hepatoblastoma
Pronunciation: hep-AT-oh-blas-TOH-mah
The most common primary liver malignancy in children, typically occurring in infants and toddlers under 3 years of age.
Full Definition
Hepatoblastoma is an embryonal tumor arising from hepatic parenchymal cells, representing approximately 1% of all childhood cancers but accounting for two-thirds of liver tumors in children. It most commonly affects children under 3 years of age, with a peak incidence in the first two years of life. The tumor is often associated with elevated alpha-fetoprotein (AFP) levels, which serve as both a diagnostic marker and a tool for monitoring treatment response. Risk factors include Beckwith-Wiedemann syndrome, familial adenomatous polyposis, and very low birth weight. Treatment typically involves surgical resection with or without neoadjuvant chemotherapy, and liver transplantation may be considered in unresectable cases.
Usage
Usage note: Distinguish from hepatocellular carcinoma, which is rare in children; AFP elevation is a key diagnostic feature.
In Context
- "Serum AFP levels were markedly elevated at diagnosis of hepatoblastoma." — Laboratory report
- "The PRETEXT staging system was used to evaluate the hepatoblastoma for surgical planning." — Surgical consultation