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Abdominal Solid Tumor:

Clinical Diagnosis and Management


Endang Windiastuti Hematology-Oncology Division Department of Child Health FMUI dr Cipto M Hospital, Jakarta

Abdominal Mass

Serious finding Very broad spectrum of pathologies


From small lesions to large ones occupying peritoneal cavity, from benign to malignant From unilocular cysts to complex solid ones Malignant ? Compressing vital organ ? There is intestinal hemorrhage ?

Need to find out if :


The older the child the more likely the mass represents a malignant process

Objective
Points to address in history taking Physical findings associated with abdominal mass Routine laboratory and imaging studies needed Overview of most common pediatric abdominal mass

General Approach
History Onset Location Associated Symptoms Physical Examination Inspection Auscultation Percussion Other Maneuvers Special investigation

General Approach
History

Onset

Location

acute chronic progression Upper Abdomen Lower Abdomen

Associated Symptoms Pain Urinary Symptoms Fever OBGY Symptoms Abd distension Endocrine/ Cardio GI Symptoms Hematologic

General Approach
History

Antenatal : Any abnormalities on antenatal Poly/oligohydramnion Ultrasound findings Duration Age : neonates and infant / older children Rate of growth Significant family history (adenomatous polyposis) Inherited predisposition

Clinical History
Age is important
Neonatal Congenital malformations (GUT / GIT abnormalities)
- malignancies uncommon

Older infants and Children (peak age 1-5 years old)

Wilms and Neuroblastoma mostly Germ cell tumors Non-Hodgkin lymphoma Non-Hodgkin lymphoma Consider : Inflammation process and pregnancy

Adolescents

Physical Examination
Inspection :

Shape of abdomen Scar Superficial lesions Bulges

Auscultation : bowel sound, bruit Palpation : tenderness, rigidity, character of mass Percussion : distinguishes causes of distention

(gas, fluid, solid)

Abdominal Examination

Site :

- central / flank
- pelvic filling - unilateral/bilateral - crossing midline

Characteristics : -size
- consistency - tender - smooth /nodular - mobile / fixed

Associated findings : ascites


pleural effusion

Initial Blood Work


Renal function/ Electrolyte Uric acid/ LDH (+ALT) FBC / Diff LFT Possible renal involvement / impairment Increased cell turn over suggesting malignancy Bone marrow involvement Liver involvement

Laboratory Examination
CBC : WBC with left shift (infection) Pancytopenia :

Bone Marrow infiltration by malignancy Marrow suppressed by infection Often seen with liver tumor (thrombopoietin produced by tumor)

Thrombocytosis

Laboratory Examination
Coagulation studies

Presence of DIC Liver dysfunction Hematuria or proteinuria (Renal and bladder function)
Urine VMA, AFP, HCG

Urinalysis

Tumor Markers :

Tumor Specific Investigation

Tumor markers AFP HepB sAg HCG Hepatoblastoma Hepatocellular Ca Germ cell tumors

Urine HVAs Ferritin Urine NMA (24 hrs) ESR

Neuroblastoma Neuroblastoma Pheochromocytoma Lymphoma

Special Investigation : bone marrow puncture / biopsy Neuroblastoma Malignant rhabdoid tumor Lymphoma Renal clear cell sarcoma Rhabdomyosarcoma

Initial Imaging for Diagnosis


X-Ray Abd XR :Controversial as to necessity
Describe location & density ?Obstruction ?Calcification

Chest XR :Metastasis
Staging Malignant effusion Exclude TB

Initial Imaging for Diagnosis


Ultrasound

Cheap and readily available Sedation not necessary Good initial imaging helpful with initial diagnosis

Organ of Origin Tissue components : cysts, hemorrhage, calcification Vascular lesions (doppler)

Further Imaging

CT-Scan :

if suspicious of malignancy Determination size and infiltration into vessel or vital organ

MIBG Scan Bone Scan

Neuroblastoma Neuroblastoma Clear cell sarcoma Lymphoma

PET Scan

Further Imaging
MRI Best imaging of abdominal tumors (abdomen & pelvis) Expensive No radiation : implications for initial and follow up scans. CT Chest Essential to assess chest metastases CT Abdomen Good imaging for blood vessels Radiation : Implication for follow up imaging

Abdominal Mass
History and PE Intra -Abdominal

Abdominal Wall Infectious Non-Infectious

Malignant

Non-Malignant Intraperitoneal Retroperitoneal

Neonatal Abdominal Masses

Renal 55% Hydronephrosis 35% Cystic disease 10% Multicystic dysplastic Polycystic dysplastic Solid Tumors 10% Mesonephric nephroma nephroblastomatosis Pelvic / Genital 15% Teratoma Ovarian Cysts Hydrometrocolpos Obstructed bladder Non-Renal Retroperitoneal 10% Adrenal Hemorrhage neuroblastoma

Gastrointestinal 15% Duplication Mesenteric omental cyst Pseudocyst from complicated obstruction Meconium ileus Hepatobiliary 5% Hepatic tumors Hemangioendothelioma Cystic mesenchymal
Choledochal cyst
hamartoma Hepatoblastoma Neuroblastoma

Kirk et al., 1981 Radiol. Clin. North Am., 19:527-545

Distribution of abdominal mass in neonates by organ system


Kirks et al. Radiol Clin North Am 1981;19:527-545

Abdominal Masses in Older Children

Renal Wilms (& other) Hydronephrosis Cystic disease

55% 25% 20% 5%

Non Renal Retroperitoneal 23% Neuroblastoma 21% Teratoma 1% Other 1%

Gastrointestinal 12% Appendiceal Abscess Lymphoma Hepatobiliary 6% Tumors Hepatoblastoma HCC Genital 4% Ovarian Cysts and Teratoma

Kirk et al., 1981 Radiol. Clin. North Am., 19:527-545

The Commonest Malignant Abdominal Masses in Children:


1. Neuroblastoma. 2. Wilms tumor (Nephroblastoma). 3. Malignant lymphoma (usually Non-Hodgkin lymphoma of the intestine). 4. Hepatoblastoma. 5. Lymphosarcoma. 6. Other Suprarenal tumors (Non Neuroblastoma). 7. Others .

