hepatoblastoma

malignant embryonal neoplasm Epidemiology: - most frequent liver tumor in children - 4% present at birth - 68% first two years - 90% by age 5 years Pathology: - variable histological patterns - pure fetal epithelial differentiation: ~ 1/3 of cases resemble hepatocytes in developing fetal liver - combined fetal and embryonal epithelial - macrotrabecular - small cell undifferentiated - mixed epithelial & mesenchymal - mesenchymal components include fibrous tissue, osteoid- like tissue & cartilagenous tissue - mixed with teratoid features - additional possibile features include striated muscle, bone, mucinous epithelium, squamous epithelium, melanin pigment Genetics: - associated with mutations in CTNNB1 Laboratory: - alpha fetoprotein elevated in 90% - anemia 70% - thrombocytopenia 50%

General

embryonal neoplasm liver cancer; hepatobiliary carcinoma

References

WHO Classification - Tumours of the Digestive System. Hamilton & Aaltonen, eds. IARC Press 2000