bubbly Lungs [Figure 6-12-13]
Figure 6-12-13
Bronchopulmonary Dysplasia
HRCT
Emphysema
Cystic or bullous changes
Septal lines
No zonal predominance
Bronchopulmonary Dysplasia
[Figure 6-12-14]
BPD: Classic Temporal

Course
BPD: Chronic Course:
Disorganized Aeration
Bronchopulmonary dysplasia.
Hyperinflation and bubbly cystic
lungs
Figure 6-12-14
BPD: Prognosis
Bronchopulmonary
dysplasia. Gross path
specimen (left ipanel)
showing fibrotic lungs.
Histologic specimen
(right panel) showing
dilated air spaces and
septa thickened by a
combination of edema,
inflammatory cells and
fibroblasts
Pulmonary function slowly improves

Radiographs normalize in most by 3 years
PFTs remain abnormal for years
Refractory pulmonary hypertension = poor prognosis
& lung transplant
Terminology: Need to recognize
BPD is a chronic insult

Any insult that increases the need for mechanical
ventilation can lead to chronic injury
Mechanical ventilation increases risk of lung
damage!
Will have the same appearance as BPD
Bronchopulmonary dysplasia. CT showing

emphysematous changes, septal lines extending
to pleural surface and bullous formation
Medical Lung Diseases: Term Neonate

Other Neonatal Lung Diseases
Retained fetal lung fluid

Aspiration syndromes
Pneumonia (term & premature neonates)
Surfactant B protein deficiency
Chylothorax
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Pediatric Radiology
Retained Fetal Lung Fluid or Transient Tachypnea of Newborn
Associated with C-section, maternal sedation,

maternal diabetes
Lung fluid normally removed by capillary and
lymphatic resorption & retrograde tracheal flow
Lack of compression by birth canal (C-section) &
decreased maternal & neonatal beta-adrenergic
responsiveness may alter clearance
Figure 6-12-15
Retained Fetal Lung

Retained Fetal Lung Fluid: Radiographic
Findings
Retained fetal lung fluid. Frontal chest radiograph

showing reticular opacities and small right pleural
effusion. Lateral radiograph showing fissural fluid

Reticular opacities
Fissural fluid
Small pleural effusions
Clearing in 24 to 48 hours
Figure 6-12-16
Retained Fetal Lung Fluid

[Figures 6-12-15 and 6-12-16]
Meconium Aspiration Syndrome
In utero defecation due to fetal distress

Usually > 34 weeks gestation
10%-15% of pregnancies have meconium stained
amniotic fluid
5% of meconium stained neonates develop
meconium aspiration syndrome
30%-50% require mechanical ventilation
Meconium Aspiration Syndrome:

Pathologic Features
Retained fetal lung fluid. Frontal chest radiograph

day 1 (left panel) showing reticular opacities.
Chest radiograph day 2 (right panel) showing
interval clearing of interstitial fluid
Figure 6-12-17
Acellular debris
Squamous epithelial cells
Tenacious green-yellow meconium plugs in airways
Pneumonitis
Hemorrhagic edema
medic.med.uth.tmc.edu/
Meconium Aspiration: Pathophysiology
Primary
Mechanical Obstruction
Chemical inflammation
Surfactant inactivation
Secondary
Air trapping
Air leak
Atelectasis
Meconium aspiration. Hyperinflated

lungs and coarse interstitial opacities
Figure 6-12-18
Meconium Aspiration: Imaging [Figure 6-12-17]
Hyperinflation
Coarse patchy opacities
Air leak (25%-40%)
Pneumomediastinum
Pneumothorax
Pleural effusion (rare)
Meconium aspiration. Hyperinflated lungs with

large pneumothoraces and pneumomediastinum
Meconium Aspiration [Figure 6-12-18]
Pediatric Radiology
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Meconium Aspiration: Treatment
Antibiotics
Surfactant
Mechanical ventilation
Inhaled nitric oxide (iNO)
May require ECMO
94% overall survival
Figure 6-12-19
Neonatal Pneumonia
0.5% of term infants

Frequently associated with neonatal sepsis
Clinical and radiographic challenging diagnosis
Neonatal Pneumonia: Etiology
In utero (hematogenous) infection

CMV, syphilis, listeriosis
Ascending infection (PPROM)
Group B beta hemolytic strep
E. coli
During delivery
Strept
Chlamydia trachomatis
Postnatal infection
Bacterial
Neonatal pneumonia. Coarse interstitial opacities,

increased lung volumes
Figure 6-12-20
Neonatal Pneumonia: Path
Air spaces contain neutrophils & squamous epithelial cells without

fibrin
Air spaces surrounded by thin cellular septa
Neonatal Pneumonia: Radiologic Findings
Nonspecific, need to correlate clinically

Diffuse granularity (may mimic RDS)
Patchy and streaky opacities (may mimic TTN or meconium)
Pleural effusion (65%)
Lung volumes usually normal, but may be increased
Neonatal pneumonia. Patchy

confluent opacities. Normal lung
volumes
Neonatal Pneumonia [Figures 6-12-19 and 6-12-20]

Figure 6-12-21
Review & Quiz Time

TTN, Pneumonia or Meconium?
Concede that lung findings are nonspecific:
Look for the clues!! [Figure 6-12-21]
Meconium, pneumonia, TTN?
Hyperinflation?
Meconium, TTN, pneumonia
Air leak?
Meconium aspiration
Normal aeration?
Pneumonia
Patchy confluent opacities?
Pneumonia
Fissural fluid?
TTN
Clues to diagnosing neonatal lung disease
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Pediatric Radiology
OR Stated Another Way
Meconium aspiration
Reticular opacities, increased lung volumes, effusion rare (<10%)
Neonatal pneumonia
Reticular or confluent opacities, normal or increased aeration, effusions
common (65%)
TTN
Reticular opacities, increased lung volumes, pleural or fissural fluid (90%)
For Comparison
Respiratory distress syndrome

Decreased long volumes
Granular lung disease
NO effusions in uncomplicated disease!!
Medical Lung Diseases: Presenting in 1st week

Chylothorax
Rupture of thoracic duct

Usually due to birth trauma
Rarely congenital anomaly
Full term infants
70% symptomatic in 1st week
Right > left pleural effusions
Rarely bilateral
Chylothorax
Thoracentesis usually yields cloudy fluid because of the high lipid content of
chyle
Treatment includes thoracentesis, chest tube drainage, and feedings of
medium-chain triglycerides
Most lymphatic ruptures seal with combined chest tube and dietary treatment
Chylothorax
Differential diagnosis includes causes of nonchylous pleural effusions

Wet-lung disease
Hydrops fetalis
Turners syndrome
Pulmonary vein obstruction
Esophageal rupture
Chylothorax [Figure 6-12-22]

Alveolar Proteinosis
Due to congenital surfactant protein B deficiency

Autosomal recessive
PAS-positive abnormal surfactant lipids & proteins accumulate in alveoli &
macrophages
Diagnosis confirmed by alveolar lavage & peripheral blood DNA analysis
Treatment - lung transplantation
Figure 6-12-22
Pediatr Radiol 2001; 31: 327-331
Chylothorax. Day 1 (left image), large right pleural effusion.

Day 7 (middle image), decreased fluid following thoracenteses.
One month (right image), resolution, following thoracentesis
and feedings of medium chain triglycerides
Pediatric Radiology
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Surfactant B Deficiency: Imaging Features
Mimics RDS, but in term infant

Chest
Low lung volumes
Hazy lungs
CT
Ground-glass opacities & septal lines
Crazy paving
Surfactant B Deficiency [Figure 6-12-23]
Mimics HMD
Crazy paving
Neonatal Medical Diseases: The Big Four
Figure 6-12-23
Surfactant B deficiency. Chest radiograph

showing low volume, hazy lungs. CT showing
ground-glass opacities and thickened septal lines
[Figure 6-12-24]
Figure 6-12-24
The top 4 neonatal medical diseases
References
1.
2.
3.
4.
5.
6.
7.
Center for Disease Control and Prevention: National Center for Disease Statistics. National Vital Statistics
Reports, Vol. 52, No. 10, Dec 17, 2003. Web: www.cdc.gov/nchs
Dinger J, Schwarze R, Rupprecht E. Radiological changes after therapeutic use of surfactant in infants with
respiratory distress syndrome. Pediatr Radiol 1997; 27:26-31.
Donnelly LF, Lucaya J, Ozelame V, et al. CT findings and temporal course of persistent pulmonary interstitial
emphysema in neonates: a multiinstitutional study. AJR Am J Roentgenol 2003; 180:1129-1133.
Medic: medical education information. University of Texas - Houston, Department of Pathology and Laboratory
Medicine. http://medic.med.uth.tmc.edu/
Newman B, Kuhn JP, Kramer SS, Carcillo JA. Congenital surfactant protein B deficiency--emphasis on imaging.
Pediatr Radiol 2001; 31:327-331.
Stocker JT. Pathologic features of long-standing "healed" bronchopulmonary dysplasia: a study of 28 3- to 40month-old infants. Hum Pathol 1986; 17:943-961.
Williams DW, Merten DF, Effmann EL, Scatliff JH. Ventilator-induced pulmonary pseudocysts in preterm
neonates. AJR Am J Roentgenol 1988; 150:885-887.
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Pediatric Radiology
Pediatric Cardiac Imaging Part I:

Vascular Anomalies
Lecture Outline
Review MRA and CTA techniques

Discuss CT and MR appearances of common thoracic vascular anomalies
MR Angiography: Basic Technique
Black blood sequence: Single shot FSE with half fourier reconstruction (RARE,
HASTE)
Evaluation of airway, vessel lumen
Bright blood sequence: Steady-state free precession (FIESTA, FISP, true
FISP)
Evaluation of shunts/jets due to dephasing associated with turbulent flow
Contrast-enhanced MR angiography
Evaluation of stenoses, bronchial collaterals, anomalous pulmonary veins
CEMRA depicts blood flow from PA to systemic arteries to

systemic veins
CT Angiography: Basic Protocol
PE protocol
Thin collimation (< 1mm)
Fast table speed
Reconstruct 1 to 2 mm intervals (3D)
Low mAs and kVp
Bolus tracking for scan initiation
Trigger @ 100-120 HU
Position of the ROI: Over the Area of Interest
Aorta and surgical shunts

Ascending aorta
Pulmonary artery
Main PA or branches
Pulmonary veins: LA
Reconstructions Increase diagnostic accuracy
Multiplanar
MIPs
Volume Rendering
Which One is Best? MRA or CTA
NO BEST ANSWER
It depends on what you need to know
Both accurate for anatomic detail
MR >CT for functional information
CT>MR for showing stents and calcifications
Applications for Thoracic MRA & CTA
Congenital vascular anomalies

Aorta
Pulmonary vessels
Vena cava
Systemic diseases
Marfan disease
Kawasaki disease
Pediatric Radiology
1453
Vascular Anomalies
Aortic Arch Anomalies
Figure 6-13-1
Symptomatic lesions
Vascular rings
Right arch with aberrant Lt SCA
Double arch
Anomalous innominate artery
Asymptomatic lesions
Left arch with aberrant Rt SCA
Cervical arch
Vascular RingsDouble Aortic Arch
Right arch is dominant

Left arch may be patent or atretic
Complete ring
Clue: 2 arches
4 artery sign (no brachiocephalic
artery)
Double Arch
Double aortic arch. Transverse CT scans showing two arches.

The right arch is larger and more superior than the left. The two
arches unite posterior to the esophagus and a single aorta
descends on the left
6 month old boy with cough
Double Arch: Patent Left Limb [Figure 6-13-1]
Figure 6-13-2
CTA: Double Aortic Arch

Double Arch
Infant with wheezing
MRA: Double Arch
Double Arch Atretic Segment [Figure 6-13-2]
Double Arch Atretic Segment
Look at the Airway
Double aortic arch with hypoplastic left segment

(arrow)
Right Aortic Arch:

Aberrant left subclavian artery
True ring-completed by ligamentum d. arteriosum

Left SCA is last vessel off aorta
Encircles trachea & esophagus
Figure 6-13-3
Right aortic arch

Right Arch Aberrant Left SCA
Right Arch Aberrant Left SCA [Figure 6-13-3]
6 wk old boy with CHD
MRA: Right arch/aberrant LT SCA
GRE MR
CE MRA
Mirror Image Right Arch
Mirror image branching

Asymptomatic
Not a true vascular ring
Congenital heart disease (98%)
Tetralogy of Fallot
Truncus arteriosus
Vascular Anomalies
Right aortic arch with aberrant left subclavian

artery, CT The aberrant subclavian artery courses
behind the esophagus and trachea. LCCA=left
common carotid artery; RCCA=right common
carotid artery; RSA=Right subclavian artery;
LSA=left subclavian artery
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Pediatric Radiology
Mirror Image Right Arch [Figure 6-13-4]
Figure 6-13-4
Tetralogy of Fallot
Innominate Artery Compression
Anterior tracheal compression by the

right innominate artery
Symptoms--respiratory obstruction,
repeated infection, stridor and on
occasion respiratory arrest
Innominate Artery Compression
Mirror image right aortic arch. Transverse CT scans showing a

right sided aortic arch without a posterior crossing vessel
[Figure 6-13-5]
Pulmonary Arterial Anomalies
Agenesis (interruption) of main PA

Pulmonary sling
Ductus arteriosus
Interrupted Pulmonary Artery
Rt or Lt PA is congenitally absent
Interrupted 1 cm beyond origin
Leads to increased systemic blood flow (collateral circulation) to affected
hemithorax
Affected lung absent or hypoplastic
Growth dependent on collateral supply
Figure 6-13-5

Hypoplastic Lung [Figure 6-13-6]
Bronchial collaterals
Absent Pulmonary Artery

Absence of lung
Pulmonary Sling
Created by anomalous course of left PA

Arises from right pulmonary artery & crosses between trachea
& esophagus
Usually symptomatic in children, dyspnea
Figure 6-13-6
Innominate artery compression.
Transverse and sagittal reconstruction
CT showing anterior compression of
the trachea by the right innominate
artery (arrow)
Interrupted pulmonary artery. Transverse CT

showing the right pulmonary artery (arrow)
abruptly interrupted about 1 cm beyond its origin.
Right lung is hypoplastic. Coronal reformation CT
showing fibrofatty tissue filling a small right
hemithorax
Pediatric Radiology
1455
Vascular Anomalies
Pulmonary Sling
3 month old girl [Figure 6-13-7]
Figure 6-13-7
Pulmonary Sling
MRA: Pulmonary Sling
Patent Ductus Arteriosus
Tubular connection between proximal descending aorta and left

pulmonary artery
Isolated lesion or associated with other anomalies
Patent Ductus Arteriosus [Figure 6-13-8]

Ductus
Calcified ductus
Figure 6-13-8
Pulmonary sling. Chest radiograph
(upper panel) showing tracheal
compression. CTA (upper right panel)
showing the left pulmonary artery
(LPA) arising from the right pulmonary
artery (RPA) and crossing behind the
trachea to reach the left hilum. 3D
rendering of the airway (lower panel)
confirming right-sided tracheal
compression
Figure 6-13-9
Patent ductus arteriosus, CTA. Multiple

transverse CT scans showing the patent ductus
(arrow) connecting the proximal descending aorta
and pulmonary artery
Patent Ductus: MRI [Figure 6-13-9]

Pulmonary Venous Anomalies
Anomalous return
Anomalous drainage to systemic veins
Anomalous drainage to left atrium
Patent ductus arteriosus, MRA. Transverse MR
images and 3D sagittal reconstruction showing the
patent ductus (arrow) connecting the proximal
descending aorta and pulmonary artery
Partial Anomalous Return

Partial Anomalous Venous Return
All PAPVR are LT to RT shunts but the shunt is

usually clinically insignificant
Some present with pulmonary hypertension
Vascular Anomalies
1456
Pediatric Radiology
RUL Anomalous Return to SVC
Figure 6-13-10
MRA: Anomalous RUL pulmonary vein

[Figure 6-13-10]
RUL PAPVR
90% have venosum ASD
RLL Anomalous Return
Transverse MR (left image) showing anomalous

right upper lobe pulmonary vein (arrow) draining
into the superior vena cava. T1-weighted image
(right image) showing associated sinus venosus
atrial septal defect (arrow)
RLL Anomalous Return [Figure 6-13-11]
MIP CTA
GRE MR
Scimitar Syndrome
Hypogenetic lung syndrome, pulmonary venolobar syndrome

Vein draining RLL enters IVC, portal vein/ hepatic vein, or right atrium
Hypoplastic lung
Small pulmonary artery
Figure 6-13-11
Scimitar syndrome
10 year old girl

Pneumonia suspected
Scimitar syndrome
Scimitar Syndrome [Figure 6-13-12]
Figure 6-13-12
Right lower lobe anomalous
pulmonary venous return. Coronal
CT showing right lower lobe vein
draining into the inferior vena cava
Scimitar syndrome. Anomalous return of the right lower lobe is

associated with a hypoplastic right lung
LUL Anomalous Return [Figure 6-13-13]

Figure 6-13-13
Anomalous left upper lobe pulmonary

venous return. Transverse CT images
showing the left upper lobe pulmonary vein
(arrow) coursing next to the aorta and
passing adjacent to the left pulmonary
artery. There is no vessel noted at the level
of the coronary sinus which helps to
differentiate this condition from left superior
vena cava
Pediatric Radiology
1457
Vascular Anomalies
LUL Anomalous Return
Figure 6-13-14
Anomalous LUL Vein [Figure 6-13-14]

Systemic Venous Anomalies
Systemic
Persistent left superior vena cava
Interrupted inferior vena cava
Left (Double) Superior Vena Cava
0.5% of general population

5% CHD patients
Drains subclavian vein
Empties into coronary sinus
Small Rt SVC, 90% of cases
Anomalous left upper lobe venous return. Axial

MR images showing the left upper lobe pulmonary
vein (arrow) coursing adjacent to the aorta and
pulmonary artery. Coronal contrast enhanced
MRA (right panel) showing the anomalous left
upper lobe vein (arrow) draining into the
brachiocephalic vein. Also noted is anomalous
drainage of the right upper lobe vein (arrowhead)
into the superior vena cava
Left Superior Vena Cava

[Figures 6-13-15 and 6-13-16]
Figure 6-13-15
Figure 6-13-16
Left superior vena cava. Transverse images

showing an enhancing left sided cava (arrow) and
a smaller right superior vena cava. The left
superior vena cava courses past the aortic arch
and left pulmonary hilum to enter into a dilated
coronary sinus
Left paramediastinal structures
Left superior intercostal vein

Anomalous LUL venous return
Another Lt-sided Vessel

Superior Lt Intercostal Vein
Superior intercostal vein

Drains left 2nd-4th intercostal spaces
Opens into brachinocephalic vein
Joins accessory hemiazygous vein
Aortic nipple shadow on CXR
Left superior vena cava. Coronal and sagittal

multiplanar CT reconstructions showing left
superior vena cava (arrows) draining into the
coronary sinus
Left Superior Intercostal Vein [Figures 6-13-17 and 6-13-18]
Vascular Anomalies
1458
Pediatric Radiology
Figure 6-13-17
Figure 6-13-18
Left superior intercostal vein creating aortic nipple

shadow (arrow)
Summary: Left Paramediastinal Structures
Left superior vena cava

Subclavian vein to coronary sinus
Anomalous LUL pulmonary vein
Left pulmonary hilum to BCV
Left superior intercostal vein
BCV to accessory hemiazygos vein
Left superior intercostal vein. The vein (arrow)

courses adjacent to the aortic arch and connects
with the accessory hemiazygous vein inferiorly
Figure 6-13-19
Review: Paramediastinal Veins [Figure 6-13-19]

Right paramediastinal structures
Azygous vein
joins hemiazygos
ascends on right
drains into SVC
Superior intercostal vein
joins azygos vein
drains 2nd-4th intercostal veins
Summary: Left image, left superior vena cava.

Middle image, anomalous left upper lobe
pulmonary venous return. Right image, superior
left intercostal vein
Figure 6-13-20
Right Azygous System

Azygous Vein [Figure 6-13-20]
Right Superior Intercostal Vein [Figure 6-13-21]
Figure 6-13-21
Normal azygous vein (arrow) draining into superior

vena cava (SVC).
Right superior intercostal vein. CT scans
showing the right superior intercostal vein
(arrow) draining into the azygous vein
Pediatric Radiology
1459
Vascular Anomalies
Azygos Continuation IVC
Figure 6-13-22
Absence of infrahepatic segment of IVC

Blood from lower half of body returns to heart via
dilated azygous and hemiazygous veins
Isolated condition or associated with CHD
Azygos Continuation IVC [Figure 6-13-22]

Dilated Azygous Vein:
Differential Diagnoses
Azygous continuation of the inferior vena cava.

CT scans showing dilated azygous and
hemiazygous veins and absence of the
infrahepatic inferior vena cava
Azygous continuation IVC

Constrictve pericarditis
Acquired obstruction SVC or IVC
Pericardial effusion
Tricuspid insufficiency
Figure 6-13-23
Acquired Vascular Lesions
Aortic aneurysm & dissection

Marfan syndrome
Coronary artery aneurysms
Kawasaki disease
Kawasaki Disease
Aka Mucocutaneous lymph node syndrome

Unknown etiology
Clinical features
fever
rash
conjunctivitis
erythema of lips & buccal mucosa
myocarditis
coronary artery aneurysms
Coronary artery aneurysm, Kawasaki disease. CT

of a neonate (left panel) showing dilated left
anterior descending and circumflex arteries
(arrows) . CT scan of an adolescent girl (right
panel) showing calcified aneurysm (arrow) of the
left coronary artery
Coronary Artery Aneurysms
Arise in proximal part of arteries

Fusiform, saccular, or cylindrical
Coronary Artery Aneurysms-Kawasaki [Figure 6-13-23]

Marfan Disease
Inherited connective tissue disorder transmitted as an autosomal dominant

trait
Localized to a mutation in chromosome 15
Cardinal features
tall stature
slender limbs and fingers
ectopia lentis
pectus excavatum
scoliosis
Common Cardiovascular Findings
Aortic-root dilatation
involves sinuses of Valsalva
prevalence 70%-80%
Aortic dissection
Ascending >> descending aorta
Mitral valve prolapse (55%-69%)
Vascular Anomalies
1460
Pediatric Radiology
Marfan Disease Aortic Aneurysm & Dissection
15 year old boy with Marfans syndrome

Dilated aortic root
Figure 6-13-24
Marfan Disease: Dissection

Marfan Disease [Figure 6-13-24]
Review: Top 10 Vascular Lesions
Double aortic arch

Right arch with anomalous SCA
Absent pulmonary artery
Pulmonary sling
Patent ductus arteriosus
Anomalous pulmonary venous return
Double SVC
Interrupted IVC
Aortic aneurysm & dissection--Marfan
Coronary artery aneurysm-- Kawasaki
Marfan disease, aortic aneurysm and dissection.

