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This PDQ cancer information summary has current information about the treatment of neuroblastoma. 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.
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 ("Date Last Modified") 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.
CONTENTS
- General Information About Neuroblastoma
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Neuroblastoma is a type of cancer that forms in neuroblasts (immature nerve tissue) in the adrenal glands, neck, chest, or spinal cord.
Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. The adrenal glands make important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress.
Neuroblastoma may be found in the adrenal glands and paraspinal nerve tissue from the neck to the pelvis. Neuroblastoma most often begins in infancy. It is usually diagnosed between the first month of life and age 5 years. The tumor is found when it begins to grow and cause signs or symptoms. Sometimes it forms before birth and is found during an ultrasound of the baby.
By the time cancer is diagnosed, it has usually metastasized (spread). Neuroblastoma spreads most often to the lymph nodes, bones, bone marrow, liver, and skin in infants and children. Adolescents may also have metastasis to the lungs and brain.
Certain genetic conditions affect the risk of having neuroblastoma.
Neuroblastoma is caused by certain changes to the way neuroblast cells function, especially how they grow and divide into new cells. There are many risk factors for neuroblastoma, but many do not directly cause cancer. Instead, they increase the chance of DNA damage in cells that may lead to neuroblastoma. To learn more about how cancer develops, see What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Some risk factors can be changed. Risk factors also include things people cannot change, like family history. It's important to learn about risk factors for neuroblastoma because it can help you make choices for screening for cancer.
Possible risk factors for neuroblastoma include the following:
Genetic testing can determine whether a child has an inherited form of neuroblastoma.
Gene mutations that increase the risk of neuroblastoma are sometimes inherited (passed from the parent to the child). In children with a gene mutation, neuroblastoma usually occurs at a younger age and more than one tumor may form in the adrenal glands or in the nerve tissue in the neck, chest, abdomen, or pelvis.
It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can discuss a child's diagnosis and the family's medical history to understand:
Genetic counselors can also help parents cope with their child's genetic testing results, including how to discuss the results with family members.
Once it is known that the child has an inherited form of neuroblastoma, other family members can be screened for the ALK or PHOX2B mutation.
Sometimes children with certain gene mutations should be checked for signs of neuroblastoma.
Children with certain gene mutations or hereditary (inherited) syndromes should be checked for signs of neuroblastoma until they are 10 years old. The following tests may be used:
Talk to your child's doctor about how often these tests need to be done.
Signs and symptoms of neuroblastoma include a lump in the abdomen, neck, or chest or bone pain.
The most common signs and symptoms of neuroblastoma are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone.
Check with your child’s doctor if your child has any of the following:
Less common signs and symptoms of neuroblastoma include the following:
These and other signs and symptoms may be caused by neuroblastoma or by other conditions. The only way to know is to see your child's doctor. The doctor will ask you when the symptoms started and how often your child has been having them as a first step in making a diagnosis.
Tests that examine many different body tissues and fluids are used to diagnose neuroblastoma.
If your child has symptoms that suggest neuroblastoma, their doctor will need to find out if these are due to cancer or another condition. They will ask about your child's personal and family medical history and do a physical exam.
Depending on your child's symptoms and medical history and the results of their physical and neurological exam, the doctor may recommend more tests to find out if your child has neuroblastoma, and if so, its extent (stage). The results of the test and procedures done to diagnose neuroblastoma are used to help make decisions about treatment.
The following tests and procedures may be used:
Getting a second opinion.
Some people want to get a second opinion to confirm their child’s neuroblastoma diagnosis and treatment plan. If you choose to seek a second opinion, you will need to get important 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 before giving a recommendation. The doctor who gives the second opinion may agree with the first doctor, suggest changes or another approach, or provide more information about your child’s cancer.
To learn more about choosing a doctor and getting a second opinion, see Finding Health Care Services. 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 appointments, see Questions to Ask Your Doctor about Cancer.
Certain factors affect prognosis (chance of recovery) and treatment options.
If your child has been diagnosed with neuroblastoma, you may 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 and treatment options depend on the following:
Prognosis and treatment options for neuroblastoma are also affected by tumor histology, which includes the following:
The tumor histology is said to be favorable or unfavorable, depending on these factors. A child with favorable tumor histology has a better chance of recovery.
In some children up to age 6 months, neuroblastoma may disappear without treatment. This is called spontaneous regression. The child is closely watched for signs or symptoms of neuroblastoma. If signs or symptoms occur, treatment may be needed.
- Stages of Neuroblastoma
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After neuroblastoma has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body.
The process used to find out the extent or spread of cancer is called staging. The information gathered from the staging process helps determine the stage of the disease. For neuroblastoma, the stage of disease affects whether the cancer is low risk, intermediate risk, or high risk. It also affects the treatment plan. The results of some tests and procedures used to diagnose neuroblastoma may be used for staging. See the General Information section for a description of these tests and procedures.
The International Neuroblastoma Risk Group staging system (INRGSS)
Stage L1
In stage L1, the cancer is in only one area and there are no image-defined risk factors. Image-defined risk factors (IDRFs) are found on MRI or CT scans done during diagnosis. IDRFs are used to determine the risk of surgery and the chance of completely removing the tumor.
Stage L2
In stage L2, the cancer is in one area, has not spread beyond nearby tissue, and there are one or more image-defined risk factors.
Stage M
In stage M, neuroblastoma has spread to areas far from the tumor. This does not include stage MS.
Stage MS
In stage MS, children younger than 18 months have cancer that has spread to the skin, liver, and/or bone marrow.
Treatment of neuroblastoma is based on risk groups.
