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Current Evidence
Tumors of the hepatobiliary tree in children are more likely to be metastases rather
than primary tumors of the liver ( Table 98.6 ). Of the primary tumors,
hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common (
Fig. 98.2 ). HB usually occurs in patients younger than 6 years and is associated
with risk factors such as overgrowth syndromes (e.g., Beckwith–Wiedemann),
prematurity, and familial adenomatous polyposis (FAP). In contrast, HCC is more
common in patients older than 6 years, particularly in children older than 10 years.
Risk factors include chronic liver injury from inborn errors of metabolism such as
tyrosinemia, glycogen storage disease type I, chronic hepatitis, chronic iatrogenic
androgen exposure, or cirrhosis for any reason. In general, these latter risk factors
rarely lead to cancer in childhood.