Advances in Pediatric Liver Tumors

Hepatoblastoma and hepatocellular carcinoma are rare pediatric tumors. We review the significant advances in hepatoblastoma and pediatric hepatocellular carcinoma prognosis and treatment. International pathologic classification and risk stratification have been extensively reviewed and redefined for hepatoblastoma via international collaborative analyses of an international hepatoblastoma database. International trials have identified patients for whom (1) no adjuvant chemotherapy is indicated, (2) neoadjuvant chemotherapy improves resectability and survival, and (3) intensified therapy improves survival (for patients with metastatic disease). Hepatocellular carcinoma studies highlight the poor prognosis for patients with nonlocalized disease emphasizing the need for future collaborative efforts exploring molecular characteristics and novel therapeutics. Recent advances have significantly improved treatment of hepatoblastoma by implementing a consensus international pathologic classification and risk stratification and identification of higher risk biological features. Advances in pediatric hepatocellular carcinoma treatment lag behind hepatoblastoma. The Pediatric Hepatic International Tumor Trial (PHITT) will focus on decreasing long-term toxicity, improving outcomes, providing surgical guidelines, and advancing the knowledge of the biology of hepatoblastoma and hepatocellular carcinoma.