Long term follow up after resection emphasizes the role of surgery in Primary Hepatic Epithelioid Hemangioendothelioma

•Primary Hepatic Epithelioid Hemangioendothelioma is an extremely rare tumor of vascular origin. In this work we present clinical data and long term results of eight patients who were surgically treated in our institution.•Liver transplantation was performed in five patients (four cadaveric and one living related) and major liver resection in three patients. After a median follow up of 100 months (48–266) all eight patients are alive. During follow up, three patients developed recurrent tumor, one in the lung and the liver, one only in the liver, and one patient developed systemic metastases.

BackgroundPrimary Hepatic Epithelioid Hemangioendothelioma is an extremely rare tumor of vascular origin.PatientsIn this work we present clinical data and long term results of eight patients who were surgically treated in our institution. Liver transplantation was performed in five patients (four cadaveric and one living related) and major liver resection in three patients. Three patients are alive with recurrent tumor. After a median follow-up of 100 months (48–266) all patients are alive.ConclusionsOnly surgery can provide cure in HEH. If the extent of the disease prohibits primary resection liver transplantation might offer the most valuable option.