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Diseases

Genetic and Rare Diseases Information Center (GARD)

Olfactory neuroblastoma


Other Names for this Disease
  • Esthesioneuroblastoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

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

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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

References
Other Names for this Disease
  • Esthesioneuroblastoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.