Einar Hafberg1, Scott C Borinstein2, Sophoclis P Alexopoulos3. 1. Division of Gastroenterology, Department of Pediatrics, Vanderbilt University Medical Center. 2. Division of Pediatric Hematology/Oncology, Department of Pediatrics, Vanderbilt University Medical Center. 3. Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Abstract
PURPOSE OF REVIEW: Hepatoblastoma is the most common primary pediatric liver malignancy. The goal of treatment in hepatoblastoma is complete surgical resection. Recently published multinational collaborative studies are better defining risk factors for disease recurrence and guide optimal treatment strategy. RECENT FINDINGS: Successful margin-negative resection of hepatoblastoma is dependent on the location and extent of disease as defined by the PRETEXT classification. Liver transplantation is an appropriate treatment modality when complete oncological resection requires total hepatectomy. In general, advanced PRETEXT class as well as histologic features, age at presentation, tumoral production of α-feto protein and the presence of metastatic disease adversely affect outcome. Hepatoblastoma is chemosensitive and significant downstaging can occur with the use of neoadjuvant chemotherapy allowing for less extensive hepatectomy. In addition, patients at moderate-to-high risk of postresection recurrence should receive neoadjuvant chemotherapy. Cisplatin-based chemotherapy can allow for resection of transplantation of patients with metastatic disease when complete metasatectomy can be achieved albeit with inferior results. SUMMARY: Treatment of hepatoblastoma with surgical resection or liver transplantation is associated with excellent long-term results in the setting of modern chemotherapy.
PURPOSE OF REVIEW: Hepatoblastoma is the most common primary pediatric liver malignancy. The goal of treatment in hepatoblastoma is complete surgical resection. Recently published multinational collaborative studies are better defining risk factors for disease recurrence and guide optimal treatment strategy. RECENT FINDINGS: Successful margin-negative resection of hepatoblastoma is dependent on the location and extent of disease as defined by the PRETEXT classification. Liver transplantation is an appropriate treatment modality when complete oncological resection requires total hepatectomy. In general, advanced PRETEXT class as well as histologic features, age at presentation, tumoral production of α-feto protein and the presence of metastatic disease adversely affect outcome. Hepatoblastoma is chemosensitive and significant downstaging can occur with the use of neoadjuvant chemotherapy allowing for less extensive hepatectomy. In addition, patients at moderate-to-high risk of postresection recurrence should receive neoadjuvant chemotherapy. Cisplatin-based chemotherapy can allow for resection of transplantation of patients with metastatic disease when complete metasatectomy can be achieved albeit with inferior results. SUMMARY: Treatment of hepatoblastoma with surgical resection or liver transplantation is associated with excellent long-term results in the setting of modern chemotherapy.