TREATMENT OF UNRESECTABLE OR METASTATIC PEDIATRIC SOFT-TISSUE SARCOMAS WITH SURGERY, IRRADIATION, AND CHEMOTHERAPY - A PEDIATRIC-ONCOLOGY-GROUP STUDY

Background. The objectives of this study were to compare vincristine/a ctinomycin D/cyclophosphamide/adriamycin (VACA) with VACA plus imidazo le carboxamide (DTIC) (VACAD) therapy in regards to complete/partial r esponse and event free survival rates in children and adolescents with metastatic non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) or previ ously chemotherapy-naive recurrent NRSTS or locally persistent gross r esidual tumor after surgery and radiation therapy. Procedures. Between 1986 and March 1994, 75 patients entered this randomized study compar ing VACA and VACAD, given at 3 week intervals. Sixty-one patients were considered eligible and received chemotherapy and radiation therapy t o the primary tumor and areas of metastases. Thirty-six patients had r egional unresected (Group III) disease, and 25 had metastatic disease (Group IV) at time of accession. Thirty-six patients received VACA (11 were not randomized), and 25 received VACAD. Results. With a median f ollow-up oi greater than 4 years, overall and event-free survival for all eligible patients are 30.6% and 18.4%, respectively (S.E: 9.5% and 6.8%). There was insufficient evidence that DTIC offered any advantag e to event free survival, bur there was evidence for better outcome fo r patients in Group III disease in comparison to patients with Group I V disease, and for patients with a Grade 1 and 2 histology in comparis on to Grade 3 lesions. Conclusions. Combination chemotherapy with VACA and VACAD were insufficient to prevent recurrent or progressive disea se in children and adolescents with high stage NRSTS. The use of vincr istine/ifosfamide/doxorubicin with cytokine support is under study. (C ) 1998 Wiley-Liss, Inc.