Ewing Tumors of the Head and Neck

Purpose: Retrospective review describing the 40-year University of Florida experience treating Ewing tumors of the head-and-neck region with a summary of the pertinent literature. Patients and Methods: Nine patients were diagnosed and treated for Ewing sarcoma of the head and neck at our institution between 1965 and 2007. Primary sites included: mandible (3 patients), calvarium (2 patients), paranasal sinus (2 patients), oral cavity (1 patient), and the extraosseous soft tissue of the neck (1 patient). The median age at diagnosis was 13.0 years (range, 6.3-20.1 years). The median observed follow-up was 10.2 years (range, 1.5-37 years). Results: All patients received multiagent chemotherapy and radiation therapy to a median dose of 55.8 Gy (range, 36-67.2 Gy). Three patients also underwent wide local excision. The actuarial 10-year overall survival, cause-specific survival, event-free survival, and local control probabilities were 66%, 66%, 56%, and 89%, respectively. Late complications included poor dentition, mild xerophthalmia, cataract, and mandibular hypoplasia. A literature search revealed 8 previous series with Ewing tumors of the head and neck analyzed as subsets of larger studies. Conclusions: In the context of limited data on head and neck Ewing tumors, combined modality therapy provides excellent local control with reasonable acute and late toxicity. Large tumors were associated with poor disease control.