Management of Monophasic Synovial Sarcoma of the Small Intestine

BACKGROUND: Reports of primary intraabdominal synovial sarcomas are extremely rare. METHODS: A literature review using PubMed was performed. A retrospective review of the one known case at our institution was completed. RESULTS: Even the most experienced pathologists report that synovial sarcomas can be very difficult to diagnose correctly. One cytogenic abnormality that is common (>90%) and pathognomonic for synovial sarcoma is a characteristic chromosomal translocation resulting in the SYT/SSX fusion gene. Wide regional excision has been performed for intraabdominal sarcoma, with improved results. Our patient is more than 24 months with no evidence of recurrent or metastatic disease. CONCLUSIONS: The prognosis for patients with intraabdominal synovial sarcoma remains poor. However, wide regional excision may allow for prolonged disease-free survival.