CHILDHOOD SOFT-TISSUE SARCOMA - A 20-YEAR EXPERIENCE

Purpose: To evaluate the disease-free and overall survival of pediatri c patients with nonrhabdomyosarcoma soft-tissue sarcomas. Methods: We retrospectively analyzed the records of 67 pediatric patients with a d iagnosis of nonrhabdomyosarcoma soft tissue sarcoma treated with curat ive intent between 1970 and 1992. Median follow-up time for the 52 sur vivors was 120 months (range, 7 to 277 months). Fifty-nine patients re ceived external beam radiotherapy, in a median dose of 5400 cCy (range , 1800 to 6560 cGy.) All patients underwent an initial surgical proced ure. Eighteen patients had gross residual disease, and 15 had gross to tal excision with microscopic residual disease or positive margins. Ad juvant chemotherapy was administered to 44 patients (65%). Results: Th e actuarial 10-year freedom from progression or recurrence and overall survival rates were 76% and 75%, and the 20-year rates were the same. Of 18 patients with gross residual disease, 9 (50%) had local progres sion and 6 died of local-only disease. By contrast, only one patient w ith microscopic residual disease who received postoperative radiothera py had a local recurrence. The disease-free survival rate also correla ted with histologic grade. Conclusions: As with adult soft tissue sarc omas, gross residual disease predicts local failure. Our results sugge st that pediatric patients with soft tissue sarcomas treated with surg ery and postoperative radiotherapy generally have a favorable overall survival rate.