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Olfactory Neuroblastoma

What is an olfactory neuroblastoma?

An olfactory neuroblastoma is a rare form of cancer. It starts in the nerves that affect your sense of smell. This type of cancer is also called esthesioneuroblastoma. An olfactory neuroblastoma often happens on the roof of the nasal cavity. It affects a bone (cribriform plate) that’s between the eyes and located deep in the skull.

What causes an olfactory neuroblastoma?

Experts don’t know what causes an olfactory neuroblastoma.

Who is at risk for an olfactory neuroblastoma?

Olfactory neuroblastomas tend to occur in people between ages 10 and 20 years old and 50 to 60 years old. Research has found that people have an increased risk if they who are exposed to harmful chemicals and particles at work or in other ways. Some possible causes include wood dust, flour, nickel and cadmium dust, glues, and formaldehyde and other solvents. Tobacco smoke may also be a contributing factor in getting this type of cancer. To protect yourself, avoid 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 an olfactory neuroblastoma?

The symptoms of olfactory neuroblastoma can include:

  • Pain around the eyes

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

  • Blockage of the nose

  • Nasal drainage in the throat (postnasal drip)

  • Watery eyes

  • Nosebleeds

  • Pus from the nose

  • Face or tooth numbness

  • Loose teeth

  • Decreased sense of smell (anosmia)

  • Loss of or change in vision (visual field defect)

  • 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 an olfactory neuroblastoma diagnosed?

Your healthcare provider may order an imaging test to look at your nasal cavity. These include X-rays, CT scans, and MRI. To get more information, your provider may do a biopsy. This is done by taking a tissue sample and checking it under a microscope to find out what kind of cancer is present.

After a diagnosis of olfactory neuroblastoma, you’ll likely need more tests. 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.

A stage grouping is then assigned. The stage is assigned after surgery has been done to remove the tumor.

How is an olfactory neuroblastoma staged?

The stage of a cancer tells your doctor 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 International Neuroblastoma Staging System (INSS) is often used for this type of cancer. INSS divides tumors into stages 1 to 4. The higher the number, the more advanced the cancer is. Letters and numbers can be used after the number to give more details.

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 an olfactory neuroblastoma treated?

In most cases, the first treatment for olfactory neuroblastoma is surgery to remove the tumor. Surgery will also often remove a wide area of tissue around the cancer. This helps make sure that all the cancer has been removed. The 2 types of surgery that are often done are wide local excision and medial maxillectomy. These may require the 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 through a nostril using a thin, flexible, lighted tube. If the tumor is present in the neck, surgery might be necessary there too.

Radiation therapy is also often part of the treatment plan for this type of cancer. This may be 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 options. Make a list of questions. Think about the benefits and possible side effects of each option. Discuss your concerns with your healthcare provider before making a decision.

How can I prevent an olfactory neuroblastoma?

Research has found that people who are exposed to harmful chemicals and particles at work or in other ways have an increased risk of getting nasal cavity cancers. Some possible causes include wood dust, flour, nickel and cadmium dust, glues, and formaldehyde and other solvents. Tobacco smoke may also be a risk factor for olfactory neuroblastoma. To protect yourself, stay away from these substances as much as possible. And always use the correct protective gear at work. This includes masks and respirators.

How do I manage an olfactory neuroblastoma?

Any cancer diagnosis is stressful, but there is hope for those with a diagnosis of olfactory neuroblastoma. The best thing you can do is be alert and aware about your health and have a positive outlook about your treatments. Eating a healthy diet and living as healthy a lifestyle as possible may also play a role in your recovery. It may also help prevent the cancer from coming back. This is a difficult condition. If you and your family have trouble dealing with this diagnosis, ask your healthcare team for referrals to a mental health provider.

When early symptoms and signs of a nasal tumor are suspected, especially nasal obstruction with or without bleeding, and loss of smell.

Key points about olfactory neuroblastoma

  • An 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 an 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 require 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: Alteri, Rick, MD
Online Medical Reviewer: Cunningham, Louise, RN
Date Last Reviewed: 3/1/2018
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