Rare Encounter With Hepatic Epithelioid Hemangioendothelioma: A Case Report

Malignant hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor with variable malignant potential, often affecting middle-aged individuals. Hepatic involvement is uncommon, presenting diagnostic challenges due to overlapping imaging features with other liver pathologies. We report a case of a 35-year-old female with HEHE initially presenting with epigastric pain and an incidental liver mass. Imaging revealed multifocal hepatic lesions, confirmed as HEHE through biopsy and immunohistochemistry. Despite initial stability on tamoxifen, disease progression occurred after seven years, manifesting as an increase in the size and number of hepatic lesions. Hepatic transplantation was planned, which was refused by the patient. Subsequently, the disease progressed to a large confluent hepatic mass with calcifications, lymphadenopathy, and pulmonary metastases. Systemic chemotherapy was henceforth initiated. This case underscores the importance of a multimodal approach integrating imaging, histopathology, and tailored therapeutic strategies for diagnosing and managing HEHE. Early detection and comprehensive management are critical to improving outcomes where liver transplantation offers a potential cure.