Clinical characteristics and treatment-associated survival of head and neck Ewing sarcoma

Ewing sarcoma of the head and neck (ESHN) is a rare malignancy for which limited data exists. Herein we examine anatomic distribution of ESHN, demographic characteristics, and multi-modal therapy as potential determinants in the overall survival (OS) of patients with ESHN.

A retrospective study of the National Cancer Database (NCDB) from 2004-2016 was performed. A multivariate Cox regression and univariate Kaplan-Meier survival analyses were conducted.

We identified 284 patients with ESHN eligible for demographic analysis and 223 for survival analysis. Approximately half (50.3%) of patients with ESHN were under the age of 18. Pediatric patients (<18) were less likely to present with distant metastases (≤8.0% vs. 9.5-19.0%, P = .006) and more likely to present with osseous disease (90.2% vs. 75.2%, P = .001) than adults. Adult age (HR: 2.727, 95% CI, 1.381-5.384, P = .004) and distant metastatic disease at the time of presentation (HR: 8.161, 95% CI, 2.922-22.790, P < .001) were independently associated with worse OS. The addition of local therapy (either surgery, radiotherapy, or both) to chemotherapy was not associated with improved survival when compared to treatment with chemotherapy alone.

Predictors of OS in patients with ESHN included age < 18 years and non-metastatic disease at the time of diagnosis. Tumor site of origin (osseous vs. extraosseous primary) or the addition of local therapy to chemotherapy had no impact on OS. Our analysis suggests that chemotherapy serves as the primary modality in treating ESHN, while further study of this rare malignancy is required to discern the utility of combined systemic and local therapy. Laryngoscope, 130:2385-2392, 2020.