Epithelioid hemangioma of the orbit or angiolymphoid hyperplasia with eosinophilia

A patient was referred to the department for an incidentally discovered right orbital mass on MRI. After surgical excision, histopathologic examination led to the diagnosis of epithelioid hemangioma, a tumor rarely located in the orbit, whose main histopathologic feature is an exuberant proliferation of small vascular channels lined by enlarged endothelial cells. The clinical characteristics and histopathology of epithelioid hemangioma allow differentiation with Kimura disease, angiosarcoma, intravascular papillary endothelial hyperplasia, and other vascular tumors that share the common feature of an abnormal proliferation of endothelial cells. Although epithelioid hemangioma is extremely rare, it should be considered in the differential diagnosis of orbital tumors.