For many types of cancer, stages are used to plan treatment. For neuroblastoma, treatment depends on the patient's risk group. The risk group is determined by the following factors:
There are three risk groups: low risk, intermediate risk, and high risk.
For more information on the risk group classification, see the Revised International Neuroblastoma Response Criteria (INRC) section in Neuroblastoma Treatment (Health Professional Version).
Sometimes neuroblastoma does not respond to treatment or comes back after treatment.
Refractory neuroblastoma is a tumor that does not respond to treatment.
Recurrent neuroblastoma is cancer that has recurred (come back) after it has been treated. The tumor may come back in the site where it began or in the central nervous system.
- Treatment Option Overview
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There are different types of treatment for patients with neuroblastoma.
There are different types of treatment for children and adolescents with neuroblastoma. You and your child's cancer 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.
Children with neuroblastoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer, especially neuroblastoma.
A pediatric oncologist, a doctor who specializes in treating children with cancer, will oversee treatment. The pediatric oncologist works with other pediatric health care professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. These may include the following specialists and others:
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 cancer 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.
The following types of treatment may be used:
Observation
Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change.
Surgery
Surgery is used to treat neuroblastoma that has not spread to other parts of the body. As much of the tumor as is safely possible is removed. Lymph nodes are also removed and checked for signs of cancer.
If the tumor cannot be removed, a biopsy may be done instead.
Learn more about Surgery to Treat Cancer.
Chemotherapy
Chemotherapy (also called chemo) 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).
The use of two or more anticancer drugs is called combination chemotherapy.
See Drugs Approved for Neuroblastoma for more information.
Learn more about Chemotherapy to Treat Cancer.
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. There are two types of radiation therapy:
Learn more about Radiation Therapy to Treat Cancer.
High-dose chemotherapy and radiation therapy with stem cell rescue
High-dose chemotherapy and radiation therapy are given to kill any cancer cells that may regrow and cause the cancer to come back. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the completion of chemotherapy and radiation therapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
Iodine 131-MIBG therapy
Iodine 131-MIBG therapy is a treatment with radioactive iodine. The radioactive iodine is given through an intravenous (IV) line and enters the bloodstream which carries radiation directly to tumor cells. Radioactive iodine collects in neuroblastoma cells and kills them with the radiation that is given off. Iodine 131-MIBG therapy is sometimes used to treat high-risk neuroblastoma that comes back after initial treatment.
Targeted therapy
Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, or other molecules involved in the growth and spread of cancer cells.
Learn more about Targeted Therapy to Treat Cancer.
Other drug therapy
Other drugs used in combination to treat neuroblastoma include the following:
New types of treatment 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. For some patients, taking part in a clinical trial may be an option. Because cancer in children is rare, taking part in a clinical trial should be considered.
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. Some clinical trials are open only to patients who have not started treatment. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
To learn more, see Clinical Trials Information for Patients and Caregivers.
Immunotherapy
Immunotherapy helps a child's immune system fight cancer.
Learn more about T-cell Transfer Therapy.
Treatment for neuroblastoma causes side effects and late 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 effects cancer treatment can have on your child. To learn more, see Late Effects of Treatment for Childhood Cancer.
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.
Follow-up tests for patients with neuroblastoma include the following:
- Treatment of Low-Risk Neuroblastoma
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For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed low-risk neuroblastoma 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 Intermediate-Risk Neuroblastoma
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For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed intermediate-risk neuroblastoma 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 High-Risk Neuroblastoma
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For information about the treatments listed below, see the Treatment Option Overview section.
Children with newly diagnosed high-risk neuroblastoma usually receive treatment in three phases which includes induction, consolidation, and maintenance.
Treatment during induction may include:
Treatment during consolidation may include:
Treatment during maintenance may include:
Treatments under study may include:
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 Stage MS Neuroblastoma
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For information about the treatments listed below, see the Treatment Option Overview section.
There is no standard treatment for newly diagnosed stage MS but treatment options 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 Recurrent Neuroblastoma
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For information about the treatments listed below, see the Treatment Option Overview section.
Patients First Treated for Low-Risk Neuroblastoma
Treatment for recurrent neuroblastoma that comes back in the area where the cancer first formed may include the following:
Treatment for recurrent neuroblastoma that comes back in other parts of the body or that has not responded to treatment may include the following:
Patients First Treated for Intermediate-Risk Neuroblastoma
Treatment for recurrent neuroblastoma that comes back in the area where the cancer first formed may include the following:
Treatment for recurrent neuroblastoma that comes back in other parts of the body may include the following:
Patients First Treated for High-Risk Neuroblastoma
There is no standard treatment for recurrent neuroblastoma in patients first treated for high-risk neuroblastoma. Treatment may include the following:
Because there is no standard treatment, patients first treated for high-risk neuroblastoma may want to consider a clinical trial. For information about clinical trials, please see the NCI website.
Patients with Recurrent CNS Neuroblastoma
Treatment for neuroblastoma that recurs (comes back) in the central nervous system (CNS; brain and spinal cord) may include the following:
Treatments Being Studied for Progressive/Recurrent Neuroblastoma
Some of the treatments being studied in clinical trials for neuroblastoma that recurs (comes back) or progresses (grows, spreads, or does not respond to treatment) 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.
- To Learn More About Neuroblastoma
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For more information from the National Cancer Institute about neuroblastoma, see the following:
For more childhood cancer information and other general cancer resources, see the following:
- About This PDQ Summary
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About PDQ
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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Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of neuroblastoma. 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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PDQ® Pediatric Treatment Editorial Board. PDQ Neuroblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389278]
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