A PILOT-STUDY OF VINCRISTINE, IFOSFAMIDE, AND DOXORUBICIN IN THE TREATMENT OF PEDIATRIC NON-RHABDOMYOSARCOMA SOFT-TISSUE SARCOMAS

Background. Standard therapy for pediatric nonrhabdomyosarcoma sort ti ssue sarcomas (PNRSTS) consists oi surgical resection with or without radiotherapy. The role of chemotherapy in the treatment of these tumor s has nor vet been defined, We investigated the efficacy and toxicity of an ifosfamide-based regimen in controlling disease in children with high-risk PNRSTS. Patients and Methods. Between January 1992 and June 1994 at St. Jude Children's Research Hospital, we treated 11 children and young adults with PNRSTS who were at high risk far treatment fail ure by using a combined modality regimen that comprised aggressive sur gery, radiotherapy, and chemotherapy including vincristine, ifosfamide , and doxorubicin (VID). Nine of these patients had grade 3 disease an d one had grade 2 tumor; due to insufficient tissue, the disease grade of the remaining patient could not be established. Metastases were pr esent at diagnosis in 2 children. Results, Therapy was generally well tolerated, with minimal morbidity and ne mortality. The most common to xicity was grade ii neutropenia, which occurred in 51% of evaluable co urses. Among 4 patients evaluable for response to chemotherapy alone, 1 child attained a partial response and 3 had stable disease. One chil d had a response to chemotherapy and concurrent irradiation. At a medi an follow-up of 30 months, 10 of 11 patients are alive; 8 of 11 patien ts are alive without evidence of disease. Conclusion. Aggressive multi modality therapy for PNRSTS is well tolerated, despite frequent and pr ofound neutropenia. Although adjuvant chemotherapy for this group oi c ancers remains unproved, the rate of tumor control achieved in this pi lot study encourages further investigation in a multi-institutional se tting. (C) 1998 Wiley-Liss. Inc.