Childhood Esthesioneuroblastoma (PDQ®)–Patient Version
What is esthesioneuroblastoma?
Childhood esthesioneuroblastoma is a rare type of cancer that forms 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.
Esthesioneuroblastoma affects adults and children. Even though it is very rare, esthesioneuroblastoma is the most common nasal cavity cancer in children and teens.
Causes and risk factors for childhood esthesioneuroblastoma
Childhood esthesioneuroblastoma is caused by certain changes to the way the cells in the olfactory nerve endings function, especially how they grow and divide into new cells. Often, the exact cause of these cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. There are no known risk factors for childhood esthesioneuroblastoma.
Symptoms of childhood esthesioneuroblastoma
Children may not have symptoms of esthesioneuroblastoma until the tumor has grown bigger. It's important to check with your child's doctor if your child has:
- a mass in the nose
- trouble breathing through the nose
- a change in or loss of the sense of smell
- nosebleeds
- a headache
- bulging of the eye
- trouble seeing
- eye pain
These symptoms may be caused by problems other than esthesioneuroblastoma. The only way to know is to see your child's doctor.
Tests to diagnose childhood esthesioneuroblastoma
If your child has symptoms that suggest esthesioneuroblastoma, their doctor will need to find out if these are due to cancer or to another condition. The doctor will ask when the symptoms started and how often your child has been having them. The doctor will also ask about your child's personal and family health history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with esthesioneuroblastoma, the results of these tests will help you and your child's doctor plan treatment.
Diagnostic tests
The following tests may be used to diagnose esthesioneuroblastoma:
Nasal endoscopy
Nasal endoscopy is 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 a pathologist checks under a microscope for signs of disease.
Magnetic resonance imaging (MRI)
MRI 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 MRI (NMRI). 
CT scan (CAT scan)
CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the head and neck or lymph nodes. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. 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. Learn more about Computed Tomography (CT) Scans and Cancer.
PET scan (positron emission tomography scan)
PET scan uses a small amount of radioactive sugar (radioactive glucose) that 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
Biopsy is a procedure in which a sample of tissue is removed from the tumor so that a pathologist can view it under a microscope to check for signs of cancer.
The following tests may be done on the sample of cells or tissues that were removed:
- Immunohistochemistry 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 is 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. Children with esthesioneuroblastoma may be eligible for genetic analysis and other types of molecular testing through the Molecular Characterization Initiative.
The Molecular Characterization Initiative offers free molecular testing to children, adolescents, and young adults with certain types of newly diagnosed cancer. The program is offered through NCI's Childhood Cancer Data Initiative. To learn more, visit About the Molecular Characterization Initiative.
- Electron microscopy is 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.
Tests to stage childhood esthesioneuroblastoma
If your child is diagnosed with esthesioneuroblastoma, they will be referred to a pediatric oncologist. This is a doctor who specializes in staging and treating esthesioneuroblastoma and other cancers. They will recommend tests to determine the extent (stage) of cancer. Sometimes the cancer is only in the olfactory nerve ending in the upper nasal cavity. Or, it may have spread from the nasal cavity to other parts of the body. The process of learning the extent of cancer in the body is called staging. It is important to know the stage of esthesioneuroblastoma to plan treatment.
Most children with esthesioneuroblastoma are at an advanced stage at the time of diagnosis. Esthesioneuroblastoma spreads most often to nearby tissue, including the olfactory bulb, nasal sinuses, or brain. It can also spread to the lymph nodes, lungs, liver, bone or bone marrow.
Learn more about a specific stage of esthesioneuroblastoma at Stages of childhood esthesioneuroblastoma.
The following tests may be used to determine the esthesioneuroblastoma stage:
Chest x-ray
An x-ray is a type of radiation that can go through the body and make pictures. A chest x-ray is one that makes pictures of the organs and bones inside the chest.
PET-CT scan
PET-CT scan combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. If there is any cancer, this increases the chance that it will be found.
- For the PET scan, a small amount of radioactive glucose (sugar) is injected into a vein. The 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.
- For the CT scan, a series of detailed pictures of areas inside the body, such as the chest or abdomen, is taken from different angles. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly.
Fine-needle aspiration (FNA) biopsy
Fine-needle aspiration biopsy uses a thin needle to remove tissue or fluid from an enlarged lymph node. A pathologist views the tissue or fluid under a microscope to look for cancer cells.
Getting a second opinion
You may want to get a second opinion to confirm your child's cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child's cancer.
