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Other Rare Unusual Cancers of Childhood

Multiple Endocrine Neoplasia Syndromes

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 Werner 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

    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:

    • Medullary thyroid cancer.
    • Pheochromocytoma (a tumor of the adrenal gland).
    • Parathyroid gland cancer.

    Symptoms of medullary thyroid cancer may include the following:

    Symptoms of pheochromocytoma may include:

    • Pain in the abdomen or chest.
    • Fast or irregular heart beat.
    • Being irritable or nervous.
    • Headache.

    Symptoms of parathyroid gland cancer may include:

    • Hypercalcemia.
    • Pain in the abdomen, side, or back that doesn't go away.
    • Pain in the bones.
    • A broken bone.
    • A lump in the neck.
    • Change in voice, such as hoarseness.
    • Trouble swallowing.

    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.

  • MEN2B 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:

    • Medullary thyroid cancer.
    • Parathyroid hyperplasia.
    • Adenomas.
    • Pheochromocytoma.
    • Nerve cell tumors in the mucous membranes or other places.
  • 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.


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