CT showing a dilated ascending aorta (A, top left
image) and a dissection of the descending aorta
(arrows)
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
Katz M, Konen E, Rozenman, et al. Spiral CT and 3D image reconstruction of vascular rings and associated
tracheobronchial anomalies J Comput Assist Tomogr 1995; 19:564-568.
Lee Ed, Siegel MJ, Hildebolt CF, Gutierrez FR, Bhalla S, Fallah JH. Multidetector CT Evaluation of Pediatric
Thoracic Aortic Anomalies: Comparison of Axial, Multiplanar, and Three-Dimensional Images. AJR 2004;
182:777-784
Lawler LP, Fishman EK. Multi-detector row CT of thoracic disease with emphasis on 3D volume rendering and
CT angiography. RadioGraphics 2001; 21: 1257-1273
Remy-Jardin M, Remy J, Mayo JR, Muller NL. Thoracic aorta. In: CT Angiography of the Chest. Lippincott
Williams & Wilkins. Philadelphia. 2001; 29-50.
Czum JM, Corse WR, Ho VB. MR angiography of the thoracic aorta. Magn Reson Clin N Am 2005; 13:41-64.
Choe YH, Kim YM, Han BK, Park KG, Lee HJ. MR Imaging In the morphologic diagnosis of congenital heart
disease. RadioGraphics 1997; 17:403-422.
Ho VS, Corse WR, Hood MN, Rowedder AM. MRA of the thoracic vessels. Semin Ultrasound CT MR 204:192216.
Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of congenital heart disease in pediatric and
adult patients. AJR 2003; 180:973-980.
Gup HW, Park I-S, Ko JK, et al. CT of congenital heart disease: normal anatomy and typical pathologic
conditions. Radiographics 2003; 23: S147-165.
Hopkins KL, Patrick LE, Simoneaux SF, et al. Pediatric great vessel anomalies: initial clinical experience with
spiral CT angiography. Radiology 1996; 200:811-815.
Stella VB, Toutouzas P. Patent arterial duct and aortopulmonary window. In: Gatzoulis MA, Wevbb GD,
Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 247-252.
Morgan-Hughes GJ, Marshall AJ, Roobottom C. Morphologic assessment of patent ductus arteriosus in adults
using retrospectively ECG-gated multidetector CT. AJR 2003; 181:749-754.
Mahnken AH, Wildberger JE, Spuntrup E, et al. Unilateral absence of the left pulmonary artery associated with
coronary-to-bronchial artery anastomosis. J Thorac Imaging 2000; 15:187-190;
Gupta H, Mayo-Smith WW, Mainiero MB, Dupuy DE, Abbott GF. Helical CT of pulmonary vascular
abnormalities. AJR 2002; 178: 487-492.
Park HS, Im JG, Jung JW, et al. Anomalous left pulmonary artery with complete cartilaginous ring. J Comput
Assist Tomogr 1997; 21:478-480.
Zwetsch B, Wicky S, Meuli R et al. Three-dimensional image reconstruction of partial anomalous pulmonary
venous return to the superior vena cava. Chest 1995; 108:1743-1735,
Dillon EH, Camputaro C. Partial anomalous pulmonary venous drainage of the left upper lobe vs duplication of
the superior vena cava: distinction based on CT findings. AJR 1993;160: 375-379.
Van Praagh S, Carrera ME, Sanders S, Mayer JE, Van Praagh R. Partial or total direct pulmonary venous drainage
to the right atrium due to malposition of septum primum. Chest 1995; 107:1488-1498.
Pediatric Radiology
1461
Vascular Anomalies
19. Woodring JH, Howard TS, Kanga JF. Congenital pulmonary venolobar syndrome revisited. Radiographics 1994;
14:349-369.
20. Remy-Jardin M, Remy J, Mayo JR, Muller NL. Superior vena cava syndromes. In: CT Angiography of the Chest.
Lippincott Williams & Wilkins. Philadelphia. 2001; 130-139
21. White CS, Blaffa JM, Haney PH, Pace ME, Campbell AB. MR imaging of congenital anomalies of the thoracic
veins. RadioGaphics 1997; 17:595-608.
22. Bass JE, Redqine MD, Kramer LA, Huynh PT, Harris JH. Spectrum of congenital anomalies of the inferior vena
cava: cross-sectional imaging findings. Radiographics 2000; 20:639-652.
23. Yamada I, Nakagawa T, Himeno Y, Numano F, Shibuya H. Takayasu arteritis.: evaluation of the thoracic aorta
with CT angiography. Radiology 1998; 209:103-109
Vascular Anomalies
1462
Pediatric Radiology
Pediatric Cardiac Imaging Part II:

Lecture Outline
Review CT & MRI techniques

Describe CT and MR appearances of common congenital heart diseases
Neonates and infants
Older children & adolescents
Discuss treatment options
Techniques: Cardiac CTA/MRA
MRA: Same as mediastinal vascular lesions (see part I), but add cine
sequence
Black blood: delineation of anatomic structures and vessel lumens
White blood: evaluation of shunts/jets as turbulent flow causes dephasing
Gd for vessels: vessel stenoses
Cine: function & flow dynamics
CT Angiography: Basic Protocol
Use a PE protocol
Thin collimation (< 1mm)
Fast table speed
Low mAs and kVp
Bolus tracking for scan initiation
Trigger @ 100-120 HU
Pediatric Cardiac Imaging: What you need to know
Neonates & Infants:

Top Ten Diagnoses
CT & MRI Features
Top 10 Congenital Heart Diseases Neonates & Infants

SHUNTS
VSD
ASD
PDA
AV Canal
OBSTRUCTIVE
Coarctation
HLHS
CYANOTIC HEART LESIONS

Tet of Fallot
TGV
TAPVR*
Tricuspid atresia*
Truncus
* Adds to 11 because TA &TAPVR have similar frequency
I. The Shunt Lesions
Atrial septal defect

Ventricular septal defect
Atrioventricular canal
(chest x-ray: cardiomegaly & increased vascularity
Atrial Septal Defects
Sinus venosus (10%)

Level of SVC
associated with PAPVR
Secundum (60%)
Level of fossa ovalis
Primum (30%)
Lower atrial septum
Part of AV canal defect
Pediatric Radiology
1463
Sinus Venosus ASD [Figure 6-14-1]
Figure 6-14-1
Sinus Venosus ASD: MRI

Secundum ASD
Large PAs
Mid septal defect
Secundum ASD [Figure 6-14-2]

Ostium Primum ASD
Low septal defect

Sinus venosus ASD. CT scan at level of superior
Just above AV valves
vena cava (left panel) shows anomalous right
Two types
upper lobe vein (arrow) draining into superior vena
Partial: involves atrial septum & mitral valve
cava. CT scan more caudal (right panel) shows
Complete: involves atrial & ventricular septums &
sinus venosus ASD (arrow)
both AV valves
Aka AV canal
Figure 6-14-2
Ostium Primum ASD: CT/MR
Partial primum ASD

Low ASD + mitral insufficiency
AV canal
Low ASD, high VSD + common AV valve
Complete Atrioventricular Septal Defect [Figure 6-14-3]

ASD: Overview [Figure 6-14-4]
ASD Repair
Occluder devices
Small secundum lesions
Amplatzer or CardioSeal
Surgical repair
Large Secundum ASD
Sinus venosus
Primum ASD
Secundum ASD (arrow). Atrial septal

defect (arrow) is at the level of fossa
ovalis and aortic valve. The right
atrium is enlarged
Figure 6-14-3
Repaired ASD
Occluder device for small secundum

ASD
Figure 6-14-4
Complete atrioventricular septal defect (aka AV canal and

endocardial cushion defect). CT scan and MRI show a low
atrial septal defect (ASD) and high ventricular septal defect
(VSD). In this lesion, there is a common atrioventricular valve
Summary. Sinus venosus ASD (left panel). Secundum ASD

(middle panel). Primum ASD (right panel)
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Pediatric Radiology
Occluder Devices [Figure 6-14-5]
Figure 6-14-5
Repaired ASD
Septal patch for large secundum ASD & venosus &

primum ASD
Ventricular Septal Defects
Most common CHD

Locations
Sub-aortic (80%)
(perimembranous)
Intramuscular)
Sub-pulmonic
RV inlet (associated with AV canal)
Amplatzer occluder for smaller ASD. CT scans

showing two disks connected by a short neck in
the location of the atrial septum. RA=right atrium,
LA=left atrium, PA=dilated pulmonary artery
Approximate Location of VSDs

Subaortic VSD [Figure 6-14-6]
Figure 6-14-6
Muscular VSD [Figure 6-14-7]

Subpulmonic ( supracristal) VSD
[Figure 6-14-8]
Overview VSD [Figure 6-14-9]
VSD
Subaortic Most Common
Muscular Multiple
RV Inlet With canal
Subpulmonic TOF
VSD-Treatment
Subaortic (perimembranous) ASD (arrow). CT and MR showing

the ventricular septal defect at the level of the aortic valve (A)
Figure 6-14-7
Small lesions may close spontaneously 30% to 40%

Small lesions that fail to close are occluded with septal occluder device
Large lesions closed with patch graft
Tubular connection between proximal descending aorta and left PA

Isolated lesion or associated with other anomalies
Figure 6-14-8
Figure 6-14-9
Subpulmonic VSD. MR
showing the septal defect
(arrow) at the level of the right
ventricular outlet in the
supracristal area
Pediatric Radiology
Intramuscular VSD (arrow).

The defect is within the
interventricular septum
Summary. Subaortic VSD (far left panel).

Muscular VSD (middle left panel). Ostium
primum (inlet) VSD (middle right
panel).Subpulmonic VSD (far right panel)
1465
PDA [Figure 6-14-10]
Figure 6-14-10
II. More Top 10 Obstructive Lesions
Coarctation of the aorta

Hypoplastic left heart
(X-ray: cardiomegaly & edema)
Obstructive Lesions Aortic Coarctation
Post-ductal
Distal to left SCA
Normal diameter arch
Collaterals common
Pre-ductal
Above left SCA
Hypoplastic arch
Post-Ductal Coarctation-CT
Clues: dilated ascending aorta, post-stenotic dilatation & collaterals
Post-ductal Coarctation-CTA [Figure 6-14-11]

MR: Postductal Coarctation
Figure 6-14-11
Preductal Coarctation-CT [Figure 6-14-12]
Patent ductus arteriosus.

Sagittal MRA showing
patent ductus between
aorta and left pulmonary
artery
Pre-ductal
Above left SCA
Arch hypoplasia
Collaterals uncommon
Coarctation Repair
Resection & end-to-end anastomosis

Stents, angioplasty, patch aortoplasty
After balloon dilatation

Complications Coarctation Repair (5-30%)
Complications
Re-stenosis
Stent fracture
Pseudo-aneurysm
5% to 12% angioplasty
33% patch aortoplasty
Figure 6-14-12
Stent Restenosis
Post-op Complication: Pseudoaneurysm
Hypoplastic left heart syndrome
Presents as CHF in neonate

Classic findings
Small or absent LV
Hypoplastic ascending aorta
Hypoplasia aortic & mitral valves
ASD & PDA
Preductal coarctation,
neonate with heart
failure. Sagittal CT
showing coarctation
(arrow) above origin of
left subclavian artery
1466
Postductal coarctation,
neonate. Transverse CT
(upper left image) shows
a small caliber
descending aorta
(arrow). Sagittal CT
(middle and right
images) show the level
of obstruction (arrow)
just below origin of left
subclavian artery
Pediatric Radiology
Hypoplastic Left Heart [Figure 6-14-13]

Part III.
Common Cyanotic Diseases
SHUNTS
VSD
ASD
PDA
AV Canal
OBSTRUCTIVE
Coarctation
HLHS
CYANOTIC HEART LESIONS

Tet of Fallot
Tricuspid Atresia
TGV
Truncus
TAPVR
Figure 6-14-13
Cyanotic CHD
Indicates that unoxygenated venous

blood is reaching aorta
Causes:
Right heart obstruction with Rt to Lt
shunting via a septation defect
TOF, tricuspid atresia
Mixing of pulmonary & systemic
blood due to incomplete separation Hypoplastic left heart syndrome. Transverse CT (left image)
of chambers
showing small left ventricle (arrow). CT (right image) at a more
TGV, Truncus, TAPVR
proximal level shows small ascending aorta (arrow)
Tetralogy of Fallot: Clues: 4 findings
Figure 6-14-14
The Tetrad:
Subaortic VSD
Infundibular pulmonic stenosis
Overriding aorta
Right ventricular hypertrophy
Tetralogy of Fallot: CTA
Membranous VSD, RVH, infundibular

PS, overriding aorta
Tetralogy of Fallot-MR [Figure 6-14-14]

Surgical Repair TOF [Figure 6-14-15]
Initial surgery is palliative

Blalock-taussig shunt
Subclavian artery to PA
Tetralogy of Fallot, MRI. Transverse MR (left panel) shows

perimembranous VSD (arrow) and right ventricular
hypertrophy (RVH). Sagittal MR (middle panel) shows
narrowed pulmonary outflow tract (arrow). (PA=normal size
main pulmonary artery). Sagittal MR (right panel) shows aorta
(Ao) overriding right and left ventricles, VSD (arrow), and right
ventricular hypertrophy
Figure 6-14-15
Blalock-Taussig shunt for palliation of

tetralogy of Fallot. 3D volume
rendered CT showing subclavian
artery (SCA) to pulmonary artery (PA)
anastomosis (arrow)
Pediatric Radiology
1467
Surgical Repair TOF [Figure 6-14-16]
Figure 6-14-16
Definitive repair
enlargement of PA via patch graft of pulmonary
valve annulus or outflow tract
closure of VSD
Tricuspid Atresia [Figure 6-14-17]
Fatty bar between RA & RV

Hypoplastic RV
Large RA
VSD
Tricuspid Atresia
RAE, fatty tricuspid valve, small RV
Tricuspid Atresia: Surgical Repair [Figure 6-14-18]
Definitive repair of tetralogy of Fallot. Transverse

CT (left image) at level of pulmonary artery shows
a normal caliber main pulmonary artery with
adjacent graft material. CT at level of ventricles
(right image) shows closure of VSD
Glen shunt
SVC to PA
Fontan
RA to PA
Figure 6-14-17
Total Cavopulmonary Fontan [Figure 6-14-19]
Cavopulmonary Fontan
Conduit between SVC & IVC which is joined to main PA
Lesions with Increased Flow

D-Transposition of Great Vessels
Ventriculoarterial discordance
Aorta arises from RV
Pulmonary artery from LV
Circuits are in parallel
ASD, VSD, PDA common
Tricuspid atresia classic features. CT

showing atretic valve replaced by
fatty tissue (arrow), right atrial (RA)
enlargement, and right ventricle
hypoplasia
Figure 6-14-18
Figure 6-14-19
Surgical repairs for tricuspid atresia. Glenn shunt

(left panel) is a superior vena cava (S) to
pulmonary artery anastamosis. Fontan proceudre
(right panel) is a right atrium (RA) to main
pulmonary artery anastomosis. Note the markedly
dilated right atrium.
(Left panel, Glenn shunt, reprinted from Core
Curriculum, Siegel M, Coley B 2005).
Total cavopulmonary shunt. Superior

vena cava (SVC) and inferior vena
cava (IVC) joined via conduit (C),
which is anastomosed to pulmonary
artery (PA)
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Pediatric Radiology
D-Loop Transposition [Figure 6-14-20]
Figure 6-14-20
Surgical Repair: Atrial Switch
Intra-atrial baffle
Blood from SVC and IVC enters superior chamber &
directed to LV and PA
Blood from pulmonary veins enters inferior chamber
& directed to RV & Aorta
Atrial Switch [Figure 6-14-21]

Mustard Procedure
Systemic venous baffle

Pulmonary venous baffle
D-Transposition of great vessels. Sagittal MRA

(left panel) showing aorta arising from right
Current procedure of choice
ventricle (RV) and pulmonary artery from left
Great artery switch
ventricle (LV). Transverse MRI and CT showing
Aorta and PA sectioned above valves & reconnected aorta (A) anterior and to right of pulmonary artery
to proper ventricles
(PA)
Coronary arteries reimplanted
Jantene Procedure
Arterial Switch: Jatene Procedure [Figure 6-14-22]
Figure 6-14-21
Total Anomalous PV Return
Pulmonary veins drain to RA (not LA)

4 types
I. supracardiac (55%)
II. cardiac (coronary sinus) (30%)
III. infracardiac (to portal vein or IVC) (12%)
IV. two of the above (3%)
ASD essential for survival
Repair: reimplant
Total Anomalous Return: Surgery
Veins reanastomosed to LA
Truncus Arteriosus
I. Single PA arises from truncus (80%)

II. R and L PA arise from posterior truncus
III. R and L PA arise from sides of truncus.
IV. R and L PA arise from descending aorta
Atrial switch, mustard

procedure. Coronal CT
(upper image) shows
systemic baffle
directing blood from
right heart to left heart
where it exits into
pulmonary arteries.
Transverse CT (lower
image) shows systemic
baffle and also
pulmonary venous (PV)
baffle which directs
blood from pulmonary
veins into right ventricle
where it exits into aorta
Figure 6-14-22
Jatene procedure, arterial switch. Pulmonary artery (PA) lies

anterior and to right of aorta (A)
Pediatric Radiology
1469
Truncus Arteriosus Type I [Figure 6-14-23]
Figure 6-14-23
Type IV Truncus (pseudotruncus)

[Figure 6-14-24]
Truncus: Surgical Repair
Pulmonary arteries detached from common artery

(truncus arteriosus) and connected to RV using a
conduit.
VSD is closed with a patch
Additional Lesions: Older Children &

Adolescents
Truncus arteriosus. Type I A single pulmonary

artery arises from the truncus (T)
Aortic stenosis
Pulmonic stenosis
Figure 6-14-24
Aortic Stenosis
Valvular >> sub- or supravalvular

Usually due to bicuspid valve
CT/MR: dilated ascending aorta
Bicuspid valve
Aortic Stenosis-CT [Figure 6-14-25]

Bicuspid Aortic Valve
Ca++ uncommon before 4th decade
Truncus arteriosus type IV. Oblique MRA showing

both pulmonary arteries arise from the descending
aorta. Transverse MR showing an associated
VSD (arrow)
AS and Bicuspid Valve-MR
Thickened, domed leaflets during systole

Thick wall left ventricle
Figure 6-14-25
Aortic Stenosis: Treatment
Balloon dilatation
Valvotomy
Valve replacement
Ross procedure
aortic valve replaced with patients pulmonary
valve
pulmonary valve replaced with cadaveric valve
Valvular Pulmonic Stenosis
Valvular stenosis most common (95%)

90% due to commissural fusion
10% due to a dysplastic valve
thickened, but non-fused commissures
Aortic stenosis. Transverse and coronal CT
showing dilated ascending aorta
Pulmonary Artery Stenosis [Figure 6-14-26]
CT/MR
Dilated main PA
Dilated Lt PA
Rt ventricular hypertrophy
Figure 6-14-26
Pulmonic valve stenosis. CT

showing dilated main (M) & left
(L) pulmonary arteries
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Pediatric Radiology
Pulmonic Stenosis: Repair
Surgical valvotomy
Percutaneous balloon valvuloplasty
Less successful in patients with valvular dysplasia
Summary
Diagnosis of CHD depends on knowledge of the anatomic abnormality, the clinical findings &
understanding of imaging findings
References
1.
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Amplatz K, Moller JH Radiology of Congenital Heart Disease Mosby Year Book Inc 1993
Boxt LM. MR imaging of congenital heart disease. Magn Reson Imaging Clin North America;1996:4:327-359
Choe YH, Kim Ym, Han BK, Park KG, Lee HJ. MR imaging in the morphologic diagnosis of congenital heart
disease. RadioGraphics 1997; 17:403-422.
Poustchi-Amin M, Gutierrez FR, Brown JJ, et al. How to plan and perform a cardiac MR imaging examination.
Radiol Clin North Am 2004; 42:497-514
Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of congenital heart disease in pediatric and
adult patients. AJR 2003; 180:973-980.
Goo HW, Park I-S, Ko J-K, et al. CT of congenital heart disease: normal anatomy and typical pathologic
conditions. Radiographics 2003; 23: S147-165
Gutierrez FR, Canter CE, Mirowitz SA: MR appearance of congenital heart defects. In: Gutierrez FR, Brown JJ,
Mirowitz SA (eds.): Cardiovascular magnetic resonance Imaging. St. Louis: Mosby Year Book, 1992;72-83.
Higgins CB: Congenital heart disease. In: Higgins CB, Hricak H, Helms CA (eds.): Magnetic Resonance Imaging
of the body. 3rd ed. Philadelphia: Lippincott-Raven, 1997; 461-518.
Lee E, Siegel MJ, Guttierez F, Hildebolt CF, Bhalla S, Fallah JH. Multidetector CT evaluation of thoracic aortic
anomalies in pediatric patients and young adults: comparison of thoracic axial, multiplanar, and 3D images. AJR
2004; 182:777-78410.
Kaemmerer H. Aortic coarctation and interrupted aortic arch. In: Gatzoulis MA, Wevbb GD, Daubeney PEF.
Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 253-264.
Becker C, Soppa C, Fink U et al. Spiral CT angiography and 3D reconstruction in patients with aortic coarctation.
Eur Radiol 1997; 7:1473-1477.
Roest AA, Helbing WA, van der Wall EE. Postoperative evaluation of congenital heart disease by magnetic
resonance imaging. J Magn Res Imag 1999; 10:656-666.
Donnelly LF, et al. MR imaging of cono-truncal abnormalities. AJR 1996; 166:925-928.
Connelly M. Common arterial trunk. In: Gatzoulis MA, Webb GD, Daubeney PEF. Adult Congenital Heart
Disease. Churchill Livingstone. Edinburgh 2003; 265-271.
Jacobs ML. Congenital heart surgery nomenclature and database project: truncus arteriosus. Ann Thorac Surg
2000; 69:S50-S55.
Kim TH, et al. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary
venous connections in neonates and infants. AJR 2000; 175: 1381-1386.
Bardo DM, et al. Hypoplastic left heart syndrome. Radiographic 2001; 21:705-717.
Mavroudis C, Backer CL, Deal BJ. Venous shunts and the Fontan circulation in adult congenital heart disease. In:
Gatzoulis MA, Wevbb GD, Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh
2003; 79-83.
Feeedom RM, Li J, Yoo S-J. The complications following the Fontan operation. In: Gatzoulis MA, Wevbb GD,
Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 85-91.
Pediatric Radiology
1471

Topics
Assessing the chest film

Acyanotic CHD with increased PBF
Cyanotic CHD with increased PBF
Cyanotic CHD with decreased PBF
Increased PBF
ACYANOTIC
VSD
ASD
PDA
ECD
AP Window
Increased PBF
CYANOTIC
TGV
TAPVR
Truncus
Tric Atresia
Single Chamber
HLHS
Decreased PBF
CYANOTIC
Tet of Fallot
Ebstein
Tric Atresia
Pulm Atresia
Assessing the Chest Film
Pulmonary blood flow (PBF) KEY!!!

Cardiomediastinal silhouette
Configuration
Size
Aortic arch side
Situs
Pulmonary Blood Flow
Normal
Increased (arterial overcirculation and venous congestion)
Requires 2 : 1 (pulmonary : systemic) shunt to see increased PBF @ CXR
Decreased
Systemic collaterals (AKA bronchial or major aorticopulmonary collaterals)
Pulmonary Blood Flow Indicators
Gestalt too big, seen too far in periphery (look at hilar vessels most
reliable and most technique - independent)
Main pulmonary artery
Interlobar artery diameter = supraaortic tracheal diameter (+/ 2
mm)
Bronchovascular couplet: diameter artery = diameter bronchus in
upper zones in normal patients (less than 2:1 shunt)
Hepatic window if vessels too large here or seen on end, there
is increased PBF
Figure 6-15-1
Chamber Assessment [Figure 6-15-1]
RA: not reliably assessed by plain film & infrequently a diagnostic

discriminator
RV: retrosternal space >1/3 filled
LA: upper 1/2 of posterior cardiac border
LV: lower 1/2 of posterior cardiac border
Does either or both:
Touch spine?
Extend beyond posterior tracheal line?
RV touches lower 1/3 of sternum; LA

is upper 1/2 and LV is lower 1/2 of
posterior cardiac border
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Pediatric Radiology
Cardiomediastinal Silhouette Size
Cardiothoracic ratio:
Infant < .65
Adult < .50
Infant cardiomegaly:
Apex touches lateral chest wall on AP
Posterior border touches spine on lateral
Cardiomediastinal Silhouette Configuration
Acyanotic CHD: usually normal shape

Cardiomegaly common
Cyanotic CHD: more often bizarre
Absence of thymus - think cyanotic disease (stressed infant, agenesis)
Acyanotic CHD with Increased PBF
Increased blood volume in lungs

Can only occur in 1 of 2 ways:
Passive congestion: impeded pulmonary venous return
Example: congestive heart failure
overcirculation: too much blood delivered to lungs
Example: ventricular septal defect
Figure 6-15-2
Acyanotic CHD with Increased PBF shunts

[Figures 6-15-2 and 6-15-3]
Unifying theme:
Pressure difference R v. L
Blood will preferentially flow thru an opening from
high pressure L to lower pressure R
Blood is recirculated thru pulmonary bed (result: increased PBF)

( L to R shunts INTRACARDIAC)
Ventricular septal defect (VSD)

Atrial septal defect (ASD)
Patent ductus arteriosus (PDA)
Endocardial cushion defect (ECD)
Aorticopulmonary window (APW)
Pressures in infant cardiac chambers

(L to R shunts EXTRACARDIAC)
Vein of Galen aneurysm

Hepatic hemangioendothelioma
Peripheral AV fistula
Figure 6-15-3
Acyanotic CHD with Increased PBF(FAILURE)
Hypoxic cardiac injury (birth asphyxia, drowning)

Anomalous origin left coronary artery
Sepsis
Endocardial fibroelastosis
Glycogen storage disease (Pompe)
Viral myocarditis
Adriamycin toxicity
Caveat
In infants, all forms of increased PBF may lead to pulmonary

edema
Cannot reliably distinguishing arterial overcirculation from passive
congestion
Consider the 3 causes of increased PBF:
Intracardiac shunts
Extracardiac shunts
Cardiac failure
Pediatric Radiology
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1473
VSD: blood flows from LV to RV

Ventricular Septal Defect (VSD) [Figure 6-15-3]
Most common CHD (25%)
VSD: Hemodynamics
Blood flows from LV to RV

Volume overload in RV
RV enlarges and hypertrophies
Blood in RV goes to lungs, LA, LV.
When it arrives in LV, it decompresses into RV
LV remains normal
4 basic types
Membranous
80%
Muscular (often multiple) 10%
Supracristal
5%
Inflow / AV canal type
5%
Size matters, not location!
Radiology [Figures 6-15-4 and 6-15-5]
Large heart
RV and LA large
VSD: Presentation
Usually in 2nd month of life

Pulmonary vascular resistance has dropped
sufficiently to allow increased shunting
Presentation
Frequent pneumonias
Failure to thrive
CHF
Figure 6-15-4
VSD: Natural History
Small: close spontaneously by 3 years

Muscular ingrowth
By 2 months of age: follow v. repair
Large: repair via trans-atrial approach
Goretex patch
Pericardial patch
VSD: cardiomegaly, large RV, and large LA
VSD
All need to be closed

Large:
Significant L to R shunting
Less turbulence
Pulmonary HTN, Eisenmenger syndrome
Small:
Less L to R shunting
More turbulence
Higher risk of bacterial endocarditis
Figure 6-15-5
Atrial Septal Defect (ASD) [Figures 6-15-6 a and b]
Incidence 10%
3 : 1 female : male
Most common shunt to present after the age of 3
Size determines shunt
Locations
Ostium secundum (fossa ovalis) 60%
Patent foramen ovale
Ostium primum (part of cushion) 30%
Sinus venosus (just below SVC, usually with partially anomalous pulm.
venous return from RUL)
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MRI of VSD
Pediatric Radiology
ASD: Hemodynamics
Figure 6-15-6a
Blood shunts LA to RA to RV to lungs to LA

RV & RA enlarge in response to increased volume
L heart: no increased volume (blood from lungs returning to LA
decompresses into RA)
LA, LV, and aorta are normal
RV large; no L-sided chamber enlargement
Figure 6-15-6b
ASD locations: uppermost is
sinus venosus, middle is
ostium secundum, lowest is
ostium primum
ASD
Figure 6-15-7
ASD: Complications
Pulmonary HTN & Eisenmenger syndrome

30% with mod-large ASD develop HTN
Atrial arrhythmias (volume overload)
Paradoxical emboli
ASD
ASD: R to L Shunting [Figure 6-15-9]
Paradoxical emboli
R to L shunting may occur:
Pregnancy (decreased SVR)
Diving,valsalva, cough
COPD, pulmonary embolus
Paradoxical Embolus
Ischemic stroke: no cause in

35-40%
Cryptogenic stroke
Paradoxical embolus from
ASD?
Pts with cryptogenic ischemic
stroke and PFO, on
anticoagulant therapy:
1.2% risk/year of TIA
3.4% risk/year of TIA or
stroke
Figure 6-15-8
Figure 6-15-9
ASD at MR
R to L shunting
across septal defect
Pediatric Radiology
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ASD
Figure 6-15-10
Repair all between 2-5 years

Repair methods:
Amplatzer occluder (secundum only)
Repair mortality <1%
Suture (small, any location)
Patch (any location)
Goretex
Pericardium
Patent Ductus Arteriosus (PDA) [Figure 6-15-10]
Incidence 8%
In utero, allows fetal blood returning from placenta to bypass
underdeveloped lungs
2:1 F:M
Birth:
Lungs aerate, fluid leaves
Pulmonary pressure drops
Ductus closes by 15 hours
All RV blood to lungs
Normal PBF
Ductus may remain open
High pulmonary pressures
Prematurity
Meconium aspiration
Pulm hypoplasia (diaph hernia,oligo, ARPKD)
Idiopathic
PDA: shunt
Shunt direction determined by:

