Skip to main content
U.S. Department of Health & Human Services
National Institutes of Health
NCATS

COVID-19 is an emerging, rapidly evolving situation.

Get the latest public health information from CDC: https://www.coronavirus.gov (link is external)
Get the latest research information from NIH: https://www.nih.gov/coronavirus (link is external)

National Center for Advancing and Translational Sciences National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences
1-888-205-2311
Menu Search
1-888-205-2311
Menu
  • Home
  • Diseases expand submenu for Diseases
    • Browse A-Z
    • Find Diseases By Category expand submenu for Find Diseases By Category
      • Autoimmune / Autoinflammatory diseases
      • Bacterial infections
      • Behavioral and mental disorders
      • Blood Diseases
      • Chromosome Disorders
      • Congenital and Genetic Diseases
      • Connective tissue diseases
      • Digestive Diseases
      • Ear, Nose, and Throat Diseases
      • Endocrine Diseases
      • Environmental Diseases
      • Eye diseases
      • Female Reproductive Diseases
      • Fungal infections
      • Heart Diseases
      • Hereditary Cancer Syndromes
      • Immune System Diseases
      • Kidney and Urinary Diseases
      • Lung Diseases
      • Male Reproductive Diseases
      • Metabolic disorders
      • Mouth Diseases
      • Musculoskeletal Diseases
      • Myelodysplastic Syndromes
      • Nervous System Diseases
      • Newborn Screening
      • Nutritional diseases
      • Parasitic diseases
      • Rare Cancers
      • RDCRN
      • Skin Diseases
      • Viral infections
    • List of FDA Orphan Drugs
    • GARD Information Navigator
    • FAQs About Rare Diseases
  • Guides expand submenu for Guides
    • Patients, Families and Friends expand submenu for Patients, Families and Friends
      • How to Find a Disease Specialist
      • Tips for the Undiagnosed
      • Support for Patients and Families
      • Tips for Finding Financial Aid
      • Help with Travel Costs
      • How to Get Involved in Research
      • FAQs About Chromosome Disorders
      • Medical and Science Glossaries
    • Healthcare Professionals expand submenu for Healthcare Professionals
      • Caring for Your Patient with a Rare Disease
      • ICD Coding for Rare Diseases
      • FindZebra Diagnosis Assist Tool
    • Researchers expand submenu for Researchers
      • Finding Funding Opportunities
    • Teachers and Students expand submenu for Teachers and Students
      • Teaching Resources
  • News expand submenu for News
    • In The Spotlight
    • News Archive
    • Connect with GARD
    • Media Requests
  • About GARD expand submenu for About GARD
    • Contact GARD
    • About GARD
    • Videos
    • Brochures and Publications
  • En Español expand submenu for En Español
    • Enfermedades
    • Contacte GARD
    • Guías de Información expand submenu for Guías de Información
      • Preguntas Más Frecuentes Sobre Enfermedades Raras
      • Como Encontrar un Especialista en su Enfermedad
      • Consejos Para una Condición no Diagnosticada
      • Consejos Para Pacientes y Familias
      • Consejos Para Obtener Ayuda Financiera Para Una Enfermedad
      • Participe en Estudios de Investigación
      • Preguntas Más Frecuentes Sobre los Trastornos Cromosómicos
    • Sobre GARD
  • Home
© Positive Exposure
banner showing people with disabilities and/or their relatives
  1. Home
  2. Diseases
  3. Olfactory neuroblastoma
print
  • Table of Contents expand submenu for Table of Contents
    • Summary
    • Symptoms
    • Cause
    • Diagnosis
    • Treatment
    • Research
    • Organizations
    • Living With
    • Learn More
    • GARD Answers
  • Browse A-Z
  • Find Diseases By Category expand submenu for Find Diseases By Category
    • Autoimmune / Autoinflammatory diseases
    • Bacterial infections
    • Behavioral and mental disorders
    • Blood Diseases
    • Chromosome Disorders
    • Congenital and Genetic Diseases
    • Connective tissue diseases
    • Digestive Diseases
    • Ear, Nose, and Throat Diseases
    • Endocrine Diseases
    • Environmental Diseases
    • Eye diseases
    • Female Reproductive Diseases
    • Fungal infections
    • Heart Diseases
    • Hereditary Cancer Syndromes
    • Immune System Diseases
    • Kidney and Urinary Diseases
    • Lung Diseases
    • Male Reproductive Diseases
    • Metabolic disorders
    • Mouth Diseases
    • Musculoskeletal Diseases
    • Myelodysplastic Syndromes
    • Nervous System Diseases
    • Newborn Screening
    • Nutritional diseases
    • Parasitic diseases
    • Rare Cancers
    • RDCRN
    • Skin Diseases
    • Viral infections
  • List of FDA Orphan Drugs
  • GARD Information Navigator
  • FAQs About Rare Diseases

Olfactory neuroblastoma


Back to List of Questions

Question Question


Listen
I think I may have an olfactory neuroblastoma. Can you provide me with more information?

Answer Answer


Listen

The following information may help to address your question:

  • What is olfactory neuroblastoma?
  • What causes olfactory neuroblastoma?
  • What are the signs and symptoms of olfactory neuroblastoma?
  • How is olfactory neuroblastoma diagnosed?
  • How is olfactory neuroblastoma staged?
  • How might olfactory neuroblastoma be treated? 

What is olfactory neuroblastoma?

