Epithelioid hemangioma of the orbit.

Objective: To describe the histopathologic features of two cases of epithelioid hemangioma occurring in the orbit and to distinguish this condition from Kimura's disease and from other vascular lesions of proliferated endothelium.

Design: Two interventional case reports.

Intervention: Treatment consisted of orbitotomy with excision of the tumor.

Main Outcome Measures: Histopathologic examination including light microscopy, immunohistochemistry, and electron microscopy and clinical follow-up.

Results: In one case, there was no local recurrence after 2 years of follow-up. In the other case, local recurrence required re-excision 2 years after surgery with no apparent recurrence 16 years later. Histopathologic examination of both tumors disclosed an epithelioid hemangioma. A characteristic finding was the presence of peculiar plump vacuolated endothelial cells lining the vascular lumina.

Conclusions: Epithelioid hemangioma is an uncommon benign vascular tumor that can occur in the orbit, and surgical excision is usually required. It is the same condition as angiolymphoid hyperplasia with eosinophilia. There are distinct clinical and histopathologic characteristics to distinguish epithelioid hemangioma from Kimura's disease and from other vascular tumors.