Current Management and Molecular Targets of Synovial Sarcoma

Synovial Sarcoma (SS) is a rare and aggressive form of soft tissue sarcoma (STS) with a high metastatic potential that is characterized by a unique translocation between SYT on chromosome 18 and SSX on chromosome X. Presently, standard of care involves surgery, radiation therapy and chemotherapy. For those patients with metastatic disease, standard of care remains enrollment in a clinical trial. While there are numerous open clinical trials for the treatment of STS in general, clinical trials designed specifically for SS remain limited. The overall low response rate to cytotoxic chemotherapies has necessitated the need for development of pathway?specific targeted therapies for SS. Deregulation of several cell signaling pathways have been identified in SS, including the SRC, Bcl?2, and MDM2 signaling pathways, which are involved with cell growth, apoptosis, and p53 regulation, respectively. Additionally, several potential enzymatic targets have been identified, including argininosuccinate synthetase 1 and histone deacetylases. Here we present an updated review of the current therapy and the prospective molecular therapeutic targets that are available for clinical trial development in SS.