Clinicopathological Features and Prognosis of Hepatic Epithelioid Hemangioendothelioma After Liver Resection and Transplantation.

Abstract

BACKGROUND Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor. We present a single-center study on hepatic EHE cases treated by hepatic resection (HR) or liver transplantation (LT). MATERIAL AND METHODS Six HR patients with hepatic EHE were identified during 5 years from 2010 to 2014; EHE represented 0.13% (6 of 4572) of all primary liver tumors. Two LT recipients were also identified during 12 years from 2003 to 2014, who represented 0.06% (2 of 3576) of all adult LT patients. RESULTS Mean age of the patients was 42.3±11.2 years and 7 were female. Preoperative diagnoses based on imaging studies were suspected liver metastasis or EHE; thus, all patients underwent liver biopsy. The extent of HR, which was determined by the location and size of tumors, consisted of right hepatectomy in two patients, right anterior sectionectomy in one, right posterior sectionectomy in one, central bisectionectomy plus partial hepatectomy in one, and right trisectionectomy plus partial hepatectomy in one. All patients recovered uneventfully from surgery. The disease-free survival rate was 83.3% at 1 year and 44.4% at 3 years in HR patients, but all patients are alive to date. Two patients underwent living-donor LT because they had multiple tumors scattered throughout the liver. Of these, one patient died from tumor recurrence at 9 months and the other is alive after 5 years without recurrence. CONCLUSIONS Hepatic EHE is often misdiagnosed as a metastatic tumor. This disease has malignant potential; thus, it requires aggressive treatment including HR, non-surgical treatment, and LT.