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This PDQ cancer information summary has current information about the treatment of childhood multiple endocrine neoplasia (MEN) syndromes. 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 Multiple Endocrine Neoplasia (MEN) Syndromes
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Multiple endocrine neoplasia (MEN) syndromes are inherited disorders that affect the endocrine system.
The endocrine system is made up of glands and cells that make hormones and release them into the blood. MEN syndromes may cause hyperplasia (the growth of too many normal cells) or tumors that may be benign (not cancer) or malignant (cancer).
There are several types of MEN syndromes and each type may cause different conditions or cancers.
The two main types of MEN syndromes are MEN1 and MEN2. MEN2 syndrome has two subgroups: MEN2A syndrome and MEN2B syndrome. MEN2A syndrome includes familial medullary thyroid cancer.
Multiple endocrine neoplasia (MEN) syndromes are rare, inherited disorders that affect the endocrine glands or organs. There are several types of MEN syndromes, and each type may cause different conditions or cancers. The two main types of MEN syndromes are MEN1 and MEN2. MEN1 syndrome usually causes tumors in the pituitary gland, parathyroid gland, or pancreas. MEN2 syndrome usually causes tumors in the thyroid gland, parathyroid gland, or adrenal gland. The tumors may be benign (not cancer) or malignant (cancer). MEN1 syndrome usually causes tumors in the parathyroid gland, pituitary gland, or islet cells of the pancreas.
A diagnosis of MEN1 syndrome is made when tumors are found in two of the following glands or organs: parathyroid gland, pituitary gland, or islet cells in the pancreas. These tumors may make extra hormones and cause certain signs or symptoms of disease. The signs and symptoms depend on the type of hormone made by the tumor. MEN1 syndrome is also called Wermer syndrome.
The prognosis (chance of recovery) is usually good.
The most common condition associated with MEN1 syndrome is hyperparathyroidism. Signs and symptoms of hyperparathyroidism (too much parathyroid hormone) include the following:
Other conditions associated with MEN1 syndrome and their common signs and symptoms are:
Children with primary hyperparathyroidism, tumors associated with MEN1 syndrome, or a family history of hypercalcemia or MEN1 syndrome may have genetic testing to check for a mutation (change) in the MEN1 gene. Parents should receive genetic counseling (a discussion with a trained professional about the risk of genetic diseases) before genetic testing is done. Genetic counseling also includes a discussion of the risk of MEN1 syndrome for the child and other family members.
Children who are diagnosed with MEN1 syndrome are checked for signs of cancer starting at age 5 years and continuing for the rest of their life. Talk to your child's doctor about the tests and procedures needed to check for signs of cancer and how often they should be done.
MEN2A syndrome may cause medullary thyroid cancer, pheochromocytoma, or parathyroid gland disease.
MEN2A syndrome is also called Sipple syndrome. A diagnosis of MEN2A syndrome may be made when the patient or the patient's parents, brothers, sisters, or children have two or more of the following conditions:
The prognosis is usually good.
A mutation in the RET gene is usually linked to medullary thyroid cancer in MEN2 syndrome. If a diagnosis of MEN2 syndrome is suspected for the child or a family member is diagnosed with MEN2 syndrome, the parents should receive genetic counseling before genetic testing is done for the child. Genetic counseling also includes a discussion of the risk of MEN2 syndrome for the child and other family members.
MEN2B syndrome causes several conditions.
Patients with MEN2B syndrome may have a slender body build with long, thin arms and legs. The lips may appear large and bumpy because of benign tumors in the mucous membranes. MEN2B syndrome may cause the following conditions:
The prognosis for MEN2B syndrome is not as good as the prognosis for MEN1 syndrome and MEN2A syndrome because medullary thyroid cancer is a more aggressive cancer.
Children with MEN2A syndrome, MEN2B syndrome, or FMTC may need genetic testing.
A mutation in the RET gene is usually linked to medullary thyroid cancer in MEN2 syndrome. If a diagnosis of MEN2 syndrome is suspected for the child or a family member is diagnosed with MEN2 syndrome, the parents should receive genetic counseling before genetic testing is done for the child. Genetic counseling also includes a discussion of the risk of MEN2 syndrome for the child and other family members.
Tests used to diagnose and stage cancers related to MEN syndromes depend on the signs and symptoms and the patient's 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:
If cancer, such as medullary thyroid cancer, has formed, 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 system for staging childhood cancers linked to MEN syndromes. The results of the tests and procedures done to diagnose cancer are used to help make decisions about treatment.
Sometimes childhood MEN syndromes recur (come back) after treatment.
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 medullary thyroid cancer spreads to the lung, the cancer cells in the lung are actually medullary thyroid cells. The disease is metastatic medullary thyroid cancer, not lung cancer.
- Treatment Option Overview
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There are different types of treatment for children with MEN syndromes.
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 MEN syndromes 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:
The treatment will depend on the type of MEN syndrome the child has.
See the treatment section of this summary for information on the treatment of MEN1 syndrome, MEN2A syndrome, and MEN2B syndrome.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Treatment for childhood MEN syndrome 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.
- Treatment of Childhood MEN1 Syndrome
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Hyperparathyroidism is the most common sign of MEN1 syndrome. Children with MEN1 syndrome and primary hyperparathyroidism may have surgery to remove at least three parathyroid glands and the thymus. Treatment is also given for pancreatic islet cell and pituitary tumors, and other conditions linked to MEN1 syndrome as needed.
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 Childhood MEN2A Syndrome
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Medullary thyroid cancer is linked to MEN2A syndrome. Children with MEN2A syndrome and certain changes in the RET gene usually have surgery to remove the thyroid before the child is 5 years old to diagnose cancer or to lessen the chance cancer will form or spread. Treatment is also given for pheochromocytoma and hyperparathyroidism as needed.
Children with MEN2A syndrome who have medullary thyroid cancer may be treated with 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.
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 Childhood MEN2B Syndrome
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Medullary thyroid cancer, an aggressive form of thyroid cancer, is also linked to MEN2B syndrome. Infants with MEN2B syndrome and certain changes in the RET gene usually have surgery to remove the thyroid to lessen the chance cancer will form.
Children with MEN2B syndrome who have medullary thyroid cancer may be treated with 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.
Treatment is also given for pheochromocytoma and other conditions as needed.
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 MEN Syndromes
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Treatment of cancer linked to MEN syndrome that has recurred (come back) may include the following:
- To Learn More About Childhood MEN Syndromes
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For more information from the National Cancer Institute about MEN syndromes, 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 multiple endocrine neoplasia (MEN) syndromes. 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 Multiple Endocrine Neoplasia (MEN) Syndromes Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/multiple-endocrine-neoplasia/patient-child-men-syndromes-treatment-pdq. Accessed <MM/DD/YYYY>.
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