Sarcoma sinoviale in età pediatrica. Esperienza dell'Istituto Nazionale Tumori, Milano

Objectives. Multimodality treatment approach has improved the prognosis of synovial sarcoma during the last two decades, but the real role of post-operative radiotherapy and adjuvant chemotherapy remains to be determined. Methods. Thirty-five children affected by synovial sarcoma were treated at our Pediatric Oncology Unit during a 25-year period: among the 34 patients with localized disease, 18 had radical surgery (2 amputation, 12 primary re-excision), 14 non-radical resection, 2 biopsy. Radiotherapy was delivered to 22 patients, chemotherapy to 31. Results. Twenty-nine patients were alive, 24 in first complete remission. With a median follow-up of 10 years, survival was 86.1% at 5 years. In univariate analysis, tumor size, local invasiveness and site of origin were the significant prognostic factors. Conclusions. In comparison to adults', childhood synovial sarcoma seems to have a more favorable outcome, probably related to the systematic use of chemotherapy and radiotherapy in the treatment programs. The pivotal role of surgery remains unquestionable. Radiotherapy seems advisable in patients considered at high risk due to size, local invasiveness and non-radical surgery. Pediatric protocols require the use of chemotherapy in all patients.