Abstract

(1) Background: Esthesioneuroblastoma (ENB) is a rare tumor entity originating from the olfactory neuroepithelium. There is a scarcity of data about different treatment strategies. Intensity modulated radiotherapy (IMRT) and carbon ion radiotherapy (CIRT) are advanced radiation techniques that might improve local tumor control. (2) Methods: This retrospective analysis contained 17 patients with ENB (Kadish stage ≥ C: 88%; n = 15). Four patients had already undergone previous radiotherapy (RT). The treatment consisted of either IMRT (n = 5), CIRT (n = 4) or a combination of both techniques (n = 8). Median follow-up was 29 months. (3) Results: In patients that had not been irradiated before (n = 13), calculated overall survival (OS) and progression free survival (PFS) rates after 48 months were 100% and 81% respectively (Kaplan-Meier estimates). Two of four patients that underwent reirradiation died after RT, presumably due to tumor progression. Besides common toxicities, five patients (30%) showed mostly asymptomatic radiation-induced brain changes, most likely due to a disturbance of the blood-brain barrier. (4) Conclusions: Our results demonstrate that IMRT, CIRT, a combined approach of IMRT and CIRT as well as reirradiation with CIRT seem to be feasible and effective treatment methods in ENB.

Highlights

  • Esthesioneuroblastoma (ENB) or olfactory neuroblastoma is a rare tumor entity that seems to originate from the olfactory neuroepithelium

  • Group A consisted of 13 patients that had not been irradiated before

  • Tumor progression could be observed in three patients

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Summary

Introduction

Esthesioneuroblastoma (ENB) or olfactory neuroblastoma is a rare tumor entity that seems to originate from the olfactory neuroepithelium. 3–6% of all cancers of the nasal cavity or paranasal sinuses can be categorized as ENB [1,2,3]. It was first described by Berger et al in 1924 [4] but still most of the published data rely on small cohorts due to the rarity of this tumor. The incidence seems to have increased in the last decade, underlining the necessity for finding appropriate, well-designed and tumor-specific treatment options [6]. Prospective data remain illusive due to the limited number of patients

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