Childhood Esthesioneuroblastoma Treatment (PDQ®): Treatment

This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.

General Information About Childhood Esthesioneuroblastoma

Esthesioneuroblastoma is a disease in which malignant (cancer) cells form in the tissues of the upper nasal cavity and base of the skull.

Esthesioneuroblastoma begins in the olfactory nerve endings in the upper part of the nasal cavity. The olfactory nerves (sense of smell) pass through the many tiny holes in the bone at the base of the brain to the olfactory bulb. Esthesioneuroblastoma is also called olfactory neuroblastoma. Drawing shows areas of the body where esthesioneuroblastoma tumors may form, including the olfactory nerve endings, olfactory bulb, nasal cavity, nasal sinuses, and brain.

Esthesioneuroblastomas form in the olfactory nerve endings in the upper part of the nasal cavity. The olfactory nerves (sense of smell) pass through the many tiny holes in the bone at the base of the brain to the olfactory bulb. Esthesioneuroblastomas may spread from the nasal cavity to the nasal sinuses or to nearby tissue. They may also spread to the brain or to other parts of the body (not shown).

Esthesioneuroblastoma affects adults and children. Even though it is very rare, esthesioneuroblastoma is the most common cancer of the nasal cavity in children and teens. This summary is about the treatment of children and teens with esthesioneuroblastoma.

Signs and symptoms of esthesioneuroblastoma include nosebleeds and the loss of sense of smell.

These and other signs and symptoms may be caused by esthesioneuroblastoma or by other conditions. Check with your child's doctor if your child has any of the following:

A mass in the nose.
Trouble breathing through the nose.
Change in or loss of the sense of smell.
Nosebleeds.
Headache.
Bulging of the eye.
Trouble seeing.
Eye pain.

Tests that examine the nasal cavity are used to help diagnose esthesioneuroblastoma.

In addition to asking about your child's personal and family health history and doing a physical exam, your child's doctor may perform the following tests and procedures.

Nasal endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. A flexible or rigid endoscope is inserted through the nose. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope by a pathologist for signs of disease.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the head and neck. This procedure is also called nuclear magnetic resonance imaging (NMRI).
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the head and neck or lymph nodes, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

Computed tomography (CT) scan of the head and neck. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.
PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
The following tests may be done on the sample of cells or tissues that were removed:
- Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient's tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
- Genetic analysis: A laboratory test in which cells or tissue are analyzed to look for changes in genes or chromosomes. These changes may be a sign that a person has or is at risk of having a specific disease or condition.
- Electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.

Certain factors affect prognosis (chance of recovery).

The prognosis depends on the following:

Whether the cancer is in the nose only or if it has spread to the nasal sinuses, lymph nodes, or to other parts of the body.
Whether the tumor was completely removed by surgery.

Stages of Esthesioneuroblastoma

After esthesioneuroblastoma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.

The process used to find out if cancer has spread to tissues near the nasal cavity or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. The results of the tests used to diagnose cancer are often also used to stage the disease. It is important to know the stage in order to plan treatment. For more information on the tests used to diagnose esthesioneuroblastoma, see the General Information section.

The following tests and procedures may be used to find out if cancer has spread:

Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
PET-CT scan: Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid from an enlarged lymph node, using a thin needle. A pathologist views the tissue or fluid under a microscope to look for cancer cells.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

Tissue. The cancer spreads from where it began by growing into nearby areas.
Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if esthesioneuroblastoma spreads to the lung, the cancer cells in the lung are actually esthesioneuroblastoma cells. The disease is metastatic esthesioneuroblastoma, not lung cancer.

The following stages are used for esthesioneuroblastoma:

Stage A

In stage A, cancer is found in the nasal cavity only.

Stage B

In stage B, cancer has spread from the nasal cavity to the nasal sinuses.

Stage C

In stage C, cancer has spread through the nasal sinuses to nearby tissue.

Stage D

In stage D, cancer has spread to distant parts of the body, such as the lymph nodes in the neck, or to the brain, bone marrow, or lung.

Sometimes childhood esthesioneuroblastoma comes back after treatment.

The cancer may recur (come back) in the original cancer site, central nervous system, or lymph nodes after it has been treated.

Treatment Option Overview

There are different types of treatment for children with esthesioneuroblastoma.

Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with esthesioneuroblastoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:

Pediatrician.
Pediatric surgeon.
Radiation oncologist.
Pathologist.
Pediatric nurse specialist.
Social worker.
Rehabilitation specialist.
Psychologist.
Child-life specialist.

Three types of standard treatment are used:

Surgery

Surgery to remove the tumor is one of the main treatments used for esthesioneuroblastoma.

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells.

Targeted therapy is being studied for the treatment of childhood esthesioneuroblastoma that has recurred (come back).

Treatment for childhood esthesioneuroblastoma may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

Physical problems.
Changes in mood, feelings, thinking, learning, or memory.
Second cancers (new types of cancer) or other conditions.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. For more information, see Late Effects of Treatment for Childhood Cancer.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back).