SYNOVIAL SARCOMA OF CHILDHOOD AND ADOLESCENCE - REPORT OF THE GERMAN CWS-81 STUDY

Background. Synovial sarcoma is the third most common pediatric soft t issue tumor. It requires an aggressive approach to achieve a cure. How ever, optimal treatment modalities adapted to disease extension and hi stologic variants have not been determined because there is little inf ormation 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 irradiatio n after initial tumor excision or biopsy. The male-female ratio in thi s group was 1:1.6 with a median age of 14 years (range, 1-19 years). T he 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 si gnificantly better prognosis than those in Group III-IV (EFS at 5 year s 84.4% and 58.3%, respectively; P = 0.024). Small tumors (< 5 cm) res ponded better than larger tumors (greater-than-or-equal-to 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 extre mity rhabdomyosarcoma in this study (EFS for Group I-IV was 82% versus 24%, P = 0.001). Conclusions. The results appeared superior to previo us experience using radical surgery alone and suggested that after ini tial, nonmutilating surgery, adjuvant chemotherapy, and irradiation co ntributed to the improved long-term survival.