Disseminated hepatic epithelioid hemangioendothelioma with multiorgan metastases achieving long-term stability with Pazopanib: a case report

Background/Objectives Epithelioid Hemangioendothelioma (EHE) is a rare malignant vascular neoplasm with an unpredictable clinical behavior and often delayed diagnosis. The malignancy arises from many anatomical sites, with the liver being the most common primary site. This report portrays a young patient who presented atypically with a multifocal hepatic epithelioid hemangioendothelioma (HEHE) and highlights the challenges faced during the diagnosis and management of such rare malignancies. Methods A 28-year-old male patient from Palestine presented with progressive headache associated with constitutional symptoms. Diagnostic imaging revealed disseminated disease throughout the body. CT-guided liver biopsy confirmed neoplastic cells arranged in small nests and individually, some of which showed intracytoplasmic vacuoles within a hyalinized stroma. Immunohistochemistry was positive for CD31, CD34 and FLI-1, supporting the endothelial origin. Metastatic HEHE was established; a biological treatment plan with Pazopanib (Votrient) was initiated, and the patient was followed for 4 years. Results During the follow-up period, serial imaging revealed no significant regression or progression of the lesions, indicating stable disease with the treatment used. Conclusion The case emphasizes the clinical intricacy of HEHE, given its rarity, the lack of standardized treatment guidelines, and the tumor’s unpredictable course. Specifically, this case illustrates these challenges within the Palestinian healthcare system.