To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI's Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child's appointments, see Questions to Ask Your Doctor About Cancer.
Stages of childhood esthesioneuroblastoma
The cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. It is important to know the stage of esthesioneuroblastoma to plan the best treatment.
There are several different staging systems for cancer. The Kadish staging system is used for esthesioneuroblastoma. Based on the diagnostic and staging test results, a Kadish stage is assigned to your child's esthesioneuroblastoma, such as stage A, stage B, stage C, or stage D. When talking with you about your child's cancer, your child's doctor may describe it as one of these stages.
Stage A esthesioneuroblastoma
In stage A, cancer is found in the nasal cavity only.
Stage B esthesioneuroblastoma
In stage B, cancer has spread from the nasal cavity to the nasal sinuses.
Stage C esthesioneuroblastoma
In stage C, cancer has spread through the nasal sinuses to nearby tissue.
Stage D esthesioneuroblastoma
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.
Types of treatment for childhood esthesioneuroblastoma
Who treats children with esthesioneuroblastoma?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of esthesioneuroblastoma. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
There are different types of treatment for children and adolescents with esthesioneuroblastoma. You and your child's care team will work together to decide treatment. Many factors will be considered, such as your child's overall health and whether the cancer is newly diagnosed or has come back.
Your child's treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child's care team before treatment begins about what to expect. For help every step of the way, see our downloadable booklet, Children with Cancer: A Guide for Parents.
Surgery
Surgery to remove the tumor is one of the main treatments for esthesioneuroblastoma.
After the doctor removes all the cancer that can be seen at the time of the surgery, your child may be given chemotherapy or radiation therapy to kill any cancer cells that are left. Treatment given after surgery to lower the risk that the cancer will come back is called adjuvant therapy.
Radiation therapy
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Esthesioneuroblastoma may be treated with external beam radiation therapy. This type of radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Radiation therapy may be given alone or with other treatments, such as chemotherapy.
Learn more about External Beam Radiation Therapy for Cancer and Radiation Therapy Side Effects.
Chemotherapy
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cells or stops them from dividing. Chemotherapy may be given alone or with other types of treatment, such as radiation therapy.
For esthesioneuroblastoma, the chemotherapy is injected into a vein. When given this way, the drugs enter the bloodstream and can reach cancer cells throughout the body.
Chemotherapy drugs used alone or in combination to treat esthesioneuroblastoma in children include:
Other chemotherapy drugs not listed here may also be used.
Learn more about Chemotherapy to Treat Cancer.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child's age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.
Treatment of childhood esthesioneuroblastoma
Treatment of newly diagnosed stage A esthesioneuroblastoma may include:
- surgery to remove the tumor followed by radiation therapy if visible tumor cells remain after surgery
Treatment of newly diagnosed stage B esthesioneuroblastoma may include:
- surgery to remove the tumor followed by radiation therapy
Treatment of newly diagnosed stage C esthesioneuroblastoma may include:
- chemotherapy followed by surgery to remove the tumor
- radiation therapy followed by surgery to remove the tumor
- chemotherapy and radiation therapy given at the same time followed by surgery to remove the tumor
Treatment of newly diagnosed stage D esthesioneuroblastoma may include:
- chemotherapy given before or after surgery to remove the tumor
- radiation therapy to the tumor and other places in the body where the cancer has spread
If the cancer comes back after treatment, your child's doctor will talk with you about what to expect and possible next steps. There might be treatment options that may shrink the cancer or control its growth. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
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.
Prognostic factors for esthesioneuroblastoma
If your child has been diagnosed with esthesioneuroblastoma, you likely have questions about how serious the cancer is and your child's chances of survival. The likely outcome or course of a disease is called prognosis. The prognosis can be affected by whether the cancer is only in the nose or if it has spread to the nasal sinuses, lymph nodes, or other parts of the body and whether the tumor was completely removed by surgery. No two people are alike, and responses to treatment can vary greatly. Your child's cancer care team is in the best position to talk with you about your child's prognosis.
Side effects and late effects of treatment
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child's treatment team about which side effects to look for and ways to manage them.
To learn more about side effects that begin during treatment for cancer, visit Side Effects.
Problems from cancer treatment that begin 6 months or later 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. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
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 the 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).
Coping with your child's cancer
When your child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child's treatment team and to people in your family and community for support. To learn more, see Support for Families When a Child Has Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources:
About This PDQ Summary
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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.
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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.
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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Esthesioneuroblastoma. 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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