Two Cases of Hepatic Epithelioid Hemangioendothelioma Misdiagnosed With Hepatic Veno-Occlusive Disease.

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin with an incidence of < 0.1/100,000. The disease is easily misdiagnosed. The aim of this article is to increase public awareness and vigilance of HEHE. We report two cases presenting with fever, abdominal discomfort, abnormal liver function, and jaundice. Computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen suggested hepatic stasis, venous compression, and ascites, and liver biopsy was initially misdiagnosed as hepatic veno-occlusive disease (VOD), which did not improve with treatment. In the first case, a male patient, after liver transplantation, pathological and immunohistochemical (IHC) analyses revealed hyperplasia of blood vessels in the liver tissue with dilated lumen and heterogeneous cells. Immunohistochemistry was performed and showed CD34 positivity, confirming the diagnosis of HEHE. The second female patient had liver bruising and ascites on imaging, and the first hepatic puncture was reported to be VOD, which did not improve with treatment. Repeat hepatic puncture was performed, and the diagnosis of HEHE was confirmed after a second repathology with additional immunohistochemistry for HEHE. These misdiagnosis cases highlight the challenge of diagnosing HEHE. This is the first report of misdiagnosis of HEHE as VOD. This article analyzes the underlying causes of misdiagnosis of HEHE and emphasizes the causes of imaging misdiagnosis and the importance of repeated hepatic puncture biopsy and immunohistochemistry in the diagnosis of HEHE.