Abstract

Primary malignant liver cancers in children have an overall incidence of two cases per million. Hepatoblastoma (HB) is the most common, and the majority will present in infants and toddlers within the first 2 years of life. On the other hand, hepatocellular carcinoma (HCC) which is the second most frequent malignant liver tumor will present later, typically in school-aged children or adolescents. The fundamental principle for treatment is complete surgical resection of the tumor. Unfortunately 50 % of children with HB and 70 % of those with HCC present initially as inoperable due to the extent of tumor involvement, vascular invasion, or distant metastasis. The prognosis for children that cannot ultimately achieve a complete tumor resection or transplant is dismal. HB is generally chemosensitive, particularly to platinum-based agents such as cisplatin. Following systemic chemotherapy up to 85 % are appropriate candidates for surgical resection. However, HCC remains relatively chemoresistant, and the fibrolamellar type of HCC most often attributable to children (13–22 % of HCC in children) does not respond any differently than the typical HCC with current therapeutic regimens, having an overall survival rate below 25 % at 3 years.

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