Synovial sarcoma: Report of a series of 25 consecutive children from a single institution

Background. The role of postoperative radiotherapy and adjuvant chemotherap y in the treatment of synovial sarcoma remains to be determined. Procedure. Twenty-five children were treated during a 23-year period with a multimoda lity approach. All of them had resection of the primary tumor (three amputa tions), followed by surgical retreatment in eight. Post-operative radiother apy was delivered to 16 patients and adjuvant chemotherapy was given to 22. Results. At the time of the report, 19 patients were alive and without evi dence of disease. Six developed distant metastases (one associated with loc al recurrence); five of them died of their disease and one was alive in com plete remission at 4 years from relapse. With a median follow-up of 9 years (range 2-23), the survival and the event-free survival at 5 years were 80% (SE 8.2) and 74% (SE 9.2), respectively. All relapsing patients had been c lassified as T2B. Conclusions. Multimodality treatment yielded satisfying s urvival results using limb-preserving surgery in most cases. Tumor size >5 cm and invasiveness, which defined stage T2B, were the most important predi ctors of poor outcome. Evaluation of the role of adjuvant chemotherapy and radiotherapy awaits prospective studies, even if T2B patients, as well as c hildren having nonradical surgery, seem worth managing by adjuvant treatmen ts. Med. Pediatr. Oncol. 32:32-37, 1999. (C) 1999 Wiley-Liss, Inc.