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. 2014 Feb;75(1):58-64.
doi: 10.1055/s-0033-1356493. Epub 2013 Sep 20.

Esthesioneuroblastoma: an update on the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy

Marc W Herr  1 , Rosh K V Sethi  2 , Joshua C Meier  1 , Kyle J Chambers  3 , Aaron Remenschneider  3 , Annie Chan  4 , William T Curry  5 , Fred G Barker 2nd  5 , Daniel G Deschler  3 , Derrick T Lin  1
Affiliations

Esthesioneuroblastoma: an update on the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy

Marc W Herr et al. J Neurol Surg B Skull Base. 2014 Feb.

Abstract

Objectives To update the Massachusetts General Hospital (MGH) and Massachusetts Eye and Ear Infirmary (MEEI) experience in the management of esthesioneuroblastoma (ENB) with multimodality therapy and to reassess treatment outcomes and complications in a larger cohort with longer follow-up times. Design A retrospective chart review. Setting A tertiary referral center. Participants All patients presenting with ENB and managed at the MGH and MEEI from 1997 to 2013. Main Outcome Measures Disease-free and overall survival. Results Twenty-two patients were identified with an average follow-up of 73 months. Ten patients presented with Kadish stage B disease and 12 with stage C disease. A total of six patients (27%) developed regional metastases. 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 86.4% and 95.2%, respectively, by Kaplan-Meier analysis. Negative margins were a significant factor in disease-free survival. One patient experienced severe late-radiation toxicity. Conclusions ENB is safely and effectively treated with CFR followed by proton beam irradiation. The high incidence of regional metastases warrants strong consideration for elective neck irradiation. Proton beam radiation is associated with lower rates of severe late-radiation toxicity than conventional radiotherapy.

Keywords: craniofacial resection; esthesioneuroblastoma; proton beam radiation; sinonasal malignancy.

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Conflict of interest statement

Disclosures The authors have no conflicts of interest to report.

Figures

Fig. 1
Fig. 1
The 5-year overall survival rate was 95.2% by Kaplan-Meier analysis.
Fig. 2
Fig. 2
The 5-year disease free survival rate was 86.4% by Kaplan-Meier analysis.
Fig. 3
Fig. 3
The 5-year disease-free survival (DFS) Kaplan-Meier curves by surgical margin status. Five-year DFS rates were 92.3% with negative surgical margins versus 77.8% with positive margins; log-rank p = 0.015.
See this image and copyright information in PMC

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