Intraneural synovial sarcoma: two cases

Abstract

We report two cases of intraneural synovial sarcoma. The first patient is a 46-year-old female who presented for several months with soft-tissue mass in the right infra-auricular region. The second patient is a 11-year-old girl who fell and then presented with pain in the area innervated by the right C7 spinal root and a nodule identified in the nerve root foramina. Both lesions were of small size and presented with features of synovial sarcoma. A biphasic variant was found in case 1 and a monophasic variant was present in case 2. Immunohistochemical studies were performed to confirm the diagnosis, excluding the main differential diagnoses, namely schwannoma and malignant peripheral nerve sheath tumor. Ultrastructural study was performed in case 2 allowing exclusion of other possible diagnoses. Molecular studies were performed on paraffin-embedded tissue in both cases and revealed the known characteristic t(X;18)(SYT-SSX) translocation.

Intraneural Synovial Sarcoma Originating From the Median Nerve -Case Report-

“…2,4,6,16,20,21,23,25,29) The macro-and microscopic observations in the present case, especially in the recurrent tumor growing from the median nerve, were consistent with the observation that synovial sarcoma can develop in any soft tissue of the body from neural origins. Accurate diagnosis of synovial sarcoma was difficult until few years ago.…”

“…26) However, only 10 cases of intraneural synovial sarcoma have been reported. 2,4,6,16,20,21,23,25,29) We treated an adult female with a synovial sarcoma originating from the right median nerve at the right wrist, which was preoperatively diagnosed as schwannoma.…”

Neuronal Differentiation of Synovial Sarcoma and Its Therapeutic Application

“…Neural crest-derived cells are precursors of neuroblastomas [41,42], malignant melanomas [18], and the Ewing family of tumors [46], which are sarcomas harboring a tumor-specific fusion oncoprotein (EWS-Fli1 or EWS-ERG) [34]. SS is known to develop at unusual anatomic sites such as the pleurae [6], visceral organs [10], bone marrow [24], and peripheral nerves [14]. Molecular diagnoses using the SYT-SSX fusion transcript as a marker have proved some renal tumors previously diagnosed as different diseases were in fact SSs [4].…”

Cervical Nerve Root Synovial Sarcoma in a Child with Chromosomal (X;18) Translocation

“…This malignant tumor is seldom encountered in children [1, 2] and was exceptionally described involving a nerve [3,4,5,6,7]. Synovial sarcoma arising within the nerve sheath [8] as well as one case of peripheral intraneural metastasis of soft tissue sarcoma have been reported [9]. However, the aggressiveness of this entity and the importance of early diagnosis have lead us to present this case report.…”