Hepatic malignancies in childhood and adolescence (hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma).

Abstract

Hepatoblastoma is the most common malignant liver tumor of childhood. Clinical trials have demonstrated its responsiveness to chemotherapy, especially with platinum-based chemotherapeutic agents. In patients with completely resected tumors, recurrent disease is effectively controlled by adjuvant chemotherapy. In patients with initially unresectable tumors, chemotherapy can induce tumor shrinkage sufficient to allow complete extirpation of tumor and also to prevent recurrent disease. The child with tumor resistant to primary therapy or with recurrent disease presents a special problem requiring individualized and innovative therapies, including consideration of orthotopic liver transplant. Hepatocellular carcinoma in children and adolescents carries a much poorer prognosis compared to hepatoblastoma. Complete resection of tumor offers the only hope of cure, but these tumors are unfortunately resistant or partially resistant to conventional doses of chemotherapy. A number of innovative treatment strategies have been employed, but optimal treatment remains elusive. Transplant for tumor localized to the liver may offer the only hope of cure. Embryonal (undifferentiated) sarcoma of the liver is a rare tumor that has not been studied prospectively in any clinical trial. Small published series indicate that it can be responsive to chemotherapy, and cure may be possible.