Periocular epithelioid hemangioma: response to bevacizumab and vascular pathogenesis.

Epithelioid hemangioma (EH), also known as angiolymphoid hyperplasia with eosinophilia, may affect the eyelids and orbit, mimic idiopathic orbital inflammation, and respond poorly to corticosteroids. We describe a patient with chronic, bilateral orbital EH unresponsive to corticosteroids. Histopathologic analysis showed lobular vascular proliferations containing plump endothelial cells with surrounding mixed leukocytic infiltrates diagnostic of EH. Successful treatment consisted of surgical debulking and intralesional bevacizumab. Periocular EH lesions of 2 additional patients treated by debulking also showed prominent lobular vascular proliferations, highlighting them as the basis of this benign tumor. We conclude that EH is primarily a vascular tumor that can respond to bevacizumab.