hepatoblastoma
Pronunciation: hep-ah-toe-blas-TOH-mah
Most common primary liver malignancy in children, arising from primitive hepatic cells.
Full Definition
Hepatoblastoma is the most common primary hepatic malignancy in children, accounting for approximately 1% of all pediatric cancers. These tumors typically arise in children under 3 years of age and originate from primitive hepatic cells or hepatocyte precursors. Histologically, hepatoblastomas are classified into epithelial and mixed subtypes, with the epithelial type further subdivided into fetal, embryonal, and small cell undifferentiated patterns. The fetal pattern generally carries the best prognosis. Risk factors include very low birth weight, Beckwith-Wiedemann syndrome, and familial adenomatous polyposis. Serum alpha-fetoprotein is characteristically elevated and serves as both a diagnostic marker and monitor of treatment response.
Usage
Usage note: Specify histological subtype (fetal, embryonal, mixed) as this affects prognosis. Usually occurs before age 3.
In Context
- "Histopathological examination revealed a mixed hepatoblastoma with both fetal and embryonal epithelial patterns." — Pediatric pathology report
- "The elevated alpha-fetoprotein level of 50,000 ng/mL was consistent with hepatoblastoma in this 18-month-old patient." — Laboratory correlation