Olfactory neuroblastoma

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.

Last updated on 05-01-20

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.

Last updated on 05-01-20

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. 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.

Last updated on 05-01-20

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. 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.

Last updated on 05-01-20

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.

Last updated on 05-01-20

Name: American Cancer Society 250 Williams Street NW
Atlanta, GA, 30329, United States
Toll Free: 1-800-227-2345 Url: https://www.cancer.org

Connect with other users with Olfactory neuroblastoma on the RareGuru app

Do you have information about a disease, disorder, or syndrome? Want to suggest a symptom?
Please send suggestions to RareGuru!

The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

People Using the App

Join the RareGuru Community

To connect, share, empower and heal today.

People Using the App