Esthesioneuroblastoma

Esthesioneuroblastoma (es-thee-zee-o-noo-row-blas-TOE-muh) is a rare type of cancer that starts in the upper part of the inside of the nose, known as the nasal cavity. Esthesioneuroblastoma also is called olfactory neuroblastoma.

This cancer usually affects adults in their 50s and 60s. But it can occur at any age. Esthesioneuroblastoma usually begins as a growth of cells, called a tumor, inside the nose. It might grow and go into the sinuses, eyes and brain. It also can spread to other parts of the body.

People with esthesioneuroblastoma can lose their sense of smell. They might get nosebleeds. And they might have trouble breathing through their nose as the tumor grows.

Esthesioneuroblastoma treatment usually includes surgery. Often, radiation and chemotherapy are part of the treatment as well.

Esthesioneuroblastoma symptoms include:

• Loss of the sense of smell.
• Frequent nosebleeds.
• Difficulty breathing through the nose.

As the cancer grows, it might cause eye pain, loss of vision, ear pain and headaches.

When to see a doctor

Make an appointment with your health care team if you have lasting symptoms that worry you.

Experts haven't found the exact cause of esthesioneuroblastoma. In general, cancer happens when cells get changes in their DNA. A cell's DNA holds the instructions that tell a cell what to do. The changes tell the cells to make many more cells quickly. The changes give the cells the ability to keep living when healthy cells would naturally die. This causes too many cells.

The cells might form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cells can break away and spread to other parts of the body.

Complications of esthesioneuroblastoma may include:

• Cancer that grows into nearby organs and tissues. Esthesioneuroblastoma can grow and get into the sinuses, eyes and brain.
• Spread of the cancer, known as metastasis. Esthesioneuroblastoma can spread to other parts of the body, such as the lymph nodes, bone marrow, lungs, liver, skin and bones.

An esthesioneuroblastoma diagnosis might involve:

• Physical exam. A member of your health care team may take a history of your symptoms and look at your eyes, nose, and head and neck.
• Endoscopic exam. A doctor may put a thin, flexible tube, known as an endoscope, into the nose. The tube has a camera attached that allows the doctor to look at the cancer and see how large it is.
• Imaging tests. Imaging tests take pictures of the inside of the body. They can show the size of the cancer, exactly where it is and whether it has spread. Imaging tests might include magnetic resonance imaging (MRI) scans, computed tomography (CT) scans and positron emission tomography (PET) scans.
• Removing a sample of tissue for testing, also called a biopsy. A biopsy is a procedure to remove a sample of tissue for testing in a lab. A biopsy might be done by putting a special tool into the nose to remove a small piece of the cancer. This procedure can often be done in the doctor's office.

Diagnosing esthesioneuroblastoma can be hard. It's rare, and it can look like other cancers that occur in the head, neck or nose. Testing can show if the cancer is esthesioneuroblastoma and it can give other information about the cancer that will help in making a treatment plan.

Esthesioneuroblastoma treatment usually involves surgery to remove the cancer. Other treatments include radiation and chemotherapy.

Treatment for esthesioneuroblastoma usually involves a team of experts with different specialties. The team might include:

• Surgeons who operate on the nervous system, known as neurosurgeons.
• Head and neck surgeons.
• Doctors who use radiation to treat cancer, known as a radiation oncologists.
• Doctors who use medicine to treat cancer, known as medical oncologists.

If the person with esthesioneuroblastoma is a child, the team also might include specialists in pediatric surgery and oncology.

Surgery

The type of surgery depends on where the tumor is and how large it is. Surgery might involve:

• Removing the part of the tumor that's in the nose. This is usually done using a thin, flexible tube, known as an endoscope. The tube has a camera that lets the surgeon see the cancer. Special surgical tools passed through the endoscope help with removing the cancer and nearby tissue.
• Opening the skull to get to the tumor, known as a craniotomy. This procedure involves removing a small piece of skull. That allows the surgeon to remove the tumor from the brain.

Surgery complications might include spinal fluid leaking into the nose, infection and vision problems.

Radiation therapy

Radiation therapy uses powerful energy beams to kill cancer cells. The energy can come from X-rays, protons or other sources. People with esthesioneuroblastoma often have radiation therapy after surgery to kill any cancer cells that might remain in the head and neck.

If surgery isn't possible, radiation therapy can be used alone or with chemotherapy.

Chemotherapy

Chemotherapy uses strong medicines to kill cancer cells. In people with esthesioneuroblastoma, chemotherapy might be used with radiation therapy after surgery to kill cancer cells that remain.

No alternative medicine treatments can cure esthesioneuroblastoma. But complementary and alternative medicine therapies may help with the side effects of treatment. Talk with your health care team about your options.

Therapies that might help during cancer treatment include:

• Acupuncture.
• Aromatherapy.
• Hypnosis.
• Massage.
• Music therapy.
• Relaxation techniques.
• Tai chi.
• Yoga.

An esthesioneuroblastoma diagnosis can feel scary. With time, you'll find good ways to cope with your diagnosis. Until you find what works for you, consider trying to:

• Learn enough about your cancer to make decisions about your care. To know more about your esthesioneuroblastoma, ask your health care provider for the details, such as the type and grade. Ask where to find good sources of information about treatments. Knowing more might help you feel better about making treatment decisions.
• Talk with others who have cancer. Talking with others going through what you're going through might help. Contact the American Cancer Society or the National Cancer Institute to find out about support groups in your area and online.
• Talk to someone about your feelings. Find a friend or family member who listens well. Or talk with a clergy member or counselor. Ask your health care team to refer you to a counselor or other professional who works with cancer survivors.

• Keep your friends and family close. Your friends and family can provide much-needed support during your cancer treatment.

  When you tell people about your esthesioneuroblastoma diagnosis, you'll likely get many offers for help. Think about what you might want help with. For example, you might want someone to talk to if you're feeling low. Or you might need rides to treatments or help with making meals.

Start by making an appointment with your primary care provider if you have symptoms that worry you. You might be referred to an ear, nose and throat specialist.

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do before you go, such as restrict your diet. Also, if possible, take a family member or friend with you to help you remember the information you get.

Make a list of:

• Your symptoms and when they began.
• Key personal information, including any major stresses or recent life changes.
• All medicines, vitamins and supplements you take, including doses.
• Questions to ask your health care provider.

For esthesioneuroblastoma, some questions to ask might include:

• What is likely causing my symptoms?
• What are other possible causes?
• What tests do I need?
• Is my condition likely to go away or be long lasting?
• What is the best course of action?
• What else might I do?
• I have other health conditions. How can I manage them together?
• Are there brochures or other printed material that I can have? What websites do you recommend?

Be sure to ask all the questions you have.

What to expect from your doctor

Your health care provider is likely to ask you several questions, such as:

• Have your symptoms come and gone or stayed steady?
• How bad are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?