Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: The St. Jude Children's Research Hospital experience

Background. Because the natural history of pediatric patients with metastat ic nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) had not been well d escribed, we retrospectively reviewed our single-institution experience wit h these tumors. Procedure. We identified 26 patients with metastatic NRSTS who were treated at St, Jude Children's Research Hospital from December 197 1 through July 1995. We evaluated the characteristics of each patient, incl uding age, sex, primary site, stage, type of therapy received, and outcome. Sites of metastatic disease at diagnosis and relapse were noted. Results. The median age of the 26 study patients at diagnosis was 14.8 years; 54% of patients were male and 69% were white. The most common histologies were sy novial sarcoma, alveolar soft-part sarcoma, and malignant fibrous histiocyt oma. Most primary tumors (73% of cases) occurred in the trunk or extremitie s. Most patients presented with large (>5 cm), high-grade lesions. All 26 p atients received chemotherapy, and 8 responded to an ifosfamide- or cycloph osphamide-doxorubicin-based regimen. Radiotherapy was administered to 15 pa tients, and 13 had a partial or complete resection of the primary tumor. Si x patients underwent thoracotomy. The estimated 2-year survival for the coh ort was 34.6% +/- 8.9%; the 2-year progression-free survival was 15.4% +/- 6.3%. The lung was the most common site of failure. Conclusions. Children w ith metastatic NRSTS have a poor outcome, which is similar to that in adult s. More effective systemic chemotherapy is needed to facilitate complete su rgical resection of primary and metastatic sites. Aggressive pulmonary meta statectomy can increase disease control. (C) 1999 Wiley-Liss, Inc.