Hepatic Epithelioid Hemangioendothelioma Mimicking Multifocal Hepatocellular Carcinoma in a Cirrhotic Patient: A Case Report and Review of the Literature

Abstract

Background: Hepatic epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm of intermediate malignant potential, positioned between benign haemangioma and angiosarcoma. Because it typically presents as multifocal, heterogeneously enhancing hepatic nodules, it is frequently mistaken for hepatocellular carcinoma (HCC) or metastatic disease, particularly when it arises on a background of chronic liver disease. Case Presentation: We report the case of a 77-year-old man with hepatitis C virus (HCV)-related cirrhosis who presented with a three-month history of epigastric pain, dyspepsia, and unquantified weight loss. Contrast-enhanced computed tomography (CT) revealed multiple hepatic lesions on a cirrhotic liver, initially considered highly suggestive of multifocal HCC, with alpha-fetoprotein mildly elevated at 16.16 ng/mL. Trans-arterial chemoembolisation, initially planned, was contraindicated because of tumoral invasion of the main portal vein. Ultrasound-guided liver biopsy demonstrated an epithelioid vascular proliferation with diffuse CD31 positivity and negative staining for CD34, pan-cytokeratin (AE1/AE3), and HHV8, confirming the diagnosis of hepatic epithelioid hemangioendothelioma. Because of extensive portal invasion, the patient was referred for systemic oncological management. Conclusion: This case illustrates a diagnostic pitfall in which hepatic epithelioid hemangioendothelioma, arising on a cirrhotic, HCV-infected liver with a mildly elevated alpha-fetoprotein, closely mimicked multifocal HCC. It underscores the necessity of histological and immunohistochemical confirmation before any invasive loco-regional therapy is undertaken in patients with atypical or multifocal liver lesions, even in a setting highly suggestive of HCC.