Rectal synovial sarcoma: A case report and review of the literature

Synovial Sarcoma (SS) is a rare and aggressive mesenchymal malignancy, representing 5-10% of all soft tissue sarcomas. Approximately 70% of patients experience local recurrence or distant metastasis. Primary SS in the gastrointestinal tract is exceedingly uncommon, with less than 1% being located in the retroperitoneal location. Morphology could be monophasic, biphasic, or poorly differentiated subtypes, having translocation of SS18 with SSX1, SSX2, or SSX4 genes most typically. A 30-year-old lady presented to our outpatient department with symptoms of rectal bleeding and perineal discomfort. Clinical examination and imaging studies suggested the presence of rectal synovial sarcoma, which had extended into the right ischiorectal and isochronal fossa. Subsequent biopsy and immunohistochemistry confirmed the diagnosis of biphasic synovial sarcoma. The patient was reviewed by a multidisciplinary tumor board, and a treatment plan involving neoadjuvant chemotherapy followed by surgery was established. After chemotherapy, she underwent abdominoperineal resection, and her postoperative recovery was uneventful. Henceforth, rectal sarcoma is a very rare malignancy with poor prognosis. However, surgery with curative intent with a negative margin may additionally confer better overall survival.