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This PDQ cancer information summary has current information about the treatment of childhood pheochromocytoma and paraganglioma. 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 Childhood Pheochromocytoma and Paraganglioma
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Pheochromocytoma forms in the adrenal gland.
Pheochromocytoma forms in the adrenal gland. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Pheochromocytoma is a tumor of the adrenal medulla.
Anatomy of the adrenal gland. There are two adrenal glands, one on top of each kidney. The outer part of each gland is the adrenal cortex and the inner part is the adrenal medulla. The adrenal glands make important hormones called catecholamines. Adrenaline (epinephrine) and noradrenaline (norepinephrine) are two types of catecholamines that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Some pheochromocytomas release extra adrenaline and noradrenaline into the blood and cause symptoms.
Paraganglioma forms in nerve tissue near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body.
Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of extra adrenaline and noradrenaline into the blood may cause symptoms.
A paraganglioma is a rare tumor that often forms in nerve tissue near the carotid artery. It may also form along nerve pathways in the head and neck and in other parts of the body. Pheochromocytoma and paraganglioma may be benign (not cancer) or malignant (cancer).
About half of all children with pheochromocytoma or paraganglioma have malignant pheochromocytoma or paraganglioma. This means that the tumor cells have spread to other parts of the body. Benign and malignant pheochromocytoma and paraganglioma require treatment because they can cause severe or life-threatening heart problems and affect many body functions.
Inheriting certain gene mutations (changes) increases the risk of pheochromocytoma and paraganglioma.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.
Having any of the following inherited syndromes or gene changes increases risk of pheochromocytoma and paraganglioma:
More than half of the children and adolescents diagnosed with pheochromocytoma or paraganglioma have an inherited syndrome or gene change that increases the risk of cancer. Genetic counseling (a discussion with a trained professional about inherited diseases) and testing is an important part of the treatment plan.
Signs and symptoms of pheochromocytoma and paraganglioma occur if too much adrenaline or noradrenaline is released into the blood.
Some tumors do not make extra adrenaline or noradrenaline and do not cause symptoms. These tumors may be found when a lump forms in the neck or when a test is done for another reason. Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma, paraganglioma, or other conditions.
Check with your child's doctor if your child has any of the following:
These signs and symptoms may come and go, but high blood pressure is more likely to occur for long periods of time in young patients. These signs and symptoms may also occur with physical activity, injury, anesthesia, surgery to remove the tumor, eating foods such as chocolate and cheese, or while passing urine (if the tumor is in the bladder).
Tests and procedures used to diagnose and stage pheochromocytoma and paraganglioma depend on the patient's signs and symptoms and family history.
Tests are done to diagnose and stage cancer. After cancer is diagnosed, more tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body. This process is called staging.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery) and treatment options.
Prognosis and treatment options depend on the following:
- Stages of Childhood Pheochromocytoma and Paraganglioma
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After pheochromocytoma or paraganglioma 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 nearby areas or to other parts of the body is called staging. There is no standard staging system for childhood pheochromocytoma and paraganglioma. The results of the tests and procedures done to diagnose cancer are used to help make decisions about treatment.
Sometimes childhood pheochromocytoma or paraganglioma recurs (comes back) after treatment. It may come back in the place it first formed or in other parts of the body.
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 pheochromocytoma spreads to the bone, the cancer cells in the bone are actually pheochromocytoma cells. The disease is metastatic pheochromocytoma, not bone cancer.
- Treatment Option Overview
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There are different types of treatment for children with pheochromocytoma or paraganglioma.
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 pheochromocytoma or paraganglioma 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:
Four types of standard treatment are used:
Surgery
Surgery to remove the tumor is the main treatment for pheochromocytoma and paraganglioma. For several days before surgery, your child may need to take blood pressure medicine to lower the risk of complications during and after surgery.
Chemotherapy
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).
High-dose 131I-MIBG therapy
131I-MIBG therapy is a treatment with high-dose 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 pheochromocytoma and paraganglioma cells and kills them with the radiation that is given off.
Targeted therapy
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.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Treatment of pheochromocytoma and paraganglioma may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page.
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 patients with pheochromocytoma or paraganglioma that causes symptoms, catecholamine levels in the blood and urine will be checked on a regular basis. Catecholamine levels that are higher than normal can be a sign that the cancer has come back. Talk to your child’s doctor about which tests should be done and how often.
Patients with pheochromocytoma or paraganglioma need lifelong follow-up.
- Treatment of Childhood Pheochromocytoma and Paraganglioma
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For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed pheochromocytoma and paraganglioma in children may include the following:
Before surgery, drug therapy with alpha-blockers to control blood pressure and beta-blockers to control heart rate are given. If both adrenal glands are removed, life-long hormone therapy to replace hormones made by the adrenal glands is needed after surgery.
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 Childhood Pheochromocytoma and Paraganglioma
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For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of recurrent pheochromocytoma and paraganglioma 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.
- To Learn More About Childhood Pheochromocytoma and Paraganglioma
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For more information from the National Cancer Institute about childhood pheochromocytoma and paraganglioma, 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.
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 pheochromocytoma and paraganglioma. 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 Childhood Pheochromocytoma and Paraganglioma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pheochromocytoma/patient/child-pheochromocytoma-treatment-pdq. Accessed <MM/DD/YYYY>.
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