Sarcomas sinoviales

Abstract

Synovial sarcomas represent 5-10% of all soft tissue sarcomas. Nevertheless, these tumors are highly aggressive, causing death in 10% of affected individuals in the first year after diagnosis. Synovial sarcomas are more prevalent in adolescents and young adults aged between 15 and 40 years old. The most common clinical presentation is a generally painful, juxta-articular, palpable mass. Due to the low index of suspicion, synovial sarcoma is often misdiagnosed as benign soft tissue processes, bursitis or arthritis and diagnosis can be delayed for more than 1 year. These tumors can occur in any site but most arise from deep soft tissues in the lower extremities. Survival rates vary significantly according to tumoral size and location, depth, histologic grade, and the presence of nodal involvement or distant metastasis. In almost all cases, synovial sarcomas contain a characteristic translocation between chromosomes X and 18. This translocation involves the SYT gene on chromosome 18 and one of several highly homologous genes (SSX1, SSX2 and SSX4) on the X chromosome. The cornerstone of treatment is surgery, along with radiation therapy and chemotherapy.