Abstract
Aim
Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors.
Method
Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. The following parameters were examined for their potential prognostic value: age at diagnosis, sex, tumour site and size, histology, histological grade, fusion type (SYT-SSX1 vs. SYT-SSX2), and surgical margin status. Mean follow-up of living patients was 17.2 years, and of dead patients 7.7 years.
Results
Mean age at diagnosis was 35.4 years (range, 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range, 0.5-14.9 years), and metastases, at a mean of 5.7 years (range, 0.5-16.3 years). Only 4 patients (7%) were treated technically correctly. Factors associated with significantly worse prognosis included larger tumour size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment.
Conclusion
In SS, metastases develop late with high mortality. Patients with SS should be followed for more than 10 years. There is a very high rate of incorrectly treated patients. Patients with suspicious lesions therefore should be sent to a referral center.