Intraneural Synovial Sarcoma of the Median Nerve in a 15-Year-Old Male: A Case Report and Review of Literature
Joshua F Scarcella, BS, Arthur Samia, BS, Richard Zeri, MD and Yifan Guo, MD, Brody School of Medicine, Greenville, NC
Introduction:
Synovial sarcomas are rare malignant soft tissue tumors, with an estimated incidence of 2.75 in 100,000.1 Prognosis is poor with a 50-60% five-year survival rate due to recurrence and proliferation of metastatic disease.2
There have been eight synovial sarcomas of the median nerve reported. We report a case of a 15-year-old male with synovial sarcoma of the right-hand median nerve.
Materials & Methods:
A 15-year-old male presented with a two-month history of enlarging mass at the base of the right thenar eminence associated with numbness in the median nerve distribution. Physical exam reveals a soft mass over the thenar eminence and paresthesia in the median nerve distribution.
He underwent excision of the tumor, which revealed a well encapsulated lesion encompassing the median nerve, involving the first, second, and radial aspect of the third web space as well as recurrent branches of the median nerve.
Following excision of the tumor, a thorough metastatic workup was negative for metastatic disease. He was staged as III, T2b, N0, M0 - poorly differentiated monophasic synovial sarcoma of the right median nerve. Post-operatively the patient was started on chemotherapy and radiation.
Results:
Intraneural synovial sarcoma is extremely rare. Our case is the youngest with the longest follow-up (table 1).
Surgical resection of the tumor within a 5cm margin is the established treatment. The efficacy and dosage of chemotherapy and radiation are not clear.3
Good prognostic factors include a tumor size less than 5 cm, age less than 25-30 years old, and histological findings without necrosis, bone invasion, or neurovascular invasion.3 Death most commonly occurs from distant metastases, oftentimes to the lungs.5
Conclusions:
We present a case of a 15-year-old male with an extremely rare presentation of a synovial sarcoma of the right median nerve who underwent resection and post-operative radiation and chemotherapy. He is currently three years status post-treatment with no signs of recurrence and excellent use of his right hand.
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