EPITHELIOID SARCOMA OF THE ORBIT

Background: Epithelioid sarcoma is a rare and aggressive malignancy co mmonly occurring in the tendon sheaths of the distal extremities of yo ung adults. This article presents the clinical and pathologic details of two patients with epithelioid sarcoma in the orbit, the first to be reported as occurring in this location. Methods: The clinical history , results of physical and radiologic examination, surgical procedures, pathologic specimens, and clinical course of each patient were review ed and recorded. Results: Biopsies were performed of the masses that w ere found in the superotemporal orbit of both patients. Results of pat hologic examination in each patient caused considerable difficulty, an d the correct diagnosis was reached only after extensive consultation. The first patient underwent wide soft tissue and bone exenteration an d is alive without recurrence at 3 years. The second patient had multi ple recurrences and died of recurrent and metastatic disease at 29 mon ths. Conclusions: Epithelioid sarcoma must be added to the differentia l diagnosis of orbital sarcomas. The histology is nodular with a pseud ogranulomatous appearance. Based on our limited experience, epithelioi d sarcoma of the orbit may have a course similar to that which occurs elsewhere in the body, requiring aggressive surgical treatment at the outset.