Management of hepatic epithelial malignancy in childhood and adolescence

This review addresses the management of epithelial liver tumors of childhood and adolescence (hepatoblastoma and hepatocellular carcinoma), which constitute approximately 90% of primary liver malignancy in this age group. The epidemiology, pathology, clinical presentation, and diagnosis are given in order to appreciate differences in biological behavior of these two neoplasms and the need for a distinct therapeutic approach to each. The multidisciplinary treatment of hepatoblastoma has become increasingly refined and long-term survival can be expected in approximately 80% of patients. Where survival once depended solely on complete surgical resection, it is now also possible in patients with initially unresectable tumors due to effective cytoreductive chemotherapy. The problem of systemic relapse following complete surgical resection has been reduced although not eliminated by adjuvant chemotherapy programs. To date, the biological behavior of hepatocellular carcinoma prohibits complete resection in the majority of children and chemotherapy has not been effective. Early detection, development of new agents and techniques such as monoclonal antibodies and total hepatectomy with autologous transplantation in selected cases may offer hope for the future.