Neuroblastoma Treatment (PDQ�)
General Information About Neuroblastoma
Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord.
Neuroblastoma most often begins during early childhood, usually in children younger than 5 years. It sometimes forms before birth but is usually found later, when the
By the time neuroblastoma is
Neuroblastoma is sometimes caused by a gene mutation passed from the parent to the child.
Neuroblastoma is sometimes
Possible signs of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.
The most common symptoms of neuroblastoma are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone. These and other symptoms may be caused by neuroblastoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
Lump in the abdomen, neck, or chest.
Dark circles around the eyes ("black eyes").
stomachand trouble breathing in infants.
Painless, bluish lumps under the skin in infants.
paralysis(loss of ability to move a body part).
Less common signs of neuroblastoma include the following:
Shortness of breath.
Easy bruising or bleeding.
Petechiae(flat, pinpoint spots under the skin caused by bleeding). High blood pressure.
Jerky muscle movements.
Uncontrolled eye movement.
Swelling of the legs, ankles, feet, or
Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma.
The following tests and procedures may be used:
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.
urinetest: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organor tissue that makes it. A higher than normal amount of the substances homovanillic acid (HMA) and vanillyl mandelic acid (VMA) may be a sign of neuroblastoma. Blood chemistry studies: A procedure in which a bloodsample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of the hormones dopamine and norepinephrinemay be a sign of neuroblastoma. Cytogenetic analysis: A laboratory test in which cellsin a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. Bone marrow aspirationand biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologistviews the bone marrow, blood, and bone under a microscope to look for signs of cancer. Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. X-ray: 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, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dyemay be injectedinto a veinor swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person?s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. Ultrasoundexam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
Immunohistochemistry study: A laboratory test in which a substance such as an
antibody, dye, or radioisotopeis added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
scan: A procedure used to find neuroendocrinetumors, such as neuroblastomas and pheochromocytomas. A small amount of radioactivematerial called MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive material and are detected by a scanner. Scans may be taken over 1-3 days. An iodinesolution may be given before or during the test to prevent the thyroid glandfrom absorbing too much of the MIBG.
Certain factors affect prognosis (chance of recovery) and treatment options.
Age of the child when diagnosed.
Stageof the cancer.
Where the tumor is in the body.
histology(the shape, function, and structure of the tumor cells).
Whether there is cancer in the lymph nodes.
Prognosis and treatment decisions for neuroblastoma are also affected by tumor biology, which includes:
The patterns of the tumor cells.
How different the tumor cells are from normal cells.
How fast the tumor cells are growing.
The tumor biology is said to be favorable or unfavorable, depending on these factors. A favorable tumor biology means there is a better chance of recovery.
In some infants, neuroblastoma may disappear without treatment. The infant is closely watched for symptoms of neuroblastoma. If symptoms occur, treatment may be needed.
Stages of Neuroblastoma
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
Bone marrow aspirationand biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologistviews the bone marrow, blood, and bone under a microscope to look for signs of cancer. Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissueunder a microscope to look for cancer cells. One of the following types of biopsies may be done: Excisional biopsy: The removal of an entire lymph node. Incisional biopsy: The removal of part of a lymph node. Core biopsy: The removal of tissue from a lymph node using a wide needle. Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluidfrom a lymph node using a thin needle. CT scan(CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-raymachine. A dyemay be injectedinto a veinor swallowed to help the organsor tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. MRI(magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
X-rays of the chest, bones, and
abdomen: 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. Ultrasoundexam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. Radionuclide scan: A procedure to find areas in the body where cells, such as cancer cells, are dividing rapidly. A very small amount of radioactivematerial is swallowed or injected into a vein and travels through the bloodstream. The radioactive material collects in the bones or other tissues and is detected by a radiation-measuring device.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
tissue. Cancer invadesthe surrounding normal tissue.
lymph system. Cancer invades the lymph system and travels through the lymph vesselsto other places in the body.
blood. Cancer invades the veinsand capillariesand travels through the blood to other places in the body.
The following stages are used for neuroblastoma:
Stage 2A: The tumoris in only one area and all of the tumor that can be seen cannot be completely removed during surgery. Stage 2B: The tumoris in only one area and all of the tumor that can be seen may be completely removed during surgery. Cancer cellsare found in the lymph nodesnear the tumor.
tumorcannot be completely removed during surgeryand has spread from one side of the body to the other side and may also have spread to nearby lymph nodes; or
the tumor is in only one area, on one side of the body, but has spread to lymph nodes on the other side of the body; or
the tumor is in the middle of the body and has spread to
tissuesor lymph nodes on both sides of the body, and the tumor cannot be removed by surgery.
stage 4, the tumorhas spread to distant lymph nodes, the skin, or other parts of the body.
stage 4S, the following are true:
the child is younger than 1 year; and
cancerhas spread to the skin, liver, and/or bone marrow; and
tumoris in only one area and all of the tumor that can be seen may be completely removed during surgery; and/or
cellsmay be found in the lymph nodesnear the tumor.
Treatment of neuroblastoma is based on risk groups.
