NORMAL ANATOMY

Mediastinal Contents
Compartment Main Strictures
Anterior Fat, lymph nodes, thymus, heart, ascending aorta
Middle Trachea, bronchi, lymph nodes, oesophagus, descending
aorta
Posterior Para vertebral soft tissues
Mediastinal Masses
Compartment % Malignant
Anterosuperior 59
Middle 29
Posterior 16
Anatomy of the Mediastinum
Normal Contents
Anterosuperior: thymus, extrapericardial
aorta and branches, IVC, SVC, lymphatic tissue
Middle: heart, intrapericardial great vessels,
pulmonary hila, pericardium, trachea
Posterior: esophagus, vagus nerves, thoracic
duct, sympathetic chain, descending thoracic
aorta, azygous venous system
10/21/2014
History and Physical
Examination
Chest pain or dyspnea may be reported
secondary to associated pleural effusions,
cardiac tamponade, or phrenic nerve
involvement.
Occasionally, hoarseness because of left
recurrent laryngeal nerve involvement .
Systemic Sx present in 50% of patients and
a lymphoproliferative disorder, compared
with only 29% of patients with other masses
(such as thymic or neurogenic lesions).




10/21/2014
HU, 2009
6
Pain
Cough
Hemoptysis
SVC syndrome
Hoarseness
Dyspnea
Horners syndrome
Dysphagia
Pleural effusion
Stridor
Myathenia Gravis
Phrenic nerve palsy
Chylothorax
Symptoms and Signs
Diagnosis
Chest PA & Lateral
Bucky film
Chest CT
Fluoroscopy
Bronchoscopy
Esophagogram


NAB
Isotope Scanning
Angiography
Thoracotomy
VATS
Medistinoscopy


Clinical Presentation
Asymptomatic mass
Incidental discovery most common
50% of all mediastinal mass are asymptomatic
80% of such mass are benign
More than half are malignant if with symptoms
Clinical Presentation
Effects on Compression or invasion of adjacent tissues
Chest pain, from traction on mediastinal mass, tissue
invasion, or bone erosion is common
Cough, because of extrinsic compression of the trachea or
bronchi, or erosion into the airway itself
Hemoptysis, hoarseness or stridor
Pleural effusion, invasion or irritation of pleural space
Dysphagia, invasion or direct invasioin of the esophagus
Pericarditis or pericardial tamponade
Right ventricular outflow obstruction and cor pulmonale
Clinical Presentation
Superior vena cava
Vulnerable to extrinsic compression and obstruction because it is
thin walled and its intravascular pressure is low, and relatively
confined by lymph nodes and other rigid structures
Superior vena cava syndrome
Results from the increase venous pressure in the upper thorax , head
and neck
characterized by dilation of the collateral veins in the upper portion
of the head and thorax and edema oand phlethora of the face, neck
and upper torso, suffusion and edema of the conjunctiva and cerebral
symptoms such as headache, disturbance of consciousness and visual
distortion
Bronchogenic carcinoma and lymphoma are the most
common etiologies
Clinical Presentation
Hoarseness, invading or compressing the nerves
Horners syndrome, involvement of the sympathetic
ganglia
Dyspnea, from phrenic nerve involvement causing
diaphragmatic paralysis
Tachycardia, secondary to vagus nerve involvement
Clinical manifestations of spinal cord compression
Clinical Presentation
Systemic symptoms and syndromes
Fever, anorexia, weight loss and other non
specific symptoms of malignancy and
granulomatous inflammation
10/21/2014
Anterior mediastinal masses
Benign
Thymoma
Thymic cyst
Thymolipoma
Thymic hyperplasia
Thyroid
Teratoma
Cystic hygroma
Parathyroid adenoma
Foramen of morgagni
hernia
Malignant
Thymic carcinoma
Thyroid carcinoma
Seminoma
Mixed germ cell
Lymphoma
Thymic carcinoid


