Epithelioid hemangioendothelioma (EHE) isan uncommon tumour of endothelial origin having borderline malignant potential which frequently is initially misdiagnosed when occurring in the liver, often as cholangiocarcinoma, metastatic carcinoma or veno-occlusive disease. The authors report two cases of hepatic EHE. The fi rst case presented in a 33-year-old female and was originally misdiagnosed as cholangiocarcinoma. Only after re-evaluation of the case seven years later, because of an unexpectedly indolent clinical course, was the correct diagnosis made. The second case presented in a 26-year-old female having nonspecific gastrointestinal complaints. The diagnosis of hepatic EHE was made from a wedge biopsy of liver taken during an exploratory laparotomy. In both cases, immunohistochemical reactivity of the tumour cells for von Willebrand factor (FY1IIR:Ag) and Ulex europeaus I lectin was key to the final diagnosis. The first patient, to this time, remains asymptomatic. The second patient underwent liver transplantation and is being followed (now one year since the initial diagnosis). Hepatic EHE can present a diagnostic dilemma. The use of immunohistochemical techniques to demonstrate the vascular origin of this tumour is essential for its diagnosis. Better awareness of this lesion and the features differentiating it from other histologically similar lesions will prevent exposing affected patients to unnecessary investigations and treatments.
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