Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

Abstract

With an estimated incidence of only 1-2 cases in every 1 million people, hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types: solitary, multiple, and diffuse. Both the etiology and characteristic clinical manifestations of HEHE are unclear. However, HEHE has a characteristic appearance on imaging including ultrasound, magnetic resonance imaging, and positron emission tomography/computerized tomography. Still, its diagnosis depends mainly on pathological findings, with immunohistochemical detection of endothelial markers cluster of differentiation 31 (CD31), CD34, CD10, vimentin, and factor VIII antigen as the basis of diagnosis. Hepatectomy and/or liver transplantation are the first choice for treatment, but various chemotherapeutic drugs are reportedly effective, providing a promising treatment option. In this review, we summarize the literature related to the diagnosis and treatment of HEHE, which provides future perspectives for the clinical management of HEHE.

Keywords: Hepatic epithelioid hemangioendothelioma; Diagnosis; Differential diagnosis; Therapy; Prognosis; Imaging

Core Tip: In this work, we review the updated diagnosis and therapy of the hepatic epithelioid hemangioendothelioma, which is an extremely rare tumor of vascular origin with an incidence of < 0.1 per 100000 population. It is hard to differentiate from other liver lesions and there is no standard strategy for treating it based on its rarity. Our work helps to better understand and treat this rare disease.