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Home > Living Well > Health Library > Childhood Esthesioneuroblastoma Treatment (PDQ®): Treatment - Patient Information [NCI]
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.
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.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 tumor 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:
Tests that examine the nasal cavity are used to help diagnose esthesioneuroblastoma.
The following tests and procedures may be used:
The following tests may be done on the sample of cells or tissues that were removed:
Certain factors affect prognosis (chance of recovery).
The prognosis depends on the following:
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. 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:
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
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.
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:
In stage A, cancer is found in the nasal cavity only.
In stage B, cancer has spread from the nasal cavity to the nasal sinuses.
In stage C, cancer has spread through the nasal sinuses to nearby tissue.
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.
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:
Three types of standard treatment are used:
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 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 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 is a type of treatment that uses drugs or other substances to identify and attack specific 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.
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:
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 for more information.
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.
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed 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.
Treatment of newly diagnosed stage B esthesioneuroblastoma may include the following:
It is not clear whether giving chemotherapy after surgery improves the child's prognosis.
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed stage C esthesioneuroblastoma may include the following:
Treatment of newly diagnosed stage D esthesioneuroblastoma may include the following:
Treatment of recurrent esthesioneuroblastoma may include the following:
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:
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government's center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood esthesioneuroblastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
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The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Esthesioneuroblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/patient/child/esthesioneuroblastoma-treatment-pdq. Accessed <MM/DD/YYYY>.
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Last Revised: 2022-01-21
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