Pulmonary vs systemic pressure
In otherwise normal infants, it will be L to R
Hemodynamics
Shunt is from Ao to PA
LA, LV, Ao: increased volume
L heart enlarges
RV: increased pressure from afterload; hypertrophy
Radiology [Figure 6-15-11]
LA, LV, Ao arch large

Some RVH
PDA
Anatomically closed by 3 weeks

If open @6 months, certainly will remain
Flow depends on:
Width
Length
Shape
Figure 6-15-11
L to R shunt: pulmonary HTN

Bacterial endocarditis
Turbulent flow
Continuous flow
0.5%/year cumulative risk
SBE prophylaxis until repair
PDA: increased PBF, enlargement of RV, LA, LV
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Pediatric Radiology
Figure 6-15-12
Treatment:
Small: coils
Medium to large:
Mesh occluder
Neonatal
Clip (any size ductus)
Prostaglandin inhibitors: only for stable
neonates
Arterial access limited by small size
Tiny, incidental PDA: Rx controversial
SBE prophylaxis if residual shunt
2
3
Spectrum of cushion defects:
Endocardial Cushion Defect (ECD) /

Atrioventricular Canal (AV Canal) [Figure 6-15-12]
Incidence 4%
Spectrum of lesions & severity:
Ostium primum ASD
+ / - common anterior and posterior mitral and
tricuspid valve leaflets
+ / - inlet VSD (most severe)
Presentation/radiographic appearance depends
on severity
Variable cardiomegaly and increased PBF
40% have Down syndrome (look for 11 or 13 rib
pairs, 6 sternal ossification centers)
Most common cardiac lesion in Down
syndrome
1-Normal
2-Moderate ECD: ASD & cleft AV valve leaflets
3-Severe ECD: ASD, VSD, common AV valve
Figure 6-15-13
ECD: increased PBF, RV and LA enlargement
Figure 6-15-14
Chamber enlargement varies with nature of ECD

Cardiomegaly & increased PBF
Gooseneck deformity at angio from LV outflow obstruction
Aorticopulmonary Window (APW) [Figures 6-15-15 and 6-15-16]
Rare
Communication between ascending aorta and pulmonary trunk or
RPA
Large L to R shunt
Predominantly L chamber enlargement
Similar to PDA
Figure 6-15-15
Figure 6-15-16
Aorticopulmonary window
Pediatric Radiology
ECD: gooseneck deformity of LV

outflow tract
AP window: increased PBF, RV, LA, & LV

enlargement
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Eisenmenger Physiology [Figures 6-15-17 and 6-15-18]
L to R shunting creates increased volume in pulmonary arteries;

results in increased pressure
Pulmonary arteries respond by undergoing hypertrophy &
endothelial thickening, creating pulmonary hypertension (a
vicious cycle) if not corrected (may begin in utero)
Pulmonary pressure may become higher than systemic pressure
Shunt switches from L to R to R to L
Deoxygenated blood from R heart shunts to L heart and
Cyanosis results (cyanosis tardive)
Figure 6-15-17
Normal (left) and hypertensive (right)

pulmonary artery, showing
endoluminal narrowing
Pruned appearance of PBF on CXR:

Dilated central arteries
Diminutive peripheral arteries
Irreversible course of events
Goal of surgery is to prevent this dreaded complication
Figure 6-15-18
1
Figure 6-15-19
Eisenmenger syndrome in VSD:

1-VSD initially shunts L to R
2-With onset of pulmonary HTN,
blood begins to shunt R to L
Figure 6-15-20
ASD with Eisenmenger syndrome:

large hilar arteries, diminutive
peripheral vessels
Cyanotic CHD [Figures 6-15-21 and 6-15-22]
Cyanosis: Hb saturation < 85%

Deoxygenated Hb (blood from R heart)
has entered systemic (L heart)
circulation
This can only happen if there is . . .
R to L shunt (from R-sided outlet
obstruction, such as Tet of Fallot,
Eisenmenger syndrome)
Mixing of R & L sided blood in
systemic circulation (eg: truncus
arteriosus)-admixture lesion
Figure 6-15-21
Pulmonary artery banding

reduces PBF, reducing risk of
Eisenmenger syndrome
Deoxygenated blood from R

side enters L side, leading to
cyanosis
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Pediatric Radiology
Admixture Lesions [Figure 6-15-23]
Figure 6-15-22
Mixing of R & L circulations, across a large VSD,

ASD, PDA, or a single chamber where there
usually are 2
Admixed blood flows to pulmonary circulation
These are the cyanotic with increased PBF lesions
Admixture Lesions
5Ts and H
Transposition of the great vessels

Total anom. pulmonary venous return
Truncus arteriosus
Tricuspid atresia
Single chamber
single ventricle
double outlet right ventricle
common atrium)
Hypoplastic left heart syndrome
R to L shunt lesions shunt PBF to L side of heart;

there is decreased PBF
Complete Transposition of Great Vessels

(d-TGV, D loop TGV) [Figure 6-15-24]
Admixture lesions allow intracardiac mixing of R

and L circulations; blood preferentially flows to
lower pressure pulmonary vascular bed,
increased PBF results and patient is cyanotic
Incidence 8%
Most common cyanotic CHD presenting in neonate
TOF most common overall
2 : 1 male : female
Classic TGV:
Aorta arises from RV
Pulm art from LV
2 closed circuits
Incompatible with life w/o L-R communication
All have ASD
Half with VSD
Figure 6-15-23
PA not border-forming on left

Mediastinum appears narrow
Egg on a string appearance
Vast majority with L aortic arch
Small to absent thymus (makes perception of narrow vascular
pedicle possible)
DORV: admixture lesion
Figure 6-15-24
Figure 6-15-25
D-TGV: cardiomegaly, narrow

mediastinum, increased PBF
D (classic) transposition
Pediatric Radiology
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Surgical Repair [Figures 6-15-26 and 6-15-27]
Arterial switch (Jatene) switching aorta &

PA to normal location
Atrial septostomy (palliative) - opens ASD to
improve admixture
Balloon septostomy = Rashkind
Atrial switch/baffle (Mustard, Senning) systemic blood returns to LA, pulmonary
returns to RA
Figure 6-15-26
Figure 6-15-27
D and L Designations
[Figures 6-15-28 and 6-15-29]
Refers to aortic or ventricular position

D may refer to:
Aortic position (D is ABNORMAL)
Ventricular looping & position (D is
NORMAL)
d-TGV = D loop TGV = complete TGV
L designation may refer to
Aorta to left and anterior to pulmonary
artery (not normal, but close)
Ventricular looping & position (L is
ABNORMAL)
Jatene switch repair of

TGV
Figure 6-15-28
Atrial baffle (Mustard

procedure) palliation of
TGV
Figure 6-15-29
Congenitally Corrected TGV (L-TGV

or L loop TGV)
Ventricular inversion key abnormality

There is great vessel transposition, too,
but...
Blood circulatory pattern normal
Radiograph: NORMAL
High incidence associated anomalies
(responsible for morbidity)
Ebstein-like changes in tricuspid valve
VSD
Pulmonary stenosis/atresia
AV valves (tricuspid & mitral) go with the
D (classic) transposition of
inverted ventricles
Ao & PA
Coronary arteries are anatomically inverted
as well
RV (inverted LV) supplied by two coronary arteries
LV (inverted RV) supplied by one
Increased mortality:
Coronary artery disease (single vessel)
L-TGV (transposition of
ventricles)
Figure 6-15-30
Total Anomalous Pulmonary Venous Return (TAPVR) [Figure
6-15-30]
Incidence 2 %
PBF returns to RA
Admixes with systemic return in RA
MUST have communication with L heart
All have ASD
Admixed blood: R to L flow
TAPVR type 2 with return of

PBF to RA
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Pediatric Radiology
Darlings Classification of TAPVR : Type 1 (supracardiac)

[Figure 6-15-31]
Figure 6-15-31
55%
Pulmonary veins drain cephalad into L SVC (aka left vertical vein), into
left BCV, then into SVC (occasionally azygos)
Snowman heart
Almost half are obstructive (from bronchial compression, vein
stenosis)
Type 2 (cardiac) [Figure 6-15-32]
30%
Pulmonary veins drain into coronary sinus or directly into RA
Non-specific appearance (like any L to R shunt). Rarely obstructive
Type 3 (infracardiac)
[Figure 6-15-33]
12%
Common pulmonary vein descends thru esophageal hiatus
Drains into portal vein/ductus venosus, hepatic vein, or IVC
Pulmonary venous return is always obstructed because:
Long course of vein
Passage through diaphragm
Return through hepatic parenchyma when draining into
PV/ductus venosus (most common)
Pulmonary edema
Heart size is NORMAL
Lungs act as capacitor for obstructed pulmonary venous
return
Distinctive appearance: normal size heart with pulmonary edema
Type 4 = combination lesion of some the above (5%)
Partial APVR (clinically insignificant) [Figure 6-15-34]
Most commonly is return of RUL pulmonary vein directly into
SVC
Scimitar syndrome is PAPVR of hypoplastic RML, RLL; drains
to IVC
Type 1 (supracardiac) TAPVR;

PBF returns to SVC
Figure 6-15-32
TAPVR: Variable Physiology
Increased PBF
Obstruction occurs in all type 3 and in 25 40% of type 1 (from
bronchial compression or intrahepatic drainage), which creates
pulmonary edema
Figure 6-15-33
Figure 6-15-34
Type 3 (infracardiac) TAPVR;

PBF returns to portal vein,
hepatic vein, or IVC
Pediatric Radiology
Type 2 TAPVR; PBF returns to RA
Partial APVR; PBF from 1-2

lobes returns to RA
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TAPVR 1: Radiology [Figures 6-15-35 and 6-15-36]
Figure 6-15-35
Cardiomegaly and increased PBF

Snowman heart from prominent left vertical vein
and distended SVC
Superior mediastinum looks wide and round
TAPVR 2: Radiology [Figure 6-15-37]
Non-specific
Looks like many L to R shunts (large heart with
increased PBF)
Figure 6-15-37
TAPVR 1: Snowman heart & increased PBF
Figure 6-15-36
TAPVR 2: increased PBF, non-specific

appearance
TAPVR 3: Radiology [Figures 6-15-38 and 6-15-39]
Always obstructive
Pulmonary edema
Heart size normal (it is not seeing increased volume)
Normal size heart + pulmonary edema = TAPVR III
TAPVR 1: MRI of L SVC

and large R SVC
Figure 6-13-39
Figure 6-15-38
TAPVR 3: normal size heart +

pulmonary edema + increased PBF
Surgical Repair
Depends on type
Aim is to patch-graft pulmonary vein to LA or create pulmonary
venous conduit to LA
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Type 3 (infracardiac) TAPVR:

pulmonary venous return to portal
vein
Pediatric Radiology
Truncus Arteriosus [Figures 6-15-40 ]
1%
Failure of septation of truncal artery into Ao & PA
Single great artery arises from heart, via single valve
Gives rise to Ao, PA, and coronary arteries
Truncal artery overrides a VSD (may have ASD and/or PDA too)
R aortic arch in 35%
Collett & Edwards classification (anatomic)
Type 1: PA arises as single main PA
Type 2: PAs arise separately but close
Type 3: PAs arise independently, with widely spaced origins
Type 4: PA arise from descending Aorta (aka pseudotruncus)
Really bronchial arteries
Figure 6-15-40
Type 1 truncus arteriosus:

single artery arises from
RV & LV
Type 2 truncus
Type 3 truncus
R aortic arch + increased PBF + cyanosis: truncus arteriosus likely

Always have large heart
Resembles TGV
Type 2 & 3 give hilar comma or hilar waterfall sign from
vertical course of pulmonary arteries descending to pulmonary
hilum
Pseudotruncus
(bronchial collaterals
arise from descending
Ao)
Figure 6-15-41
Repair [Figure 6-15-43]
Rastelli: RV to PA Conduit
Figure 6-15-43
Truncus with R Ao arch
Figure 6-15-42
Truncus arteriosus: cardiomegaly +

increased PBF
Rastelli repair
Pediatric Radiology
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1.5%
Very variable
Appearance
Physiology
Associated defects
Blood flows RA to LA (across ASD) to LV to RV (across VSD) and
out PA; admixture occurs in LA
RV rudimentary
Classified into 2 types: [Figure 6-15-45]
With TGV (25%) (increased PBF)
Without TGV (75%) (decreased PBF)
Therefore, the great vessel served by RV is usually
underperfused. This would be the:
Pulmonary artery in normally related great vessels (therefore
PBF is decreased)
Aorta in TGV (therefore PBF is increased)
Figure 6-15-44
Figure 6-15-45
Non-specific
Increased or decreased PBF
depending on transposition
Difficult dx to exclude!
Tricuspid atresia with normally related

Ao & PA (decreased PBF)
Figure 6-15-46
Surgical Repair
[Figure 6-15-47]
Correction with Fontan

(conduit between RA and
main PA and closure of VSD)
Single Ventricle/DORV/
Common Atrium
[Figure 6-15-48]
All have single dominant

chamber (where normally
there are 2 separate L and R
chambers) into which all
blood flows
Tricuspid atresia with transposition
Admixture occurs in this
of Ao & PA (increased PBF)
single chamber
Tricuspid atresia with TGV:

cardiomegaly + increased PBF
Figure 6-15-47
Figure 6-15-48
Single chamber lesions: single ventricle, double outlet right ventricle,

& common atrium
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Fontan repair of tricuspid atresia
Pediatric Radiology
Single Ventricle [Figures 6-15-49]
Figure 6-15-49
All blood returns to the atria and into

single ventricle (admixture occurs here),
and is pumped from this into the great
vessels
May have increased or decreased PBF
depending on outflow tract obstructions
Radiographic appearance hence very
variable
Double Outlet Right Ventricle

[Figure 6-15-50a]
Origin of both great vessels from RV

Single ventricle
Usually with VSD
Admixture occurs in RV and admixed
blood is pumped to systemic and pulmonary circulations
Single Ventricle vs DORV

[Figure 6-15-50b]
To the patient, not much physiologic

difference
Both have:
Admixture of circulations in dominant
ventricle
A functional single ventricle that
pumps admixed blood to both
pulmonary and systemic circulations
Single ventricle: cardiomegaly +

increased PBF
Figure 6-15-50b
Figure 6-15-50a
Common Atrium [Figures 6-15-51]
Essentially huge ASD permitting

significant admixture between RA & LA
No gradient L-R
Very uncommon lesion
Large heart with increased PBF (nonspecific)
Double outlet right

ventricle
DORV: cardiomegaly + increased
PBF
Figure 6-15-51
Figure 6-15-52
Common atrium (similar appearance to large ASD)
Hypoplastic Left Heart Syndrome: HLHS [Figure 6-13-52]
8%
Most common cause cardiac death 1st week of life
Hypoplastic LV, AoV, proximal Ao, LA, MV (degrees variable)
L-sided outflow tract obstruction
Variable severity
Systemic perfusion of aorta entirely thru PDA @ pulmonary pressures
RV failure rapidly ensues, especially as ductus closes in 24 48 hours
Infants are dusky (poorly perfused with admixed blood)
They are too poorly perfused to appear cyanotic
Dusky infant in failure in first 48 hrs of life = HLHS
Pediatric Radiology
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syndrome
Typically very large heart

Failure
One of very few conditions that cause failure in first 24 hours of
life
Figure 6-15-53
Surgical Repair
Cardiac transplant
Norwood (3 stage repair)
Stage 1: (birth)
Conduit RV to Ao root
Divide PA from RV and ductus
Perfuse PA via BT shunt
Stage 2: (6 mos)
HLHS: cardiomegaly + pulmonary
Construct Glenn shunt (SVC to PA) to reduce RV volume
edema
load
Stage 3: (18 mos)
Figure 6-15-54
Extracardiac Fontan. Direct blood from IVC & SVC thru RA to PA
Conduit RV to Aorta: newborn [Figure 6-15-54]

Blalock-Taussig Shunt: newborn [Figure 6-15-55]
Bidirectional Glenn Shunt: 4-6 mos [Figure 6-15-56]
Extracardiac Fontan: 2 years [Figure 6-15-57]
Fontan: 2+ years [Figure 6-15-58]
Figure 6-15-56
Figure 6-15-55
Rastelli repair for HLHS:
conduit from RV to Aorta
Figure 6-15-57
BT shunt: subclavian
artery to ipsilateral PA
Figure 6-15-58
Glenn shunt: SVC to R PA

(bidirectional = perfuses R
and L PA)
Extracardiac Fontan: SVC

and IVC grafted to PA
(bypasses RA & RV)
Fontan: RA to PA graft
(adds atrial kick to perfusion
pressure)
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Pediatric Radiology
Cyanotic CHD with Decreased PBF R to L Shunt Lesions

[Figure 6-15-59]
Patients with decreased PBF are ALWAYS cyanotic

Blood shunts right to left (bypasses lungs so is not oxygenated);
there is decreased PBF
Figure 6-13-59
R to L Shunts
2 common features:
Opening between R and L sides of heart (allows R to L
shunting)
R-sided outflow tract obstruction (pulmonary stenosis/ atresia,
tricuspid stenosis/atresia)
R to L Shunt Lesions TET P
Tetralogy of Fallot
Ebstein malformation
Tricuspid atresia
Pulmonary atresia
Tetralogy of Fallot [Figure 6-15-59]
Incidence 9%
Most common cyanotic CHD
Associated with Down Syndrome
ECD most common cardiac lesion in Down syndrome
Tetrad:
VSD (usually large)
Infundibular pulmonary stenosis
Overriding aorta
R ventricular hypertrophy
Tetralogy of Fallot
Figure 6-15-60
R aortic arch in 25% (usually mirror image branching)

R arch also seen in 3540% Truncus (most highly associated)
But TOF much more common
Pulmonary valvular stenosis (90%)
Peripheral pulmonary stenoses (75%)
ASD (10%) (w/ VSD=pentalogy of Fallot)
Enlarged systemic collateral arteries
Physiology
Large VSD means pressure LV = RV

Severity of pulmonary stenosis dictates amount of R to L shunt
Mild stenosis = little/ no shunting = pink tet)
Tet spells paroxysmal dyspnea progressing to cyanosis and
unconsciousness (unknown etiology)
Squatting: patient squats to increase systemic resistance,
decrease R to L shunting, and improve PBF. Especially important
when exercising / playing (SVR decreases with exercise, so R to
L shunting worsens)
Tetralogy: coeur en sabot heart
Figure 6-15-61
Boot-shaped heart (coeur en sabot or heart in a boot)

Concave PA segment gives unusually straight (horizontal) upper
L cardiac border
The more severe the pulmonary outflow obstruction, the more
classic the X-ray appearance
Pediatric Radiology
1485
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Tetralogy: angiogram of large VSD,

overriding Ao, pulmonary stenosis
Surgical Repair [Figure 6-15-62]
Figure 6-15-62
Corrective:
Patch VSD
Ao isolated to LV
Widen infundibulum,
Repair other anomalies
Waterston [Figure 6-15-63]

Potts [Figure 6-15-64]
Figure 6-15-63
Figure 6-15-64
Tetralogy corrective repair
Figure 6-15-65
Waterston palliative shunt
Potts palliative shunt
Surgical Complications [Figure 6-15-65]
RV outflow tract aneurysms

Obstruction of a shunt
Look for asymmetry of PBF
Change from baseline
Ebstein Malformation [Figure 6-15-66]
2 tricuspid valve leaflets displaced into RV

RV functionally small
Atrialization of RV
RV outflow tract obstructive
Tricuspid valve insufficiency
ASD shunts R to L
RA is large
Figure
Cardiomegaly
RV outflow tract aneurysm, S/P Tet

repair
6-15-66
Figure 6-15-67
Cardiomegaly (RA dilation)

Decreased PBF
Ebstein malformation
1486
1488
Ebstein malformation: large heart,

decreased PBF
Pediatric Radiology
Figure 6-15-68
Incidence 1.5%
75% of TA have decreased PBF (they do NOT have
associated TGV)
ASD shunts R to L
Small VSD
PA arises from diminutive RV
PBF decreased
Mild cardiomegaly
Increased or decreased PBF
Depends on whether PA or Ao arises from RV
Very variable
Tricuspid atresia:
1-With transposition (increased PBF)
2-With normal great vessels (decreased PBF)
Surgical Repair [Figure 6-15-70]
Palliative L to R conduits
Fontan:
Conduit from RA to PA
RV usually closed off
Figure 6-15-69
Figure 6-15-70
Single Ventricle Physiology
Common disorders:
Tricuspid atresia
Pulmonary atresia
DORV
Increased or decreased pulmonary
vascularity
Determined by R outflow
obstruction (eg.: pulmonary
stenosis)
Single Ventricle Protocol

All: single ventricle serves Ao
Tricuspid atresia with normally related

vessels: decreased PBF
Step 1 (newborn): adjust PBF

BT shunt for decreased PBF
Banding, other Rx for increased PBF
Step 2 (4-6 mos): take volume load off heart
Bidirectional Glenn shunt
Step 3 (2 yrs):
IVC and SVC serve PA
Via RA = Fontan
Directly = extracardiac Fontan
Fontan palliation of
tricuspid atresia
Figure 6-15-71
Pulmonary Atresia with Intact Ventricular Septum [Figure 6-15-71]
Incidence 1%
Pulmonary atresia with VSD = tet physiology
Similar to tricuspid atresia physiologically
Lungs perfused via PDA
Ductal dependent lesion
Appearance variable
Usually large heart
Pediatric Radiology
Pulmonary atresia with

intact ventricular septum
1487
1489
Top 10 CHD
1. VSD
2. ASD
3. Tetralogy of Fallot
4. PDA
5. TGV
6. Hypopl. L Heart
7. Coarctation
8. AV Canal Defects
9. TAPVR
9. Tricuspid Atresia
10.Truncus
25%
10
9
8
8
8
5
4
2
1.5
1
Syndromes & Cardiac Lesions

Down
DiGeorge
Ellis-van Creveld
Holt-Oram
Noonan
Rubella
Turner
Williams
ECD, tetralogy, VSD

Interrup. Ao arch, truncus
ASD, common atrium
ASD
Pulmonary stenosis
Peripheral PS, PDA
Coarctation Ao
Supravalv. Ao stenosis
References
Textbooks
1. Gedgaudas E. Cardiovascular radiology. Philadelphia, Pa: WB Saunders, 1985.
2. Tonkin, ILD. Pediatric cardiovascular imaging. Philadelphia, Pa: WB Saunders, 1992.
3. Amplatz K, Moller JH. Radiology of congenital heart disease. St Louis, Mo: Mosby, 1993.
Journal Articles
1. Alexiou C, Mahmoud H, Al-Khaddour A, et al. Outcome after repair of tetralogy of Fallot in the first year of life.
Ann Thorac Surg. 2001;71:494-500
2. Bichell DP, Geva T, Bacha EA, Mayer JE, Jonas RA, del Nido PJ. Minimal access approach for the repair of atrial
septal defect: the initial 135 patients. Ann Thorac Surg. 2000;70:115-8.
3. Coussement AM, Gooding CA. Objective radiographic assessment of pulmonary vascularity in children.
Radiology 1973;109:649-654.
4. El-Najdawi EK, Driscoll DJ, Puga FJ, et al. Operation for partial atrioventricular septal defect: a forty-year review.
J Thorac Cardiovasc Surg. 2000;119:880-889.
5. Fisher RG, Moodie DS, Sterba R, et al. Patent ductus arteriosus in adults - long term follow-up: nonsurgical versus
surgical treatment. J Am Coll Card. 1986;8:280-284.
6. Harvey JR, Teague SM, Anderson JL, et al. Clinically silent atrial septal defects with evidence for cerebral
embolization. Ann Intern Med. 1986;105:695-687.
7. Jacobs ML, Pourmoghadam KK. The hemi-Fontan operation. Semin Thorac Cardiovasc Surg, 2003;6:90-97.
8. Kreutzer C, De Vive J, Oppido G, et al. Twenty-five-year experience with Rastelli repair for transposition of the
great arteries. J Thorac Cardiovasc Surg. 2000 Aug;120:211-223.
9. Murphy JG, Gersh BJ, McGoon MD, et al. Long term outcome after surgical repair of isolated atrial septal defect.
N Engl J Med 1990;323:1645-1650.
10. Ohye RG, Bove EL. Advances in congenital heart surgery. Curr Opin Pediatr. 2001;13(5):473-481.
11. Thibeault DW, Emmanouilides GX, Nelson RJ, et al. Patent ductus arteriosus complicating the respiratory distress
syndrome in preterm infants. J Pediatr 1975; 86:120-126.
12. Williams DL, Gelijns AC, Moskowitz AJ, et al. Hypoplastic left heart syndrome: valuing the survival. J Thorac
Cardiovasc Surg. 2000;119(4 Pt 1):720-731.
1488
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Pediatric Radiology
Forensic Radiology of Child Abuse

Figure 6-16-1
Scope of the problem1: U.S. Data for the year 2000
3 million cases reported

879,000 substantiated cases
~1200 fatalities (U.S.) in the year 2000 from abuse
1.22% of all children in US are abused
1Child Maltreatment 2000: Reports from the States to the National Child Abuse
and Neglect Data System. In: US Department of Health and Human Services
Childrens Bureau (online). Available at:
http://www.calib.com/nccanch/prevmnth/scope/ncands/cfm
Common radiographic findings in abuse
Long bone fx (shaft & metaphyseal)

Rib fx
Skull fx
Subdural & subarachnoid hemorrhage
Cerebral edema
Visceral injury
Long bone shaft fracture [Figure 6-16-1]
MOST common fracture in abuse (?4x more common than metaph. fx?) when
all ages considered
Infants: metaphyseal, rib, & skull fx more common1,2
Not specific for abuse . . .
Except in the very young
Shaft fx (esp spiral) in a non-walking infant is suggestive of abuse w/o
convincing & verifiable history
Most common sites: femur, humerus
Inflicted femoral shaft

fracture
Figure 6-16-2
1Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Inflicted skeletal injury: a
postmortem radiologic-histopathologic study in 31 infants. AJR 1995; 165:647-650.
2Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-
accidental injury in children: a comparative study. Br Med J 1986; 293:100-102.
Spiral Fracture [Figure 6-16-2]
Figure 6-16-3
Developmental Milestones
4 mos
56 mos
89 mos
15 mos
18 mos
24 mos
36 mos
raises head 90
rolls over
sits alone
walks alone
climbs stairs
runs well
alternates feet up stairs
Metaphyseal Fracture1 [Figure 6-16-3]
High specificity for abuse

