Nonrhabdomyosarcoma Soft Tissue Sarcomas in Children

Abstract

Eighty-six children to 18 years of age were treated for nonrhabdomyosarcoma soft tissue sarcomas of the trunk and extremities. Synovial sarcoma (31), fibrosarcoma (13), malignant fibrous histiocytoma (11), epithelioid sarcoma (10), and clear cell sarcoma (7) were the most common diagnoses. Four patients presented with metastatic disease. A high percentage of patients presented after biopsy by the referring physician, although this could not be shown to affect outcome. Patients were treated with wide removal of the tumor when possible, with judicious use of adjuvant radiation, or with chemotherapy in selected cases. Mean followup was 11 years. Five- and 10-year survival was 92% and 84%, respectively. Tumors larger than 5 cm were associated with a worse prognosis. When compared with published data in adults, the prognosis of primary, localized nonrhabdomyosarcoma soft tissue sarcomas in children appears to be more favorable.