Long-term outcomes of patients with esthesioneuroblastomas: A cohort from a single institution

Objective: Esthesioneuroblastoma is a rare cancer. The purpose of this study was to review the long-term outcomes of patients with esthesioneuroblastomas (ENBs) who were treated at a single institution. Materials and methods: One hundred thirteen patients with biopsy-proven ENBs between June of 1979 and November of 2014 were retrospectively reviewed. There was 1 patient at stage A, 23 stage B and 89 stage C according to Kadish classifications. The initial treatments included pre-operative radiotherapy (RT) followed by surgery in 11 patients, surgery followed by post-operative RT in 51, primary RT in 47, and surgery in 3, and only a single patient was treated with palliative chemotherapy alone. Results: The median follow-up was 75 months, 5-year overall survival (OS), loco-regional control rate (LRC) and distant metastasis-free survival were 65%, 73% and 67%, respectively. The OSs at 5 years were 91% in the pre-operative RT group, 82% in the post-operative RT group, and 50% in the primary RT group (p < 0.001). Regarding the patients in early disease stages (Kadish A/B), no survival differences were observed between primary RT and combination treatment. Regarding the node-negative Kadish C disease patients, combination of surgery and RT elicited superior survival, and pre-operative RT yielded the best prognoses. Distant failure rate is over 60% for N-positive disease, chemotherapy may play a more important role. Conclusions: The optimal treatment policy for ENBs remains the combination of surgery and radiotherapy. When choosing the most adequate therapy for ENBs, disease stage, age and lymph nodes status should be taken into consideration. (C) 2015 Elsevier Ltd. All rights reserved.