- 3rd most common intra-abdominal malignancy (after Neuroblastoma and Wilms
- Median age of dx is: 12 months
presents with abdominal
distention and hepatomegaly; a discrete tumor mass generally is not palpable.
- The mass
may move more with inspiration than do
renal or adrenal masses.
- Usually arises from the right lobe and is unifocal.
- What % have jaundice at time of dx? 5%
- Mets at diagnosis in 10%-> local invasion, regional lymph nodes, lungs
- Which tumor marker is increased? Increase in AFP in 70-90%
- Small subset secretes B-HCG: Can lead to precocious puberty
- Thrombocytosis is characteristic (>1 million)
- Associated with Beckwith-Wiedemann syndrome
- It may be
difficult to differentiate hepatoblastoma initially from neuroblastoma,
lymphoma, or leukemia with hepatic involvement.
- Treatment is excision and chemotherapy
- Survival is 90% with multimodal therapy
Hepatoblastoma vs Hepatocarcinoma
- Age of presentation: HB 1 yr, HC 12 yrs
- Liver distribution: HB unifocal, HC multifocal
- Associations: HB Beckwith-Wiedemann, HC Hep B
- Survival: HB 90%, HC 30%
||Age of Presentation (years)
||bone, BM, liver
||LN, lungs, BM, Bones
CHLA Board Review 2005