Esthesioneuroblastoma: An Update on the Massachusetts Eye and Ear Infirmary and Massachusetts General Hospital Experience with Craniofacial Resection, Proton Beam Radiation, and Chemotherapy

Objective: To update the Massachusetts General Hospital (MGH) and Massachusetts Eye and Ear Infirmary (MEEI) experience in the management of esthesioneuroblasoma (ENB) with multimodality therapy, including craniofacial resection and proton beam radiotherapy with or without chemotherapy.

Study Design: A retrospective chart review was performed of all patients presenting with ENB and completely managed at the MGH and MEEI from 1997 to 2011.

Setting: A tertiary referral center.

Main Outcome Measures: Disease-free and overall survival.

Participants: All patients presenting with ENB and completely managed at the MGH and MEEI from 1997 to 2011.

Results: Twenty-three patients were identified with a median follow-up time of 42 months (range, 8 to 180 months). Average age at presentation was 44 years (range, 11 to 77 years). Nasal obstruction was the most common presenting symptom. Nine patients presented with Kadish stage B disease and 14 with stage C disease. Three patients had evidence of cervical metastases at presentation. Treatment for all patients included craniofacial resection (CFR) followed by proton beam irradiation with or without chemotherapy. The 5-year disease-free and overall survival rates were 87.0% and 95.7%, respectively, by Kaplan-Meier analysis. No patient suffered severe radiation toxicity.

Conclusion: ENB is safely and effectively treated with CFR followed by proton beam irradiation. Proton beam radiation is associated with minimal, and generally temporary, radiation toxicity. Chemotherapy may be beneficial for patients with regional extension of disease or positive margins. The role of induction chemotherapy remains unclear.