Olfactory neuroblastoma is a rare cancer of the upper part of the nasal cavity called the cribiform plate, which is a bone deep in the skull between the eyes, and above the ethmoid sinuses. It accounts for about 5% of all cancers of the nasal cavity and paranasal sinuses. It develops in nerve tissue associated with the sense of smell (olfactory nerve). It can occur at any age, but typically is found in adulthood.[1][2][3] Symptoms may be nonspecific and include a blockage of the nasal passageways due to the tumor, facial pain, runny nose, and nosebleeds. Treatment usually includes surgery to remove the tumor, radiation therapy, and sometimes chemotherapy.[2]
Last updated: 6/29/2016

What causes olfactory neuroblastoma?

The cause of olfactory neuroblastoma is not well understood. No specific environmental or genetic causes have been confirmed, although comprehensive genetic testing of affected individuals has identified regions within chromosomes 2, 5, 6, 7, and 20 that may be involved.[3][4]
Last updated: 6/29/2016

What are the signs and symptoms of olfactory neuroblastoma?

The most common symptom of olfactory neuroblastoma is blockage of the nasal passageway secondary to the tumor. Other signs and symptoms may include:[2][4]
  • Loss of smell (anosmia) 
  • Chronic sinus infections (sinusitis)
  • Nasal bleeding
  • Nasal discharge
  • Pain
Some symptoms might be associated with a spread (metastasis) of the tumor to other structures in the face. These symptoms might include:[2][4]
  • Sinus pain and headache 
  • Visual changes
  • Ear pain 
Last updated: 6/29/2016

How is olfactory neuroblastoma diagnosed?

A diagnosis of olfactory neuroblastoma may be suspected based on presenting signs and symptoms and imaging studies such as MRI and CT scans. The diagnosis is confirmed through biopsy of the tumor.[2]
Last updated: 6/29/2016

How is olfactory neuroblastoma staged?

There is no standard staging system for individuals with olfactory neuroblastoma, however, the Kadish system is the most commonly used clinical staging system.[2] The "stage" refers to the extent of the cancer, such as how larger the tumor is. It additionally includes whether the cancer has spread (metastasis). The Kadish system classifies stages based on the extent of the primary tumor, lymph node involvement, and distant metastasis.  
  • Stage A-Tumors limited to the nasal cavity
  • Stage B-Tumors that involve the nasal cavity and extend into the paranasal sinuses
  • Stage C-Extension beyond the nasal cavity and the paranasal sinuses
  • Stage D-Regional lymph node involvement or distant metastasis

Most patients present with stage B or C disease.[5]

Last updated: 6/29/2016

How might olfactory neuroblastoma be treated? 

There are no standard guidelines for treatment of olfactory neuroblastoma. Treatment is dependent on the stage of the cancer. Most patients are initially treated with surgical removal of the tumor if possible. Radiation therapy is most commonly administered after surgery. The role of chemotherapy for olfactory neuroblastoma remains unclear. Although several studies have utilized chemotherapy, it is not clear whether it improves the long-term outlook (prognosis) compared to surgery and radiation therapy.[2][4]
Last updated: 6/29/2016

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

Please see our Disclaimer.

References References


  1. Nasal Cavity and Paranasal Sinuses Cancer. American Cancer Society. 3/2/2015; http://www.cancer.org/cancer/nasalcavityandparanasalsinuscancer/detailedguide/nasal-cavity-and-paranasal-sinuses-cancer-what-is-nasal-paranasal.
  2. Carl Snyderman, Derrick Lin, Bruce E Brockstein, Robert Foote, Jerome B Taxy. Olfactory neuroblastoma (esthesioneuroblastoma). UpToDate. Jan 8, 2016; http://www.uptodate.com/contents/olfactory-neuroblastoma-esthesioneuroblastoma.
  3. Michael Somenek. Esthesioneuroblastoma. Medscape. April 14, 2015; http://emedicine.medscape.com/article/278047.
  4. Nalavenkata SB, Sacks R, Adappa ND, Palmer JN, Purkey MT, Feldman MD, Schlosser RJ, Snyderman CH, Wang EW, Woodworth BA, Smee R, Havas TE, Gallagher R, Harvey RJ. Olfactory Neuroblastoma: Fate of the Neck--A Long-term Multicenter Retrospective Study.. Otolaryngol Head Neck Surg.. December 15, 2015; 154(2):283-9. http://www.ncbi.nlm.nih.gov/pubmed/26671900.
  5. Staging. National Cancer Institute. March 9, 2015; http://www.cancer.gov/about-cancer/diagnosis-staging/staging.

Share this content:

Share this content:

twitter-icon facebook-icon contact-icon link-icons

Copy Link

Link copied to your clipboard.

printer-icon

You May Be Interested In

How to Find a Disease Specialist

How to Find a Disease Specialist

Tips for the Undiagnosed

Tips for the Undiagnosed

Support for Patients and Families

Support for Patients and Families

Tips for Finding Financial Aid

Tips for Finding Financial Aid

Help with Travel Costs

Help with Travel Costs

How to Get Involved in Research

How to Get Involved in Research

FAQs About Chromosome Disorders

FAQs About Chromosome Disorders

Medical and Science Glossaries

Medical and Science Glossaries

Caring for Your Patient with a Rare Disease

Caring for Your Patient with a Rare Disease

ICD Coding for Rare Diseases

ICD Coding for Rare Diseases

FindZebra Diagnosis Assist Tool

FindZebra Diagnosis Assist Tool

Finding Funding Opportunities

Finding Funding Opportunities

Teaching Resources

Teaching Resources

placeholder for the horizontal scroll slider Back to top
GARD Home NCATS Home Site Map Browse Glossary A-Z Privacy Notice Disclaimer Accessibility FOIA OIG

If you have problems viewing PDF files, download the latest version of Adobe Reader

For language access assistance, contact the NCATS Public Information Officer

Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311

contact gard Office of Rare Disease Research Facebook Page Office of Rare Disease Research on Twitter
Listen