Adult Ewing sarcoma: Survival and local control outcomes in 36 patients with metastatic disease

Objectives: To assess the clinical features and outcomes in adult patients with metastatic Ewing sarcoma (ES).

Methods: The records of 36 ES patients with metastatic disease ≥18 years seen from 1977 to 2007 at the Mayo Clinic were studied retrospectively. Factors relevant to prognosis, survival, and local control (LC) were analyzed.

Results: The 4-year overall survival (OS) and event-free survival (EFS) rates were 20% and 11%, respectively. Patients treated from 1993 to 2007 had significantly improved outcomes compared with those treated from 1977 to 1992:4-year OS and EFS of 31% and 16%, respectively, versus 0% (P = 0.01). Primary tumor (P= 0.005 and 0.04) and metastatic sites (P= 0.05) were independent EFS prognostic factors. Four patients (11%) received surgery, 18 (50%) radiation therapy (RT), 4 (11%) surgery+radiation therapy (S + RT), and 10 (28%) received no LC. The 4-year EFS rates were 0% for surgery, 21% for RT, 0% for S+ RT, and 0% for no LC (P= 0.0001). OS in patients who received vincristine, doxorubicin, and cyclophosphamide alternating with ifosphamide and etoposide (VDC/IE) chemotherapy was improved (P = 0.04). Relapses were documented in 18 patients (excluding patients who received no LC). In total, 44% of patients had all of their metastatic site(s) treated. Patients who received treatment to all extrapulmonary metastases had significantly improved outcomes compared with those who did not receive treatment to all sites: 4-year OS and EFS of 33% and 11%, respectively, versus 0% (P= 0.04 and 0.02).

Conclusions: Our results suggest outcomes for adult patients with metastatic ES are similar to pediatric cohorts in the modern era. VDC/IE chemotherapy and treatment to all metastatic lesions is associated with improved outcomes.