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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Esthesioneuroblastoma


Other Names for this Disease
  • Olfactory neuroblastoma
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Your Question

I think I may have an esthesioneuroblastoma. 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 esthesioneuroblastoma?

Esthesioneuroblastoma 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 develops in nerve tissue associated with the sense of smell and can occur in people of any age. This cancer is very uncommon, accounting for 7 percent of all cancers of the nasal cavity and paranasal sinuses.[1] Although it generally grows slowly, an esthesioneuroblastoma can sometimes grow very quickly. Fast-growing tumors can metastasize (spread) even many years after treatment of the initial tumor.[2]
Last updated: 1/13/2009

What causes esthestioneuroblastoma?

The cause of esthesioneuroblastoma is currently unknown.[3]
Last updated: 1/13/2009

What symptoms are associated with esthesioneuroblastoma?

Symptoms of esthesioneuroblastoma may include one or more of the following:[3]
  • Nasal obstruction
  • Loss of smell
  • Chronic sinus infections (sinusitis)
  • Nasal bleeding
  • Sinus pain and headache 
  • Visual changes
Last updated: 1/13/2009

How is esthesioneuroblastoma diagnosed?

Diagnosis is typically obtained through clinical examination, biopsy, and MRI and CT scans.[2]
Last updated: 1/13/2009

How is esthesioneuroblastoma staged?

The Kadish system is most commonly used to classify the clinical extent of disease for esthesioneuroblastoma. Tumors limited to the nasal cavity are classified as stage A. Stage B includes tumors that involve the nasal cavity and that have extended into the paranasal sinuses. Finally, stage C includes disease that has extended beyond the nasal cavity and the paranasal sinuses. Most patients present with stage B or C disease.[3]
Last updated: 1/13/2009

How is esthesioneuroblastoma usually treated?

Various treatment regimens for esthesioneuroblastoma have been used through the years. Early treatment included using either surgery or radiation therapy, but, for the most part, these regimens resulted in high rates of recurrence. Subsequently, multimodality therapy with surgery and radiation therapy has been more frequently administered, and some institutions recommend trimodality therapy, with the addition of chemotherapy to surgery and radiation therapy.[3]

Most patients are initially treated with surgical removal if possible. Radiation therapy is most commonly administered after surgical removal of the tumor. The role of chemotherapy for esthesioneuroblastoma remains poorly defined. Many institutions incorporate chemotherapy into the treatment regimen, especially for stage C disease, whereas others have not noted any substantial clinical response to chemotherapy.[3]

Last updated: 1/13/2009

References
  • Detailed Guide: Nasal Cavity and Paranasal Cancer. American Cancer Society Web site. Available at: http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=75. Accessed November 11, 2008..
  • Esthesioneuroblastoma. Mayo Clinic Web site. Available at: http://www.mayoclinic.org/esthesioneuroblastoma/. Accessed November 11, 2008..
  • Murphy BA, Aulino JM, Chung CH, Ely K, Sinard R, Cmelak AT. Esthesioneuroblastoma. In: Raghavan D, Brecher ML, Johnson DH, Meropol NJ, Moots PL, Rose PG, eds. Textbook of Uncommon Cancer, third edition. Wiley & Sons; 2006: 133-142..