PEDIATRIC SOFT-TISSUE SARCOMAS OF NONMYOGENIC ORIGIN

Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) are relatively rare t umors, which nonetheless constitute 50% of the soft tissue sarcomas se en in the pediatric population. The prognosis for these tumors is good , with 92% of patients in our series alive and 61% free of their disea se at 5 years follow-up. The most important prognostic factor among ou r 35 patients was the grade of the tumor. More than 70% of our patient s with grade I or II lesions are disease-free at 5 years, compared to only 39% of patients with grade III lesions. The patients with the bes t outlook are those who can be treated with surgery alone as the defin itive care for this disease. Resection remains the primary treatment m odality in NRSTS, whereas, unlike the treatment of rhabdomyosarcomas, the value of radiation therapy and chemotherapy in treating NRSTS rema ins undefined. (C) 1993 Wiley-Liss, Inc.