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Detailed information of HEPATOBLASTOMA
HEPATOBLASTOMA
DEFINITION:
A malignant tumor of the liver usually presenting as a right
upper quadrant (RUQ) mass.
EPIDEMIOLOGY:
- incidence: 0.9/million
- age of onset:
- median age: 12 months; majority detected by 18 months of age
- risk factors:
- M > F (1.5-1.7:1)
- maternal use of gonadotropins or alcohol
PATHOGENESIS:
- hepatoblastomas have been associated with:
- Beckwith-Wiedemann Syndrome
- Wilms' Tumor
- Rhabdomyosarcoma
- Aicardi Syndrome
- Familial Adenomatous Polyposis
- hepatoblastomas and the first three disorders may share a
common chromosomal anomaly -> abnormal gene product -> tumor
CLINICAL FEATURES:
- RUQ mass
- +/- abdominal pain (15-20% of patients)
- +/- abdominal distension
- hepatomegaly/splenomegaly
- digital clubbing
- jaundice (in <5% of cases)
- tumor rupture with acute abdominal signs/symptoms
2. Others
- anorexia, nausea/vomiting, weight loss
- hemihypertrophy (in 2-3% of cases)
- congenital hemangiomas
2. Endocrine
- isosexual precosity (virilization)
- in 10% of male patients
- genital enlargement, pubic hair
3. Metastases
INVESTIGATIONS:
- 4 cell subtypes:
- fetal
- embryonal
- macrotrabecular
- small-cell undifferentiated
- highly pedunculated and vascular with areas of focal
necrosis and vascular invasion
- multicentric > right lobe > left lobe
2. Imaging Studies
- solid tumor with calcifications in 30%
2. Chest X-Ray/CT/MRI
- 10% will have pulmonary metastases
3. Skeletal X-Rays
- osteopenia, multiple pathologic fractures, vertebral
compression (hepatoblastomas may be associated with an
abnormal calcium metabolism)
3. Serum
- elevated alpha-fetoprotein in 90-95% of cases
- elevated beta-hCG (in virilizing males)
- elevated transaminases and bilirubin in 20% of cases
MANAGEMENT:
- right and left partial and complete lobectomy and
trisegmentectomy
2. Liver Transplantation
2. Chemotherapy
- used pre and post resection
- can reduce size of the primary tumor by 90% and pulmonary
metastases
- doxorubicin, cisplatin
3. Prognosis
- 5 year survival rate is 35%
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Pediatric Database - HEPATOBLASTOMA
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