Pediatric soft tissue sarcomas of nonmyogenic origin

Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) are relatively rare tumors, which nonetheless constitute 50% of the soft tissue sarcomas seen in the pediatric population. The prognosis for these tumors is good, with 92% of patients in our series alive and 61% free of their disease at 5 years follow-up. The most important prognostic factor among our 35 patients was the grade of the tumor. More than 70% of our patients 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 best outlook are those who can be treated with surgery alone as the definitive care for this disease. Resection remains the primary treatment modality in NRSTS, whereas, unlike the treatment of rhabdomyosarcomas, the value of radiation therapy and chemotherapy in treating NRSTS remains undefined.