Immunotherapy for synovial sarcoma

Synovial sarcoma (SS) is a relatively common subtype of soft tissue sarcoma that typically affects young adults. Nearly all tumors harbor a translocation between SS18 and SSX1/SSX2 and the vast majority express the cancer testis antigen (CTA) NY-ESO-1. While patients with small, non-metastatic tumors are often cured surgically, outcomes remain poor for patients with locally advanced or metastatic disease, even when aggressive chemotherapy and radiotherapy are employed. Therefore, innovative systemic therapies that target the biology of the disease are needed to improve outcomes for these higher risk patients. One such category is tumor-directed immune therapies. In this review, we will discuss the current status of immunotherapy for SS, including the recent trial results, ongoing challenges, and future directions.