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Alternative Names Return to topWermer syndrome; MEN I
Definition Return to top
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:
Causes Return to top
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:
Symptoms Return to top
Symptoms vary from person to person, and may include:
Exams and Tests Return to top
Signs may include:
Tests to diagnose MEN I may include:
Treatment Return to top
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.
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
Recurrent tumors may develop.
When to Contact a Medical Professional Return to top
Call your health care provider if you notice symptoms of MEN I.
Prevention Return to top
Screening close relatives of people affected with this disorder is recommended.
References Return to top
Kronenberg HM. