Synovial sarcoma of childhood and adolescence. Report of the german CWS-81 study

Abstract

Background. Synovial sarcoma is the third most common pediatric soft tissue tumor. It requires an aggressive approach to achieve a cure. However, optimal treatment modalities adapted to disease extension and histologic variants have not been determined because there is little information about prospectively treated patients. Methods. A multicenter trial for soft tissue sarcomas (Protocol CWS 81) was conducted in West Germany between 1981–1985, and 31 patients with synovial sarcoma were registered. Treatment included multiagent chemotherapy and irradiation after initial tumor excision or biopsy. The male–female ratio in this group was 1:1.6 with a median age of 14 years (range, 1–19 years). The median follow-up time after diagnosis was 101 months (range, 77–131 months). Results. The overall event-free survival (EFS) for patients with synovial sarcoma was 74.2% at 5 years. Group I–II tumors had a significantly better prognosis than those in Group III–IV (EFS at 5 years 84.4% and 58.3%, respectively; P = 0.024). Small tumors (< 5 cm) responded better than larger tumors (≥ 5 cm; EFS, 93% versus 58%; P = 0.029). Synovial sarcoma involved the extremities in 28 patients who had a better outcome compared with those with extremity rhabdomyosarcoma in this study (EFS for Group I–IV was 82% versus 24%, P = 0.001). Conclusions. The results appeared superior to previous experience using radical surgery alone and suggested that after initial, nonmutilating surgery, adjuvant chemotherapy, and irradiation contributed to the improved long-term survival.