Primary synovial sarcoma.

PURPOSE: To describe the clinicopathologic and immunohistochemical features and treatment of a rare case of primary synovial sarcoma of the orbit. DESIGN: Retrospective interventional case report. PARTICIPANT: 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. METHODS: The patient was treated with orbital exenteration and followed up for 18 months at regular intervals. RESULTS: The tumor was excised completely, and the patient has done well during the initial 1-year follow-up with no sign of recurrence. CONCLUSIONS: We report the fourth case of synovial sarcoma of the orbit and, at 14 years of age, the youngest patient reported.