Epithelioid sarcoma of the orbit

Epithelioid sarcoma is an aggressive and rare malignancy first recognized by Enzinger in 1970. It is known most commonly to affect the distal upper extremities in young adults. The classical “distal” form has a male predominance and can also involve other less frequent sites including lower extremities, proximal upper extremities, and the trunk. The “proximal” variant of this tumor is deep seated, tends to occur in older patients and predominantly develops in the pelvis, perineum, and genital tract. In the orbit, only a single report of two cases, which had a typical histopathologic appearance, has been previously published. We present the third case of orbital primary epithelioid sarcoma.