EWINGS-SARCOMA OF SOFT-TISSUES IN CHILDHOOD - A REPORT FROM THE INTERGROUP RHABDOMYOSARCOMA STUDY, 1972 TO 1991

Purpose: One hundred thirty of 2,792 patients (5%) registered on three Intergroup Rhabdomyosarcoma Study clinical trials (IRS-I, -II, and -I II) from 1972 to 1991 had an extraosseous Ewing's sarcoma (EOE). We re port here the results of multimodality therapy for this tumor. Patient s and Methods: The 130 patients were less than 21 years of age; 70 (54 %) were motes. Primary tumor sites were on the trunk in 41 patients, a n extremity in 34, the head/neck in 23, the retroperitoneum/pelvis in 21, and other sites in 11. One hundred fourteen patients had no metast ases at diagnosis. In 21 patients, the tumor was completely resected; in 30, the localized or regional tumor was grossly resected, and in 63 patients, grossly visible sarcoma was left behind. Sixteen patients ( 12%) had distant metastases at diagnosis. All patients were given mult iagent chemotherapy and most received irradiation (XRT); none were tre ated with bone marrow transplantation. Results: One hundred seven pati ents (82%) achieved a complete response. At 10 years, 62%, 61%, and 77 % of the patients were alive after treatment an IRS-I, IRS-II, or IRS- III therapeutic protocols, respectively, similar to figures obtained i n all IRS patients. At last follow-up evaluation, 42 patients had died of progressive tumor and one of infection. Survival at 10 years was m ost likely for patients with tumor that arose in the head and neck, ex tremities, and trunk, and for those who underwent grossly complete tum or removal before initiation of chemotherapy. For patients with locali zed, gross residual tumor, adding doxorubicin (DOX) to the combination of vincristine, dactinomycin, cyclophosphamide (VAC), and XRT did not significantly improve survival in 39 patients (62% alive at 10 years) compared with that of 24 patients treated with VAC and XRT without DO X (65% alive at 10 years, P = .93). Conclusion: This series indicated that EOE in children is similar to rhabdomyosarcoma (RMS) in its respo nse to multimodal treatment. No benefit was apparent from the addition of DOX to VAC chemotherapy in patients with gross residual EOE. (C) 1 997 by American Society of Clinical Oncology.