Advances in treatment of synovial sarcoma / 肿瘤

ABSTRACT

Synovial sarcoma (SS) is a type of rare, yet highly malignant soft tissue sarcoma (STS). SS is characterized by a pathognomonic translocation between chromosomes X and 18, involving genes SS18 [also known as synovial sarcoma translocation (SYT )] and synovial sarcoma X chromosome breakpoint (SSX ) 1, 2, or 4, and then a SS 18 SSX fusion gene is formed. At present, the comprehensive treatment is a mainstream treatment strategy for SS, however the individual plan is varied. SS patients show better responses to chemotherapy as compared with other STS patients. For the patients with localized SS, the wide surgical resection of tumors combined with radiation and/or (new) adjuvant chemotherapy (when necessary) is regarded as a standard treatment. For the patients with advanced SS, the cytotoxic chemotherapy is often at the first consideration, while the other palliative treatment options are varied. For the advanced patients with metastasis, the first-line chemotherapy is represented by the combined or sequential treatment with doxorubicin and ifosfamide, and the second-line treatment regimens include trabectedin and pazopanib. This article reviews the recent progress in the treatment of SS, focusing on the current status of pharmacologic management of SS, and the new potential therapies related to molecules and immunological features of SS are also introduced.