Also known as corner and buckethandle fractures
Most common in lower extremity
Knee
Ankle
Fracture through most immature bone
Primary spongiosa (metaphysis)
1Kleinman PK, Marks, SC, et al. AJR 1986;
146:895-905
Pediatric Radiology
Spiral humeral fracture
Metaphyseal fracture
1489
1491
Shaking mechanism [Figure 6-16-4]
From rapid, forceful acceleration and deceleration:

Shearing forces oriented perpendicular to long axis of bone
Child may be held around chest (or by limb) & shaken violently
Microfracture thru primary spongiosa
Thicker collar of metaphyseal bone at periphery
Periosteum usually normal except with extensive injury
Figure 6-16-4
Subtlest radiographic finding: metaphyseal lucency (non-specific,

seen in leukemia, stress)
Corner fracture and bucket-handle fracture are different
projections of the same fracture
Appearance depends on shape of metaphysis itself & projection
of fracture
Healing of metaphyseal fractures
Callus unusual difficult to date

eal quickly (10 days to several wks)
prompt radiography is ESSENTIAL
The younger the infant, the quicker the healing
Usually no deformity or sequela except in the unusual case of a
hip metaphyseal fracture (may cause coxa vara)
Shaking mechanism
Rib fracture [Figures 6-16-4, 6-16-6 and 6-16-7
Fracture anywhere on rib

Posterior fracture: high specificity for abuse
All locations: very suspicious
Occur with chest compression, typically during
violent shaking
Seen in 35 60% of abused infants (<18 mos)1,2
Lateral rib fracture: AP compression folds rib
laterally, causing fx
Posterior rib fracture: posterior compression levers
rib end over transverse process
Fx most pronounced at ventral cortex
Posterior compression/impact necessary for
fracture to occur
Figure 6-16-5
1 Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Lateral and frontal views of metaphyseal fracture
Inflicted skeletal injury: a postmortem radiologic-histopathologic
study in 31 infants. AJR 1995; 165:647-650.
2Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-accidental
injury in children: a comparative study. Br Med J 1986; 293:100-102.
Figure 6-16-6
Figure 6-16-7
Cross sectional diagram of chest squeeze by

adult hands
Multiple bilateral rib fractures

1490
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Pediatric Radiology
Figure 6-16-8
Visualization of posterior rib fractures1

[Figures 6-16-8 and 6-16-10]
High miss rate acutely unless oblique views or bone scan

performed
Production of callus (best at 7-14 days) aids visualization
To increase detection:
Skeletal technique chest film (NOT chest radiograph
technique)
Bone scintigraphy
Chest CT
Oblique chest films (bone technique)
Consider as part of original skel survey
Follow-up films in > 7 days
Posterior rib fractures without

evidence of healing
1Kleinman PK, Marks SC, Adams VI, Blackbourne BD. Factors affecting
Figure 6-16-9
visualization of posterior rib fractures in abused infants. AJR 1988; 150:635-638.
Rib fracture & CPR
Posterior compression NOT a

feature of CPR
Experimental studies fail to
reproduce posterior fractures
with CPR or report their
occurrence1,2,3
Rib fractures (anterior and
lateral) rarely seen after CPR in
normally mineralized bones of
infants & young children
Occult posterior rib fractures seen at scintigraphy
Figure 6-16-10
1Kleinman PK, Schlesinger AE. Mechanical factors associated with posterior rib fractures:
laboratory and case studies. Pediatr Radiol 1997; 27:87-91.
2Feldman KW, Brewer DK. Child abuse, cardiopulmonary resuscitation, and
rib fractures. Pediatrics 1984; 73:339-342.
3Spevak MR, Kleinman PK, Belanger PL, Primack C, Richmond JM.
Cardiopulmonary resuscitation and rib fractures in infants. A postmortem

radiologic-pathologic study. JAMA 1994; 272:617-618.
Healing of fractures1
No callus fx < 14 days old

Callus
fx > 7 days old
Caveat: these are general estimates and should not be thought of
as fixed time frames, as healing & callus formation are a
continuum
The younger the child, the faster the healing
CT of healing left posterior rib

fracture
1OConnor JF, Cohen J., in: Kleinman PK Diagnostic Imaging of Child Abuse, 2nd
ed. Mosby, 1998 168177.
Figure 6-16-11
Spine injury [Figure 6-16-11]
Abuse (typically shaking) results in:

Compression & superior endplate fxs at
thoracolumbar junction from hyperflexion1

Avulsion injury to interspinous ligament and
spinous process cartilage (hyperflexion
mechanism)2
Spinous process fractures are high specificity,
though unusual
1Kleinman PK, Marks SC. Vertebral body fractures in child
abuse. Radiologic-histopathologic correlates. Invest Radiol

1992; 27:715-722.
2Swischuk LE. Spine and spinal cord trauma in the battered
child syndrome. Radiology 1969; 92:733-738.
Pediatric Radiology
1491
1493
Vertebral compression fractures

Scintigraphy: Advantages
More sensitive than plain films for rib fractures

Probably slightly more sensitive for detection of some other fractures
Scintigraphy: Limitations [Figure 6-16-12]
Higher radiation dose

Insensitive for skull fxs (always need supplementary plain films of skull)
Less sensitive than plain films for the detection of metaphyseal & vertebral fx
Cant determine age or type of fracture
More technically & professionally demanding & more costly
Figure 6-16-12
Evaluation of skeletal injury
Skeletal survey with high-detail film (mammography

film or high-detail extremity film ideal)
At least 2 views of any abnormality
Do all of above even if post-mortem!
consider chest CT, scintigraphy, or repeat CXR to
visualize missed posterior rib fxs
Specimen radiography of abnormalities in postmortem child with subsequent dissection & dating
The skeletal survey (all: bone technique)
Skull fracture missed at scintigraphy
AP thorax
AP humeri
AP forearms
Oblique hands
AP feet
AP femora
AP & LAT tibiae
AP & LAT skull
AP pelvis
LAT C-spine
LAT thorax
LAT L-spine
High specificity
Metaphyseal fracture
Posterior rib fracture
Spinous process fracture
Sternal fracture
Scapular fracture
Moderate specificity
Multiple fractures, especially bilateral

Fractures of different ages
Epiphyseal separation
Vertebral body fracture & subluxation
Digital fractures
Complex skull fractures
Common, but low specificity
Clavicular fracture
Long bone shaft fracture
Linear skull fracture
Cranial injury
Leading cause morbidity & mortality

Mortality peaks at 6 months
Mechanisms:
Shaking
Direct blow
Strangulation / suffocation
Shaking alone is sufficient to cause fatal CNS injury (shearing forces)
1492
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Pediatric Radiology
In the first year of life1:

64% of all head injuries needing admission or with positive CT
findings are inflicted (excluding simple skull fracture)
95% of all serious head injuries are inflicted
Figure 6-16-13
1Billmire ME, Myers PA. Serious head injury in infants: accident or abuse?
Pediatrics 1985; 75:340-342.
Specific injuries [Figures 6-16-13 and 6-16-14]
Edema
Most common, but non-specific as to mechanism (blow,
strangulation, post-traumatic apnea, etc)
Shear injury at grey-white junction and in large WM tracks (c.
callosum)
Cortical contusion, laceration
SAH & SDH (when interhemispheric, very worrisome for abuse)1
1Zimmerman RA, Bilaniuk LT, Bruce D, Schut L, Uzzell B, Goldberg HI.
Computed tomography of craniocerebral injury in the abused child. Radiology

1979; 130:687-690.
Interhemispheric extra-axial hemorrhage
Left cerebral edema and SDH
Figure 6-16-14
[Figures 6-16-15 to 6-16-17]
Blood adjacent to falx, usually asymmetric & posterior

Difficult to distinguish SAH from SDH here
From violent shaking: hemispheres impact falx, on rebound
bridging veins to sagittal sinuses are torn
May be associated with posterior convexity and tentorial
hematomas
Interhemispheric extraaxial hemorrhage may be difficult to
distinguish from normal falx (caveat: normal falx may appear
strikingly bright in presence of global cerebral edema)
Thicker than expected
Irregular thickness
Asymmetric thickness
Extension into a posterior convexity SDH
Figure 6-16-15
Left frontal white matter tear
Figure 6-16-16
Diagram of SDH
Figure 6-16-17
Interhemispheric hemorrhage
Interhemispheric and left tentorial

extraaxial hemorrhage
Pediatric Radiology
1493
1495
Figure 6-16-18
The reversal sign1,2 [Figure 6-16-18]

AKA the bright cerebellum sign
Diffuse cortical and subcortical WM edema

Basal ganglia, thalami, brainstem, & cerebellum retain normal
attenuation so appear bright
Dismal prognosis: hemispheric injury may go on to multicystic
encephalomalacia, atrophy
1Han BK, Towbin RB, De Courten-Myers G, McLaurin RL, Ball WS, Jr.
Reversal sign on CT: effect of anoxic/ischemic cerebral injury in

children. 1990; 154:361-368.
2Harwood-Nash DC. Abuse to the pediatric central nervous system.
AJNR 1992; 13:569-575.
The reversal sign
Basal ganglia, thalami, brainstem, & cerebellum increased

relative attenuation may be due to
Neovascularity
Neuronal preservation
Vascular engorgement
Mineralized neurons
Petechial hemorrhages
Cerebral edema, parenchymal

hemorrhage, and extraaxial
hemorrhage
Cerebral edema
Not specific for abuse

May be seen in:
Drowning
Non-abusive head trauma
When abuse-related, may be from:
Direct brain injury
Strangulation
Venous pressure from chest compression
Post-traumatic apnea
Sequellae
CT
Acute
Polytrauma
MRI
Delayed presentation
Differing ages of injuries/blood collections
Normal or equivocal CT with high suspicion (better than CT for
shear and SDH)
Sequela
Dating of intracranial blood1

1Bradley WG, Radiology 1993 189: 15
Stage
Form
T1 MR
T2 MR
hyperacute (<12-24 hrs) oxy Hb
iso-low
high (slightly)
acute (1-3 days)
iso-low
low
deoxy Hb
early subacute (3-7 days) intracell met Hb high
low
late subacute (1-2 wks)
extracell met Hb high
high
chronic (>2 wks)
ferritin,
hemosiderin,
approaches
CSF with time
iso >>>> low high in center

low in rim
1494
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Pediatric Radiology
Hemorrhage dating by MR [Figure 6-16-19]
Figure 6-16-19
An inexact science
Affected by
Technical aspects MR scanner, sequences
Concentration of Hb
Relative concentrations of degradation products
(a continuum)
Arachnoid tear with leak of CSF into SDH
Rebleeding in a Chronic SDH
Outer membrane itself shown to bleed frequently1

Elevated tissue plasminogen activator (TPA) found in
chronic SDH fluid1
Little (if anything) in the literature to support a single, significant
rebleed as cause of rapidly enlarging SDH2
No documented cases of significant rebleed occurring in safe
environment
Right SDH
Figure 6-16-20
1Chen JC, Levy ML. Causes, epidemiology, and risk factors of chronic
subdural hematoma. Neurosurg Clin N Am 2000; 11:399-406.

2Block RW. Child abuse--controversies and imposters. Curr Probl Pediatr
1999; 29:249-272.
Sinus Thrombosis & Infarct [Figure 6-16-20]

Cord injury
In 9 of 11 abuse fatality autopsies in infants1,2

Subarachnoid
Subdural
Epidural
4 of 6 with ventral cord contusion
Probably overestimates real incidence
1Hadley MN, Sonntag VK, Rekate HL, Murphy A. The infant whiplash-shake
injury syndrome: a clinical and pathological study. Neurosurgery 1989;

24:536-540.
2Feldman KW, Weinberger E, Milstein JM, Fligner CL. Cervical spine MRI in
Right transverse sinus thrombosis
abused infants. Child Abuse Negl 1997; 21:199-205.
Figure 6-16-21
Skull fracture [Figure 6-16-21]
Overall, poorly correlated with CNS injury

(linear fx most common in NAT)
10% of all abuse cases have skull fx
Non-specific (altho common) unless:
Stellate / eggshell
Multiple
Diastatic (>3 mm)
Occipital
Inconsistent history (rolled off changing table)
Skull fracture from falls & stairs
Falls:
In 529 falls from heights up to 150 cm, 4 skull, 4 clavicle, 1
Diastatic eggshell skull fractures
humerus (1.7% incidence) fractures and no significant
neurologic injuries occurred
Conclusion: household falls rarely associated with fx,
almost never with any intracranial injury1,2,3
Stairs: more injurious, though significant injuries usually single (not multiple
body parts)4,5
1Helfer RE, Slovis TL, Black M. Injuries resulting when small children fall out of bed.
Pediatrics 1977; 60:533-535.
Pediatric Radiology
1495
1497
2Nimityongskul P, Anderson LD. The likelihood of injuries when children fall out of bed. J
Pediatr Orthop 1987; 7:184-186.

3Lyons TJ, Oates RK. Falling out of bed: a relatively benign occurrence. Pediatrics 1993;
92:125-127.
4Joffe M, Ludwig S. Stairway injuries in children. Pediatrics 1988; 82:457-461.
5Chiaviello CT, Christoph RA, Bond GR. Stairway-related injuries in children. Pediatrics
1994; 94:679-681.
Visceral Injury
Seen in all ages of abused children

Usually blunt trauma (punch or kick to abdomen, rapid deceleration after being
thrown)
50% mortality rate of clinically apparent visceral injury
Delay in seeking treatment pivotal!
Estimated to account for 12% of all abuse fatalities
2-4% of all abusive injuries
Small Intestine [Figure 6-16-22]
Hematoma (prox SB), perforation (distal SB)

Intramural hematoma duod & proximal jejunum most common
abdominal injury
90% in duod & prox jejunum, most near ligament of Treitz
? is bowel most tethered most vulnerable?
Pt presents with pain, vomiting
Mural, asymmetric mass on UGI
Coiled spring appearance of heaped-up proximal mucosa
+/- free air if perforation
Sepsis
Bowel Injury
Figure 6-16-22
Duodenal hematoma
Pancreas
Probably compressed against spine with blunt trauma

Abuse is a common cause of pancreatitis (trauma is most
common cause in children)
May develop pseudocysts
Figure 6-16-23
Pancreatic Injury Radiography [Figures 6-16-23 and 6-16-24]
Ascites
Decreased pancreatic echogenicity at US, ductal dilation
Peripancreatic inflammation
Other abdominal injuries [Figure 6-1-25]
Liver: contusion, laceration

Kidney: contusion, laceration, fx
Bladder: rupture
Adrenal: hemorrhage
Pancreatic laceration
Figure 6-16-24
Figure 6-16-25
Pancreatitis
Liver contusion
1496
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Pediatric Radiology
Hypoperfusion complex
Figure 6-16-26
Severe abdominal injury resulting in hemodynamic instability

Imaging:
Small caliber Ao and IVC (intravascular volume depletion)
Fluid-filled bowel with enhancing wall (concentrated contrast)
Ascites
Intensely enhancing kidneys
Shock Bowel (Hypoperfusion Complex) [Figure 6-16-26]

Differential diagnosis of abuse injuries
Birth trauma: clavicle & humerus fxs

Normal variant: periosteal new bone in infants 28 mos of life
Hypoperfusion complex
(single layer, smooth, bilaterally symmetric)
Osteogenesis imperfecta: fxs, periosteal rxn
(should also see osteopenia, +/ wormian bones, blue sclera)
Rickets: widened, irregular physes with metaphyseal irregularity
(look for osteopenia)
Scurvy: white metaphyseal line of Frankel with sub-metaphyseal lucency,
spurs, periostitis (look for osteopenia, Wimberger ring around epiphyses)
Vit A intoxication: periosteal rxn
(pronounced periostitis in ulnae & tubular bones of hands & feet)
Caffeys (presumed viral illness of infancy rarely seen today):
periostitis mandible, clavicle, ulna, irritable, febrile child < 6 mos old
Syphilis: metaphyseal lucencies, periostitis
(destructive metaphyseal osteomyelitis lesions), + serology
Leukemia: metaphyseal lucency
(see lucent bands symmetrically, osteopenia)
Menkes kinky hair: metaphyseal spurring, flared anterior rib ends
(no true metaphyseal fx, findings bilateral & symmetric, characteristic hair)
Remember to consider:
congenital insensitivity to pain
spinal dysraphism
Child abuse
We as radiologists are uniquely able to diagnose abuse

We may be the 1st to recognize abuse
Radiographic findings in abuse are among the most specific & diagnostic in medicine
Our findings may be PIVOTAL to investigation & prosecution
References
1.
2.
3.
4.
Billmire ME, Myers PA. Serious head injury in infants: accident or abuse? Pediatrics 1985; 75:340-342.
Block RW. Child abuse--controversies and imposters. Curr Probl Pediatr 1999; 29:249-272.
Bradley WG. MR appearance of hemorrhage in the brain. Radiology 1993 189: 15-26
Chen JC, Levy ML. Causes, epidemiology, and risk factors of chronic subdural hematoma. Neurosurg Clin N Am
2000; 11:399-406.
5. Chiaviello CT, Christoph RA, Bond GR. Stairway-related injuries in children. Pediatrics 1994; 94:679-681.
6. Child Maltreatment 2000: Reports from the States to the National Child Abuse and Neglect Data System. In: US
Department of Health and Human Services Children's Bureau (online). Available at:
http://www.calib.com/nccanch/prevmnth/scope/ncands/cfm
7. Feldman KW, Brewer DK. Child abuse, cardiopulmonary resuscitation, and rib fractures. Pediatrics 1984; 73:339342.
8. Feldman KW, Weinberger E, Milstein JM, Fligner CL. Cervical spine MRI in abused infants. Child Abuse Negl
1997; 21:199-205.
9. Hadley MN, Sonntag VK, Rekate HL, Murphy A. The infant whiplash-shake injury syndrome: a clinical and
pathological study. Neurosurgery 1989; 24:536-540.
10. Han BK, Towbin RB, De Courten-Myers G, McLaurin RL, Ball WS, Jr. Reversal sign on CT: effect of
anoxic/ischemic cerebral injury in children. 1990; 154:361-368.
11. Harwood-Nash DC. Abuse to the pediatric central nervous system. AJNR 1992; 13:569-575.
12. Helfer RE, Slovis TL, Black M. Injuries resulting when small children fall out of bed. Pediatrics 1977; 60:533-535.
Pediatric Radiology
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13. Joffe M, Ludwig S. Stairway injuries in children. Pediatrics 1988; 82:457-461.

14. Kleinman PK, Marks SC, Adams VI, Blackbourne BD. Factors affecting visualization of posterior rib fractures in
abused infants. AJR 1988; 150:635-638.
15. Kleinman PK, Marks SC, Blackbourne B. The metaphyseal lesion in abused infants: a radiologic-histopathologic
study. AJR 1986; 146:895-905.
16. Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Inflicted skeletal injury: a postmortem radiologichistopathologic study in 31 infants. AJR 1995; 165:647-650.
17. Kleinman PK, Marks SC. Vertebral body fractures in child abuse. Radiologic-histopathologic correlates. Invest
Radiol 1992; 27:715-722.
18. Kleinman PK, Schlesinger AE. Mechanical factors associated with posterior rib fractures: laboratory and case
studies. Pediatr Radiol 1997; 27:87-91.
19. Lyons TJ, Oates RK. Falling out of bed: a relatively benign occurrence. Pediatrics 1993; 92:125-127.
20. Nimityongskul P, Anderson LD. The likelihood of injuries when children fall out of bed. J Pediatr Orthop 1987;
7:184-186.
21. OConnor JF, Cohen J., in: Kleinman PK Diagnostic Imaging of Child Abuse, 2nd ed. Mosby, 1998 168-177.
22. Spevak MR, Kleinman PK, Belanger PL, Primack C, Richmond JM. Cardiopulmonary resuscitation and rib
fractures in infants. A postmortem radiologic-pathologic study. JAMA 1994; 272:617-618.
23. Swischuk LE. Spine and spinal cord trauma in the battered child syndrome. Radiology 1969; 92:733-738.
24. Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-accidental injury in children: a
comparative study. Br Med J 1986; 293:100-102.
25. Zimmerman RA, Bilaniuk LT, Bruce D, Schut L, Uzzell B, Goldberg HI. Computed tomography of craniocerebral
injury in the abused child. Radiology 1979; 130:687-690.
1500
Pediatric Radiology
Neonatal Brain: Radiologic Evaluation

Dorothy I Bulas, MD
Learning Objectives
Review the differences in cranial anatomy of the preterm and term infant
Review hemorrhagic (IVH) & nonhemorrhagic (PVL) injuries due to partial
asphyxia.
Understand radiologic work up of hypoxic ischemic injury of the neonate
including neurosonographic techniques (transmastoid view, Doppler)
Figure 6-17-1
Sonographic Technique
Anterior fontanelle
Coronal and sagittal planes
Axial views
Posterior and mastoid fontanelle
Doppler
MCA/ACA resistive index
Color Doppler of fluid collections
Posterior Fontanelle [Figure 6-17-1]

Mastoid Fontanelle Scanning
Ultrasound image via the posterior

fontanelle demonstrates the occipital
horn filled with choroid
24/200 patients with PF abnormality

Mastoid view:
Improved visualization (96%)
Increased diagnostic confidence (75%)
Only technique to show abnormality (46%)
Figure 6-17-2
Luna & Goldstein, AJR 2000; 174
Transmastoid view -Trapped fourth ventricle

[Figure 6-17-2]
Sagittal Sinus Thrombosis [Figure 6-17-3]

Resistive Index MCA or ACA
Peak Systole End Diastole
Peak Systole
Minimize affect of angulation
Age dependent values
Term infants 0.7 + 7%
By age 2
0.5+ 15%
An increase in diastolic flow results in decrease RI
A decrease in diastolic flow results in increase RI
As ICP increases above mean arterial pressure, diastolic flow
reverses w/ RI > 1.0.
Transmastoid view demonstrates

lateral and fourth ventriculomegaly
Figure 6-17-3
Normal Anatomy: Premature Brain
Preterm cerebral vessels penetrate from meninges to

periventricular walls
single vessel walls
Term cerebral vessels-watershed at cortex
smooth muscle AA, collagen VV
Nelson et al AJNR 1991:215

Longitudinal ultrasound via the
anterior fontanelle demonstrates
irregular flow of the sagittal sinus
consistent with sagittal sinus
thrombosis
Pediatric Radiology
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Neonatal Brain
Neonatal Hypoxic-Ischemic Injury
Figure 6-17-4
Injuries are the result of partial or global asphyxia due to various

causes: intrauterine, sepsis, apneic episodes
Partial asphyxia: IVH, PVL
Global asphyxia: multifocal ischemic necrosis
Injury patterns: Term infant [Figure 6-17-4]
Prolonged Partial Asphyxia

Watershed infarction (ACA-MCA-PCA; parasagittal) - cortex
/ white matter
Profound asphyxia:
Basal ganglia,
Rolandic cortex
Germinal Matrix
Origin of neuronal/glial development.

Rich arterial supply perforators of ACA, MCA, PCA.
Drain to deep venous system.
Regression posterior to anterior b/w 24-28 wks
Involutes by 32 wks
Axial CT demonstrates diffuse cortical

infarction in a term infant with severe
hypoxic ischemic injury
Germinal Matrix Hemorrhage
Most common origin of preterm hemorrhages

20% of preterms
<1500 gms
Venous origin
Capillaries and sinusoids in caudothalamic groove with poor stromal support

Vessels converge to form draining veins
Theory- increased flow vv rupture at convergence points.
Nakamura et al Mod Path 1991;475
Germinal Matrix Hemorrhage: FACTORS
Asphyxia-abolishes autoregulationCBF varies with SBP > injury to endothelium of germinal matrix vessels.
Hypoxia- decrease myocardial energy reserve > circulatory failure >
hypotension > cerebral ischemia > venous hypertension
Funato M, Munksgard 1994:456; Milligan D Lancet 1980:26:896
O2 delivery reduced w/ hemorrhage.

