Hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant vascular tumor that frequently poses a diagnostic challenge due to its close resemblance to metastatic carcinoma, particularly on limited biopsy material. We report the case of a 42-year-old woman who presented with constitutional symptoms and gastrointestinal bleeding. Imaging revealed multiple bilateral hepatic lesions, clinically interpreted as metastases from an unknown primary. The liver biopsy revealed cords and tubular structures composed of epithelioid cells within a reactive hepatic background. Immunohistochemistry revealed isolated CK7 positivity, leading to an initial impression of metastatic carcinoma or intrahepatic cholangiocarcinoma. Extensive clinic-radiological work-up failed to identify a primary malignancy. Review of histology highlighted blister cells and intracytoplasmic vacuoles, and subsequent endothelial marker positivity confirmed the diagnosis of HEHE. This case underscores the importance of recognizing histologic clues and the diagnostic pitfall in HEHE, which can lead to misdiagnosis and inappropriate management.
Mitra et al. (Mon,) studied this question.