Non-metastatic Ewing's sarcoma: twenty years of experience suggests that surgery is a prime factor for successful multimodality therapy.

Eighty-five patients (37 female, 48 male; median age 14 years) with non-metastatic Ewing's sarcoma received definitive treatment at the University of Texas M.D. Anderson Cancer Center between 1969 and 1988. Multidisciplinary therapy was administered as follows: combination chemotherapy (CC) and local radiotherapy (XRT): 65 patients; CC, XRT and surgery, 19 patients; and XRT and surgery, 1 patient. This permitted a 10-20 year follow-up for 75% of our patients. The overall survival at 5 and 10-20 years was 46.1%, and 37.2%, respectively. At 5 years, 80.5% of live patients had control of local disease. The influence of sex, age, ethnicity, primary site, size, lactic dehydrogenase (LDH) level, presence or absence of systemic symptoms, and XRT dose (<60 Gy and </=60 Gy) was analyzed and was not found to be of prognostic significance in survival. The presence of a soft tissue mass at diagnosis was found to be a significant unfavorable prognostic variable. Nine of 11 patient who underwent resection after CC and/or XRT had residual tumor in the surgical specimen. Patients who received surgery as part of the planned treatment of their primary tumor had significantly better local control and disease-free survival than those who did not undergo resection. Complications in long-term survivors are described.