Hepatic epitheloid hemangioendothelioma: A rare indication for liver transplantation

Abstract

Hepatic epithelioid hemangioendothelioma is a rare malignancy arising from the vascular endothelial cells within the liver. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Because the disease is rare, there is no clearly established therapeutic algorithm. Hepatic epithelioid hemangioendothelioma seems to be resistant to chemotherapy and radiotherapy. Either surgical resection or orthotopic liver transplantation is generally recommended as a curative treatment for this disease. However, in patients who have multifocal or diffuse enlargement, liver resection is frequently futile. We herein present a case with irresectable and metastatic hepatic hemangioendothelioma who underwent orthotopic liver transplantation and survived for 4 years.