Childhood Esthesioneuroblastoma Treatment (PDQ®)

Last modified: 2018-04-10
Last downloaded: 2018-06-23

   

General Information

   

Esthesioneuroblastoma is a disease in which malignant (cancer) cells form in the tissues of the olfactory bulb.

The olfactory bulbs are found in the top part of the nasal cavity near the bone that separates the nasal cavity from the brain. The olfactory bulbs connect to the olfactorynerves (nerves that control smell), which pass through small holes in the bone and go to the brain. Esthesioneuroblastoma is also called olfactory neuroblastoma.

Esthesioneuroblastoma affects adults and children. Even though it is very rare, esthesioneuroblastoma is the most common tumor of the nasal cavity in children, especially 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:

  • Change in or loss of the sense of smell.
  • Blocked nose.
  • Nosebleeds.
  • Headache.
  • Bulging of the eye.
  • Frequent sinusinfections.
   

Tests that examine the nasal cavity are used to help detect (find) and diagnose esthesioneuroblastoma.

The following tests and procedures may be used:

Computed tomography (CT) scan of the head and neck. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the head and neck.

Computed tomography (CT) scan of the head and neck. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the head and neck.

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • 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 x-ray pictures of the inside of the head and neck.
  • 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 in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test is used to check for certain changes in the cells and to tell the difference between different types of cancer.
    • 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 (chance of recovery) depends on the following:

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

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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 olfactory bulb or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section.)

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

Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

  • 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.
  • 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 malignanttumorcells 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.
  • 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.
  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the abdomen, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
  • 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 nasalsinuses.

Stage C 

In stage C, cancer has spread through the nasalsinuses 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 bone, liver, or lung.


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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:

   

Children and adolescents may have treatment-related side effects that appear months or years after treatment for esthesioneuroblastoma.

Regular follow-up exams are very important. Treatment can cause side effects long after it has ended. These are called late effects.

Late effects of cancer treatment may include:

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. See the PDQ summary on Late Effects of Treatment for Childhood Cancer.

   

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 after surgery 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 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 attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

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

   

Treatment for childhood esthesioneuroblastoma may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.

   

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.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order 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). These tests are sometimes called follow-up tests or check-ups.


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Treatment Options for Childhood Esthesioneuroblastoma

For information about the treatments listed below, see the Treatment Option Overview section.

Stage A Esthesioneuroblastoma

Treatment of stage A esthesioneuroblastoma may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Stage B Esthesioneuroblastoma

Treatment of stage B esthesioneuroblastoma may include the following:

It is not clear if giving chemotherapy after surgery improves the child’s prognosis.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Stage C Esthesioneuroblastoma

Treatment of stage C esthesioneuroblastoma may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Stage D Esthesioneuroblastoma

Treatment of stage D esthesioneuroblastoma may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.


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Treatment Options for Recurrent Childhood Esthesioneuroblastoma

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of recurrentesthesioneuroblastoma may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.


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To Learn More About Esthesioneuroblastoma

For more information from the National Cancer Institute about esthesioneuroblastoma, see the following:

For more childhood cancer information and other general cancer resources, see the following:


Back to Top Source: The National Cancer Institute's Physician Data Query (PDQ®) Cancer Information Summaries ( http://www.cancer.gov/cancertopics/pdq)