Abstract
INTRODUCTION: Malignant epithelioid hemangioendothelioma (EHE) is an uncommon and grave vascular tumor. EHE is frequently angiocentric and is associated with a medium sized vessel, especially a vein. No definite etiological associations have been ascribed to this tumor so far, except an association with oral contraceptives in EHE of liver. PRESENTATION OF CASE: A 47 year old man presented with the complaint of intermittent black stool over the past two weeks. Occasionally, he experienced pain in left lower abdomen. On Computed Tomography (CT), it showed hypervascular lesion in the ileum with persistent enhancement. An exploratory laparotomy was performed with short segmental resection and functional end-to-end anastomosis. It was diagnosed finally with the histopathological and immunohistochemical analysis as a malignant EHE. DISCUSSION: EHE is an uncommon endothelial tumor that most frequently arises in soft tissue, liver, lung and skeleton. It behaves biologically in between benign epithelioid hemangioma and the more aggressive epithelioid angiosarcoma. Although a standard systemic treatment for malignant EHE has not been fully established, complete surgical excision is strongly recommended if feasible. CONCLUSION: EHE has a variable presentation and CT is helpful in identifying ileal EHE timely in the early stage, even when there is no obvious mass formation, however the diagnosis can be confirmed only after histopathological and immunohistochemical studies.