Health Encyclopedia

Multiple endocrine neoplasia (MEN) I

Definition

Multiple endocrine neoplasia (MEN) 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:

Adrenals
Pancreas
Parathyroid
Pituitary
Thyroid

Alternative Names

Wermer syndrome; MEN I

Causes, incidence, and risk factors

MEN I is caused by a defect in a gene called RET. 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.

Risk factors for MEN I include:

Family history of this disorder
Pituitary tumor
Zollinger-Ellison syndrome

Symptoms

Symptoms vary from person to person, and 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, loss of body or facial hair (in men)
Fatigue
Headache
Lack of menstrual periods, infertility, or failure to produce breast milk (in women)
Loss of appetite
Loss of coordination
Loss of underarm or pubic hair
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
Kidney stones
Low blood pressure
Low blood sugar
Pituitary problems (such as too much prolactin, a hormone that controls breast milk production)

Tests to diagnose MEN I may include:

Cortisol
Cranial CT scan
CT of abdomen
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)

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, it is difficult for the body to regulate calcium levels without these glands.

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

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

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.

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.

Review Date:9/4/2008
Reviewed By:Sean O. Stitham, MD, private practice in Internal Medicine, Seattle, Washington; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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