MULTIMODAL THERAPY FOR CHILDREN AND ADOLESCENTS WITH EWING-SARCOMA - RESULTS OF THE FIRST NATIONAL CHILEAN TRIAL (1986-1991)

Thirty-seven patients with Ewing sarcoma were treated in the First Nat ional Chilean Trial for Ewing's Sarcoma (1986-1991), which comprised t he St. Jude Ewing's 78 Study. All patients received cyclophosphamide, doxorubicin, vincristine, and Dactinomycin for a total treatment perio d of about 10 months, and ail prescribed therapy was administered. Loc al therapy consisted of irradiation (RT) to the primary tumor, complet e surgical resection, or a combination of both surgery and RT. Twenty- nine of these patients had localized tumors, 24% had pelvic primary tu mors, 21 were males, and 20 were greater than 10 years of age at diagn osis. Twenty-one patients had tumors that were greater than 8 cm in la rgest diameter. Fourteen of the 29 patients with localized disease rem ain disease free at 23 to 91 months from diagnosis. Fourteen patients have died of tumor-related complications and 1 of a second-ary maligna ncy. Relapse was local only in 4, metastatic in 9, and local plus meta static in 1. Only 1 of the 8 patients with metastatic disease at prese ntation remains disease free. Toxicity consisted primarily of myelosup pression and mucositis. We conclude that this form of relative intense multimodal therapy for children/adolescents with localized Ewing sarc oma is curative in about half of affected children as in the original St. Jude study, and that it can be safely given in a developing countr y, provided that careful attention to supportive care and treatment pl anning is given. Although these results represent improvement in outco me for our patients, more effective therapy is needed for children wit h Ewing sarcoma, especially those with metastatic disease at presentat ion. (C) 1997 Wiley-Liss, Inc.