Recurrent primary intracranial synovial sarcoma, a case report and review of the literature

Abstract

Synovial sarcoma (SS) occurs in various parts of the body, predominantly in the extremities. It also occurs in organs without synovial structures. The intracranial disease has been reported as metastasis, but primary intracranial SS has been reported rarely. We report a patient with hemiplegia and a mass on the brain CT. Pathology showed SS with no extracranial pathology.

Ambivalent and Conjectural-Synovial Sarcoma

“…Characteristic morphological features emerge as monotonous spindle-shaped cells permeated with vesicular, plump and overlapping nuclei. Cellular component is intermingled with haemangiopericytoma-like vascular articulations [1,2].…”

“…Around ~18% instances occur within children and adolescents. A mild male predominance is observed with male to female proportion of ~1.2:1 [1,2]. Synovial sarcoma preponderantly incriminates extremities, trunk, head and neck, intrathoracic or intra-abdominal region or may arise within diverse locations although no site of tumour emergence is exempt.…”

“…Soft tissue sarcomas depicting chromosomal translocation t (X;18; p11; q11) are contemplated to be synovial sarcomas. Synovial sarcoma is posited to occur due to chromosomal translocation t(X;18) (p11; q11) incriminating genes SS18 along with SSX1, SSX2 or SSX4 [1,2]. Majority (~90%) of synovial sarcomas manifest characteristic chromosomal translocation t(X;18) (p11.2; q11) along with SYT-SSX1 genetic fusion.…”

“…Majority (~90%) of synovial sarcomas manifest characteristic chromosomal translocation t(X;18) (p11.2; q11) along with SYT-SSX1 genetic fusion. Besides, chromosomal translocation t(X;18) (p11.21; q11) and SYT-SSX2 genetic fusion may occur [1,2]. Synovial sarcoma frequently depicts p16INK4A genetic deletion.…”

“…Synovial sarcoma frequently depicts p16INK4A genetic deletion. Enhanced expression of EZH2 differentiates poorly differentiated neoplasms from monophasic or biphasic synovial sarcoma [1,2]. Aforesaid chromosomal translocation variably influences diverse oncogenetic pathways as initiation of SWI/SNF chromatin remodelling complex, polycomb repressor complex or canonical Wnt pathway.…”