Hepatic epithelioid hemangioendothelioma: long-term results of surgical management.

BACKGROUND AND OBJECTIVES Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular neoplasm of the liver. Its therapeutic management remains difficult to define in curative intent. The aim of this study was to report long-term results of surgically managed patients. METHODS From 1990 to 2006, nine patients (25-64 years) were retrospectively enrolled in this study. Intrahepatic disease extent was monolobar and bilobar in two and seven patients, respectively. As primary treatment, liver resection (LR) and liver transplantation (LT) were performed in three (two monolobar and one bilobar extent) and six patients, respectively. RESULTS Postoperative mortality was nil. During a median follow-up of 117 months, four patients developed intrahepatic and/or extrahepatic recurrence. One resected patient (with bilobar extent) presented with intrahepatic recurrence was secondary treated by LT. At the time of the follow-up, seven out of the nine patients treated (two after LR, and five after LT) were alive and disease-free. CONCLUSIONS Surgical treatment offers good long-term results in patients suffering from HEHE when LR is tailored to the intrahepatic disease extent: LT has to be considered in patients with bilobar intrahepatic disease whereas LR should be strictly limited to patients presenting with localized and monolobar intrahepatic disease.