Olfactory neuroblastoma (esthesioneuroblastoma)
- Carl Snyderman, MD, MBA
Carl Snyderman, MD, MBA
- Professor, Departments of Otolaryngology and Neurological Surgery
- University of Pittsburgh School of Medicine
- Derrick Lin, MD
Derrick Lin, MD
- Associate Professor
- Harvard Medical School
- Bruce E Brockstein, MD
Bruce E Brockstein, MD
- Section Editor — Cancer of the Head and Neck
- Clinical Associate Professor of Medicine
- University of Chicago Pritzker School of Medicine
- Robert Foote, MD, FACR, FASTRO
Robert Foote, MD, FACR, FASTRO
- Professor and Chair, Department of Radiation Oncology
- Mayo Clinic College of Medicine
- Section Editors
- Jay S Loeffler, MD
Jay S Loeffler, MD
- Section Editor — Neurooncology
- Professor of Radiation Oncology
- Harvard Medical School
- Patrick Y Wen, MD
Patrick Y Wen, MD
- Section Editor — Neurooncology
- Professor of Neurology
- Harvard Medical School
- Julie R Park, MD
Julie R Park, MD
- Section Editor — Lymphoma; Pediatric Oncology
- Professor of Pediatrics
- University of Washington
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 .
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".)
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 . 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].
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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- CLINICAL MANIFESTATIONS
- DIAGNOSIS AND STAGING
- Imaging studies
- Differential diagnosis
- Primary tumor
- - Surgery
- - Radiation therapy
- - Surgery plus RT
- - Adjuvant chemotherapy
- Management of the neck
- Systemic disease
- - Cytotoxic chemotherapy
- - Molecularly targeted therapy
- POSTTREATMENT SURVEILLANCE
- SUMMARY AND RECOMMENDATIONS