Two Cases of Hepatic Epithelioid Hemangioendothelioma Misdiagnosed With Hepatic Veno‐Occlusive Disease

TL;DR

This paper reports two cases where a rare liver tumor was initially misdiagnosed as a different liver condition, highlighting the importance of repeat biopsies and specialized tests for accurate diagnosis.

Contribution

The first report of hepatic epithelioid hemangioendothelioma being misdiagnosed as hepatic veno-occlusive disease, emphasizing the need for repeated biopsies and immunohistochemistry.

Findings

Two cases of HEHE were initially misdiagnosed as hepatic veno-occlusive disease based on imaging and initial biopsies.

Accurate diagnosis of HEHE was confirmed through repeat biopsies and immunohistochemistry showing CD34 positivity.

The study highlights the importance of repeated hepatic puncture and immunohistochemistry in diagnosing HEHE.

Abstract

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.

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