Ewing's tumors with primary lung metastases:: Survival analysis of 114 (European Intergroup) cooperative Ewing's sarcoma studies patients

Purpose: To analyze event-free survival (EFS) and prognostic factors in patients who present with Ewing's tumors (ET) of bone and synchronous pulmonary and/or pleural metastases (ppm). Patients and Methods: Of l,270 patients (pts) registered at the continental office of the German/European Intergroup Cooperative Ewing's Sarcoma Studies (CESS81, CESS86, EICESS92), 114 were diagnosed ET with ppm. patients underwent neoadjuvant therapy and local treatment of the primary tumor. Whole-lung irradiation 15 to 18 Gy was applied to 75 ppm-pts, EFS and 95% confidence intervals (CIs) were estimated according to the Kaplan Meier method, and prognostic factors were analyzed by log-rank tests and Cox and logistic regression procedures. Results: On November 1, 1997, at a median time under study of 5.9 years, the 5 year EFS was 0.36 (95% CI, 0.26 to 0.46) and the 10-year EFS was 0.30 (95% CI, 0.19 to 0.41), Thirty-seven of 59 (63%) first relapses involved lung and/or pleura, and the lungs were the only site of relapse in 26 of 59 (44%) ppm-pts, Risk factors identified in univariate and multivariate tests were poor response of the primary tumor toward chemotherapy, metastatic lesions in both lungs, and treatment without additional lung irradiation. Conclusion: Chemotherapy response of the primary tumor is a prognostic factor in patients with ET with ppm. Strategies of treatment intensification warrant further evaluation. J Clin Oncol 16:3044-3052, (C) 1998 by American Society of Clinical Oncology.