Overview

Esthesioneuroblastoma (es-thee-zee-o-noo-row-blas-TOE-muh) is a rare type of cancer that begins in the upper portion of the nasal cavity.

The area where esthesioneuroblastoma begins is separated from the brain by a bone that contains tiny holes that allow the nerves that control smell (olfactory nerves) to pass through. Esthesioneuroblastoma is also called olfactory neuroblastoma.

Esthesioneuroblastoma, which can occur at any age in adults, generally begins as a tumor in the nasal cavity and may grow or extend into the sinus, eyes and brain. People with esthesioneuroblastoma can lose their sense of smell, have frequent nosebleeds and experience difficulty breathing through their nostrils as the tumor grows.

Esthesioneuroblastoma can also spread to the lymph nodes in the neck and the parotid glands. In advanced cases, esthesioneuroblastoma can spread to other parts of the brain and other parts of the body, such as the lungs, liver and bones.

Esthesioneuroblastoma treatment usually includes surgery. Often, radiation and chemotherapy are recommended, as well.

Symptoms

Esthesioneuroblastoma symptoms include:

• Losing the sense of smell
• Frequent nosebleeds
• Difficulty breathing through the nose

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

When to see a doctor

Make an appointment with your doctor if you have any persistent signs or symptoms that concern you.

Causes

The exact cause of esthesioneuroblastoma has not been found. In general, cancer begins with a genetic mutation that allows normal, healthy cells to continue growing without responding to the signals to stop, which is unlike what normal cells do. Cancer cells grow and multiply out of control. The accumulating abnormal cells form a mass (tumor).

Complications

Complications of esthesioneuroblastoma may include:

• Cancer that grows to invade nearby structures. Advanced esthesioneuroblastoma can cause complications if it grows large enough to invade nearby structures, such as the eyes and brain.
• Spread of the cancer (metastasis). Esthesioneuroblastoma may spread (metastasize) to other parts of the body, such as the lymph nodes, bone marrow, lungs, liver, skin and bones.
• Loss of the sense of smell. The tumor can obstruct the nasal passage, which can block the flow of air through the nose.
• Complications from surgery. These complications can include spinal fluid leakage, infection, vision problems, and abscess.

Diagnosis

An esthesioneuroblastoma diagnosis may involve:

• Physical examination. A careful history of your signs and symptoms and an examination of your eyes, nose, and head and neck give doctors important information to understand the tumor's extent and aid in diagnosis.
• Using a tiny, flexible camera to see in your nose. During an endoscopic examination, a thin, flexible tube (endoscope) is inserted in your nose. The tube is attached to a camera that allows doctors to look at the extent of the tumor in the nose, back of the nose (nasopharynx) and in the sinus area of the nasal cavity.
• Imaging tests. Imaging tests such as MRI and positron emission tomography (PET) help to determine the location and extent of your esthesioneuroblastoma and help determine whether it has spread.
• Removing a sample of tissue for testing. A biopsy is a procedure to remove a sample of the tumor tissue for pathological analysis. A biopsy may be performed by inserting special instruments through your nose to remove a small piece of the cancer. This procedure can often be done in a doctor's office.
• Testing a tissue sample in a lab. Accurate testing and analysis of the biopsy sample is necessary to distinguish esthesioneuroblastoma from other tumors that may appear to look similar. Diagnosing esthesioneuroblastoma is difficult since it is very rare and can have a similar appearance to other cancers that occur in the head, neck or nasal areas. Pathological analysis also determines the aggressiveness (grade) of the tumor.

Treatment

Esthesioneuroblastoma treatment usually involves an operation to remove the cancer. Other treatments include radiation with high-powered energy beams and chemotherapy with powerful drugs.

Surgery

Surgical techniques vary, depending on the tumor's location, and generally include procedures performed by:

• Removing the nasal portion of the tumor. This is normally done as endoscopic surgery. The surgeon uses a long, thin tube (endoscope) equipped with a camera inserted through the nose to assess the cancer. Special surgical tools are passed through the endoscope to visualize the area and assist with the removal of the cancer and the surrounding tissue.
• Opening the skull to gain access to the tumor. A craniotomy is a procedure to remove a small portion of the skull in order to remove the tumor and separate it from the brain.

Treatment for esthesioneuroblastoma usually involves experts from multiple specialties, such as neurosurgeons, head and neck surgeons, radiation oncologists and medical oncologists.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays or protons, to kill cancer cells. People with esthesioneuroblastoma often undergo radiation therapy after surgery to kill any microscopic cancer cells that might remain in the head and neck.

Radiation therapy can also be used alone or in combination with chemotherapy if surgery isn't an option due to other health concerns or if the cancer is too advanced to be removed through an operation.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells. In people with esthesioneuroblastoma, chemotherapy is combined with radiation therapy after surgery to kill any cancer cells that may remain, especially for cancers that are very aggressive or extensive.

Alternative medicine

No alternative medicine treatments have been found to cure esthesioneuroblastoma. But complementary and alternative medicine therapies may help you cope with side effects of treatment when combined with your doctor's care.

Therapies that may be helpful during cancer treatment include:

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

Coping and support

An esthesioneuroblastoma diagnosis can be overwhelming. And just when you're trying to cope with the shock and the fears about your future, you're asked to make important decisions about your treatment.

Every person finds his or her own way of coping with a cancer diagnosis. Until you find what works for you, it might help to:

• Learn enough about your cancer to make decisions about your care. If you'd like to know more about your esthesioneuroblastoma, ask your doctor for the details of your cancer — the type and grade. Ask for good sources of up-to-date information on your treatment options.

  Knowing more about your cancer and your options may help you feel more confident when making treatment decisions.

• Talk with other cancer survivors. You may find it helpful and encouraging to talk to others in your same situation. Contact the American Cancer Society or the National Cancer Institute to find out about support groups in your area and online.
• Find someone to talk with about your feelings. Find a friend or family member who is a good listener, or talk with a clergy member or counselor. Ask your doctor for a referral to a counselor or other professional who works with cancer survivors.

• Keep your friends and family close. Your friends and family can provide a crucial support network for you during your cancer treatment.

  As you begin telling people about your esthesioneuroblastoma diagnosis, you'll likely get many offers for help. Think ahead about things you may want assistance with, for example, having someone to talk to if you're feeling low or getting help preparing meals.

Preparing for an appointment

Start by making an appointment with your doctor if you have any signs or 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

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you are experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, vitamins or supplements you are taking.
• Take a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time. List your questions from most important to least important in case time runs out. For esthesioneuroblastoma, some basic questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• What are other possible causes for my symptoms or condition?
• What kinds of tests do I need?
• Is my condition likely temporary or chronic?
• What is the best course of action?
• What are the alternatives to the primary approach that you're suggesting?
• I have these other health conditions. How can they best be managed together?
• Are there any restrictions that I need to follow?
• Should I see a specialist? What will that cost, and will my insurance cover it?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions anytime you don't understand something.

What to expect from your doctor

Your doctor is likely to ask you several questions, such as:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?