Abdominal Masses

Question 1

Abdominal Mass work up

Answer 1

• Prenatal US
• US with doppler
• MRI
• Location
• Cystic/complex/solid

Question 2

Hepatoblastoma

Answer 2

• A/w premie and low birth weight
• Genetic predisposition: Beckwith-Wiedemann syndrome, FAP.
• Reasonable chance of cure when complete resected. 90% survival for stage I/II, 50% for stage III/IV

Question 3

Neuroblastoma

Answer 3

• Adrenal: 40%
• ABD: 25%
• Thoracic: 15%
• Cervical: 5%
  Presentation: Mass, Pain, Obstruction, Diarrhea

Question 4

Wilm’s Tumor (Nephroblastoma)

Answer 4

• Most common RENAL tumor
• 4th most common childhood cancer.
• Majority present < 10 years old
• Bilateral presentation in 10%
• Ethnic variation: AA > White > Asian.

Question 5

Wilm’s tumor clinical features

Answer 5

• Often asymptomatic upper and mass in well appearing child.
• 25% have pain.
• 25% hematuria (tumor extension into renal pelvis)
• anemia if bleeding into the tumor.
• fever in 25%
• Hypertension in 25% due to distortion of renal vasculature.

Question 6

Wilm’s Tumor associations

Answer 6

• Beckwith-Wiedemann Syndrome. Macroglossia, hemi-hypertrophy, organomegaly, ~10% develop Wilm’s tumor. Patients with Beckwith-Wiedemann Syndrome can also develop hepatoblastoma, adrenal-cortical carcinoma.

-WAGR (mutration in WT1). 30% have Wilm’s Tumor, also Aniridia, GU malformations, mental retardation.

• Isolated hemihypertrophy: ~4% develop Wilms Tumor

Question 7

Lymphomas

Answer 7

• present as palpable RLQ mass (can be confused with Crohn’s disease).
• Burkett’s lymphoma: ~30% of pediatric lymphomas. aggressive B cell non-Hodgkin’s lymphoma. It is sporadic, endemic (African), immunodeficiency.
  Peak: 11 years old. Rapid growth. Presentation: ascites, bowel obstruction, GI bleeding.

Question 8

Teratomas

Answer 8

• Rare. Usually male, usually <2 years old, (Majority <3 months).
• Composed of mesodermal, endodermal, and ectodermal elements.
• Irregular soft tissue mass: both solid and cystic components and calcifications on imaging.
• Teratomas require complete resection.

Question 9

Enteric Duplication Cysts

Answer 9

• Uncommon: 1-5,000-10,000
• Presentation: Mass, obstruction, peptic ulceration
• tubular/spherical: anywhere from mouth to anus,
• most common location: Ileocecal.
• well developed smooth muscle layer, epithelial lining usually resembles adjacent intestine
• attachment to part of GI tract, blood supple shared with adjacent normal structures, usually not in continuity with intestinal lumen.
• mesenteric side of SI. Anti-mesenteric side of colon.

Question 10

Mesenteric/Omental cysts

Answer 10

• Lymphatic in origin.
• No Communication with the lymphatic system. Range from few mm to 40cm
• usually present in ideal mesentery.
• can be confused with chylous ascites.
• mean age of presentation: 4 years.