Esthesioneuroblastoma

What is esthesioneuroblastoma?

Esthesioneuroblastoma (es-THEE-zee-oh-NOOR-oh-blas-TOH-muh) is a rare type of head and neck cancer. Healthcare providers may call it olfactory neuroblastoma. In esthesioneuroblastoma, you have cancerous tumors in your nasal cavities, which are hollow areas inside your nose. The tumors affect nerves and tissues that are responsible for your sense of smell.

This condition mostly affects adults, but it can affect children and adolescents. Healthcare providers treat it with surgery to remove the tumor, followed by radiation therapy. Esthesioneuroblastoma may grow back (recur), so healthcare providers recommend people have regular checkups to confirm it hasn’t come back.

How common is esthesioneuroblastoma?

It’s rare, affecting about 1 in 2.5 million people worldwide each year. Most people are in their 50s to 70s when they receive their diagnosis, but it can affect children and teenagers. Esthesioneuroblastoma is the most common cancer of the nasal cavity in children and adolescents.

What are the symptoms of esthesioneuroblastoma?

It may make you feel like you have a sinus infection or other sinus issues, including:

• Anosmia (loss of sense of smell).
• Nasal congestion (stuffy nose).
• Recurrent epistaxis (nosebleeds).

What causes esthesioneuroblastoma?

There’s no known cause for this condition. Experts are researching possible chromosomal changes or mutations that may play a role in causing esthesioneuroblastoma.

What are the complications of esthesioneuroblastoma?

It often comes back after treatment and may come back years after you’ve finished treatment. Recurrent esthesioneuroblastoma may come back in the original site or in nearby tissue. Rarely, it comes back in more distant areas of your body.

Without treatment, it can spread (metastasize) to your brain or other areas of your body. Studies show esthesioneuroblastoma most often spreads to your sinuses, but it can spread to lymph nodes in your neck, your lungs and your bones.

How is esthesioneuroblastoma diagnosed?

Healthcare providers may diagnose it by doing various imaging tests and, if appropriate, a biopsy. Imaging tests may include:

• Computed tomography (CT) scan.
• Magnetic resonance imaging (MRI) scan.
• Nasal endoscopy.
• Positron emission tomography (PET) scan.

What are the stages of esthesioneuroblastoma?

Healthcare providers use cancer staging systems to plan treatment and a prognosis. Esthesioneuroblastoma stages are:

• Stage A: Cancerous cells develop into a tumor in your nasal cavity.
• Stage B: The tumor spreads directly from your nasal cavity to your nasal sinuses.
• Stage C: The tumor spreads directly from your nasal cavity and sinuses towards your eyes or your brain.
• Stage D: Tests show signs that cancerous tumors have spread to other areas of your body, such as the lymph nodes in your neck, your bone marrow or your lungs.

What are treatments for esthesioneuroblastoma?

Healthcare providers typically do surgery to remove cancerous tumors. They may do:

• Endoscopic surgery.
• Craniofacial surgery.
• Craniotomy.

Other treatments are:

• Radiation therapy to kill any cancerous cells that remain after surgery.
• Chemotherapy to treat esthesioneuroblastoma if tumors can’t be safely removed with surgery.

How long does it take to recover from surgery for esthesioneuroblastoma surgery?

That depends on the type of surgery that you have, but people remain in the hospital for two to five days before continuing to recover at home. It may be one to two months before they receive radiation therapy.

What are treatment side effects?

Surgery, radiation therapy and chemotherapy have different side effects:

• Surgery side effects include pain, nasal stuffiness and reaction to anesthesia.
• Common chemotherapy and radiation side effects may include fatigue, diarrhea, nausea and vomiting.

What are treatment complications?

Surgery complications may include:

• Infection.
• Excessive bleeding.
• Permanent loss of smell. There’s a close connection between your sense of smell and your sense of taste, so you may also lose your sense of taste.
• Cerebrospinal fluid (CSF) leak.
• Drainage in your nose that may feel crusty and dry.

What’s the prognosis for esthesioneuroblastoma in adults?

Your prognosis (outlook) is your provider’s estimate of how a disease will affect you after treatment. Every person is different, and prognosis will depend on several factors, like:

• Where the tumor is in your body.
• If the cancer has spread to other parts of your body.
• How much of the tumor was taken out during surgery.
• Results of lab analysis that show tumor cell features.

If you have questions about your prognosis, talk to your surgeon and other people on your care team. They know you and your situation and are your best source of information.

What is the prognosis for esthesioneuroblastoma in children?

Like adults, the prognosis depends on children’s specific situation, including factors like tumor location, specific tumor cell features, whether the tumor has spread and how much of the tumor was removed during surgery.

What’s the survival rate of esthesioneuroblastoma?

Healthcare providers estimate survival rates by looking at the experiences of people who have the condition. In this case, 50% to 90% of people with this condition were alive five years after diagnosis.

It can be confusing and stressful to try to figure out what survival rate data means. Here’s the thing about survival rates: they’re estimates based on other people’s experiences. What was true for them may not be true for you. If you have questions, don’t hesitate to talk to your provider. They’ll explain survival rate estimates and how those estimates factor into your situation.

Is esthesioneuroblastoma fatal?

It can be. Esthesioneuroblastoma can spread into your lungs or other areas of your body. It can come back after treatment and develop in other areas. Metastatic or recurrent esthesioneuroblastoma that goes undetected could be life-threatening. That’s why regular checkups are important so your provider can do imaging and other tests that may detect cancer early on.

When should I see my healthcare provider?

Esthesioneuroblastoma can come back, so you should plan on seeing your care team at least once a year for the rest of your life. Your team will check on your overall health. They may do imaging tests to look for signs of recurring esthesioneuroblastoma.

What questions should I ask my healthcare provider?

Esthesioneuroblastoma is rare. You may not know much about it. Here are some questions you might want to ask your healthcare provider:

• How large is the tumor in my nasal cavity?
• Will I need surgery?
• What kind of surgery will I need?
• If I have surgery, will my sense of smell ever come back?
• What are alternatives for surgery?

A note from Cleveland Clinic

Esthesioneuroblastoma is a rare cancer that develops in your nose. It affects nerves and tissues that manage your sense of smell. It develops over time. Sometimes, people think its symptoms — stuffy noses, nosebleeds and losing your sense of smell — aren’t serious issues that they should discuss with a healthcare provider.

And yes, often a nosebleed is just a nosebleed. But if your nosebleed is constant, your stuffy nose never clears up and you think you’ve lost your sense of smell, talk to a healthcare provider. They evaluate your situation and recommend next steps.