10/21/2014
14
HU, 2009
Anterior Mediastinal Masses: (4 T's)
(30% of mediastinal masses)

Thymoma
Teratoma
Thyroid (Ectopic)
(Terrible) Lymphoma

Middle Mediastinal Masses (A + B) (30%
of mediastinal masses)
Adenopathy (infection [bacterial,
granulomatous], neoplasm [leukemia /
lymphoma, metastases])
Bronchopulmonary foregut malformations
(Esophageal duplication cyst, bronchogenic cyst,
sequestration)

Posterior Mediastinal Masses: (N) (40%
of mediastinal masses)
Sympathetic ganglion tumors: neuroblastoma,
ganglioneuroblastoma, ganglioneuroma (95% of
posterior mediastinal masses)
Neurofibroma
Neurenteric cyst
Extramedullary hematopoesis
Paravertebral soft tissue mass from infection

Approach/Discussion:

PA and lateral chest films are the first step in
distinguishing from which mediastinal
compartment the mass is arising from.
Computed tomography or magnetic resonance
imaging is the next step, better characterizing
the nature and extent of the lesion, thus
narrowing the differential diagnosis. MRI is
especially good at looking for spinal canal
invasion in posterior mediastinal masses
Tissue biopsy is required for definitive diagnosis,
and surgical resection for definitive cure.
Thymoma (Staging)
Stage I : contained within an intact
capsule
Stage II: extension through the
capsule to surrounding fat, pleura,
pericardium
Stage III : Intrathoracic metastasis
Stage IV: Extrathoracic Metastasis
Thymoma(Treatment)
Stage I : Surgical resection Recurrence 2-12%
Stage II & III : Surgery + Radiotherapy
Stage IV : Multimodality Induction
chemotherapy, surgery + post op Radiotherapy
5-year Survival 12 54 %, not affected by the
presence of Myasthenia Gravis

Lymphoma
Metastatic is most common
5-10% is mediastinal primary
Second moost common Anterior
Mediastinal Mass in Adults
Malignant > Hodgkins
Dx: Mediastinoscopy, thoracotomy
NAB : Usually not confirmatory
Hodgkins Lymphoma
mediastinal widening
Germ Cell Tumors
Anterior Mediastinal location
Mainly in late teens 15 %of Ant. Med. Tumors
in Adults, 24 % in children
1/5 is Malignant
Cystic Teratoma(Dermoid Cyst) vs. Solid
tumor (Teratoma)
Solid tumor : 1/3 malignant
Radiosensitive
Teratoma, Malignant teratoma,
Seminoma(dysgerminomas)
Substernal Thyroid Tissues
Develops from cervical goiter or
intrathoracic remnants
Can be diagnosed without biopsy by
Radioactive iodine scan
No treatment unless symptomatic,
usually pressure symptoms
Rtrosternal Goiter
Neurogenic Tumors
Posterior mediastinal location
1/5 of mediastinal tumor
Originate in neural crest
Ganglioheuroma : most common in the
textbook
Neurilemmoma most common in Korea :
Dumb bell Tumor, neural sheath origin
Poosterior Mediastinal Tumor
( Neurillemmoma) )
Dumb-bell
Tumor
Neurilemmoma(Schwannoma)
Mesenchymal Tumors
Lipoma, Fibroma, Mesothelioma
Superior or Anterior mediastinal location
Diagnosis with CT scan
May cause Hypoglycemia

Mediastinitis
Acute : endoscopy complication,
Boerhaaves SD, operation, esophageal
rupture, median sternotomy
Chronic : Tbc, histoplasmosis, silicosis,
fibrosing mediastinitis

Fibrosing Mediastinitis
20- 40 years
Cough, Dyspnea, or Hemoptysis
Most common cause of Benign SVC
syndrome
Almost always remote Histoplasmosis
Plain X-rays may be normal or only
minimal changes
Partially calcified Mass on CT is
diagnostic
Pneumomediastinum
Spontaneous : mainly in young male
adults
Hamman sign
Present along the Left sternal border
Substernal pain, cough, Dyspnea,
Dysphagia

