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Multiple endocrine neoplasia (MEN) syndromes
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 syndrome and each type may cause different conditions or cancers. Patients and family members with an increased risk of these syndromes should have genetic counseling and tests to check for the syndromes.
The two main types of MEN syndromes are MEN1 and MEN2:
MEN1 syndrome is also called Wermer syndrome. This syndrome can cause tumors in the parathyroid, pancreas, and pituitary glands. A diagnosis of MEN1 syndrome is usually made when tumors are found in two or three of these glands. MEN1 syndrome may also cause tumors in the adrenal glands, gastrointestinal tract, fibrous tissue, and fat cells. The prognosis (chance of recovery) is usually good.
The most common sign of MEN1 syndrome is hypercalcemia. Hypercalcemia may cause weakness, feeling very tired, nausea and vomiting, loss of appetite, being very thirsty and urinating more than usual, and constipation.
Children who are diagnosed with MEN1 syndrome are checked for signs of cancer starting at age 5 and continuing for the rest of their life. Talk to your doctor about the tests and procedures that should be done to check for signs of cancer and how often they should be done.
MEN2 syndrome includes three subgroups:
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 tumors:
Symptoms of medullary thyroid cancer may include the following:
Symptoms of pheochromocytoma may include:
Symptoms of parathyroid gland cancer may include:
Family members of patients with the MEN2A syndrome should have genetic counseling and be tested in early childhood, before age 5, for the gene changes that lead to this type of cancer.
A small number of medullary thyroid cancers may occur at the same time as Hirschsprung disease (chronic constipation that begins when a child is an infant), which has been found in some families with MEN2A syndrome. Hirschsprung disease may appear before other signs of MEN2A syndrome do. Patients who are diagnosed with Hirschsprung disease should be checked for certain gene changes that cause MEN2A syndrome.
Patients with MEN2B syndrome may have a slender body build with long, thin arms and legs. The lips may appear thick and bumpy because of benign tumors in the mucous membranes. MEN2B syndrome may cause the following conditions:
Familial medullary carcinoma of the thyroid (FMTC)
This type of MEN2 syndrome causes medullary thyroid cancer. A diagnosis of FMTC may be made when 2 or more family members have medullary thyroid cancer and no family members have parathyroid or adrenal gland problems.
Tests used to diagnose and stage MEN syndromes depend on the symptoms and the patient's family history. They may include:
Other tests and procedures used to diagnose MEN syndromes include the following:
There are several types of MEN syndrome, and each type may need different treatment:
For more information on Multiple Endocrine Neoplasia Syndromes click here
This link is to the National Cancer Institute (NCI) cancer website in the United States. There may be references to drugs and clinical trials that are not available here in Australia.
Page last updated: 30/04/2020