For many types of cancer, stages are used to plan treatment. For neuroblastoma, treatment depends on risk groups. The stage of neuroblastoma is one factor used to determine risk group. Other factors are the age of the child, tumor
There are 3 risk groups: low risk, intermediate risk, and high risk.
Low-risk and intermediate-risk neuroblastoma have a good chance of being
High-risk neuroblastoma may be difficult to cure.
Treatment Option Overview
There are different types of treatment for patients with neuroblastoma.
Different types of treatment are available for patients with
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 neuroblastoma should have their treatment planned by a team of doctors with expertise in treating childhood cancer.
Treatment will be overseen by a
Medical oncologist. Hematologist. Pediatric surgeon. Radiation oncologist. Endocrinologist. Neurologist. Neuropathologist. Neuroradiologist. Pediatric nurse specialist. Social worker. Rehabilitation specialist. Psychologist.
Children who are treated for neuroblastoma may be at higher risk for second cancers.
Some cancer treatments cause
Changes in mood, feelings, thinking, learning, or memory.
Second cancers(new types of cancer).
Some late effects may be treated or controlled. It is important that parents of children who are treated for neuroblastoma talk with their doctors about the possible late effects caused by some treatments. See the
Five types of standard treatment are used:
The use of two or more anticancer drugs is called
See Drugs Approved for Neuroblastoma for more information.
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 Web site.
Monoclonal antibody therapy is a cancer treatment that uses
High-dose chemotherapy and radiation therapy with stem cell transplant
Other drug therapy
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a
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
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from
Follow-up tests may be needed.
Some of the tests that were done to
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 condition has changed or if the cancer has
Treatment Options for Neuroblastoma
A link to a list of current
Treatment of low-risk
Watchful waitingalone for certain infants. Surgeryfollowed by watchful waiting.
Surgery followed by
combination chemotherapy, when serious health problems occur. Radiation therapyto treat tumorsthat are causing serious health problems and do not respondquickly to chemotherapy.
clinical trialof surgery followed by chemotherapy. Radiation therapy is given to treat tumors that are causing serious health problems and do not respond quickly to surgery and chemotherapy.
Treatment of intermediate-risk
Surgery followed by
chemotherapy. Sometimes a second surgery is needed.
Surgery followed by chemotherapy.
Radiation therapymay given to treat tumorsthat are causing serious health problems and do not respondquickly to surgery and chemotherapy.
Chemotherapy followed by surgery.
Radiation therapy to treat tumors that are causing serious problems and do not respond quickly to chemotherapy.
clinical trialof lower dosesof chemotherapy.
Treatment of high-risk
Combination chemotherapyfollowed by surgeryto remove as much of the tumoras possible, followed by high-dose chemotherapyand stem cell transplant. Radiation therapyto the tumor site and, sometimes, if needed, to other parts of the body with cancer. 13-cis retinoic acidafter recoveryfrom previous treatment.
clinical trialof new regimensof chemotherapyand radiation therapy with stem cell transplant.
A clinical trial of
monoclonal antibody therapy, biologic therapy, and 13-cis retinoic acid after chemotherapy.
A clinical trial of radiation therapy with
radioactive iodineand chemotherapy before stem cell transplant.
Patients with Recurrent CNS Neuroblastoma
Surgeryto remove the tumorfollowed by radiation therapy.
Radiation therapy to the brain and spinal cord.
Surgery, radiation therapy, and
chemotherapyfollowed by targeted therapywith a radioactivesubstance linked to a monoclonal antibody.
Patients First Treated for Low-Risk Neuroblastoma
Surgeryfollowed by watchful waitingor chemotherapy. High-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.
clinical trialof surgery and if needed, chemotherapy and another surgery.
Treatment for recurrent neuroblastoma that has spread to other parts of the body may include the following:
Surgery followed by chemotherapy.
High-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.
A clinical trial of a new treatment.
Patients First Treated for Intermediate-Risk Neuroblastoma
Surgery, with or without chemotherapy.
clinical trialof surgery and more courses of chemotherapy.
A clinical trial of a new treatment.
For recurrent neuroblastoma that has spread to other parts of the body, treatment is usually
Patients First Treated for High-Risk Neuroblastoma
A second course of
high-dose chemotherapyand stem cell transplant. Chemotherapy.
Because there is no
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with neuroblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
To Learn More About Neuroblastoma
For more information from the
Neuroblastoma Home Page
Drugs Approved for Neuroblastoma
Targeted Cancer Therapies
Understanding Cancer Series: Targeted Therapies (Advances in Targeted Therapies )
New Approaches to Neuroblastoma Therapy (NANT)
For more childhood
What You Need to Know About? Cancer
CureSearch for Children's Cancer
Late Effects of Treatment for Childhood Cancer
Adolescents and Young Adults with Cancer
Young People with Cancer: A Handbook for Parents
Care for Children and Adolescents with Cancer
Understanding Cancer Series: Cancer
Coping with Cancer: Supportive and Palliative Care
Information for Survivors/Caregivers/Advocates
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Changes to This Summary (10/13/2011)
Changes were made to this summary to match those made to the health professional version.
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