Recurrent Primary Synovial Sarcoma of Median Nerve: A Case Report and Literature Review

Abstract

We report a case of intraneural synovial sarcoma of the median nerve in a 39 year old lady with multiple local recurrences over thirteen years with no distant metastasis. The diagnosis of biphasic type of synovial sarcoma was confirmed by histopathology and immunohistochemistry. At the time of the fourth recurrence below elbow amputation was performed. This case is being reported for its rarity and propensity for local recurrence without distant metastasis.

Intraneural Synovial Sarcoma of the Median Nerve in a 15-Year-Old Male: A Case Report and Review of Literature

“…Four of the 6 cases (including ours) reported monophasic histologic findings. 3,[6][7][8][9] However, interestingly the biphasic type is the most commonly reported histologic finding for all SSs. 9 Three cases reported patients that underwent total resection of the tumor and one who underwent extended resection.…”

“…Only 2 cases were positive for the SS18-SSX fusion gene. 3,[6][7][8][9] The treatment of SSs is multifaceted, often incorporating surgical resection, local radiation therapy, and chemotherapy. Primary, localized SS is approached with wide, local surgical resection.…”

“…1 Synovial sarcoma most commonly affects patients younger than 30 years of age, and is associated with a poor prognosis (50%-60% 5-year survival rate) despite radical surgical resection, local radiation therapy, and chemotheraphy. 2,3 However, prognosis is more favorable among younger patients and when the tumor affects the extremities. 1,2 Extremities are the most common site of origin, but these tumors have been known to arise for alternative site such as the heart, lung, and small intestines.…”

“…In the rarest of occasions, the tumors may arise from peripheral nerves. 3 Synovial sarcoma is named after its resemblance to synovial cells on histopathologic examination; however, interestingly, it is not derived from synovial cells. A majority of SSs can be identified by the chromosomal translocation t(X;18)(p11.2;q11.2).…”

Intraneural synovial sarcoma of the tibial nerve

“…1 While the majority of synovial sarcomas occur in the skeletal muscle and supporting connective tissue of the extremities (most often the legs), 1,2 they have also been reported in many other anatomical locations such as the mediastinum, head, neck, esophagus, pleura, heart, small intestine, and lungs. 3–6…”