The contribution of transplantation to the treatment of liver tumors in children

Major progress has been achieved during the last decades in the treatment of malignant liver tumors in children, both in chemotherapy and surgical management. Chemosensitivity varies between tumor types, and radical resection remains essential to effect a cure. In tumors extensively involving a normal liver, in a diffuse or multifocal manner, radical resection cannot be accomplished with a partial hepatectomy. This has been the case for some instances of advanced hepatoblastoma and epithelioid hemangioendothelioma. In hepatoblastoma, current experience shows that results of primary liver transplantation with neoadjuvant chemotherapy are excellent with around an 80% 5-to-10-year disease-free survival rate. Epithelioid hemangioendothelioma is very rarely seen in children and may have a more malignant behavior than in adult patients, and liver transplantation may not be the best management option. In nonresectable hepatocellular carcinoma (HCC) developed on an otherwise normal liver, the results of liver transplantation are similarly poor to those obtained in adult patients, except in a few highly selected series fulfilling the Milano criteria. The experience with HCC is still very scarce in children. Incidental HCC associated with chronic liver disease does not seem to impact posttransplant survival. When they are symptomatic, however, indications for transplantation should be very selective regarding tumor size, multifocality, vascular invasion and distant metastases.