17. Other Malignant Primary Tumors of the Liver

• The most common malignant hepatic tumor is by far hepatocellular carcinoma, followed by intrahepatic cholangiocarcinoma. However, other primary malignancies can occur and require specific approaches. • Cystadenocarcinoma is a cystic tumor that usually develops from hepatic biliary cystadenoma. In both cases, radical surgical resection is the treatment of choice. • Primary hepatic lymphomas are very rare (contrary to the frequent secondary involvement of the liver in disseminated non-Hodgkin lymphoma) and frequently associated with hepatitis C virus infection. Chemotherapy is efficient for most tumors and surgical resection indicated for complete resection of limited tumors or to reduce tumor burden before systemic therapy. • Most cases of hepatoblastoma occur in childhood. Prognosis in adults is much poorer due to late diagnosis. Treatment is based on complete surgical resection. • Hepatic epithelioid hemangioendothelioma is a rare vascular tumor with variable degrees of aggressiveness. Surgical resection (including liver transplantation) is the best approach when no extrahepatic involvement is present. • Angiosarcoma, the most common primary hepatic sarcoma, represents up to 2% of primary liver malignancies, occurs more frequently in aged patients, and carries a very poor prognosis. Surgical resection is the best option in resectable cases, although recurrence is very common. Chemotherapy and trans-arterial therapy are used alone (to prevent bleeding and avoid tumor growth) or in combination with surgery.