• 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.

17. Other Malignant Primary Tumors of the Liver

Matteo Cescon;Valentina Bertuzzo;Gian Luca Grazi;Antonio Daniele Pinna
2016

Abstract

• 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.
Principles of Hepatic Surgery
280
294
Matteo Cescon, Valentina Bertuzzo, Gian Luca Grazi, Antonio Daniele Pinna
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11585/655639
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