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Olfactory neuroblastoma (esthesioneuroblastoma)

INTRODUCTION

Olfactory neuroblastoma (ONB), also referred to as esthesioneuroblastoma, is a rare malignant tumor of neuroectodermal origin. Olfactory neuroblastomas are thought to arise from the olfactory epithelium [1].

The clinical presentation, pathology, treatment, and follow-up of patients with olfactory neuroblastomas will be reviewed here. Other malignancies of the nasal cavity and skull base are discussed elsewhere. (See "Tumors of the nasal cavity" and "Paranasal sinus cancer" and "Chordoma and chondrosarcoma of the skull base".)

EPIDEMIOLOGY

The most representative data on the epidemiology of olfactory neuroblastomas come from an analysis of 311 cases in the Surveillance, Epidemiology, and End Results (SEER) database over a 30 year period [2]. In this study, the mean age at presentation was 53 years, with most cases occurring in patients between 40 and 70 years. There was a moderate male predominance with a 55:45 male:female ratio.

Other small, contemporary single institution series have yielded similar observations regarding age and gender distribution [3-6].

CLINICAL MANIFESTATIONS

Nasal obstruction due to the presence of a mass is the most common symptom with olfactory neuroblastoma and is present in the majority of cases. Other manifestations of local disease include epistaxis, nasal discharge, and/or pain. When nasal symptoms are present, physical examination usually reveals a red-brown, polypoid mass located high in the nasal cavity.

                  

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Literature review current through: Jun 2014. | This topic last updated: Sep 23, 2013.
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