Hepatocellular carcinoma. Review of 32 cases in childhood and adolescence.

The clinical and pathologic features of 32 children and adolescents with hepatocellular carcinoma (HCC) are reviewed. Their average age at diagnosis was 9.7 years (range, 5 months to 21 years) and there was a slight predilection for males in a ratio of 1.7 to one. Of eight patients with associated or underlying abnormalities, five had cirrhosis, two had an antecedent (or coexisting) tumor fulfilling pathologic criteria for hepatic adenoma, and one developed HCC ten years after nephrectomy and radiation therapy for a Wilms' tumor. Our data reaffirm the high mortality associated with HCC (91%). Three of five tumors classified as fibrolamellar type were amenable to surgical resection while only 15% of the remaining HCC were operable. The average duration of disease for patients with conventional HCC was 4.2 months, while those with the fibrolamellar variant had a more lingering course (average, 28.5 months). Available data indicate that the fibrolamellar variant should be distinguished from HCC with more conventional histology because of greater resectability and improved overall survival.