Hepatic Epithelioid Hemangioendothelioma: A Review of Three Liver Transplant Recipients

Abstract

Introduction: Hepatic epithelioid hemangioendothelioma (HEHE) is a discrete vascular tumor with an unpredictable natural course. This rare tumor is commonly found incidentally and not too often is mistaken radiologically and histologically for another tumor. No single treatment strategy has yet been established for it, partly due to its variable clinical course, ranging from an indolent tumor with prolonged survival to an aggressive, fatal disease. Case Presentation: Among 1,029 liver transplantation cases performed at our hospital between January 2001 and November 2019, three were done for HEHE. In this study, we present these three cases and review their clinical and histopathologic characteristics. Conclusions: Epithelioid hemangioendothelioma (EHE) of the liver is a low-grade malignant tumor with different presentations, treatment strategies, and outcomes. The histopathologic characteristics of HEHE can hide its vascular nature, thus leading to its confusion with other lesions. This tumor is unique in that its clinical and histopathologic features do not always correlate with its biologic behavior. There are not reliable criteria in predicting the clinical outcome of HEHE, which needs further research.