Switch from aerobic to anaerobic metabolism
Increase lactate formation.
Blood ruptures into ventricles
Dilatation,
Disrupt ependymal lining
Figure 6-17-5
Modified Papile Classification
Grade I
Subependymal hemorrhage
Grade II
Intraventricular blood w/ no/min ventricular
dilatation
Grade III
Intraventricular blood w/ prominent
ventricular dilatation
Grade IV/PHI
Parenchymal hemorrhage associated with
IVH
Sagittal US demonstrates a Grade 1

subependymal hemorrhage
Grade I IVH [Figure 6-17-5]
Neonatal Brain
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Pediatric Radiology
Grade II IVH w/out dilatation [Figure 6-17-6]
Figure 6-17-6
Grade II IVH w/ distended

ventricle [Figure 6-17-7]
Grade III - IVH w/ distended
ventricle [Figure 6-17-8]
Periventricular Hemorrhagic
Infarction [Figure 6-17-9]
Figure 6-17-9
Coronal and sagittal US images demonstrate Grade II
intraventricular hemorrhage with blood extending into the
ventricle
Figure 6-17-7
Coronal sonogram demonstrates a

heterogeneous region in the right
parietal periventricular white matter
consistent with a periventricular
hemorrhagic infarction and
ventriculomegaly
Coronal US and axial CT images of Grade III

intraventricular hemorrhage with blood filling and
dilated the ventricles
Periventricular Hemorrhagic
Infarction (PHI/ Grade IV IVH)
Figure 6-17-8
15% with IVH dev PHI

Same side as IVH, after IVH has occurred
Usually unilateral or asymmetric
Periventricular Hemorrhagic Infarction (PHI/ Grade IV

IVH)
Hemorrhagic necrosis in PVWM dorsal/lat of lateral ventricle

where medullary veins confluent.
Periventricular Hemorrhagic Infarction
PET scans demonstrate parenchymal ischemia beyond

boundaries of hemorrhage
Further injury may be due to accumulation of metabolic toxins due
to impaired blood flow
Volpe JJ. Neurology of the Newborn 1987
Periventricular Hemorrhagic Infarction Sonography

Time
Pathology
Early
hemorrhagic infarcts
PVWM
surrounding ischemia
Subacute cystic cavities
diminished myelin
Chronic
porencephalic cyst
gliosis
Pediatric Radiology
Coronal sonogram demonstrate

dilated ventricles filled with
hemorrhage consistent with an
evolving grade III intraventricular
hemorrhage
US findings
echogenic areas
mass effect
retraction of clot,
porencephalic cyst
ventricular debri/dilat
porencephalic cyst
atrophy
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Neonatal Brain
IVH: Sonography
Figure 6-17-10
US very useful -lesions well visualized deep in the brain

Difficult to make diagnosis clinically
Screening recommendations
Preterms <32 wks
Birth wt <1.5 kg
?When- majority of bleeds occur 3-4 days after birth.
First screening US 4-7 days after birth
IVH ? CT/MRI
Difficult transporting unstable preterms

CT may miss bleed after several days
MRI - Hemosiderin staining can be seen up to 1 yr
May demonstrate additional abnormalies
Focal WM loss
Diffuse hypoxic-ischemic changes
Pulsed Doppler demonstrates no flow

during diastole with resistive index of 1
IVH/PVH: Follow up [Figure 6-17-10]
Doppler useful to differentiate HC /atrophy

RI >.8 sign of increased ICP.
IVH/Periventricular Hemorrhagic Infarction: Outcome
Normal US
Grade 1
Grade 2
Grade 3
PHI
90% nl neurologic outcome

50-70% nl outcome, 8% mortality
90-100% major motor deficit,
64% dec cognitive function,
60% mortality
IVH: Outcome
3 mechanisms for poor outcome

1. Damage from ventricular dilatation-may be reversible if shunted
2. Shunt related complications: infection, sepsis, obstruction, seizures.
3. Hypoxic ischemic injury-white matter edema, gliosis, axonal swelling
Spastic hemipareses, intellectual deficits.
Prognosis parallels size of parenchymal echodensity
Periventricular Leukomalacia: Etiology
?Arterial ischemia?
Size of zones decrease with gestational age
Older infants w/PVL - strong hx of hypotension
Very premature infant-no hx of hypotension
?Cytokine response?
Maternal chorioamniotitis assoc. w/ PVL
?fluctuation in cerebral blood flow
?Inflammatory cytokine response to infection
Prevalence in preterms before 25%
Now reported as low as 7%.
Yet reports of increasing survival of very low weight preterms with increase
rate of CP
?are we accurately identifying PVL sonographically?
Size of zones decrease w/ gestational age
Older infants w/PVL - strong hx of hypotension
Very premature infant-no hx of hypotension
Resultant injury of PVL > than border zones
?Intrinsic vulnerability of periventricular glial cells and intrinsic metabolic
properties.
O2 reduced >lactic acid accumulation
Neonatal Brain
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Pediatric Radiology
Glial cells are differentiating to astrocytes & oligodendroglia with active

myelination. Intense metabolic activity w/ high O2 demandsvulnerable to hypoxia [Figure 6-17-11]
Figure 6-17-11
Periventricular Leukomalacia:Sonography
Infarction occurs in deep cerebral WM

Bilateral symmetric
US not sensitive in detecting PVL
Difficult to distinguish from anisotropic effect of periventricular
halo
Echodensities resolve and can be missed
Periventricular Leukomalacia:Sonography
Time
Pathology
US Findings
Acute
1-3 days
focal necrosis
petechial hemor
patchy areas of inc

echogenicity PVWM
Subacute
1-2 wks
2-3 wks
diminished myelin
cystic cavities
decreasing echogenicity
may be normal
small cysts Swiss cheese
Chronic
cysts disappear
gliosis
cysts disappear
vent dilatation
Coronal sonogram demonstrates

multiple cysts in the periventricular
white matter consistent with
periventricular leukomalacia
Periventricular Leukomalacia
Less severe PVL-diminished myelin result in dilated ventricles.

Severe PVL will cavitate
Cysts eventually resolve -gliotic scarring
Figure 6-17-12
Periventricular Leukomalacia:CT
[Figures 6-17-12 and 6-17-13]
Difficult transporting premature infants

Due to high water content, difficult differentiating acute PVL from nl
preterm brain
Chronic: CT useful extent of lesions, atrophy
Irregular lateral ventricles
Prominent sulci
Subcortical gray matter abuts ventricles
Small cysts missed
Figure 6-17-13
Axial CT image demonstrates

diffuse periventricular and cortical
edema with intraventricular
hemorrhage
Axial image demonstrates

ventricular dilatation with irregular
walls and marked loss of
periventricular white matter
Pediatric Radiology
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Neonatal Brain
Periventricular Leukomalacia: MRI
Figure 6-17-14
[Figure 6-17-14]
Acute/subacute: dec. sig T1, inc. sig T2 in PVWM

High water content makes edema difficult to detect on T1, due to
delayed myelination, high signal on T2 apparent. Periatrial gliosis;
involves the subependymal periatrial white matter (tapedum)
Distal corpus callosum (pre-splenium) thinned
Periventricular Leukomalacia:Outcome
Outcome correlates with cystic cavitation

PVWM traversed by fibers of motor cortex results in spastic
diplegia
Lower> Upper extremities
Infants with larger lesions often delayed
Nonhemorrhagic Infarction
Rare, term infants

90% major handicaps
Perinatal asphyxia
Emboli
Congenital Heart disease
Meningitis
Polycythemis
Axial T2w image demonstrates high

signal in the periventricular white
matter
Profound Asphyxia
Energy requirements related to state of myelination.

Most metabolically active and mature regions with most advanced
Figure 6-17-15
myelination,
perfusion
glucose uptake
are regions that suffer the most damage
Complete arrest - injury determined by
metabolic maturity of brain
myelination
autoregulation
watershed pattern
excitatory neurotransmitter release
Coronal US demonstrates diffuse increased
severity and duration of event
echogenicity of the cerebral cortex with slit
ventricles
Profound Asphyxia: Sonography
Anterior cerebral artery Resistive Index < .5
Diffuse hypoxic ischemic encephalopathy often
consistent with arterial vasodilatation due to loss
superimposed on IVH/PVL
of sutoregulation
Profound Asphyxia in Preterms
Preterm have low O2 demand and immature cardiopulmonary control

Neuronal injury can occur in ventral pons, inferior olivary nuclei, subiculum of
hippocampus
Unlike term infant, less involvement of basal ganglia, thalami, brainstem
PVWM injury dominating
Profound Asphyxia in Term Infants
Involvement of basal ganglia, brainstem

hippocampus
posterior and medial lentiform nuclei
lateral thalami
cortical gyri
Profound asphyxia Term: Sonography [Figure 6-17-15]
Acute - increased echogenicity

small vents/sulci
Doppler - Loss of autoregulation RI < .6
Neonatal Brain
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Pediatric Radiology
Figure 6-17-16
Profound asphyxia: CT [Figure 6-17-16]
Acute-low attenuation cortex, basal ganglia, periventricular white

matter, laminar necrosis
Chronic-diffuse atrophy prominent extraaxial fluid spaces and
sulci
Profound asphyxia:
MRI findings in Preterm Infants
PVWM changes dominate

T1,T2 shortening in peritrigonal WM
T1,T2 prolongation in cerebral WM
Chronic - atrophy
Relative sparing of cerebral cortex
Hippocampal and brainstem atrophy
Thin corpus collosum
Profound asphyxia: MRI findings in term infants
Short T, at times short T2 relaxation times

Basal ganglia
Hippocampus
Posterior and medial lentiform nuclei
Lateral thalami
Cortical gyri
Axial CT images demonstrate low

attenuation of the cortex, basal
ganglia as well as periventricular
white matter.in this term infant
following profound asphyxia
Figure 6-17-17
Cerebellar Infarction [Figure 6-17-17]
Cerebellum felt to be less vulnerable to anoxic damage - sparing

during hypoxic ischemic episodes
May not be rare in preterms
Due to echogenicity of CBL, infarction and hemorrhage easily
missed US
Most common watershed distribution - between the superior
cerebellar artery and the PICA
Can be lobar /holohemispheric;
+/- hemorrhagic
Most in early premature (28-32) with hx of hypotension
Mercuri Ped Rad 1997;27:139

Tsuru Acta Neuropath 1995:90;400
US- Posterior Fossa Hemorrhages [Figures 6-17-18 and 6-17-19]
Coronal MRI demonstrates right

cerebellar infarction in a child with a
history of prematurity
Easy to miss
Close evaluation of sagittal view
Additional views
transmastoid view
posterior fontanelle
Figure 6-17-19
Figure 6-17-18
Lateral image of the

skull demonstrates
where the
transducer is placed
for imaging via the
mastoid .B. Axial
sonogram
demonstrates a
heterogenous lesion
in the posterior fossa
consistent with a
posterior fossa
hemorrhage
Sagittal midline image of a normal brain clearly

demonstrates the vermis (arrow). B. Sagittal
midline image demonstrates a heterogeneous
region in the posterior fossa with no visualization
of the vermis in an infant with a posterior fossa
hemorrhage
Pediatric Radiology
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Neonatal Brain
Conclusions
Neurosonology is an integral part of care in the neonate.

Type of hypoxic injuries vary with gestational age
US sensitive for IVH screening, less sensitive for identifying PVL,
infarcts and posterior fossa hemorrhages
Flexibility in technique Doppler, transmastoid view- important
in identifying subtle anomalies
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Barkovich AJ, Sargent SK: Profound asphyxia in the premature infant: imaging findings AJNR 1995;16;1837.
Benson JE et al. Intracranial Neonatal Neurosonography: An Update. Ultrasound Quarterly 2002: 18;89
Boo NY et al. Early cranial US changes as predictors of outcome during first yr of life. J Ped Child Healthy 2000;36:363
Bulas DI, Taylor GA, Fitz CR, Revenis ME, Glass P, Ingram JD. Posterior fossa intracranial hemorrhage in infants
treated with extracorporeal membrane oxygenation: Sonographic findings. AJR 1991; 156:571.
Bulas DI. Vezina G: Anoxic injury in the Preterm infant Radiologic evaluation Radiologic Clinics of North America,
Vol 37, Nov 1999:1147.
Bulas DI. TCD: Practical Applications in Pediatrics. Applied Radiology 1999, April 7-15.
Chadduck WM, Duong DH Kast JM et al: Pediatric cerebellar hemorrhage. Child Nerv Syst 1995;110:579.
Perlman JM, Rollins N: Surveillance protocol for the detection of intracranial abnormalities in premature neonates. Arch
Pediatr Adol Med 2000;154:822.
Rumack CM et al. Timing and course of neonatal intracranial hemorrhage using US. Radiology 1985:154:101
Rumack C, Drose J. Neonatal and Infant Brain Imaging ed Rumack et al. Diagnostic Ultrasound. Elsevier Mosby
2005
Taylor GA. Recent advances in neonatal cranial ultrasound and Doppler techniques. Clin Perinataol 1997;24:677
Seibert JJ et al. Use of power Doppler in pediatric neurosonography: a pictorial essay. Radiographics 1998;18:879
Volpe JJ: Neurobiology of periventricular leukomalacia in the premature infant Pediatr Res 2001;50:553-562
Vohr B, Allan WC, Scott DT et al: Early onset IVH in preterm neonates: Incidence of neurodevelopmental handicap.
Semin Perinatol 1999;23:212.
Neonatal Brain
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Pediatric Radiology
Pediatric Liver Tumors

Pediatric Liver Tumors: GOALS
Types and presentations

Pathologic features
Clinical features
Imaging
Therapeutic implications/correlation
Pediatric Liver Tumors: ABCs of Liver Tumors
Age of patient
Biologic imaging features
Chemistry-blood
Alpha-fetoprotein
Endothelial growth factor
Categorization
Benign Epithelial Tumors

Benign Mesenchymal Tumors
Malignant Epithelial Tumors
Malignant Mesenchymal Tumors
Metastases
Benign Epithelial Tumors

Hepatic cysts
Benign Mesenchymal Tumors
Mesenchymal Hamartoma
Hemangioma
Malignant Epithelial Tumors
Hepatoblastoma
Malignant Mesenchymal Tumors
Undifferentiated embryonal sarcoma

Embyronal Rhabdomyosarcoma
Angiosarcoma
Metastases
Neuroblastoma
Burkitts Lymphoma
Sarcomas
Wilms tumor
Other
Mesenchymal tissue, disorganized bile ducts, hepatocytes, fluid filled spaces

Developmental disturbance:
Portal/biliary obstruction, lymphangiomatous tissue
Pediatric Radiology
1509
Mesenchymal Hamartoma: Pathology [Figures 6-16-1 and 6-16-2]
Multicystic, variable size: 225cm

1530% pedunculated
80% right lobe, slow growing- fluid accumulation
Sharply demarcated, lobulated
Mesenchymal stroma, hepatocytes, cysts:
Biliary/lymphangiomatous origin (Portal tract)
Figure 6-18-1
Figure 6-18-2
Hepatic mesenchymal hamartoma

with smoothly marginated nonaggressive cysts within a fibrous
stroma
Figure 6-18-3
Photomicrograph with fibrous stroma

and cysts predominantly lined by flat
vascular endothelium from the
lympatic component of the
hamartoma
Mesenchymal Hamartoma: Clinical

Enlarging abdominal mass
Child < 2 yrs old
Can Dx in utero
Otherwise asymptomatic
Negative alpha-fetoprotein
Sonography with smooth walled cysts

with thin septations, similar to those
seen in lymphatic malformations
Mesenchymal Hamartoma: Imaging

[Figures 6-18-3 to 6-18-6]
Figure 6-18-5
Hepatomegaly, abdominal mass

US: Multilocular cysts, hypoechoic
Less frequent-solid mass with few cysts
Rare to find hemorrhage, Ca +2
MR: Cysts-low T1, high T2 signal
Figure 6-18-4
CT with few macrocysts in the

mesenchymal hamartoma
Figure 6-18-6
Sonography demonstrating
microcystic component in the
hamartoma
Cystic mesenchymal hamartoma with single

macrocyst with normal adjacent liver
1508
1510
Pediatric Radiology
Mesenchymal Hamartoma: Treatment
Figure 6-18-7
SURGICAL
Enucleation, partial hepatectomy
Incision/drainage of cysts
Marsupialize large cyst(s)
Age: Young (less than 3y/o)

Biologic imaging: Cystic
Chemistry: Normal alpha-fetoprotein
Infantile Hemangioendothelioma
Most common hepatic tumor- first year of life

Usually Dx in first few months of life
Female predominance 1.5:1
Growing mass, clinically symptomatic
Vascular, high flow, mass-stroma
History of involution in first year, if survivor
Large vascular spaces in

hemangioendothelioma with normal
adjacent liver
Figure 6-18-8
Infantile Hemangioendothelioma: Pathology

[Figures 6-18-7 and 6-18-8]
Solitary, may be multicentric

0.215 cm diameter
May be well demarcated, no capsule
Dilated vascular spaces- anastomosing,+ stroma
Central necrosis/fibrosis, hemorrhage, Ca2+
Evidence of regression
Infantile Hemangioendothelioma: Clinical
Infant, usually less than 2 months

Abdominal mass, CHF, Kasabach/Merritt, DIC
Occasionally asymptomatic hepatomegaly
Massive hemoperitoneum
Multiple cutaneous angiomas-40%
Normal alpha-fetoprotein (positive EGF)
Infantile Hemangioendothelioma: Imaging
Top micrograph with small tight

vascular spaces in a
hemamgioendothelioma from a child
with no heart failure. Bottom
specimen from a child with high
output failure from left-to-right
shunting in the
hemangioendothelioma; note the
large vascular spaces associated
with arteriovenous anastomoses
[Figures 6-18-9 to 6-18-15]

US: Heterogeneous, speckled Ca2+
High flow, venous/arterial: A-V shunts
Aortic caliber decrease-after celiac artery
CT: Hypodense, 40% Ca2+, periph. enhancement
MR: Low T1, High T2 signal, +/ hemorrhage
Angio: Vascular, A-V shunting, large celiac A.
Figure 6-18-9
Flow-failure chest radiographic image

from left-to-right shunt at the level of
the liver in patient with hepatic
hemangioendothelioma
Pediatric Radiology
Figure 6-18-10
Large vascular spaces in

hemangioendothelioma prior to color
flow and duplex Doppler interrogation
1509
1511
Figure 6-18-11
Figure 6-18-12
Decrease in abdominal aortic caliber

from left-to-right shunting of blood at
the level of the celiac artery in patient
with hemanioendothelioma
Figure 6-18-13
Diffuse hepatic involvement of
hemangioendothelioma
Figure 6-18-14
Multiple discrete liver hemangiomas
in patient who is clinically
asymptomatic
Figure 6-18-15
Draping peripheral feeding vessels in

liver hemangioma
Dynamic CT with centripetal contrast

enhancement of liver multiple
hemangiomas
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Pediatric Radiology
Infantile Hemangioendothelioma: Treatment
Figure 6-18-16
Spontaneous involution in 1218 months

CHF- embolotherapy with interferon
Interferon-alpha 1, 2a, 2b response in weeks
Embolize hepatic artery- coils, balloons PRN
A-V shunts
Particles (large) where possible
May couple with surgery PRN
Age: Young (Infants)

Biologic imaging:
Vascular-High flow state
Solid stroma
Chemistry: Normal alpha-fetoprotein
Positive endothelial growth factor
Photomicrograph of hepatoblastoma
demonstrating cords and nests of
malignant cells
Figure 6-18-17
Hepatoblastoma
Most common primary liver tumor in childhood

Third most common abdominal malignancy
After neuroblastoma, Wilms tumor
May be familial
Associations: Trisomy 18, Beckwith-Wiedemann,
hemihypertropy, familial polyposis, exposure- metals,
petroleum products, paints, oral contraceptives, Fetal
Alcohol Syndrome
Hepatoblastoma: Pathology- Gross
Solitary 80%, right lobe predominance

May be lobulated
520 cm
Nodular with fibrous bands throughout
Fleshy; +/ hemorrhage, necrosis, Ca2+
Rare diffuse infiltration, adjacent liver-normal
Slide left with right hepatic lobe nodular solid

hepatoblastoma in an otherwise normal liver.
Slide right with same tumor demonstrating the
typical nodular appearance in the gross specimen
Hepatoblastoma: Pathology-Histologic [Figure 6-18-16]

Epithelial
Fetal 30%, Pink-cytoplasm, sparse mitoses
Embryonal 20%- blastemic appearance (blue-H&E)
Macrotrabecular- cords of tumor cells- 3%
Small cell- 3%
Mixed epithelial-mesenchymal- 45%; Teratoid
osteoid differentiation, also muscle, cartilage
AFP stain positive
Figure 6-18-18
Hepatoblastoma: Clinical
Abdominal mass, hepatomegaly

Anemia
Child 90% < 5 yrs old, 6570% under 2 yrs
Males> females 2:1
Positive alpha-fetoprotein
No prior history of liver disease
Hepatoblastoma: Imaging
[Figures 6-18-17 to 6-18-19]
Hepatomegaly, abdominal mass, 1015% Ca2+

US: Heterogeneous, predominantly solid
CT: Heterogeneous, lobulated
MR: Low T1, High T2
Angio: Tumor neovascularity
Pediatric Radiology
Unenhanced CT with focus of

calcification in a teratoid variety of
hepatoblastoma. Heterogeneous
enhancement of same tumor involving
left and right hepatic lobes
1511
1513
Hepatoblastoma: Treatment [Figure 6-18-20]
Figure 6-18-19
Pre-op chemotherapy ( 50%90%)

Surgical: Resectable 90%; 35% mortality
Fetal best prognosis
Vascular definition- multisegmentectomy
Mets: Pulmonary, periaortic nodes, brain
Chemoembolization/RF after surgery PRN
Hepatoblastoma
Heptoblastoma with solid nature in CT and gross

specimen. Cystic change is irregular due to
central necrosis of the solid tumor
Age: Young (infants, young children)

Biologic imaging features: Solid
Chemistry: Positive alpha-fetoprotein
Figure 6-18-20
#2 primary liver malignancy in childhood

Often underlying liver disease/cirrhosis
Tyrosinemia, alpha-1-antitrypsin deficiency, glycogen storage
dz, biliary atresia, chronic hepatitis (HBsAg +), Fanconi
anemia, methotrexate induced hepatic fibrosis
May be primary
Hepatocellular Carcinoma: Pathology [Figure 6-18-21]
Multinodular, diffuse; less common solitary

225 cm
Hemorrhage, cysts scattered with nodules
Fibrous/cirrhotic background
Adjacent liver abnormal
Cords and nest- malignant hepatocytes
Fibrolamellar variant: favorable prognosis
Chemoembolization of residual tumor

focus following surgery in patient who
is not a liver transplantation candidate
Hepatocellular Carcinoma: Clinical
Abdominal mass, hepatomegaly

Abdominal pain
Child > 4 yrs old, 1214 yrs mean age
Male predominance > 2:1
+ prior history of liver disease
Figure 6-18-21
Hepatocellular Carcinoma: Imaging

[Figures 6-18-22 and 6-18-23]

US: Heterogeneous, mostly solid, hypoechoic
CT: Hypodense/isodense
MR: Low T1, High T2 (esp Fat Sat FSE),
PV invasion
Similar appearance to aggressive hepatoblastoma
Figure 6-18-22
Nodular
appearance of
solid epithelial
tumor
(hepatocellular
carcinoma) in
14 year old
patient, with
positive AFP
1512
1514
Nodular nature(cords and nests of

tumor cells) of solid hepatocellular
carcinoma
Figure 6-18-23
Nests of inhomogeneously enhancing

HCC
Pediatric Radiology
Hepatocellular Carcinoma: Treatment [Figures 6-18-24 to 6-18-26]

Pre-op chemotherapy
Surgical: Resectable 20%, >75% mortality
Fibrolamellar- best prognosis
Vascular definition- multisegmentectomy
Chemoembolization/RF after surgery PRN
Figure 6-18-24
Age of patient: Teens

Biologic imaging: Solid, multifocal
Chemistry-blood
Undifferentiated Embryonal Sarcoma
RF ablation of focal hepatoma (left image) with

needle seen during US guided RF ablation (right
image).
#4 liver malignancy in childhood

AKA malignant mesenchymoma
Highly aggressive neoplasm
Most children 610 yrs old, M=F
Abdominal mass, fever, wt loss
Normal alpha-fetoprotein
Figure 6-18-25
Undifferentiated Embryonal Sarcoma:

Pathology
Solid, globular, areas of necrosis/hemorrhage

230 cm
Right lobe dominance (75%)
Fibrous pseudocapsule, occasional-pedunculated
Spindle/stellate shaped sarcomatous cells
Myxoid background
Local recurrence and metastasis
Undifferentiated Embryonal Sarcoma:

Imaging [Figures 6-18-27 to 6-18-30]

US: Complex mass
CT: Hypodense-heterogeneous enhancement
MR: Low T1, High T2
Angiography: Hypovascular
Left slide with small echogenic focus of

microbubbles form at the tip of needle (arrow) in
the earliest phase of coagulation necrosis. The
small focus of microbubbles grows to become a
large area of echogenic necrotic tumor (right)
Figure 6-18-26
Figure 6-18-27
Figure 6-18-28
Focal scar 1 year following RF tumor

ablation
Complex US appearance of
embryonal sarcoma in 11 year female
Demonstrable pseudocapsule
anteriorly surrounding mesenchymal
sarcoma
Pediatric Radiology
1513
1515
Figure 6-18-29
Figure 6-18-30
MR with heterogeneous cystic and solid foci in

embryonal sarcoma
Multiple areas of cystic change in
embryonal sarcoma in 10 y/o male
Undifferentiated Embryonal Sarcoma: Treatment
Surgical: Resection
Chemotherapy, XRT
Poor prognosis
Mean survival = 12 months
Recurrence Dx 1216 months after surgery
Figure 6-18-31
Undifferentiated Embryonal Sarcoma
Age of patient: Adolescents/teens

Biologic imaging: Mixed solid/cystic
Chemistry-blood
Negative alpha-fetoprotein
Liver Metastases [Figures 6-18-31 and 6-18-32]
Neuroblastoma- Stage 4 and 4s

Burkitts lymphoma
Wilms tumor
Leukemia (AML)
Sarcomas
Other malignancies
Studded appearance of stage 4S

neuroblastoma diffusely involving the
liver
ABCs of Pediatric Liver Tumors
Figure 6-18-32
Age of patient
Biologic imaging features
Chemistry-blood
Alpha fetoprotein
Endothelial growth factor
Pediatric Liver Tumors: Summary
Summary chart of classic features of common pediatric liver tumors

Tumor
Age
Inf. Hemangio
< 1 yr
Mes Hamartoma
< 2 yr
Hepatoblastoma
HCC
Emb Rhabdo
UES
Metastases
< 3 yr
> 4 yr
< 5 yr
> 6 yr
any
Characteristics
Solid, Ca+2, vasc, AFP (-), involutes

with interferon
Cystic > solid, AFP (-)
Ca+2, solid, vasc, AFP(+) solitary
Solid, vasc, AFP (+), multifocal

Solid > cystic, mild vasc, AFP(-)
Cystic > solid, AFP (-)
Solid or cystic
1514
1516
Typical appearance of Burkitt

Lymphoma involving both the liver
and the posterior gastric wall in
patient with fatty liver
Pediatric Radiology
References
1.
2.
3.
4.
Von Schweinitz, D. Management of liver tumors in childhood. Semin Ped Surg 2006; 15(1):17-24
Stocker JT. Hepatic tumors in children. Clin Liver Dis. 2001 Feb;5(1):259-81, viii-ix.
von Schweinitz D. Neonatal liver tumours. Semin Neonatol. 2003 Oct;8(5):403-10.
Tiao GM, Bobey N, Allen S, Nieves N, Alonso M, Bucuvalas J, Wells R, Ryckman F. The current management of
hepatoblastoma: a combination of chemotherapy, conventional resection, and liver transplantation. J Pediatr. 2005
Feb;146(2):204-11.
5. Burrows PE, Dubois J, Kassarjian A. Pediatric hepatic vascular anomalies. Pediatr Radiol. 2001 Aug;31(8):533-45.
6. Dubois J, Hershon L, Carmant L, Belanger S, Leclerc JM, David M. Toxicity profile of interferon alfa-2b in children:
A prospective evaluation. J Pediatr. 1999 Dec;135(6):782-5.
7. Dachman AH, Pakter RL, Ros PR, Fishman EK, Goodman ZD, Lichtenstein JE. Hepatoblastoma: radiologic-pathologic
correlation in 50 cases. Radiology. 1987 Jul;164(1):15-9.
8. Gerber DA, Arcement C, Carr B, Towbin R, Mazariegos G, Reyes J. Use of intrahepatic chemotherapy to treat advanced
pediatric hepatic malignancies. J Pediatr Gastroenterol Nutr. 2000 Feb;30(2):137-44.
9. Sun XY, Wu ZD, Liao XF, Yuan JY. Tumor angiogenesis and its clinical significance in pediatric malignant liver tumor.
World J Gastroenterol. 2005 Feb 7;11(5):741-3.
10. Rhim H, Dodd GD 3rd, Chintapalli KN, Wood BJ, Dupuy DE, Hvizda JL, Sewell PE, Goldberg SN. Radiofrequency
thermal ablation of abdominal tumors: lessons learned from complications. Radiographics. 2004 Jan-Feb;24(1):4152.
11. Iannitti DA, Dupuy DE, Mayo-Smith WW, Murphy B. Hepatic radiofrequency ablation. Arch Surg. 2002
Apr;137(4):422-6.
Pediatric Radiology
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1517
Pediatric Hip Sonography:

Practical Radiologic Pathology
Figure 6-19-1
Ultrasound of the Hip
Developmental dysplasia
The irritable hip
Septic arthritis, toxic synovitis,
Arthritis, LCP, hemophilia
US guided intervention
Practical points
DDH - Risk Factors
Family History: 12-36%

Breech: up to 23% (esp female)
Torticollis, metatarsus adductus, oligohydramnios
DDH - Ultrasound Technique

High frequency linear
Static views (Graf)
Coronal
Dynamic views (Harcke)
Transverse and coronal
Barlow maneuver
Standard minimum exam
Coronal left hip sonography:

Transducer in right hand, left hand
pistons the left leg/hip
Figure 6-19-2
Dynamic Standard Minimum Exam [Figures 6-19-1 and 6-19-2]
Static coronal image

Static transverse image
Transverse image with stress
Coronal Image [Figures 6-19-3 and 6-19-4]
Transverse right hip sonography:

Transducer in left hand, right hand
pistons the right leg/hip
Figure 6-19-3
Figure 6-19-4
Coronal hip anatomy illustrates hyaline cartilage of

the acetabular roof, femoral head, and triradiate
cartilage; fibrocartilaginous acetabular labral tip
Pediatric Hip Sonography
Essential static coronal hip sonogram

with Graf alpha angle. Note the
critical horizontal orientation of the
iliac body for accurate depiction of
normal and pathologic anatomy
1516
1518
Pediatric Radiology
Static Transverse Image [Figure 6-19-5]
Figure 6-19-5
DDH - Coronal
Rigidly standardized protocol

Exam technique, interpretation
Alpha, beta angles
Reproducible
Large European experience
DDH - Practical Grading
Normal - Graf I
Physiologically Immature - Graf IIa
Dysplasia - Graf IIb and above
The Irritable Hip
Toxic synovitis
Septic arthritis
Arthritis - JRA, post-infectious
Legg-Calve-Perthes
Hemophilia
Note femoral metaphysis in view in

the transverse image obtained with
the hip flexed. Femoral head deeply
seated in the hip socket
The Irritable Hip
Pain
Limitation of motion
Fever, WBC, ESR, CRP
50% with effusion
Hip Effusion - Ultrasound
Direct visualization
More sensitive than plain film
Guided aspiration
Pediatric Radiology
1517
1519
Figure 6-19-6
High frequency linear

Supine, hip neutral
Anterior, parallel femoral neck
Comparison views
Fluid distends joint capsule

>2mm difference abnormal
Fluid echogenicity unreliable
Doppler flow unreliable
Septic Arthritis [Figures 6-19-6 and 6-19-7]
Fever
Elevated WBC, ESR, CRP
Symptoms more severe
Staph, strep, H. flu
Must obtain fluid for Dx
Essential Principles of US Guidance
Cursors delineate the effusion in the

anterior hip joint space
[Figure 6-19-8]
Figure 6-19-7
Figure 6-19-8
Small hip effusion in left image;

right image with needle aspiration of the effusion
Critical principles of sonographic guidance; needle

aligned in the center of the sound beam, straight
longitudinal alignment of needle along the
transducer face/sound beam short axis
References
1. Harcke HT, Grissom LE. Pediatric hip sonography. Diagnosis and differential diagnosis. Radiol Clin North Am.
1999 Jul;37(4):787-96.
2. Grissom LE, Harke HT. Developmental Dysplasia of the Pediatric Hip with Emphasis on Sonographic Evaluation.
Semin Musculoskelet Radiol. 1999;3(4):359-370
3. ACR Practice Guideline for the Performance of the Ultrasound
Examination for Detection of Developmental Dysplasia of the Hip. American College of Radiology Practice
Standards/Guidelines 2004. American College of Radiology, Reston, VA.Headquarters Office: 1891 Preston White
Dr, Reston, VA 20191, (703) 648-8900 2004 American College of2004 American College of Radiology
4. Eich GF, Superti-Furga A, Umbricht FS, Willi UV. The painful hip: evaluation of criteria for clinical decisionmaking. Eur J Pediatr. 1999 Nov;158(11):923-8.
5. Buchmann RF, Jaramillo D. Imaging of articular disorders in children. Radiol Clin North Am. 2004 Jan;42(1):15168, vii.
6. Givon U, Liberman B, Schindler A, Blankstein A, Ganel A. Treatment of septic arthritis of the hip joint by repeated
ultrasound-guided aspirations. J Pediatr Orthop. 2004 May-Jun;24(3):266-70.
1518
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Pediatric Radiology
Pediatric Seminar 1: Pulmonary Infections

CASE 1: 5 month with fever and cough
Normal thymus mimicking RUL pneumonia
Follow-up
Follow-up radiograph in one month shows expected decrease

in size of the thymus and the thymic sail sign
Normal Thymus
Sail sign, wave sign, notch or sulcus

No mass effect on trachea or vessels
Homogeneous in density
Commonly seen on CXR up to age 5 years then regresses in latter half of first
decade
Thymic rebound
Masses can arise in thymus leukemia/lymphoma, teratoma, thymolipoma,
lymphatic malformation
Pediatric Radiology
1519
1521
CASE 2: 12 yo with fever and cough

Round Pneumonia
Unique to children usually < 8 yo

Usually posterior lower lobes
Almost always bacterial
pneumococcus
Exclude bone erosion ddx: neoplasm
CT reveals a complex mass in the

right posterior mediastinum with
calcifications and extension through
the chest wall and into the spinal
canal. This is neuroblastoma
Tumors that look like pneumonia
Neuroblastoma
Chest wall masses
Parenchymal mass
Pleuropulmonary blastoma
Cyst
In the differential of round pneumonia is mediastinal or chest

wall mass. PA and lateral chest radiographs show a round
mass in the region of the medial right lower lobe. Note the
splaying of the ribs on the right adjacent to the mass. This is
therefore a mass and not a pneumonia
Pulmonary Blastoma
Arises from primitive mesenchymal blastema

Histologically reminiscent of Wilms tumor
Unlike other embryonal tumors, it is more commonly found in adults
May present as solitary nodule or huge mass
Heterogeneous, cystic areas
Plasma Cell Granuloma
AKA inflammatory pseudotumor

Localized proliferation of a variety of cells, mostly plasma cells
Reactive, organizing pneumonia
Patient often asx
Chunky calcifications
May be cystic
May have spiculated margins or air bronchograms
1520
1522
Pediatric Radiology
CASE 3: 17 mo with fever and cough without improvement after

1 week of oral abx
PA and lateral chest radiographs show dense opacification of the right

lower lobe with silhouetting of the right hemidiaphram. Right lateral
pleural thickening is also seen
Complicated Pneumonia
Suspect in cases of near white out

H. flu less than 2 y.o.
Pneumococcus
S. aureus
CT vs. US
Ultrasound may be
better determining
which fluid collections
need drainage
Ultrasound of the right chest

shows pleural fluid with
septations and consolidated,
airless lung that transmits sound.
The hyperechoic foci with ringdown artifact represent round air
collections
Multiple axial CT images show the

pleural fluid adjacent to the right
lower lobe consolidation. Note that
the lung enhances and is not
necrotic. The fluid-filled cavities with
non-nondependent air collections are
therefore most consistent with small
abscesses rather than necrotic
cavities
CASE 4: 17 yo with fever and cough, seizure disorder and DM
Round cavity in superior segment of

right upper lobe. Clinical history
suggests mild immunocompromise
and possibility of aspiration
Pediatric Radiology
1521
1523
Pulmonary abscess
Cavitary pulmonary necrosis
Pneumatocele
Pulmonary Abscess
CT fluid or air-filled cyst with enhancing, thick, irregular wall

Both necrosis and small abscesses in children have good outcomes with
antibiotics only
Pneumatoceles
Thin walled cavity seen in the recovery phase usually

of infection
Staphylococcus, pneumococcus, tuberculosis
Blunt chest trauma, hydrocarbon pneumonitis,
Bronchial obstruction leading to air trapping and
alveolar rupture
Pulmonary necrosis. Contrast this appearance to
Pneumothorax or mediastinum
case #3 with empyema and abscesses. Note
decreased enhancement of surrounding
parenchyma compared to more anterior
parenchyma with preservation of enhancement of
the visceral pleura (arrow)
CASE 5: Adult with history of pulmonary infection as a child
Left PA chest radiograph shows asymmetric density of lungs and

smaller left PA compared to right. Right expiratory PA chest
radiograph shows air trapping in left lung
Idiopathic, viral, toxic inhalation,drug reaction, collagen vascular dz,
transplant, chronic aspiration.
Adenovirus as child unilateral hyperlucent lung
Small hyperlucent lung, hypoplastic ipsilateral artery
Reticular nodular pattern with hyperinflation, central bronchiectasis
1522
1524
Pediatric Radiology
CASE 6: Adult with right chest pain
A round mass is seen in the right phrenicovertebral angle posterior to

the right atrium
Bronchopulmonary foregut
malformation
CPAM
Neurogenic tumor
Sequestration
Sequestration
Area of pulmonary tissue that

does not have a normal
connection to the bronchial tree
Systemic arterial supply
Can present as an infant or
young child or as a young adult
Often a history of recurrent
infections in the same lobe
May contain air
Sequestration
LLL most common

Multiloculated cystic or solid
mass
Intralobar vs. extralobar
CT reveals a predominantly cystic mass with focal nodular thickening
Pleural investment
along the posterior wall in the right posterior mediastinum. The lower
Extalobar own pleural
right image reveals an additional simple cyst in the left superior
investment
mediastinum
Extralobar systemic venous
drainage
Extralobar infants, associated anomalies
Extralobar-congenital/intralobar-acquired
CT vs. MRI vs. US define vascular supply and drainage
Arteriogram shows the blood supply to the right lower

lobe sequestration is coming from the celiac axis below
the diaphragm. Abdominal blood supply frequent, and
alters the surgical approach. The superior mediastinal
lesion was a foregut duplication cyst
Pediatric Radiology
1523
1525
CASE 7: 9 yo with SOB
PA and lateral chest radiographs show bilateral lower lobe

consolidation with silhouetting of both hemidiaphragms. Also note the
large cardiac silhouette, absence of the splenic shadow, and
cholecystectomy clips (unusual in children)
Acute Chest Syndrome
Fever, chest pain, shortness of breath

Rib infarction with splinting and atelectasis versus infection
Treated with oxygen, antibiotics and pain medication +/- plasma exchange
Look for associated findings of SS
CM
Absent splenic shadow
Evidence of gallbladder disease
Skeletal findings
CASE 8: 5 yo with fever and SOB

Pediatric TB Clinical Findings
Asymptomatic
Cough
Fever
Malaise / FTT
Respiratory distress
Lethargy
Pediatric TB Radiologic Findings
Normal
Focal infiltrate Ghon lesion
Unilateral hilar adenopathy Ranke
complex
Paratracheal adenopathy
Subcarinal adenopathy
Calcified granulomatous nodes
Left image shows unilateral hilar and right paratracheal

adenopathy.
Right image also shows secondary RML atelectasis
1524
1526
Pediatric Radiology
Pediatric Seminar 2: Skeletal Dysplasia

Radiologic Approach
Assess Proportion
Rhizo-, meso-, or acromelia
+/- platyspondyly
Assess Components of Bone
Epiphyses small or irregular epiphyseal dysplasia
Metaphyses widened, flared, or irregular metaphyseal dysplasia
Diaphyses widened or thickened diaphyseal dysplasia
Achondroplasia Group
All have abnormalities of the same chromosomal locus and gene product,
fibroblast growth factor receptor 3 (FGFR3)
Thanatophoric dysplasia
Achondroplasia
Hypochondroplasia
Thanatophoric Dysplasia
AD
Probably the most common lethal bone dysplasia
Skull - kleeblatschadel in type II
Thorax - very short ribs and handlebar clavicles
Spine small flat vertebral bodies with round
anterior ends, U or H-shaped on AP
Thanatophoric Dysplasia
Pelvis
Small, flared iliac bones
Very narrow sacrosciatic notches, flat dysplastic
acetabula
Extremities telephone receiver
Femora
Thanatophoric dysplasia. Note small flat vertebral

bodies, very short ribs, and telephone receiver
femora
Achondroplasia
Most common nonlethal skeletal dysplasia

AD, spontaneous mutation rate 8-%
Skull
Large with midface hypoplasia
Small skull base and foramen magnum
Achondroplasia
Spine
Very short pedicles risk of spinal canal stenosis
Decrease in interpediculate distance lumbar spine
Pelvis
Elephant-ear iliac wings
Flat acetabular roofs
Narrow sacrosciatic notches
Achondroplasia
Extremities
Rhizo- > meso- and acromelia
Hands brachydactyly with metaphyseal cupping of MCs
Knees chevron and inverted chevron deformities
Hips proximal femoral fade out and hemispheric capital femoral epiphyses
Pediatric Radiology
1525
1527
Case 1 5 yo with short stature
Achondroplasia. AP view of the extremities show short widened

bones with flared metaphyses and chevron deformities of the
distal femora and proximal tibiae
Short Rib-Polydactyly Group
Achondroplasia. AP view of the pelvis

shows flared elephant ear iliac
wings, shallow acetabular roof,
narrowed sacrosciatic notches
conferring a champagne-glass
configuration to the pelvis, and
narrowing of the interpediculate
distance in the lower lumbar spine
Includes
SRP I-IV - some with, some without polydactyly
Asphyxiating thoracic dysplasia
Chondroectodermal dysplasia
Shortest ribs of all dysplasias
Short Rib-Polydactyly
Thorax shortest ribs, horizontal ribs

Pelvis small ilia, notched acetabula
Extremities
Micromelia
Rolling pin-shaped or round-ended or spiked
femora
Ovoid tibiae
Polydactyly in some types
Asphyxiating Thoracic Dysplasia (Jeune

Syndrome)
Mixed prognosis
Some succumb early from respiratory
compromise
Others die later from progressive nephropathy
Thorax
Long and barrel-shaped
Handlebar clavicles
Short horizontal ribs with flared ant ends
Achondroplasia. AP views of the hands show

brachydactyly with cupping of the metacarpal
metaphyses
Asphyxiating Thoracic Dysplasia (Jeune Syndrome)
Spine normal
Pelvis
Trident acetabular roof
Flared iliac wings
Narrowed SS notches
Extremities cone-shaped epiphyses in hands
1526
1528
Pediatric Radiology
Case 2 Newborn with severe respiratory distress
Asphyxiating thoracic dystrophy.

Gross photo shows bilateral
polydactyly of the hands and very
small chest
Asphyxiating thoracic dystrophy
Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome)
Nonskeletal findings important in diagnosis

Hair, nail and teeth abnormalities
Congenital heart disease
Thorax small with short ribs
Pelvis
Trident acetabula
Small, flared iliac wings
Narrowed SS notches
Extremities
Generalized shortening
Exostosis of proximal medial tibia
Post-axial polydactyly
Capitate-hamate fusion
Extra carpal bone
Cone-shaped epiphyses
Case 3 6 year old with history of congenital heart disease
Chondroectodermal dysplasia. AP
views of the hands show bilateral
postaxial polydactyly and cone
shaped epiphyses (arrows)
Pediatric Radiology
1527
1529
Chondrodysplasia Punctata Group
All have epiphyseal stippling

Rhizomelic
AR, death in first year
Spine coronal clefts
Symmetric bilateral shortening of femora
Conradi-Hunermann
X-linked dominant
Asymmetric shortening of limbs
Diffuse stippling of the spine
Case 4 newborn boy with respiratory distress
Chondroectodermal dysplasia. AP
view of pelvis and LEs shows short
long bones and short, flared iliac
wings, with trident-shaped acetabular
roofs
Chondrodysplasia punctata. Note diffuse stippled

epiphyses and coronal clefts in the thoracic spine
Metaphyseal Chondrodysplasia Group
All have normal spine and wide irregular metaphyses

Jansen-type
Most severe
Infantile presentation
AD
Extremities extensive irregular, expanded metaphyses
Hyperparathryroidism
Metaphyseal Chondrodysplasias
Schmid-type mildest, metaphyseal flaring, especially around knees

Shwachman-Diamond AR
Pancreatic insufficiency malabsorption and lipomatosis of pancreas
Cyclic neutropenia recurrent infections
Metaphyseal Chondrodysplasias
McKusick-type
Cartilage-hair hypoplasia
High frequency in the Amish and Finnish populations
Hirschprung disease
Immune deficiency and increased risk of malignancy, especially leukemia
and lymphoma
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Pediatric Radiology
Case 5 12 yo boy with short stature and unusual hair

McKusick-Type
Spine square vertebral bodies

Extremities flaring, cupping and fragmentation of
metaphyses, especially at the knees
Hands shortening with metacarpal and phalangeal
cupping and coning
Dysplasias with Prominent Membranous

Bone Involvement
AD, marked variability in expression
Metaphyseal chondrodysplasia, McKusick type.
Drooping narrow chest, hypermobile shoulders,
Note widening and irregularity of metaphyses
and dental anomalies
about the knees
Mild short stature
Skull wormian bones and wide, open anterior fontanelle
Dysplasias with Prominent Membranous Bone Involvement
Thorax hypoplasia or absence of clavicles, downward sloping ribs
Spine posterior wedging of vertebral bodies
Pelvis - high, narrow iliac wings, absence or hypoplasia of pubic bones
Extremities tapered distal phalanges
Case 6 Fretful 8 mo whose pediatrician thinks he has bilateral

clavicular fractures
Cleidocranial dysplasia with wormian bones
Cleidocranial dysplasia. With

hypoplastic clavicles with
pseudarthroses
Cleidocranial dysplasia. Note

absence of ossified pubic bones,
narrowed sacrosciatic notches and
narrow ilia
Pediatric Radiology
Cleidocranial dysplasia.
Tapered distal phalanges
1529
1531
Dysostosis Multiplex Group
Mucopolysaccharidoses and mucolipidoses

All AR
All produce similar radiographic complex of findings
Hurler Syndrome
Present in infancy or early childhood

Skull J-shaped sella
Thorax
Short thick clavicles
Oar-shaped ribs
Spine
Gibbus deformity
Inferior beaked T-L vertebral bodies
Hurler Syndrome
Pelvis small flared iliac wings with inferior tapering and steep acetabular
roofs
Extremities
Wide diaphyses of long bones and metacarpals
Pointed proximal metacarpal poles
Case 7 short 3 yo with unusual facial appearance
Dysostosis multiplex due to Hurler syndrome.

AP chest shows thick clavicles and paddleshaped ribs. Lateral spine shows gibbus
deformity at thoracolumbar junction and
inferior beaking of vertebral bodies
Hurler syndrome. AP pelvis shows
flared iliac wings with inferior tapering
and steep acetabular roofs
Hurler syndrome. Another patient

with a J-shaped sella. Dental
abnormalities are related to
enlargement of the tongue
Another patient with Hurler syndrome showing the
pointed proximal poles of the metacarpals
1530
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Pediatric Radiology
Morquio Syndrome
No J-shaped sella
Vertebral beak is in the middle
Ribs are widened but not oar-shaped
Proximal metacarpal poles are rounded
Dysplasias with Decreased Density
Very large group of conditions that share an abnormality of type I

collagen
Osteogenesis Imperfecta Type II
Invariably lethal
Hurler syndrome. Second patient with
coarsening of the soft tissues of the
Skull poor or absent ossification
face including the tongue
Thorax small chest with beaded ribs
Osteogenesis Imperfecta
Type II
Spine very poor ossification with collapse of vertebral bodies
Extremities accordion femora
Other types
Skull more than 8-10 wormian bones, variable ossification
Extremities variable osteoporosis and fractures
Case 8 31-week fetus
Osteogenesis imperfecta. Prenatal ultrasound shows no

shadowing by the skull. The near side of the brain is much too
well visualized. Also the transducer is indenting the skull
Increased Bone Density
Osteopetrosis (Albers-Schonberg Disease)

Failure to resorb primary spongiosa
Severe precocious type AR
Delayed type AD
Reduced bone marrow space anemia and extramedullary
hematopoesis
Osteopetrosis Radiographic Findings
Generalized increased bone density

Skull thick and dense especially at the base with foraminal
narrowing
Spine sandwich or picture-frame vertebral bodies
Extremities
Widened metaphyses with dense bands
Bone-within-bone appearance
Pediatric Radiology
1531
1533
OI. No ossification of the

membranous portions of the skull.
Small chest with beaded ribs and
accordioned long bones due to
multiple in utero fractures
Osteopetrosis with diffusely dense

bones, bone-within-bone
appearance, and deformity due to
pathologic femoral neck fractures
Osteopetrosis. Acute
femoral neck fracture on
earlier film
Osteopetrosis with sandwich

vertebrae
Pyknodysostosis
AR, presents in infancy

Micrognathia, short fingertips, fractures
Generalized osteosclerosis
Skull
Wormian bones
Marked delay in closure of sutures and fontanelles
Obtuse mandibular angle
Thorax resorption of acromial ends of clavicles
Extremities resorption of phalangeal tufts
Case 9 17 year old with short stature
Pyknodysostosis. Skull shows

persistent unfused sutures and
very obtuse mandibular angle
Pyknodysostosis. Diffuse increased

bone density and resorption of
phalangeal tufts
1532
1534
Pediatric Radiology
Pediatric Seminar 3: Cystic Fibrosis and

Pulmonary Infections of the
Immunocompromised Child
Case 1 15 yo with fever and respiratory distress
Cystic Fibrosis Epidemiology1
Most common lethal autosomal

recessive disease in white populations
(1:2500 live births)
Rare in blacks (4% of cases)
Very rare in Asians (0.2% of cases)
Exceedingly rare in Native Americans
(0.02% of cases)
1Cystic Fibrosis Foundation Registry 1990
Annual Data Report.
Cystic Fibrosis Epidemiology1

Mean age at diagnosis 6 mo
80% diagnosed by age 3 years
10% of newly diagnosed are 18 years or
older
52.8% male
11% of new diagnoses due to family
history of CF
PA chest radiograph shows

bilateral hyperinflation and
right much greater than left
linear and nodular opacity
predominantly in the upper
lobes.
Waters view of the paranasal
sinuses demonstrates
complete opacification of the
paranasal sinuses
1Cystic Fibrosis Foundation Registry. 2004 Annual Data Report.
Cystic Fibrosis CF Gene
Long arm of chromosome 7

Encodes a large single chain protein, CF transmembrane conductance
regulator (CFTR)
CFTR forms cell membrane chloride channel
3 base pair deletion (delta F508 mutation) accounts for 70% CF cases
Remaining cases due to over 1100 different mutations
Cystic Fibrosis Cellular Physiology
Normal CFTR: Epithelial chloride channel supplies luminal water by osmosis

Abnormal CFTR: Decreased water flow produces viscous inspissated luminal
secretions
Exocrine duct obstruction
Enhanced bacterial colonization
Cystic Fibrosis Exocrine Sites
Bronchioles and small bronchi

Pancreas
Intestinal crypts
Biliary ducts
Vas deferens
Cervix
Sweat and salivary glands
Pediatric Radiology
1533
1535
Pediatric Seminar 3: Cystic Fibrosis
Cystic Fibrosis Clinical Presentation
Neonatal intestinal obstruction in 16-20%

Respiratory manifestations & pansinusitis
Failure to thrive
Malabsorption
Unexplained hypochloremic acidosis
Positive family history
Cystic Fibrosis Protean Expressions
Pulmonary Manifestations
Recurrent infection
Pulmonary insufficiency
Gastrointestinal Manifestations
Pancreatic abnormalities
Intestinal obstruction
Nonobstructive bowel manifestations
Hepatobiliary disease
Gastrointestinal malignancy
Cystic Fibrosis Lung Disease
Principal cause of morbidity and mortality

Abnormal mucus obstructs terminal airways
Decreased mucociliary transport
Air trapping & increased dead space
Colonization by S. Aureus, H. flu, Pseudomonas sp., atypical mycobacteria,
Burkholderia cepacia complex
Bronchiectasis
Alveoli usually spared
Cystic Fibrosis Pathogenesis of Lung Disease
Airway macrophages promote neutrophil (PMN) influx

Elastase from autolyzed PMNs digests elastin, causing bronchiectasis and
fibrosis
Elastase is powerful mucus secretagogue
Neutrophil death release high molecular weight DNA (Pulmozyme)
Abnormal CFTR protein may bind pathogens (mucoid strain of Pseudomonas)
Cystic Fibrosis Pulmonary Complications
Pneumothorax
Allergic bronchopulmonary aspergillosis
Acute and chronic respiratory failure
Hemoptysis from dilated bronchial arteries
Pulmonary hypertension
Cor pulmonale
Lung Disease in CF Pathologic Features
Bronchi filled with mucoid exudate laden with degenerating neutrophils

Bronchial mucosa features increased goblet cells & focal metaplastic
squamous epithelium
Ciliary changes
Bronchiectasis
1534
1536
Pediatric Radiology
Case 2 18 yo with respiratory distress
PA chest radiograph shows tunneled

central venous catheter, bilateral
linear and confluent opacities, and
bilateral hilar enlargement
Lung Disease in CF Radiographic Features

Bronchial impaction (finger in glove appearance)
Hilar adenopathy
Saccular bronchiectasis with upper lobe preponderance
Thin-walled subpleural cysts
Case 3 - 14 yo with fever and cough

Lung Disease in CF Cysts
Cyst seen on CXR - saccular bronchiectasis vs abscess

Less likely pneumatocele
AFL may be seen in bronchiectasis or abscess
Abscess rare in older children except with CF
Subpleural blebs may be seen on HRCT
Case 4 13 yo with recurrent respiratory tract infections
PA radiograph of the chest shows a

round mass with air fluid level in the
right middle lobe
Pediatric Radiology
1535
1537
High resolution chest CT image on the left is in expiratory

phase of respiration and shows mosaic pattern typical of small
airways disease. Image on right shows dilated bronchi with
thickened walls due to bronchiectasis
Lung Disease in CF - HRCT
Currently CT is not part of the routine follow-up of CF patients. CXR and PFT
are.
HRCT is much more sensitive than CXR for bronchiectasis 90%
HRCT is much more sensitive for early and reversible changes of CF than
CXR or PFT
HRCT is becoming an outcome surrogate for CF
Objective evaluation of HRCT is prerequisite
Case 5 14 yo with malabsorption
Axial IV contrast enhanced CT image

shows complete replacement of the
pancreas with fat
Exocrine Pancreatic Insufficiency
> 80% have clinical pancreatic insufficiency