The commonest benign masses


1.

Hydronephrotic kidney (Peliviureteric junction obstruction), polycystic kidney. Mesenteric mass and cysts. Intestinal Duplication cysts. Hydatid cysts in the older children

2. 3. 4.

A mass in the toddlers (1-3 years).

Triad of :
1. 2. 3.

Abdominal Neuroblastoma Wilms Tumor. Hydronephrosis.

Minimal local or general symptoms, usually the mass discovered by the mother.

Possible Diagnoses of Abdominal Masses in Infancy and Childhood


Region
Epigastrium Flank

Organ
Stomach Pancreas Kidney Adrenal Retroperitoneal Ovary Kidney Urachus Omentun,mesentery Bladder, prostate Uterus, vagina Biliary tract Liver Intestine Spleen Appendix Ileum Lymphatic Colon Lymphatic

Diagnosis
Distended stomach from pyloric stenosis, duplication Pseudocyst hydronephrosis, Wilms tumor, dysplastic kidney, ureteral duplication Neuroblastoma, ganglioneuroblastoma, ganlioneuroma Neuroblastoma, ganglioneuroblastoma, ganglioneuroma, teratoma Desmoid, teratoma, ovarian tumors, torsion of ovary Pelvic kidney Urachal cyst Omental, mesenteric, peritoneal cysts Obstructed bladder, rhabdomyosarcoma Hydrometrocolpos, hydrocolpos,rhabdomyosarcoma Cholecystitis, choledochal cyst Hepatomegaly (congestion, hepatitis), hamartoma, hemangioendothelioma, hepatoblastoma, hepatocelullar Ca, abscess,cyst intussusceptions, duplication Splenomegaly (infection, leukemic infiltration, abscess, cyst) Appendiceal abscess Meconium ileus, inflammatory mass Lymphoma, lymphangioma Fecal impaction Lymphoma, lymphangioma Atlas of Pediatric Physical Diagnosis, Fourth edition

Lower abdomen

Pelvic Right upper quadrant

Left upper quadrant Right lower quadrant

Left lower quadrant

Work up two components


Staging
X-ray of primary site CT chest, abdomen, & pelvis CXR (baseline) Bone scan Specialty tests Gallium, MIBG, PET Bone marrow ESR

Evaluate for complications of the tumor


CBC with diff. count Others LDH, uric acid tumor lysis, rapid cell growth Creatinine renal function Transaminases hepatic involvement Specialty tests Tumor markers
HCG, AFP HVA / VMA

Tissue Diagnosis

Incisional biopsy Excisional biopsy Special cases Calicified suprarenal mass + bone scan might consider getting dx from bone marrow FNA vs excisional biopsy Bias towards excisional sufficient sample to be representative and to send for special research studies (histology, chromosomes, special studies, research studies)

Algorithm for Evaluation of a Neonatal Mass


Abdominal Mass Abdominal Radiograph

Bowel obstruction Surgery Consider : Contrast studies

Bowel gas displacement

Calcifications Consider :

ULTRASOUND Confirmed

Neuroblastoma Teratoma Hepatoblastoma Meconium peritonitis

Flank Mass Adrenal Origin Renal Other

Intraperitoneal Mass Solid Cystic Complex

Pelvic Mass Solid Cystic

Solid

Cystic

Schwatz Mz, Shaul DB. Ped in Rev 1989;11:172-9

Algorithm for Evaluation of a Neonatal Mass


Flank Mass

Adrenal Origin

Renal

Other

Solid CT-scan

Cystic Adrenal hemorrhage?

Solid CT-scan
Mesoblastik Nephroma Wilms Tumor

Cystic Normal Ureter IVP, Renal Scan Dilated Ureter No reflux


Obstruction Neurogenic Bladder

Solid CT-scan
Teratoma, Sarcoma Neurobl

Cystic
Lymphangio ma

Neuroblastom Observation Surgery

Reflux
Neurogenic Bladder

Surgery

Surgery

Multicystic kidney Obstruction

Surgery Surgery
Schwatz Mz, Shaul DB. Ped in Rev 1989;11:172-9

Surgery

Algorithm for Evaluation of a Neonatal Mass


Intraperitoneal Mass
Solid Cystic Complex

CT

Ovarian
mesentric omental

Organomegaly Tumor : Hepatoblastoma Hemangioma

Intestinal duplication Spleen Surgery Angiogram ?

Ovarian teratoma Meconium cyst

Surgery

Surgery

Schwatz Mz, Shaul DB. Ped in Rev 1989;11:172-9

Conclusion
Abdominal masses in neonates and children reflect a wide spectrum of diseases The patients age is among the most important factors that help narrow the potential etiologies of an abdominal mass Plain abdominal radiographs should be the first imaging studies to evaluate an abdominal mass It is important for physician to determine the nature of the mass in a timely, safe, and cost-effective manner.

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