Benign Cysts
Most Common in Middle mediastinum
20% of mediastinal masses
Less common in Korea
Usually asymptomatic
Bronchogenic cyst(32%), pericardial
cyst(35%), enteric cyst(12%), thymic cyst,
and thoracic duct cyst

Pericardial Cyst
Thin-walled, mesothelial cell lining
most common in Right C-P angle
Simple cysts are almost always
asymptomatic
Rare cardiac impingement
Bronchogenic Cysts
30 - 60% of all mediastinal cysts
Lined by ciliated respiratory epithelium
May contain cartilages or mucous
Communicate with tracheobronchial trees
May become infected
Wheezing, dyspnea, recurrent pulmonary
infections
LABORATORY
INVESTIGATIONS
Hemoglobin, hematocrit, and white blood
cell count Red blood cell aplasia is found in
approximately 5% of patients with thymoma
and manifests as a normochromic-normocytic
anemia. Although rare, neutropenia can be
found in association with thymomas.

Gamma globulin levels
Hypogammaglobulinemia is associated with
some cases of thymoma

LABORATORY INVESTIGATIONS
Beta human chorionic gonadotropin

Elevated levels of bhCG virtually always are
found in association with non seminomatous
germ cell tumors.
Seminomas generally do not produce elevated
levels of this substance. Less than 10% of
patients with seminoma have an elevated
bhCG level, and the measured level is usually
much lower than that found with non
seminomatous tumors.

LABORATORY INVESTIGATIONS
Alpha-fetoprotein
AFP almost always is elevated in individuals
with non seminomatous germ cell tumors.
Elevation of the AFP level is not found in
individuals with pure seminoma.

Lactate Dehydrogenase

LABORATORY INVESTIGATIONS
Thyroid-stimulating hormone and thyroid
function studies Functioning thyroid
adenomas can develop in ectopic mediastinal
thyroid tissue and can cause thyroid
stimulating hormone suppression and
associated elevation of serum
triiodothyronine levels.
Serum and 24-hour urinary catecholamine
levels should be measured in all infants and
children who present with a posterior
mediastinal or paravertebral mass. These
levels are frequently elevated in patients with
neuroblastoma and ganglioneuroblastoma.

Adrenocorticotropic hormone (ACTH)
levels The thorax should always be
investigated for the source of ectopic
ACTH production. A neuroendocrine or
carcinoid tumor of the thorax should be
excluded. These tumors occur in the
mediastinum, particularly in the thymus
gland, and in the lung.

Antidiuretic hormone levels: These may
be elevated with some neuroendocrine
tumors of the thymus.

LABORATORY INVESTIGATIONS
10/21/2014
Anterior mediastinal masses
Benign
Thymoma
Thymic cyst
Thymolipoma
Thymic hyperplasia
Thyroid
Teratoma
Cystic hygroma
Parathyroid adenoma
Foramen of morgagni
hernia
Malignant
Thymic carcinoma
Thyroid carcinoma
Seminoma
Mixed germ cell
Lymphoma
Thymic carcinoid


10/21/2014
42
HU, 2009
10/21/2014
Thymoma
Most common tumor of the anterior
mediastinum.
Usually in adults with a median age of 50 years
with no gender preference.
40% of patients have PNS such as MG (30%),
pure red cell aplasia, or hypogammaglobulinemia
(5%-10%).
Other PNS, such as SLE, Cushing's syndrome,
and SIADH.
10/21/2014
43
HU, 2009
10/21/2014
Thymoma
MG is most frequent in , and symptoms include
diplopia, ptosis, dysphagia, weakness, and fatigue.
Diagnosis:
Serum anti-acetylcholine receptor antibody test
even if they are asymptomatic.
CT-Chest with contrast.

