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\nNeuroblastoma 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.
\n \nNeuroblastoma 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.
\nBy 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.
\nNeuroblastoma 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?
\nA 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.
\nPossible risk factors for neuroblastoma include:
\nGene 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.
\nIt 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:
\nGenetic counselors can also help parents cope with their child's genetic testing results, including how to discuss the results with family members.
\nOnce it is known that the child has an inherited form of neuroblastoma, other family members can be screened for the ALK or PHOX2B mutation.
\nChildren 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:
\nTalk to your child's doctor about how often these tests need to be done.
\nThe 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.
\nCheck with your child's doctor if your child has any of the following:
\nLess common signs and symptoms of neuroblastoma include the following:
\nThese 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.
\nIf 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.
\nDepending 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). If neuroblastoma is diagnosed, the results of these tests and procedures will help you and your child's doctor make decisions about treatment.
\nThe following tests and procedures may be used:
\nChildren up to age 6 months may not need a biopsy or surgery to remove the tumor because the tumor may disappear without treatment.
Children with newly diagnosed high-risk neuroblastoma may be eligible for 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.
To check for these biomarkers, samples of tissue containing neuroblastoma cells are removed during a biopsy or surgery and tested in a laboratory.
Neuroblastoma biomarker testing includes:
\nSome people want to get a second opinion to confirm their child\u2019s 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\u2019s cancer.
\nTo 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 appointments, see Questions to Ask Your Doctor about Cancer.
\nIf 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.
\nThe prognosis and treatment options depend on:
\nPrognosis and treatment options for neuroblastoma are also affected by tumor histology, which includes:
\nThe 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.
\nIn 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.
\nThe 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.
\nIn stage L1, the cancer is in only one area, and there are no image-defined risk factors (IDRFs). 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.
\nIn stage L2, the cancer is in one area, has not spread beyond nearby tissue, and there are one or more IDRFs.
\nIn stage M, neuroblastoma has spread to areas far from the tumor. This does not include stage MS.
\nIn stage MS, children younger than 18 months have cancer that has spread to the skin, liver, and/or bone marrow.
\nFor 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:
\nThere are three risk groups: low risk, intermediate risk, and high risk.
\nFor more information on the risk group classification, see the Revised International Neuroblastoma Response Criteria (INRC) section in Neuroblastoma Treatment (Health Professional Version).
\nRefractory neuroblastoma is a tumor that does not respond to treatment.
\nRecurrent 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.
\nThere 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.
\nA 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:
\nYour 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.
\nObservation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change.
\nSurgery 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.
\nIf the tumor cannot be removed, a biopsy may be done instead.
\nLearn more about Surgery to Treat Cancer.
\nChemotherapy (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).
\nThe use of two or more anticancer drugs is called combination chemotherapy.
\nSee Drugs Approved for Neuroblastoma for more information.
\nLearn more about Chemotherapy to Treat Cancer.
\nRadiation 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 different types of radiation therapy:
\nLearn more about Radiation Therapy to Treat Cancer.
\nHigh-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.
\nIodine 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.
\nTargeted 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.
\nLearn more about Targeted Therapy to Treat Cancer.
\nOther drugs used in combination to treat neuroblastoma include:
\nA 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.
\nUse 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.
\nTo learn more, see Clinical Trials Information for Patients and Caregivers.
\nImmunotherapy helps a child's immune system fight cancer.
\nCAR T-cell therapy is being studied to treat neuroblastoma that has come back after treatment or has not responded to treatment.
Learn more about T-cell Transfer Therapy.
\nTo learn more about side effects that begin during treatment for cancer, visit Side Effects.
\nSide 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:
\nSome 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.
\nAs your child goes through treatment, they will have follow-up tests or checkups. Some tests that were done to diagnose or stage 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. These tests are sometimes called follow-up tests or check-ups.
\nSome 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).
\nFollow-up tests for patients with neuroblastoma include:
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nTreatment of newly diagnosed low-risk neuroblastoma may include:
\nUse 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.
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nTreatment of newly diagnosed intermediate-risk neuroblastoma may include:
\nUse 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.
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nChildren with newly diagnosed high-risk neuroblastoma usually receive treatment in three phases: induction, consolidation, and maintenance.
\nTreatment during induction may include:
\nTreatment during consolidation may include:
\nTreatment during maintenance may include:
\nTreatments being studied may include:
\nUse 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.
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nThere is no standard treatment for newly diagnosed stage MS, but treatment options include:
\nUse 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.
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nPatients First Treated for Low-Risk Neuroblastoma
\nTreatment for recurrent neuroblastoma that comes back in the area where the cancer first formed may include:
\nTreatment for recurrent neuroblastoma that comes back in other parts of the body or that has not responded to treatment may include:
\nPatients First Treated for Intermediate-Risk Neuroblastoma
\nTreatment for recurrent neuroblastoma that comes back in the area where the cancer first formed may include:
\nTreatment for recurrent neuroblastoma that comes back in other parts of the body may include:
\nPatients First Treated for High-Risk Neuroblastoma
\nThere is no standard treatment for recurrent neuroblastoma in patients first treated for high-risk neuroblastoma. Treatment may include:
\nBecause 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.
\nPatients with Recurrent CNS Neuroblastoma
\nTreatment for neuroblastoma that recurs (comes back) in the central nervous system (CNS; brain and spinal cord) may include:
\nTreatments Being Studied for Progressive/Recurrent Neuroblastoma
\nSome 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:
\nUse 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.
\nFor more information from the National Cancer Institute about neuroblastoma, see the following:
\nFor more childhood cancer information and other general cancer resources, visit:
\nPhysician 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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\nThis 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.
\nEditorial 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.
\nThe 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.
\nA 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.
\nClinical 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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\nThe best way to cite this PDQ summary is:
\nPDQ\u00ae 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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\nUpdated:
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