Primary Synovial Sarcoma

To describe the clinicopathologic and immunohistochemical features and treatment of a rare case of primary synovial sarcoma of the orbit. Retrospective interventional case report. A 14-year-old young man with histologically proven synovial sarcoma of the orbit. The diagnosis was confirmed by demonstration of a specific chromosomal translocation by polymerase chain reaction studies. The patient was treated with orbital exenteration and followed up for 18 months at regular intervals. The tumor was excised completely, and the patient has done well during the initial 1-year follow-up with no sign of recurrence. We report the fourth case of synovial sarcoma of the orbit and, at 14 years of age, the youngest patient reported.