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Olfactory Neuroblastoma

What is an olfactory neuroblastoma?

Olfactory neuroblastoma is a very rare type of cancer that affects adults and children. It's also called esthesioneuroblastoma. It starts in the olfactory nerves that are responsible for your sense of smell. These nerves are in the roof of the nasal cavity, which is where the tumor forms. These tumors eat away the bone that’s deep in the skull between the eyes (the cribriform plate).

What causes an olfactory neuroblastoma?

Experts don’t know what causes olfactory neuroblastomas.

Who is at risk for olfactory neuroblastoma?

A risk factor is anything that may increase your chance of having a disease. The exact cause of someone’s cancer may not be known. But risk factors can make it more likely for a person to have cancer. Some risk factors may not be in your control. But others may be things you can change.

Olfactory neuroblastomas tend to occur in people between ages 10 and 20 years old and 50 to 70 years old.

Research has found that adults have an increased risk if they're exposed to harmful chemicals and particles at work or in other ways. Some possible causes include wood dust, flour, nickel and cadmium dust, glues, formaldehyde, and other solvents. Tobacco smoke may also be a risk factor for this type of cancer. To protect yourself, stay away from these substances as much as possible.

Talk with your healthcare provider about your risk factors for olfactory neuroblastomas and what you can do about them.

What are the symptoms of olfactory neuroblastoma?

The symptoms of olfactory neuroblastoma can include:

  • Blockage of the nose or trouble breathing through the nose

  • Nosebleeds

  • Pain around the eyes or bulging eyes

  • Headache

  • Nasal drainage (postnasal drip) and runny nose

  • Stuffiness or congestion that gets worse or doesn't get better

  • Watery eyes

  • Pus from the nose

  • Face or tooth numbness

  • Loose teeth

  • Decreased sense of smell (anosmia)

  • Loss of or change in vision

  • Double vision

  • Ear pain or pressure

  • Trouble opening the mouth

  • Enlarged lymph nodes in the neck

Many of these may be caused by other health problems. But it’s important to see a healthcare provider if you have these symptoms. Only a healthcare provider can tell if you have cancer.

How is olfactory neuroblastoma diagnosed?

Your healthcare provider will order a CT scan or MRI imaging to look at your nasal cavity. This can tell you if your symptoms are caused by something other than a tumor.

Imaging is also important for staging the olfactory neuroblastoma These help your healthcare providers learn more about the cancer. They can help show if the cancer has grown into nearby areas or spread to other parts of your body. The test results help your healthcare providers decide the best ways to treat the cancer.

The diagnosis of olfactory neuroblastoma needs a tissue biopsy, usually through an endoscope. An endoscope is a thin, flexible, lighted tube. Small samples of tissue will be removed. A pathologist will look at the sample under the microscope to see what kind of tumor it is.

How is an olfactory neuroblastoma staged?

The stage of a cancer tells your healthcare provider how much and how far it has spread in your body. The stage of a cancer is one of the most important things to know when deciding how to treat it. The stage of an olfactory neuroblastoma is assigned after surgery has been done to remove the tumor.

The staging system most often used for this type of cancer divides tumors into stages A, B, C, and D:

  • Stage A means the tumor is only in the nasal cavity.

  • Stage B tumors have spread into the sinuses around the nose (the paranasal sinuses).

  • Stage C cancer has spread beyond the nasal cavity and paranasal sinuses to nearby tissue.

  • Stage D means cancer cells have spread to distant lymph nodes or other parts of the body, like the lungs. liver, bones, or brain.

Your healthcare provider will talk with you about what your cancer stage means for your treatment. Ask any questions or talk about your concerns.

How is olfactory neuroblastoma treated?

Surgery to remove the tumor is usually the first treatment for olfactory neuroblastoma. A wide edge (margin) of tissue around the cancer is also removed. This helps make sure that all the cancer cells have been taken out. The 2 types of surgery that are often done are wide local excision and medial maxillectomy. Both may need reconstruction of part of the face. But these offer the best chance of long-term survival. In some cases, less-invasive surgeries may be possible. These include endoscopic surgery, which is done through the nose using a thin, flexible, lighted tube. If the cancer is also in the neck, surgery might be needed there too.

Radiation therapy is often part of the treatment plan for this type of cancer. Sometimes it's the main treatment. Or, it might be used after surgery to reduce the chances of the cancer returning. In some cases, chemotherapy may also be used to treat olfactory neuroblastoma.

You may need a combination of treatments involving radiation and chemotherapy after surgery to give you the best chance of survival.

Talk with your healthcare providers about your treatment choices. Make a list of questions. Think about the benefits and possible side effects of each choice. Discuss your concerns with your healthcare provider before making a decision.

What are possible side effects and complications of olfactory neuroblastoma?

All cancers and cancer treatments have short-term and long-term side effects. Most can be treated, and many get better over time after treatment ends. Your healthcare provider will talk with you about these based on the type of treatment you're getting.

Possible complications of treatment for olfactory neuroblastoma include:

  • Changes in the way you look

  • Brain damage

  • Leakage of cerebrospinal fluid

  • Vision changes

  • Glaucoma

  • Bone damage

  • Cancer recurrence (it comes back after treatment)

  • Infection at the surgical site

How can I prevent olfactory neuroblastoma?

There's no sure way to prevent this cancer. But if you work with harmful chemicals and particles, always use the correct protective gear. This includes masks and respirators.

Not smoking and staying away from the tobacco smoke from other people may also help decrease risk.

How do I manage olfactory neuroblastoma?

Any cancer diagnosis is stressful, but there's hope for those with a diagnosis of olfactory neuroblastoma. The best thing you can do is be aware of your body and talk with your treatment team about any changes you notice. Eating a healthy diet and living as healthy a lifestyle as possible may also play a role in your recovery. It might also help keep the cancer from coming back. If you and your family have trouble dealing with this diagnosis, ask your healthcare team for help. Talking with a mental health provider or visiting a support group can help you find ways to cope with cancer and the changes it brings to you and your family.

Key points about olfactory neuroblastoma

  • Olfactory neuroblastoma is a rare form of cancer. It starts in the nerves that affect your sense of smell.

  • Experts don’t know what causes olfactory neuroblastoma. But it is linked to exposure to harmful chemicals or particles.

  • In most cases, the first treatment is surgery to remove the tumor.

  • The most common surgeries used may need the reconstruction of part of the face. But they offer the best chance of long-term survival.

  • You may need a combination of treatments, such as radiation and chemotherapy after surgery.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

  • Know the reason for your visit and what you want to happen.

  • Before your visit, write down questions you want answered.

  • Bring someone with you to help you ask questions and remember what your provider tells you.

  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also, write down any new instructions your provider gives you.

  • Know why a new medicine or treatment is prescribed, and how it will help you. Also, know what the side effects are.

  • Ask if your condition can be treated in other ways.

  • Know why a test or procedure is recommended and what the results could mean.

  • Know what to expect if you do not take the medicine or have the test or procedure.

  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.

  • Know how you can contact your provider if you have questions.

Online Medical Reviewer: Jessica Gotwals RN BSN MPH
Online Medical Reviewer: Luc Jasmin MD
Online Medical Reviewer: Sabrina Felson MD
Date Last Reviewed: 1/1/2023
© 2000-2024 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.
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