Prognostic factors in head and neck Ewing sarcoma family of tumors

OBJECTIVES/HYPOTHESIS: Data on the Ewing sarcoma family of tumors (ESFT) of the head and neck region with uniform chemotherapy protocols are minimal. We evaluated outcome and prognostic factors in these patients treated with a uniform chemotherapy protocol. STUDY DESIGN: Single institution observational study. METHODS: This is a single-institution review of patients treated between June 2003 and November 2011. Patients received neoadjuvant chemotherapy (NACT), surgery, and/or radiotherapy as a local treatment followed by adjuvant chemotherapy. RESULTS: Thirty-five cases of head and neck ESFT were treated with a uniform chemotherapy protocol. The median age was 12 years (range, 1-43 years); three (9%) had metastases. Nine patients underwent surgery, of which eight received adjuvant radiotherapy; 23 received definitive radiotherapy post-NACT. At a median follow-up of 58 months (range. 3.7-133.7 months), 5-year event-free survival (EFS), overall survival (OS), and local control rate were 55.1 ± 9.2%, 68.3 ± 8.3%, and 74.1 ± 8.5%, respectively. Multivariate analysis showed that baseline white blood cell (WBC) count independently prognosticated EFS (P = .04), with patients who had WBC ≤11,000/µL had superior EFS, although no difference for OS was observed. CONCLUSIONS: This is one of the largest studies of head and neck ESFT treated with a uniform chemotherapy protocol with intent-to-treat analysis. Within the limitations of the small size, baseline low WBC count appeared to have a superior outcome.