Nonrhabdomyosarcoma soft tissue sarcomas in children - The Mayo Clinic experience

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 pati ents presented with metastatic disease. A high percentage of patients prese nted after biopsy by the referring physician, although this could not be sh own to affect outcome. Patients were treated with wide removal of the tumor when possible, with judicious use of adjuvant radiation, or with chemother apy in selected cases. Mean followup was 11 years. Five- and 10-year surviv al was 92% and 84%, respectively. Tumors larger than 5 cm were associated w ith a worse prognosis. When compared with published data in adults, the pro gnosis of primary, localized nonrhabdomyosarcoma soft tissue sarcomas in ch ildren appears to be more favorable.