Background: Synovial sarcoma is the fourth most commonly occurring sarcoma, accounting for 8-10% of all sarcomas. They arise from unknown pleuripotent stem cells that are capable of differentiating into mesenchymal and/or epithelial structures. Synovial sarcoma is characterized by specific chromosomal translocation t (X; 18)(p11, q11).
Case: We report the first case of monophasic synovial sarcoma arising in soft tissues of the vulva in a 33-year-old female. Complete excision of the mass was possible with tumor-free margins.
Conclusion: Only four previous cases of biphasic synovial sarcoma arising in the vulva have been reported. The finding of the SYT-SSX2 translocation is generally associated with a better prognosis, besides tumor negative margins after excision offer the possibility of a better outlook for this patient.