The Profuse Bleeding Potential of Orbital Epithelioid Hemangioendotheliomas

Abstract

Epithelioid hemangioendothelioma is a rare vascular tumor originating from vascular endothelial or pre-endothelial cells. We present the case of a 4-month-old male with a rapidly enlarging left zygomatico-orbital tumor causing mass effect on the eye globe. Examination revealed a large, nontender, solid lesion. CT angiography showed no major feeder or intralesional vessels. Complete surgical excision was performed, which was complicated by life-threatening intraoperative bleeding and successfully controlled with electrocautery. Microscopically, tumor cells exhibited varying morphologies. Immunohistochemistry confirmed the diagnosis of epithelioid hemangioendothelioma (positive for CD31 and CD34, negative for CK AE1/AE3). We also highlight 2 similar case reports with life-threatening bleeding complications. Surgeons should be aware of this condition and optimize surgical preparation, including blood products, to manage potential bleeding complications.