10/21/2014
44
HU, 2009
10/21/2014
Noninvasive Thymoma: well encapsulated,
rounded or slightly lobular shape, and usually
manifest as a solid lesion with areas of hemorrhage,
necrosis, or cystic degeneration.
10/21/2014
45
HU, 2009
10/21/2014
Invasive thymomas: infiltration of the adjacent
structures, irregular margins, areas of low
attenuation, and multifocal calcification.
10/21/2014
46
HU, 2009
10/21/2014
Thymic Carcinoma
Has cytologic and histologic features
pathognomonic of malignancy and present
a strong tendency toward early local
invasion and metastatic spread.
Rare, predominantly in middle-aged men.
Calcification in 10% to 40% and
mediastinal LAP is seen in 40% of patients.
PNS are rare.
Most patients are asymptomatic at
diagnosis.

10/21/2014
47
HU, 2009
10/21/2014
Germ cell tumors
Group of tumors with histology identical to that
of some neoplasms of the testicle and the ovary,
all of which are believed to derive from
common primary germ cells.
The mediastinum, is the most common site for
extragonadal localization.
10% to 15% of masses of the anterior
mediastinum.
Manifest in young adults in the 3
rd
-5
th
decade.
10/21/2014
48
HU, 2009
10/21/2014
Germ cell tumors
Classification:
Benign tumors (80%) are mature teratomas,
Malignant include:
Seminomas.
Nonseminomatous dysembryomas.
Malignant (immature) teratomas
Associated with Klinefelter's syndrome in 8% of
cases and hematologic malignancies.
Testing of serum -fetoprotein and (-HCG) is
mandatory


10/21/2014
49
HU, 2009
10/21/2014
Teratoma
Almost exclusively found in the anterior
mediastinum.
Often diagnosed in 3
rd
and 4
th
decade.
=
May have a solid or cystic appearance.
Mature teratomas are the most common variant,
accounting for 70% of mediastinal germ cell
tumors in children and 60% in adults.
10/21/2014
50
HU, 2009
10/21/2014
Teratoma
They are well delimited in relation to the
surrounding mediastinal structures and may be
cystic.
Incidentally discovered. However, they can reach a
remarkable size and can give rise to local symptoms.
Histologically, mature teratomas consist of
irregularly arranged, well-differentiated adult tissues
of ectodermal, mesodermal and endodermal origin.

10/21/2014
51
HU, 2009
10/21/2014
Teratoma
CT:
Well-defined, smooth or lobulated margins.
They are encapsulated and display heterogeneous
attenuation due to the combination of soft tissue,
fluid, fat, and calcific components.
They are typically multilobulated cystic tumors with
walls of varying thickness. Cough productive of hair
or sebum is a pathognomonic sign of rupture into the
tracheobronchial tree.

10/21/2014
52
HU, 2009
10/21/2014
Teratoma
Immature teratomas are made up of the same
differentiated tissues as mature forms in
association with poorly organized fetal-type
tissue.
In childhood, prognosis is good, whereas at any
other age, their behavior is often aggressive.



10/21/2014
53
HU, 2009
10/21/2014
Teratoma
Teratomas with malignant
transformation, teratocarcinomas, contain
a malignant component, most commonly
sarcoma.
These tumors tend to be larger than
benign forms and are often found to
invade adjacent structures at the time of
diagnosis.