Insufficient lipolytic & proteolytic enzymes for normal digestion & absorption of
nutrients
Steatorrhea correlates with enzyme output < 10% of normal
Delta 508 mutation higher incidence of pancreatic insufficiency and earlier
onset of lung disease and colonization with pseudomonas
Pancreatic Sufficiency
10-15% of CF patients
Do not require enzyme supplements
Better nutritional status
Older at diagnosis later onset of lung disease
Lower Pseudomonas colonization rates
Better prognosis
May convert to pancreatic insufficiency with age (genetically determined)
Cystic Fibrosis Endocrine Dysfunction
Glucose intolerance in 30-50%

Diabetes mellitus develops in 1% of children & 13% of adults
Screened annual starting at age 14
DM due to pancreatic fibrosis & other unknown factors
1536
1538
Pediatric Radiology
Cystic Fibrosis of the Pancreas Pathologic Features
Proximal duct obstruction from inspissated pancreatic juice

Acinar atrophy & inflammation
Progressive interstitial fibrosis
Fatty replacement
Duct ectasia
Micro- & macrocysts
Calcification punctate and diffuse or chunky
Cystic Fibrosis of the Pancreas Imaging Findings
Radiographs: Punctate pancreatic calcifications

US: Small echogenic pancreas
CT: Fat attenuation, +/- calcifications, small cysts, complete pancreatic
replacement by macrocysts (rare)
MRI: Variable signal intensity depending on amount of fat & fibrosis
Case 6 - 12 yo with failure to thrive
Sonogram on the left shows simple cyst anterior to the SMV

and posterior to the left lobe of the liver. CT shows two simple
cysts in the pancreas
Case 6 Newborn with emesis and abdominal distension
KUB of infant shows many loops of

dilated, unfolded bowel with soap
bubble lucencies in the right lower
quadrant
Pediatric Radiology
Contrast enema in the same patient

showing a microcolon. The ileum is of
larger caliber than the colon and
shows multiple filling defects (arrow)
1537
1539
GI Manifestations of CF Intestinal Obstruction
Meconium ileus
Meconium plug syndrome
Distal intestinal obstruction syndrome (meconium ileus equivalent)
Intussusception
Fibrosing colonopathy
GI Manifestations of CF Meconium Ileus
Earliest clinical manifestation of CF

10-15% of CF patients present with meconium ileus
All pts. with meconium ileus have CF
Dysfunction of secretory intestinal epithelium plus panc enzyme insufficiency
Distal small bowel obstruction from dessicated meconium pellets
Meconium Plug Syndrome
Colonic obstruction in neonates

Not a syndrome but a symptom
25% have CF
The rest have functional immaturity of the colon or Hirshprung disease
Contrast enema may relieve obstruction
Distal Intestinal Obstruction Syndrome (DIOS)
Formerly termed meconium ileus equivalent

Reserve dx for patients with obstruction
10-15 % of CF pts (usually adolescents & adults)
Results from fluid loss and poor compliance with pancreatic
enzyme replacement
May mimic appendicitis (appy uncommon in CF)
Distal obstruction pattern on plain films
Fecal mass in RLQ
Enema may be therapeutic but usually treated medically
GI Manifestations of CF Rectal Prolapse
Occurs in 20% of CF patients

Presents in first years of life
Resolves spontaneously by approx. 5 years
Associated with bulky stools, diarrhea, or constipation
Improved by pancreatic enzyme supplementation
GI Manifestations of CF Intussusception
Occurs in approx. 1% of pts. with CF

Mean age of presentation = 10 years
Usually ileocolic
Lead points: adherent fecal residue, enlarged lymphoid follicles,
chronically distended appendix, or DIOS
GI Manifestations of CF Fibrosing Colonopathy
Contrast enema shows shortening

and loss of haustration in ascending
colon with short focal narrowing. Also
there is a large round filling defect
proximal to the narrowing.
Colonscopy revealed the narrowing
was a stricture and the filling defect
was an inflammatory pseudopolyp
Usually right colon

High-strength pancreatic enzyme supplementation compounded by high
protease intake strongly implicated
Submucosal fibrosis, fatty infiltration, mural thickening, haustral loss,
shortening, stricture formation
1538
1540
Pediatric Radiology
Case 7 10 yo with abdominal pain
Air lucency is seen in the wall of the

colon in the left upper quadrant on
this CT image representing
pneumatosis
Cystic Fibrosis Nonobstructive Bowel Manifestations
Thickened nodular mucosal folds in duodenum & small bowel

Jejunization of colon
Duodenal ulcer seen at autopsy in 10%
Gastroesophageal reflux
Barrett esophagus
Pneumatosis intestinalis
Cystic Fibrosis Hepatobiliary Disease
Cholelithiasis (cholesterol stones) in 12-24%

Microgallbladder at autopsy in 25%
Atrophy or obstruction of cystic duct
Distal CBD stricture
Fatty liver
Focal biliary cirrhosis and portal hypertension
Case 8 16 year old with hematemesis
Left images shows longitudinal

linear filling defects in the
esophagus indicating varices.
Also note bronchial artery
embolization coils. Axial image
from a contrast enhanced CT
shows fat density in the liver,
cholecystectomy clips, and a
large coronary vein in the left
upper quadrant. (Patient
underwent splenectomy as an
infant.)
Focal Biliary Cirrhosis
Pathognomonic for CF
Up to 40% of CF pt
Attributed to thickened intrahepatic bile duct secretions
Periductal inflammation, focal biliary fibrosis, & ductular proliferation
Multinodular cirrhosis in 5 - 12%
Portal hypertension and end-stage liver disease 1%
Pediatric Radiology
1539
1541
Radiologic Pathology 2006-2007 - Volume III - INDEX

1st Branchial Cleft Cysts 1278
Aberrant Internal Carotid Artery 1073
Aberrant LSCA 1377, 1454
Aberrant RSCA (Right Subclavian Artery) 1375
Abnormal Ureteral Insertion 1332
Abnormal Axis of Intrarenal Collecting System 1333
Abscess 1133
Abscess (Intracranial) 1234
Absent Internal Carotid Artery 1073
Absent Pulmonary Artery 1455
Absent Pulmonary Valve Syndrome 1380
Abuse injuries (Child) - Differential Diagnosis 1499
Accessory Parotid Tissue 1275
Achondroplasia 1527
Acinic Cell Carcinoma 1280
Acoustic Schwannoma 1079
Acquired Cholesteatoma 1076
Acquired Renal Cysts 1382
Active plaques (MS) 1038
Acute Chest Syndrome 1526
Acute disseminated encephalomyelitis 1040
Acute Disseminated Encephalomyelitis (ADEM) 1040
Acute GI Disorders (Infants and Children) 1353
Acute Meningitis 1232
Acute Pulmonary Interstitial Emphysema 1446
Acute Pyeloneprhitis 1335
Acyanotic CHD 1472
Acyanotic CHD with Increased PBF 1473
ADC (Aids Dementia Complex) 1237
Adenoid Cystic Carcinoma 1280
Adenoid Enlargement 1368
Adenoma 1251
Adenoma (Pleomorphic) 1279
Adenoma Sebaceum 1198
Admixture Lesions 1479
Adrenal
Hematoma 1410
Hemorrhage 1410
Masses (Pediatric) 1402
Medullary Tumors 1402
Metastases 1410
Adrenocortical Cancer 1409
Adrenocortical Tumors 1409
Aggressive Fibromatosis 1276
AIDS Dementia Complex (ADC) 1237
AIDS related infections (Intracranial) 1231
Air Leak 1445
Pseudocysts 1447
Pneumomediastinum 1447
Pneumothorax 1447
Airway (Pediatric) 1363
Albers-Schonberg Disease 1533
Alcohol 1041
Allergic fungal sinusitis 1244
with polyps 1244
Alobar Holoprosencephaly 1310
Alveolar Proteinosis 1451
AMEN Differential Diagnosis 1079
Amyloid angiopathy 1039
Anencephaly 1307
Aneurysm (suprasellar) 1255
Aneurysms (Intracranial) 1210
Deconstructive Therapy 1218
Dissecting 1212
Saccular 1212
Angiomyolipoma 1201, 1399
ANGIOMYOMATOSIS vs. LYMPHANGIOMYOMATOSIS
1202
Annular Tear/Fissure 1290
Annulus of Zinn 1089
Anomalous innominate artery 1454
Antenatal Pelvicaliectasis 1336
Anterior chamber: aqueous humor 1088
Antoni A and B fibers 1079
Antral Web 1342
Antrochoanal polyp 1245, 1368
Anus (Imperforate) 1351
Aortic Arch 1378
Double 1378
Embryology 1374
Anomalies 1454
Aortic Coarctation 1466
Aortic Stenosis 1470
Aorticopulmonary Window 1477
Appendicitis 1360
Aqueous humor (Globe) 1088
Aqueous Protein Solution 1163
Arachnoid Cyst 1109
Arnold Chiari Malformation 1308
Arrested Pulmonary Development 1439
Arteriosclerosis 1039
Arteriosclerosis / venous collagenosis 1039
Arteriovenous Fistula (Orbit) 1100
Arteriovenous Malformation (Lung) 1442
Ash-Leaf Spots 1199
Asphyxia 1506
Asphyxiating Thoracic Dysplasia (Jeune Syndrome) 1528
Aspirated Foreign Body 1365
Aspiration syndromes 1448
Astrocyte Mutation 1142
Astrocytoma 1058, 1141, 1109, 1145
Circumscribed 1139
Diffuse 1142
Pilocytic 1139
Atresia
Colonic 1349
Duodenal 1345
Esophageal 1341
Ileal 1347
Jejunal 1347
Tricuspid 1468
Atrial Septal Defect 1463, 1474
Atrial Switch 1469
Atrioventricular Canal 1477
Atrioventricular Septal Defect 1464
Atypical Teratoid / Rhabdoid Tumor 1053
Autosomal Dominant Polycystic Kidney Disease (ADPKD)
1387
Autosomal Recessive Polycystic Kidney Disease (ARPKD)
1383
AVM (Brain) 1222
AVM Grading (Intracranial) 1222
Azygos Continuation 1460
Azygous Vein 1459
Basal Ganglia Thalamus 1119
Beckwith-Wiedemann syndrome 1388
Benign Astrocytoma 1139, 1143
Benign Lymphoepithelial Lesions 1278
I1
Benign Sacrococcygeal Teratoma 1422

Benign sinus lesions 1247
Berdon Syndrome 1349
Bezoar 1356
Bezold abscess 1078
Biliary Cirrhosis (Cystic Fibrosis) 1541
Binswanger's 1039
Bladder Diverticula 1332
Blood Brain Barrier 1131
Blowout Orbital Trauma 1093
Blunt trauma 1498
Blyth and Ockenden Clinical Classification (ARPKD) 1384
Bone Marrow Components 1425
Bone Marrow Imaging (Pediatric) 1425
Bony Orbit 1088
Borden Classification (Dural AVF) 1225
Borrelia burgdorferi 1237
Bourneville Disease 1197
Brachial Plexus Traction Injury 1296
Brain (Congenital Abnormalities) 1307
Brain Tumor 1137
Branchial Cleft Cyst 1269, 1278
Bright cerebellum sign 1496
Bronchial Atresia 1372
Bronchopulmonary Dysplasia 1447
Butterfly pattern 1046
Callosal Dysgenesis 1316
Calyceal Diverticulum 1381
Capillary Telangiectasia (Brain) 1229
Carbon monoxide poisoning 1041
Carcinoma (Choroid Plexus) 1151
Cardiac Imaging (Pediatric) 1453
Cardiomediastinal Silhouette Size 1473
Carotid Artery 1073
Carotid Body Paragangliomas 1272
Carotid Space 1269
Catecholamine Production 1402
Caudal Regression Syndrome 1265
Cavernous Angioma (Brain) 1228
Cavernous Hemangioma 1098
Cavernous Malformation (Brain) 1229
Cavernous Sinus Invasion (Pituitary Macroadenoma) 1254
Cellulitis (Orbit) 1101
Central Neurocytoma 1058, 1060, 1061
Central Posterior Fossa Lesion 1109
Cephaloceles 1307
Cerebellar Infarction 1507
Cerebellar Liponeurocytoma 1050
Cerebellopontine Angle Masses 1079
Cerebral edema 1496
Cerebral Hemiatrophy (Dyke-Davidoff-Masson Syndrome)
1312
Cerebral Infarction 1123
Cerebral Intraventricular Neoplasms 1058
Cerebral Neuroblastoma 1157
Cerebritis 1231
Ceruminoma 1079
Cervical aortic arch 1378
Cervical Fascia 1266
Chamber Assessment 1472
CHARGE Syndrome 1366
Chemotherapy 1042, 1155
Chiari I Malformation 1308
Chiari II Malformation - (Arnold Chiari Malformation) 1308
Chiari III Malformation 1309
Chiari IV Malformation 1309
Chiasmatic-hypothalamic glioma 1256
Child Abuse 1491

Childhood Neck Neoplasms 1370
Choanal Atresia 1366
Cholesteatoma 1074, 1076
Cholesterol Granuloma (Cyst) 1083
Chondrodysplasia Punctata 1530
1529
Chondrosarcoma 1084
Chordoma 1158
Choriocarcinoma 1178
Chorioretinitis 1092
Choroid Plexus
Carcinoma 1063, 1064
Metastasis 1065
Neoplasms 1151
Papilloma 1063
Tumors 1063
Choroidal Detachment 1090
Chylothorax 1448, 1451
Clear Cell Sarcoma (Kidney) 1396
Cleft Brain 1314
Clivus 1115
Cloquets canal 1091
Closed-Lip (Fused) 1314
CNS Bacterial Infections 1130
CNS infections (acquired) 1232
CNS Lyme Disease 1237
CNS Lymphoma 1045
CNS Neoplasms Chromosome Loss of Heterozygosity 1192
CNS Neoplasms: Chromosomes 1184
CNS Tuberculosis 1235
Coats Disease 1092
Cochlea 1068
Colloid Cyst 1058, 1062, 1116, 1163
Colonic Aganglionosis (Total) 1351
Colonic Atresia 1349
Common Atrium 1485
Complete Atrioventricular Septal Defect 1464
Complete Labrynthine Aplasia 1072
Complete Transposition of Great Vessels 1479
Complicated Pneumonia 1523
Compression Fractures 1430
Conal Lesions 1094
Congenital
Abnormalities of the Brain 1307
Adrenal Hyperplasia 1411
Anomalies (UTI) 1329
Cholesteatoma (Epidermoid) 1074
Dehiscence of Tegmen Tympani 1074
Heart Disease 1379, 1463, 1472
hyperplasia (Adrenal) 1410
Lobar Emphysema 1435
Lung Malformations 1435
Megacalyces 1339
Megacystis-Megaureter 1339
Midline Nasal Mass 1367
Spinal Anomalies 1260
Congenitally Corrected TGV 1480
Contrast Enema (Malrotation) 1344
Contrast Enhancement 1126
Contrast Enhancement- Abscess 1133
Contrast Enhancement: Hematoma 1134
Contusion (CNS) 1324
Convexity Extraaxial Differential 1124
Convexity Intraaxial 1123
I2
Cord Herniation (Idiopathic Transdural) 1296

Cord injury 1497
Coronary Artery Aneurysms-Kawasaki 1460
Cortical Tubers 1200
Cranial injury 1494
Cranial
Nerve Enhancement 1129
Nerves 1107
Nerves III, IV, VI 1090
Vascular Development 1194
Craniopharyngioma 1114, 1253, 1255, 1319
Creutzfeldt-Jakob Disease 1235
Crohn Disease 1360
Crohn's 1037
Cryptococcus meningitis 1238
Cryptococcus neoformans 1238
CSF
Dissemination 1156
Homeostasis 1152
Spread - Zuckerguss (Sugar Icing) 1157
CT Angiography: Basic Protocol 1453
Cyanotic CHD 1467, 1472, 1478
Cyanotic CHD with Decreased PBF 1487
Cyst (Neurenteric) 1264
Cystadenoma (Ovary) 1418
Cystic
Adenomatoid Malformation 1436
Fibrosis 1535
CF Gene 1535
of the Pancreas 1539
Masses (Pediatric Renal Tumors) 1398
neoplasms (Ovary) 1415
Neoplasms: Ovarian Teratoma 1417
nephroma 1398
Partially Differentiated Nephroblastoma 1398
Renal Disease of Childhood 1381
Renal Tumor (Multilocular) 1398
Cysticercosis (Intracranial) 1236
Cysts of the CNS 1160
Dandy-Walker Malformation 1312
Darlings Classification 1481
Dating of intracranial blood 1496
DAVF (Dural AVF) 1225
Dawson's fingers 1038
DBOs MR Signal Abnormalities 1191
Deep and Periventricular 1121
Degenerative Disc Disease 1290
Degenerative Disease (Spine) 1290
Dehiscence of Tegmen Tympani 1074
Dehiscent jugular bulb 1073
DeMorsiers Syndrome (Septo-Optic Dysplasia) 1310
Demyelinating Diseases 1037
Demyelination 1039, 1040, 1041
Imaging 1043
Dermal Sinus (Dorsal) 1264
DERMOID 1162
Dermoid / Epidermoid (Orbit) 1101
Dermoid Cysts 1287
Desmoplastic Infantile Ganglioglioma / Astrocytoma 1048
Differential diagnosis of abuse injuries 1499
Dilated Azygous Vein 1460
Dirty retrobulbar fat (Grave's Disease) 1094
Disc
Disease 1290
Extrusion with Migration 1291
herniations and types 1291
Disorders of Neuronal Proliferation 1313, 1314
Distal Intestinal Obstruction Syndrome (DIOS) 1540

Dorsal Induction (Disorders of) 1307
Double Aortic Arch 1378
Double Arch 1454
Double Outlet Right Ventricle 1485
Double Ring sign 1085
Drug abuse 1041
Ductus Arteriosus 1456
Duodenal
Atresia/Stenosis/Web 1345
Hematoma 1356
Duplication Cyst (GI tract location) 1360
Dural Tail 1131, 1169
Dyke-Davidoff-Masson Syndrome 1196, 1312
Dysembryoplastic Neuroepithelial Tumor 1051
Dysgenesis (Callosal) 1316
Dysgerminoma 1419
Dysostosis Multiplex 1532
Dysphagia 1374
Dysplasias with Prominent Membranous Bone Involvement
1531
Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos
Disease) 1049
EAC cholesteatoma 1078
Ear 1068
Ebstein Malformation 1488
Echogenic Kidneys in Neonate 1386
Ectopic Ureter 1332
Eisenmenger Physiology 1478
Elevated Prolactin 1113
Ellis-van Creveld Syndrome 1529
Embryonal
Sarcoma 1515
small cell tumor 1053
Emphysema (Pulmonary Interstitial) 1446
Encephalitis 1040, 1231
Encephalocele 1074
Endocardial Cushion Defect 1477
Endolymphatic Sac Tumor 1205
Enteric Duplication Cyst 1360
Enteric Fistula (Dorsal) 1263
Enterocolitis (Necrotizing) 1353
Enterocolitis (Neutropenic) 1361
Ependymitis granularis 1037
Ependymoma 1058, 1109, 1112, 1149
Epidermoid 1081, 1161
Epidermoid Inclusion Cyst 1109
Epidermoid vs. Arachnoid Cyst 1109
Epidural abscess 1231
Epidural Phlegmon / Abscess 1295
Epiglottitis 1364
Epstein-Barr virus (EBV) 1037
Esophageal Atresia 1341
Esthesioneuroblastoma 1248, 1368
Ethmoid sinus 1240
Exocrine Pancreatic Insufficiency 1538
Exostoses 1288
Exostosis (External Ear) 1078
External auditory canal (EAC) atresia 1071
External Ear Masses 1078
External Ear Neoplasms 1079
External Otitis 1078
Extraaxial Lesions 1106
Extraaxial Tumors 1158
Extraconal Lesions 1100
Extralobar Sequestration 1440
Extraocular muscles (EOM) 1089
Extrapontine myelinolysis 1041
I3
Facet Joint Synovial Cysts 1292

Facial Nerve Palsy 1081
Failed Back Surgery Syndrome (see also FBBS) 1292
Fallot 1487
FBSS (Failed Back Surgery Syndrome) 1293
Cervical Spinal Canal Stenosis 1293
Ossification (Posterior Longitudinal Ligament) 1294
Posterior Longitudinal Ligament (Ossification) 1294
Type I Arachnoiditis 1293
Type II Arachnoiditis 1293
Fibromatosis (Aggressive - Masticator Space) 1276
Fibromatosis Colli 1267, 1371
Fibrosing Colonopathy (Cystic Fibrosis) 1540
Fibrous Dysplasia (Paranasal Sinuses) 1246
Fibrous Histiocytoma (Orbit) 1099
Filum Terminale (Congenital Anomalies) 1263
Fissures of Santorini 1078
Focal Biliary Cirrhosis 1541
Follicular cysts 1415
Foreign Body 1365, 1366
Forensic Radiology of Child Abuse 1491
Fourth Ventricle 1109
Fractures (Healing) 1493
Fried egg appearance 1061
Frontal horn capping 1037
Frontal Lipoencephalocystocele 1307
Frontal sinus 1241
Functional Ovarian Cyst 1416
Fungal sinusitis 1102, 1245
Ganglioglioma / Gangliocytoma 1047
Ganglioneuroblastoma 1402, 1408
Ganglioneuroma 1402
Gastric Atresia 1342
Gastrostomy Tubes 1356
Gastrulation 1263
Gaucher Disease 1431
Germ Cell Tumors 1176, 1418
Germ Cell Tumors-AFIP Series 1177
Germinal Matrix 1502
Germinoma 1121, 1177
Germinoma (Infundibular) 1257
Ghost tumor 1046
GI Disorders (Acute - Infants and Chidren) 1353
GI Tract Obstruction 1341
Glioblastoma Multiforme 1119, 1147, 1322
Glioma 1109
Glioma (Chiasmatic-hypothalamic) 1256
Globe 1088
Globus pallidus 1191
Glomerulocystic Disease 1386
Glomus jugulare 1082
Glomus tympanicum 1082
Gradenigos syndrome 1078
Grading Problems in Gliomas 1140
Graf System (Pediatric Hip) 1519
Granulocyte Colony Stimulating Factor 1429
Granulosa-theca Cell Tumor 1419
Graves 1037
Graves Disease 1094
Great Vessels (Transposition) 1468
Grey Matter Heterotopias 1315
HAART 1239
Hamartoma 1510
Hamartoma (hypothalamic) 1257
Healing of fractures 1493
Healing of metaphyseal fractures 1492
Hemangioblastoma 1109, 1111, 1203, 1320
Hemangioblastomatosis 1202
Hemangioendothelioma 1511
Hemangioma 1370
Hemangiomas (suprahyoid Neck) 1266
Hemangiopericytoma 1173
Hemangiopericytoma vs. Meningioma 1173
Hematoma (Duodenal) 1356
Hemimegalencephaly 1313
Hemorrhage 1502
Adrenal 1410
Child Abuse 1495
Intracranial 1221
Hemorrhagic Cysts (Ovary) 1416
Hemorrhagic Infarction 1131
Hemorrhagic Ovarian Cysts 1416
Henoch-Schnlein Purpura 1356
Hepatobiliary Disease (Cystic Fibrosis) 1541
Hepatoblastoma 1513
Hernia (Inguinal) 1357
heroin 1041
Herpes encephalitis 1234
Heterotopias (Gray Matter) 1315
High Jugular Bulb (Megabulb) 1073
Highly Active Antiretroviral TX (HAART) 1239
Hip Effusion 1519, 1520
Hip Sonography 1518
Hirschprung Disease 1350
HIV encephalitis 1040, 1041
HIV Leukoencephalopathy 1238
Holoprosencephaly (Alobar) 1310
Holoprosencephaly (Semilobar) 1310
Horseshoe kidney 1330
HSV Encephalitis 1131
Hurler Syndrome 1532
Hyaline Membranes 1444
Hydranencephaly 1310
Hydrocarbon aspiration 1366
Hydrocephalus 1181
Hydrocolpos 1420
Hydronephrosis 1338
Hyperostosis in Meningiomas 1171
Hypertrophic Pyloric Stenosis 1354
Hypogenetic Lung Syndrome 1439
Hypoperfusion complex (Child Abuse) 1499
Hypopharyngeal cyst 1369
Hypoplastic left heart syndrome 1466, 1485
Hypothalamic Hamartoma 1257
Hypothalamus 1115
Hypoxic-ischemic encephalopathy 1039
Iatrogenic Demyelinating Disorders: Chemotherapy 1042
Iatrogenic white matter degeneration 1037
Idiopathic Transdural Cord Herniation 1296
Ileal Atresia 1347
Ileus (Meconium) 1348
Imaging Studies (UTI) 1331
Immaturity of the Colon (Functional) 1349
Immune reconstitution syndrome 1239
Immunocompromised Child 1535
Immunocompromised Patient 1045
Imperforate Anus 1351
Incudostapedial disruption 1085
Infantile Hemangioendothelioma 1511
Infarction 1506, 1507
Infections (Intracranial) 1231
Infections (Spine) 1290, 1295
Inferior orbital fissure 1088
Inflammatory disease of the salivary glands 1369
Infratentorial 1106
I4
Inguinal Hernia 1357

