Chest wall Ewing sarcoma: a population-based analysis

BackgroundThe globally low incidence of pediatric chest wall Ewing sarcoma (CWES) has limited prior studies of this disease to mostly small, single-institution reviews. Our objective was to assess incidence, demographics, treatment patterns, and long-term survival of this disease through a population-based analysis.Materials and methodsThe Surveillance, Epidemiology, and End Results database was used to identify patients aged 0-21 y diagnosed with CWES from 1973 to 2011. Patients were grouped by decade to assess changes in treatment patterns and outcomes. The effects of clinical, demographic, and treatment variables on overall survival (OS) were assessed by the computation of Kaplan–Meier curves and the log-rank test, with Cox proportional hazard regression used for multivariable analysis.ResultsA total of 193 pediatric patients with histologically confirmed CWES were identified. The disease was more common in men (61%), whites (92%), and 11- to 17-y olds (49%). It was metastatic at presentation in 37% of patients. When grouped approximately by decade, 10-y OS improved progressively from 38% in 1973-1979 to 65% in 2000-2011 (P = 0.033). The use of radiation decreased from 84% in the earliest period to 40% in the most recent, whereas the proportion of patients receiving surgery increased from 75% to 85%. When controlling for covariates in multivariable analysis, male patients were found to have a higher mortality than female patients (hazard ratio: 2.4; confidence interval: 1.4, 4.4; P = 0.0028).ConclusionsThis population-based analysis of CWES demonstrated an impressive trend of improving OS, with increasing use of surgery and decreasing use of radiation therapy. Our study demonstrated a gender difference in survival of CWES, with females having a better prognosis. The presence of metastatic disease is a very important prognostic factor for this illness.