10/21/2014
HU, 2009
54
10/21/2014
Lymphoma
Is a relatively common mediastinal tumor.
20% of all mediastinal tumors in adults and
50% in children.
They are mostly Hodgkin's lymphomas and
seldom are confined only to the
mediastinum at diagnosis.
The most common variants of non-
Hodgkin's lymphomas that primarily affect
the anterior mediastinum are large B-cell
lymphoma and lymphoblastic lymphoma
10/21/2014
55
HU, 2009
10/21/2014
Middle mediastinal masses
Benign
Benign adenopathy
Cysts
Esophageal mass
Hiatal hernia
Cardiac and vascular structures
Lipomatosis
Cardiophrenic fat pad
Foramen of morgagni hernia
Ectopic thyroid
Malignant
Lymphoma
Metastases
Esophageal cancer
Thyroid carcinoma




10/21/2014
HU, 2009
56
10/21/2014
Enlarged Lymph Nodes
Located around the tracheobronchial tree, the
lower esophagus, or the aortopulmonary window.
DDx: malignancy (e.g., lymphoma, metastatic
spread from lung cancer or other malignancies)
and inflammatory lymph node changes due to
infection (tuberculosis, histoplasmosis) or
granulomatous disease (sarcoidosis).
CT scans may show typical patterns such as
calcifications (tuberculosis) or necrosis
(lymphomas).

10/21/2014
HU, 2009
57
10/21/2014
Bronchogenic Cysts
Result from abnormal budding of the tracheal
diverticulum between the 3
rd
&6
th
wks of
gestation.
5% to 10% of all mediastinal lesions.
Usually found adjacent to the tracheobronchial
tree but can be also found in the posterior
mediastinum or within the lungs.
Cause symptoms in adults in 30% to 45% of
cases
10/21/2014
HU, 2009
58
10/21/2014
Bronchogenic Cysts
At CT, well-defined, round masses with a
homogeneous density similar to water.
However, density and the heterogeneous aspect
can make diagnosis difficult
If there is a direct communication with the
tracheobronchial tree, air-fluid levels can be
seen.

10/21/2014
HU, 2009
59
10/21/2014
Pericardial Cysts
Benign intrathoracic lesions and constitute 7% of
all mediastinal tumors.
Typically located at the right cardiophrenic angle
(50%-70%) or at the left cardiophrenic angle
(30%-40%), or rarely in other mediastinal
locations not adjacent to the diaphragm.
Their size varies from a few centimeters to 30
cm.
They are usually congenital but may also be
acquired after cardiothoracic surgery.
10/21/2014
HU, 2009
60
10/21/2014
Pericardial Cysts
Most pericardial cysts are asymptomatic.
Patients may present with:
Chest discomfort
Dyspnea
Cough
Life-threatening complications such as cardiac
tamponade have been reported.
10/21/2014
HU, 2009
61
10/21/2014
Pericardial Cysts
Definitive diagnosis by use of CT may be
challenging.
Echocardiography was shown to be a
superior noninvasive modality in selected
cases to delineate the exact location of the
cyst and to differentiate it from other
potential diagnoses such as fat pad,
ventricular or aortic aneurysm, and solid
tumors

10/21/2014
HU, 2009
62
10/21/2014
Pericardial Cysts
10/21/2014
HU, 2009
63
10/21/2014
Posterior mediastinal masses
Benign
Neurofibroma
Schwannoma
Ganglioneuroma
Pheochromocytoma
Chemodectoma
Foramen of bochdalek hernia
Meningocele
Malignant
Neurofibrosarcoma
Neuroblastoma
Ganglioneuroblastoma
M.pheochromocytoma
M. chemodectoma


10/21/2014
HU, 2009
64
10/21/2014
Neuroblastoma
Highly malignant.
Most common extracranial solid malignancy in
pediatric patients.
The most common intrathoracic malignancy of
childhood.
Adrenal gland is a common primary site, but
14% of all neuroblastomas arise in the thorax,
where the tumors are commonly associated with
extension into the spinal canal and osseous
invasion.
10/21/2014
HU, 2009
65
10/21/2014
Neuroblastoma
Is not as recalcitrant to chemotherapy and
surgical resection as are other chest
malignancies.
They are more likely to be resectable, with less
invasion of surrounding organs.
>1/2 occur in children <2 years of age.
90% arise within the first decade of life

10/21/2014
HU, 2009
66