Inner Ear Anomalies 1071
Innominate Artery Compression 1455
Innominate Artery Compression Syndrome 1379
Interhemispheric extra-axial hemorrhage 1495
Internal Auditory Canal 1107
Internal Carotid Artery 1073
Interrupted Pulmonary Artery 1455
Intestinal Obstruction (Neonatal) 1341
Intraaxial Lesions 1106
Intraconal Lesions 1097
Intracranial
Aneurysms 1210
blood (Dating) 1496
Germ Cell Tumors 1176
Germinoma 1177
Infections 1231
Lipoma 1182
Vascular Malformations 1220
Intralobar Sequestration 1440
Intramural Hemorrhage (GI - Differential) 1356
Intrarenal Collecting System (Abnormal Axis) 1333
Intrarenal Reflux 1334
Intrasellar Pathology 1251
Intraventricular Lesions 1106
Intraventricular Meningioma 1065
Intussusception 1357
Cystic Fibrosis 1540
Reduction 1358
Inverted Papilloma (Paranasal Sinuses) 1247
Irritable Hip 1519
Ischemic Enhancement 1131
Jantene Procedure 1469
Jejunal Atresia 1347
Jeune Syndrome 1528
JNA (Juvenile Nasopharyngeal Angiofibroma) 1246
Jouberts Syndrome 1313
Jugular bulb 1073
Jugular
Diverticulum 1073
Foramen Masses 1083
Paragangliomas 1270
Jugulotympanic Paraganglioma 1082
Juvenile Angiofibroma 1367
Juvenile Nasopharyngeal Angiofibroma (JNA) 1246
Kawasaki disease 1460
Keratosis obturans 1078
Kernohan-Sayre (AFIP) Grading System 1137
Kidney (Medullary Sponge) 1382
Kidney (Multicystic Dysplastic) 1399
Kidney (Rhabdoid Tumor of) 1395
Labrynthine Aplasia 1072
Lacrimal Gland Lesions 1102
Lacrimal Sac Lesions 1103
Langerhans Cell Histiocytosis (Sella) 1258
Large Endolymphatic Duct and Sac (LEDS) 1072
Laryngeal- Tracheopapillomatosis 1372
Laryngomalacia 1371
Laryngotracheal cleft 1371
Left (Double) Superior Vena Cava 1458
Left paramediastinal structures (Differential Diagnosis) 1458
Left Superior Vena Cava 1458
Leukocoria 1090
Leukoencephalopathy (HIV) 1238
Lhermitte-Duclos Disease 1049
Lingual Thyroid 1370
Lingual Thyroid Gland 1285
Lipoencephalocystocele (Frontal) 1307
Lipoma (Intraspinal) 1262

Liposarcoma (Suprahyoid Neck) 1267
Lissencephaly 1314
Listeria Monocytogenes 1130
Liver Metastases 1516
Liver Tumors (Pediatric) 1509
Lobar emphysema 1435
Long bone shaft fracture 1491
Long Parotid Tails 1275
Low Back Pain 1290
Low Intestinal Obstruction 1347
Lumbar 1292
Facet Arthropathy 1292
Spinal Canal and Foraminal Stenosis 1292
Lung Agenesis 1439
Lung Disease(Cystic Fibrosis) 1537
Lung Diseases in Neonates 1444
Lyme disease 1231
Lymphangioma (Orbit) 1100
Lymphatic Malformations (SupraHyoid Neck) 1267
Lymphocytic hypophysitis 1252
Lymphoepithelial Lesions (Benign - Suprahyoid Neck) 1278
Lymphoma 1324, 1371
CNS 1045
Orbit) 1098
Pediatric Renal Tumors) 1400
Sella) 1258
in AIDS 1324
vs. Toxoplasmosis 1324
Macroglossia 1369
Madelungs Disease 1267
Malfixation (Duodenum) 1343
Malignant
Astrocytoma 1145
Compression Fracture 1430
Germ Cell Tumors 1418
Meningioma 1172
Sinus Lesions 1247
Malrotation UGI 1344
Malrotation (Duodenum) 1343
Mandibular Hypoplasia 1369
Marburg 1039
Marchiafava-Bignami disease 1042
Marfan syndrome 1460
Marrow
Components 1425
Conversion 1427
Depletion Fatty Replacement 1432
Distribution 1427
Reconversion 1428
Replacement or Infiltration 1429
Masses - Ring Enhancing (CNS) 1322
Masticator Space 1274
Mastoiditis 1078
Mature Teratoma (Ovary) 1417
Maxillary sinus 1241
McKusick 1531
Measles 1037
Meckel Diverticulum 1359
Meckel-Gruber Syndrome 1388
Meconium Aspiration Syndrome 1449
Meconium Ileus 1348
Cystic Fibrosis 1540
Meconium Peritonitis 1348
Meconium Plug Syndrome 1540
Mediastinal Bronchogenic Cysts 1438
Medullary Cystic Disease Complex 1387
I5
Medullary Sponge Kidney 1382

Medullary Tumors (Adrenal) 1402
Medulloblastoma 1109, 1154, 1318
Medulloblastoma - Desmoplastic 1157
Megabulb 1073
Megacalyces 1339
Megalencephaly (Unilateral) 1313
Megaureter 1339
Melanoma (Uveal) 1092
Membranous Tracheitis 1365
Meninges (Neoplasms) 1164
Meningioma 1065, 1081, 1108, 1125, 1164
MENINGIOMA
*Imaging Features: CT vs. MR 1169
Angiography Transit Time 1170
Atypical Imaging 1172
Dural Tail 1131
Hyperostosis 1171
Vasogenic Edema 1167
Suprahyoid Neck 1270
Suprasellar 1254
Tentorial 1320
MR Imaging 1167
Meningitis 1231
Meningocele 1262
Mesenchymal Hamartoma 1509
Mesoblastic Nephroma 1396
Metabolic imaging 1043
Metaphyseal Chondrodysplasia 1530
Metaphyseal fracture 1491-1492
Metastasis (Pituitary and Infundibulum) 1259
Metastatic Lesions (Orbit) 1100
Methotrexate 1042
Michels deformity 1072
Microangiopathy 1039
Microgastria 1342
Microgyria 1314
Middle Ear 1068
Midgut Loop (Normal Rotation) 1343
Midgut Volvulus 1343
Migraine 1039
Mirror Image Right Arch 1376
Modic Changes 1291
Modified Papile Classification 1502
Brain (Neonatal) 1501
Mondinis dysplasia 1072
Monosymptomatic demyelinating 1037
Morquio Syndrome 1533
MR Angiography: Basic Technique 1453
Mucocele 1246
Mucoepidermoid Carcinoma 1281
Mucopolysaccharides 1094
Multicystic Dysplastic Kidney 1382, 1399
Multilocular Cystic Renal Tumor 1398
Multiple sclerosis 1037, 1039, 1325
MR 1038
Mustard Procedure 1469
myasthenia gravis, 1037
myelinolysis 1041
myelitis 1040
Myelocystocele (Terminal) 1262
Myelofibrosis 1431
Myeloid Depletion: MRI 1432
Myelomeningocele 1260
Nasal
Cycle 1128
Dermoid 1367
polyps 1368
Nasopharyngeal Carcinoma (NPSCCa) 1283
Neck Neoplasms (Child) 1370
Necrotizing Enterocolitis 1353
Necrotizing External Otitis 1078
Neonatal
Brain 1501
GI Tract Obstruction 1341
Hypoxic-Ischemic Injury 1502
Low Intestinal Obstruction (Differential Diagnosis) 1351
Pneumonia 1450
Respiratory Distress 1444
Lung Diseases 1444
Neoplasms (Benign - Masticator Space) 1276
Neoplasms (Malignant - Masticator Space) 1277
Neoplasms of the Meninges 1164
Nephroblastoma (Cystic Partially Differentiated) 1398
Nephroblastomatosis 1394
Cortical Nodule 1395
Diffuse 1394
Nephrogenic Rests: Location 1394
Nephroma (mesoblastic) 1396
Nerve Sheath Tumors 1187
Neuroblastic Tumors 1402
Neuroblastoma 1371, 1402
Stage Distribution 1408
Neuroectodermal Tumor 1053
Neuroepithelial Tumors 1047
Neurofibroma vs. Schwannoma 1188
Neurofibromas (Pelvis) 1423
Neurofibromatosis 1185
Type 2 1191
Type 1 or von Recklinghausen Disease 1185
Neuromyelitis optica (Devic syndrome) 1039
Neuronal Proliferation (Disorders of) 1313, 1314
Neutropenic Enterocolitis 1361
Non-Astrocytic Gliomas 1149
Non-Glial Lesions 1158
Nonhemorrhagic Infarction 1506
Non-Hodgkin Lymphoma (NHL) -Pharyngeal Mucosal Space
1283
Non-Lissencephalic Cortical Dysplasias
Microgyria/Polymicrogyria 1314
Normal
Cranial Nerve Enhancement 1129
Enhancement 1128
Marrow (MR Features) 1426
Pineal Calcification 1175
Thymus 1521
Vertebral Marrow: MRI 1428
Norries 1091
Obstruction (GI Tract - Neonatal) 1341
Olfactory Neuroblastoma 1248
Olidodendroglioma 1152
Oncocytoma (Suprahyoid Neck) 1280
Ophthalmic veins 1088
Opportunistic neoplasm 1045
Optic Nerve
Glioma 1097
Sheath Meningioma 1097
Optic neuritis 1040
Oral Cavity Normal Anatomy 1286
Orbit 1088
Orbital
Cellulitis 1101
fissures 1088
Lymphoma 1098
I6
septal system 1088

Trauma 1093
Varix 1099
Organic toxins 1041
Oropharynx 1284
Osmotic myelinolysis 1041, 1042
Ossicular Derangement 1085
Ossifying Renal Tumor of Infancy 1397
Osteogenesis Imperfecta 1533
Osteoma (Paranasal Sinuses) 1246
Osteomyelitis 1431
Osteomyelitis (Spine) 1295
Pyogenic 1295
Tuberculous 1295
Osteopetrosis (Albers-Schonberg Disease) 1533
Osteoporotic Fracture 1430
Ostiomeatal complex (Paranasal Sinuses) 1241
Ostium Primum ASD 1464
Otitis 1078
Otosclerosis 1085
Otospongiosis 1085
Outer Ear Anomalies 1071
Ovarian
Cancer 1420
Cyst 1416
Cystadenoma 1418
Maturation 1414
Tumors 1415
Ovary (Prepubertal vs Postpubertal) 1414
Palatine Tonsil Enlargement 1368
Pancreas (Child Abuse) 1498
Pancreatic injury 1498
Papillary Cystadenoma Lymphomatosum 1280
Papillary Endolymphatic Sac Tumor 1083
Papilloma 1151
Papovavirus 1238
Paradoxical Embolus 1475
Paragangliomas 1082, 1270
Paranasal Sinuses 1240
Paraovarian cysts 1415, 1417
Parapharyngeal Abscess 1365
Parapharyngeal Space 1267, 1268
Parasellar Region 1250
Parinaud Syndrome 1175
Parotid Space 1278
Parotid Tail 1275
Partial Anomalous Venous Return (Pulmonary) 1456
Patent Ductus Arteriosus 1456, 1476
Patterns of Enhancement 1126
Patterns of Location 1106
Pediatric
Adrenal Masses 1402
Airway 1363
Hip Sonography 1518
Liver Tumors 1509
Pelvic Masses 1414
Posterior Fossa Tumors 1318
Renal Tumors 1390
Tuberculosis 1526
Pelvic Masses (Pediatric) 1414
Pelvicaliectasis (Antenatal) 1336
Peritonitis (Meconium) 1348
Periventricular Hemorrhagic Infarction 1503
Sonography 1503
Periventricular Leukomalacia 1504
periventricular white matter 1039
Persistent hyaloid (Cloquets) canal 1091
Persistent Hyperplastic Primary Vitreous (PHPV) 1091
Persistent Interstitial Pulmonary Emphysema 1446

Persistent Stapedial Artery 1073
Petrous Apex (Differential Diagnosis) 1083
Phakomatoses 1184
Pharyngeal Mucosal Space 1282
Pharyngeal Perforation 1342
Phlegmon / Abscess (Epidural) 1295
Pial A-V Fistula 1225
Pilocytic Astrocytoma 1110, 1134, 1139
Pilocytic Astrocytoma (Juvenile Pilocytic) 1141
Pineal Calcification 1175
Pineal Cyst 1121, 1181
Pineal
Neoplasms Laboratory Tests 1179
Parenchyma 1180
Region Masses 1175, 1320
Region Neoplasms 1178
Pineal/Quadrigeminal Cistern Region 1121
Pinealomas 1121, 1175
Pineoblastoma 1121, 1180
Pineocytoma 1121, 1180
Pituitary 1250
Pituitary
Adenoma 1113, 1253
Apoplexy 1254
Macroadenoma 1253
Neoplasms 1251
Plasma Cell Granuloma 1522
Pleomorphic Adenoma 1279, 1288
Pleomorphic Xanthoastrocytoma 1051, 1141
Plexiform Neurofibromatosis 1423
Pneumatoceles 1524
Pneumonia 1523
Pneumonia (term & premature neonates) 1448
Polycystic Kidney Disease (autosomal Recessive) 1383
Polymicrogyria 1314
Port Wine Stain 1193
Post fossa cysts 1109
Posterior chamber 1088
Posterior Fossa
Malformations 1312
Tumors (Pediatric) 1318
Posterior Hyaloid Detachment 1090
Posterior Reversible Encephalopathy Syndrome (PRES)
1039, 1040
Posterior rib fractures (visualization) 1493
Posterior Urethral Valves 1337
Precocious Puberty 1175
Premature Births 1444
Premature Brain 1501
Prepubertal ovary 1414
Primary Megaureter 1339
Pringles Disease 1199
Profound Asphyxia 1506
Progressive multifocal leukoencephalopathy 1040
Progressive Multifocal Leukoencephalopathy (PML) 1041
Prolactin 1113
Prolactinoma 1251, 1253
Proteinosis (Alveolar) 1451
Proximal Neonatal Intestinal Obstruction 1347
Pseudotumor (Orbit) 1095
Pubertal ovary 1414
Pulmonary
Abscess 1524
Arterial Anomalies 1455
Artery Stenosis 1470
I7
Atresia with Intact Ventricular Septum 1489

AVM 1442
Blastoma 1522
Blood Flow 1472
Hypoplasia 1439
Infections 1521
Infections (Immunocompromised Child) 1535
Interstitial Emphysema 1446
Sequestration 1440
Sling 1379, 1455
Underdevelopment 1439
Venous Anomalies 1456
Pulsatile Tinnitus Lesions 1074
Pyeloneprhitis 1335
Pyknodysostosis 1534
Pyloric Stenosis 1354
Pyogenic Abscess (Intracranial) 1234
Pyogenic Osteomyelitis 1295
Pyriform Aperture stenosis 1367
Radiation 1155
Injury (Brain) 1043
Necrosis vs. Tumor (CNS) 1323
Ranulas 1287
Rathke Cleft Cyst 1252
Reactive Airways Disease 1365
Rebleeding 1497
Rectal Prolapse (Cystic Fibrosis) 1540
Rectus: medial, lateral, superior, inferior 1089
Red Marrow Signal 1427
Reflux Nephropathy 1335
Renal Agenesis 1329
Renal Cell Cancer 1399
Renal Cyst 1381
Renal Ectopia 1330
Renal Ectopia and Fusion 1330
Renal Tumors (Infancy and Young Children) 1390
Respiratory Distress (Neonatal) 1444
Respiratory Distress Syndrome (RDS) 1444
Retained fetal lung fluid 1448, 1449
Retina 1089
Retinal Detachment (RD) 1090
Retinoblastoma 1091
Gene 1091
Retinopathy of prematurity 1092
Retrobulbar (Postseptal) Space 1089
Retropharyngeal Cellulitis 1364
Reversal sign 1496
Rhabdoid Tumor 1054
Rhabdoid Tumor of Kidney 1395
Rhabdomyosarcoma 1368, 1420
Orbit 1101
Male Bladder & Prostate 1421
Rhabdomyosarcomatoid variant of Wilms tumor 1053
Rhombencephalosynapsis 1313
Rib fracture 1492
Rib Notching 1189
Right aortic arch 1454
Right Arch 1376
Right paramediastinal structures (Differential Diagnosis)
1459
Right to Left Shunts 1487
Ring Enhancing Mass 1132
Ring Lesion Features For Infection 1132
Ring Lesions Differential 1132
Ring-enhancing Masses (CNS) 1322
Risk Factors (subarachnoid Hemorrhage) 1211
Rotation of Midgut Loop 1343

Round Pneumonia 1522
Rules for Ring Enhancing Mass 1132
Sacrococcygeal Teratoma 1421
SAH (Subarachnoid Hemorrhage) 1210
Aneurysms (Intracranial) - Infectious 1218
Aneurysms (Intracranial) - Treatment Options 1218
Clinical Grading Scale 1213
CT 1214
CTA 1215
DSA 1215
induced Vasospasm 1214
Infectious Intracranial Aneurysms 1218
Lumbar puncture 1214
MRA 1215
MRI 1215
Outcomes 1213
Patterns 1213
Radiologic Grading Scale 1213
Risk Factors 1210
Screening 1217
Salivary glands (Inflammatory disease) 1369
Salt and pepper appearance 1082
Sarcoidosis (CNS) 1321
Sarcoidosis (Sella) 1258
SATCHMO 1319
SCCa (Squamous Cell Carcinoma) 1248
Schizencephaly 1314
Schwannoma 1107, 1188, 1192
Acoustic - Vestibular 1079
Orbit 1098
Scimitar Syndrome 1457
Sclera 1089
Scutum 1076
Second Branchial Cleft Cyst 1269
Secundum ASD 1464
Segmental Spinal Dysgenesis 1265
Sella 1250
Sella/Parasellar Region - Differential 1113
Sellar Masses: SATCHMO 1319
Semicircular Canals (SCC) 1068
Semilobar Holoprosencephaly 1310
Senescent White Matter Changes 1039
Senile Macular Degeneration 1090
Septic Arthritis (Hip) 1520
Septo-Optic Dysplasia (DeMorsiers Syndrome) 1310
Sequestration 1525
Sertoli-Leydig cell tumor 1419
Sex Cord-Stromal Tumors 1419
Shaking mechanism 1492
Short Rib-Polydactyly 1528
Shunt Lesions 1463, 1487
Sickle Cell Anemia 1429
Simple Renal Cyst 1381
Single Ventricle 1484
Physiology 1489
Sinus Mass Differential 1368
Sinus Venosus ASD 1464
Sinuses (Paranasal) 1240
Sinusitis 1243
Sinusitis (Fungal) 1102
Sjogrens Syndrome 1279
Skeletal Dysplasia 1527
Skeletal injury (evaluation) 1494
Skull fracture 1497
SLE 1037
Soap-bubble appearance 1051
I8
Spetzler-Martin Grading System (Intracranial Vascular

Malformations) 1222
Sphenoid sinus 1242
Spina Bifida 1262
Spinal Anomalies (Congenital) 1260
Caudal Regression Syndrome 1265
Chiari II Malformation 1261
Complex Dysraphic States 1263
Dorsal Dermal Sinus 1264
Dorsal Enteric Fistula 1263
Fibrolipomatous Infiltration of Filum 1263
Gastrulation 1263
HemiMMC/Hemimyelocele 1261
Intraspinal Lipoma 1262
Lipoma with Dorsal Defect 1261
Lipomyelomeningocele 1261
Meningocele 1262
Myelocele (Myeloschisis) 1261
Myelomeningocele 1260
Neurenteric Cyst 1264
Persistent Terminal Ventricle 1263
Posterior Spina Bifida 1262
Segmental Spinal Dysgenesis 1265
Spinal Dysraphism 1260
Split Cord Malformation 1264
Terminal Myelocystocele 1262
Tight Filum Terminale 1263
Spinal Dysraphism 1260
Spine 1290
Spine injury 1493
Split Cord Malformation 1264
Squamous cell carcinoma 1248
Stapedial Artery 1073
Stridor 1363, 1374
Sturge-Weber Syndrome 1193
Subacute sclerosing panencephalitis 1040
Subarachnoid Hemorrhage 1210
Subdural empyema 1231
Subependymal Giant Cell Astrocytoma 1058, 1061
Subependymal Nodules 1200
subependymal veins 1039
Subependymoma 1058, 1059
Subepidermal Fibrosis 1199
Subglottic edema 1363
Subglottic Hemangioma 1370
Sugar Icing 1156
Superior and inferior ophthalmic veins 1088
Superior Left Intercostal Vein 1458
Superior orbital fissure 1088
Suprahyoid Neck 1266
Suprasellar Masses 1253
Supratentorial 1106
Supratentorial Primitive Neuroectodermal Tumor 1052
Surfactant 1445
Surfactant B protein deficiency 1448
Swyer-James Syndrome 1436, 1524
Systemic Gas Embolism 1447
Systemic Venous Anomalies 1458
Taenia solium 1236
Tegmen Tympani 1074
Temporal Bone Fracture 1084
Temporal Bone:
Anatomy 1068
Congenital Lesions 1068
Infectious Lesions 1076
Neoplastic Lesions 1076
Temporomandibular joint (TMJ) anomalies 1071

Tenons capsule 1089
Tentorial Meningioma 1320
Teratoid Tumor 1053
Teratoma 1178, 1371
Ovary 1417
Sacrococcygeal 1421
vs. Dermoid (Pineal Region) 1178
Tetralogy of Fallot 1455, 1467, 1487
Thanatophoric Dysplasia 1527
Thiamin deficiency 1042
THIRD VENTRICLE 1116
Thoracic MRA & CTA 1453
Thymus (Pediatric - Normal) 1521
Thyroglossal Duct Cysts 1284
Thyroid Gland (Lingual) 1285
Thyroid Orbitopathy (Graves Disease) 1094
Time Density Curves 1126
Tinnitus 1074
Tongue Base Mass 1369
Top 10 Pelvic Lesions 1423
Total Anomalous Pulmonary Venous Return 1480
Total Anomalous PV Return 1469
Total Colonic Aganglionosis 1351
Toxic Demyelination 1041
Toxocara canis 1092
Toxocariasis 1092
Toxoplasmosis 1119
Intracranial 1238
Tracheal bronchus 1372
Tracheal Stenosis 1372
Tracheomalacia 1371
Transient Tachypnea of Newborn 1449
Transposition of Great Vessels 1468
Transverse Myelitis 1039
Tricuspid Atresia 1468, 1484, 1489
Trilateral Retinoblastoma 1180
Truncus Arteriosus 1469, 1483
Tuberculosis (Intracranial) 1231
Tuberculosis Pediatric 1526
Tuberous Sclerosis or Bourneville Disease 1197
Tumefactive Demyelination 1134
Tumor Blush 1171
UGI (Upper GI Tract - Malrotation) 1344
ulcerative colitis 1037
Uncommon Neuroepithelial Tumors 1045
Undifferentiated Embryonal Sarcoma 1515
Unilateral Megalencephaly (Hemimegalencephaly) 1313
Upper esophageal foreign body 1366
Ureterocele 1333
Ureteropelvic duplication 1333
Ureteropelvic Junction Obstruction 1338
Urethral Valves (Posterior) 1337
Urinary Tract Infection (Child) 1329
US Guidance 1520
Uterine Morphology: Maturation 1415
Uvea: choroid 1089
Uveal Melanoma 1092
Uveal Metastasis 1093
VACTERL 1342
Vagal paraganglioma 1082
Vagale Paragangliomas 1271
Vaginal Rhabdomyosarcoma 1421
Vallecular Cyst 1369
Valvular Pulmonic Stenosis 1470
Varix (Orbit) 1099
Vascular Anomalies (Pediatric Cardiac Imaging) 1453
Vascular Malformations (Intracranial) 1220
I9
Vascular Mediated Disorders (Bone Marrow) 1433

Edema 1433
Ischemia 1433
Ischemia & Edema: Causes (Bone Marrow) 1433
Vascular Rings and Slings 1374
Vascular White Matter Disease 1039
Vasculitis 1039
Vein Of Galen Malformation 1181
Venous Anomaly (Intracranial - Developmental) 1226
Venous Collagenosis 1039
Ventral Induction (Disorders of) 1310
Ventricular Septal Defect 1474
Ventricular Septal Defects 1465
Vesicoureteric Reflux 1332
Vestibular Schwannoma 1079, 1108
Vestibule 1068
Viral and Postviral Demyelination 1040
Viral Croup 1363
Virchow-Robin spaces 1037
Visceral injury 1498
Vitreous body 1088
Volvulus (Midgut) 1343
von Hippel-Linmdau Syndrome: NIH Classification 1202
von Recklinghausen Disease 1185
Warburgs 1091
Warthins Tumor 1280
Wernicke encephalopathy 1042
White Matter Changes (Senescent) 1039
WHO 2000 Brain Tumor Classification 1137
Whole-body MRI 1426
Wilms Tumor 1390
Wishart Disease 1191
Wolman Disease 1411
Xanthoastrocytoma 1052
Xanthoastrocytoma (Pleomorphic) 1141
Yellow Marrow Signal 1427
Zellweger Syndrome 1388
I 10

Radiologic Pathology

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Radiologic Pathology

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Radiologic

Ellen M. Chung, LTC, MC, USA

Six Week Course Director

Chief, Genitourinary Radiology

Department of Radiologic Pathology

American Registry of Pathology

Chief, Pulmonary and Mediastinal Radiology

Director of Chest Radiology

Staff Radiologist and Medical Illustrator

Washington Radiology Associates, PC

Chairman and Professor of Radiology

Melissa L. Rosado de Christenson, MD, FACR

Acting Chief, Genitourinary Radiology

Clinical Associate Professor of Radiology

Chief Molecular Imaging Program

Chairman and Gastrointestinal Radiology Section Chief

Chief, Genitourinary Radiology

Associate Professor of Radiology

Chairman, Department of Radiology

Associate Professor of Diagnostic Radiology

Table of Contents VOLUME 1

An Approach to Diffuse Lung Disease, Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3

Pulmonary Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .131

Melissa L. Rosado de Christenson, MD, FACR

Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .148

Pneumonia: Usual and Unusual Organisms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .178

Uncommon Malignant Tumors of the Lung . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .192

Leonard M. Glassman, MD (Mammography)

Classic Breast Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .220

Benign Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .267

GI Seminar 6: Beyond Appendicitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .427

Cholelithiasis and Cholecystitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .438

Inflammatory Diseases of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .444

Pancreatitis: Imaging Has Made a Difference . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .460

Small Bowel Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .475

The Spleen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .531

Imaging of Uterine Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .551

Cystic Diseases of the Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .614

Radiographic Evaluation of Urinary Stone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .624

Testicular Torsion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .630

Imaging of Ovarian Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .637

Jade Wong You Cheong, MD

Non-Neoplastic Disorders Of The Ovary And Adnexae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .653

The Neglected Nephrogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .674

GU Seminar 1: MSAFP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .691

An Approach to Diffuse Lung Disease,

The Alveolar vs. Interstitial Problem

An Approach to Diffuse Lung Disease

Radiology and pathology form

Reduced [Figure 1-1-2]

Airways disease results in increased

An Approach to Diffuse Lung Disease

Distribution: Upper vs Lower

Plain Film and CT Opacities

Plain Film and CT Opacities

An Approach to Diffuse Lung Disease

Anatomy - Secondary Lobule [Figure 1-1-5]

The secondary lobule with lymphatics in the

Septal pattern on HRCT and gross specimen

An Approach to Diffuse Lung Disease

[Figure 1-1-10and 1-1-11]

Multisystem granulomatous disorder

ATS Statement on Sarcoidosis 1999

Sarcoidosis: Clinical Features