Abstract

ObjectivesTo summarize the characteristics and long–term outcomes of olfactory neuroblastoma through the analysis of 13 cases in single institution, with the assessment of treatment modality, prognostic factors.MethodA retrospective study of thirteen cases diagnosed as olfactory neuroblastoma and underwent combined treatments during the period 2000–2010. Statistical analysis was performed to search for prognostic factors and compared different treatment modalities.Results13 patients were enrolled in this study, including 8 male and 5 female, ranging from 15 to 69 (median 43) years old. One patient at stage A was only treated with endoscopic endonasal surgery (EES). Seven patients were treated with preoperative radiotherapy and EES, two with EES and postoperative radiotherapy, and the other three with combined radiotherapy and chemotherapy. The range of follow-up time varied from 23 to 116 months (median 65 months). The 5-year overall survival rate was 46.2% (6/13). To date, these thirteen patients have not suffered local recurrences while two patients had lymph node recurrences and one had distant metastasis in the bone marrow. In 13 patients, 61.5% were diagnosed as late T stage (T3/4), 69.2% late Kadish stage (C/D) and 53.8% were high Hyams grade (I/ II), which indicated poor prognosis. Related prognostic factors were the TNM stage (T stage P = 0.028, N stage P = 0.000, M stage P = 0.007), Kadish stage (P = 0.025) and treatment modality (P = 0.015).ConclusionLate stage of TNM and Kadish staging system indicated a poor prognosis. Combined treatment modality, including endoscopic endonasal surgery, achieved a better outcome than non-surgical approach.

Highlights

  • Olfactory neuroblastoma (ONB), known as esthesioneuroblastoma (ENB), is a rare malignant neoplasm arising from the upper nasal cavity

  • One patient at stage A was only treated with endoscopic endonasal surgery (EES)

  • Seven patients were treated with preoperative radiotherapy and EES, two with EES and postoperative radiotherapy, and the other three with combined radiotherapy and chemotherapy

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Summary

Introduction

Olfactory neuroblastoma (ONB), known as esthesioneuroblastoma (ENB), is a rare malignant neoplasm arising from the upper nasal cavity It was originally described by Berger and colleagues in 1924 using the term “esthesioneuroepitheliome olfactif ”[1]. In 1992, Dulguerov and Calcetta proposed a tumor, node and metastasis (TNM) staging system, largely based on computed tomography(CT) and magnetic resonance imaging (MRI) scans, allowing for cervical lymph node involvement and distant metastasis [5]. These tumors have a tendency towards local invasion (commonly orbit and anterior skull base) and distant metastases (commonly neck, lungs, liver and bones). We performed a systematic review of previous published studies to compare our findings with them

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