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Multiple endocrine neoplasia (MEN) I

Definition

Multiple endocrine neoplasia (MEN) type I is an inherited disorder in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include:

• Pancreas
• Parathyroid
• Pituitary

Alternative Names

Wermer syndrome; MEN I

Causes, incidence, and risk factors

MEN I is caused by a defect in a gene that carries the code for a protein called menin. The condition causes tumors of various glands to appear in the same person, but not necessarily at the same time.

The disorder may occur at any age, and it affects men and women equally. A family history of this disorder raises your risk.

Symptoms

Symptoms vary from person to person, and depend on which gland is involved. They may include:

• Abdominal pain
• Anxiety
• Black, tarry stools
• Bloated feeling after meals
• Burning, aching, or hunger discomfort in the upper abdomen or lower chest that is relieved by antacids, milk, or food
• Decreased sexual interest
• Fatigue
• Headache
• Lack of menstrual periods, infertility, or failure to produce breast milk (in women)
• Loss of appetite
• Loss of body or facial hair (in men)
• Loss of coordination
• Mental changes or confusion
• Muscle pain
• Nausea and vomiting
• Sensitivity to the cold
• Unintentional weight loss
• Vision problems
• Weakness

Signs and tests

Signs may include:

• Coma (if low blood sugar is untreated)
• High blood calcium level
• Kidney stones
• Low blood pressure
• Low blood sugar
• Pituitary problems (such as breast discharge from too much prolactin, a hormone that controls breast milk production)

Tests to diagnose tumors involved with MEN I may include:

• Blood cortisol level
• CT scan of abdomen
• CT scan of the head
• Fasting blood sugar
• Insulin test
• MRI of the abdomen
• MRI of the head
• Parathyroid biopsy
• Serum adrenocorticotropic hormone
• Serum calcium
• Serum follicle stimulating hormone
• Serum gastrin
• Serum glucagon
• Serum luteinizing hormone
• Serum parathyroid hormone
• Serum prolactin
• Serum thyroid stimulating hormone (rarely used)
• Ultrasound of the neck

Treatment

Surgery to remove the diseased gland is the treatment of choice. A medication called bromocriptine may be used instead of surgery for pituitary tumors that release the hormone prolactin.

The parathyroid glands, which control calcium production, can be removed. However, because it is difficult for the body to regulate calcium levels without these glands, a total parathyroid removal is usually not done initially.

There is now effective medication to reduce the excess stomach acid production caused by some tumors, and to reduce the risk of ulcers.

Hormone replacement therapy is given when entire glands are removed or do not produce enough hormones.

Support Groups

Expectations (prognosis)

Pituitary and parathyroid tumors are usually noncancerous (benign), but some pancreatic tumors may become cancerous (malignant) and spread to the liver. These can lower life expectancy.

The symptoms of peptic ulcer disease, low blood sugar, excess calcium in the blood, and pituitary dysfunction usually respond well to treatment.

Complications

Recurrent tumors may develop.

Calling your health care provider

Call your health care provider if you notice symptoms of MEN I or have a family history of this condition.

Prevention

Screening close relatives of people affected with this disorder is recommended.

References

Kronenberg HM. Polyglandular disorders. in: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 250.

National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. National Comprehensive Cancer Network; 2009. Version 2.2009.