Primary intracranial synovial sarcoma: A case report and review of literature

Abstract

Background: Primary intracranial synovial sarcomas (PrISS) are unusual dural based mesenchymal tumors seen most commonly in the supratentorial compartment. They can mimic a spontaneous intracranial hemorrhage or a high-grade glioma on imaging. Case Description: A 31-year-old male presented with headache and right hemiparesis for 2 weeks. CT brain revealed a left frontal spontaneous intracerebral hemorrhage. PrISS revealed a heterogeneously ring enhancing solid cystic lesion with attachment to convexity dura. Intraoperatively, it mimicked a high-grade glioma. Histopathology report showed features of a synovial sarcoma, which was later confirmed with IHC. Classical SYT-SSX2 translocation was confirmed only on RTPCR after fluorescent in situ hybridization (FISH) was negative for same. Whole body positron emission tomography (PET-CT) did not show any extracranial tumor. Despite radiotherapy, there were recurrence and tumor progression at 6 months and the patient succumbed 11 months later. Conclusion: PrISS is an unusual aggressive intracranial neoplasm that carries a worse prognosis when compared nonintracranial synovial sarcomas. Molecular cytogenetics (FISH and RTPCR) are essential for confirming the diagnosis, though FISH seems to have a lower sensitivity and can yield false negative results as was noted in this case.

Olfactory groove monophasic sinovial sarcoma and von Recklinghausen’s disease: A case report and literature review

“…In addition, intracranial lesions present with a high aggressiveness and a rapid decline in neurological function, and dural adherence is described in most cases reported in the literature. [ 9 , 31 ] The histological analysis of SS comprise a wide spectrum of lesions that divide similarities in clinical evaluation, immunohistochemistry, and genetics. This analysis divides the classification of SSs into two groups: single-phase and biphasic sarcomas, although other subtypes of rarer lesions are also described, such as poorly differentiated sarcomas and myxoid types.…”

“…[20] However, the description of cases of primary intracranial SSs still remains a fairly rare entity. [1,2,12,[14][15][16][17][18][21][22][23]31,32,35,36] e present work is a case report of a final diagnostic patient of monophasic SS (MSS) in olfactory groove and with neurofibromatosis Type 1 (NF1). It is well-described that patients with chromosome 17 disorder with NF1, also known as von Recklinghausen's disease, have a predisposition to the development of central nervous system tumors.…”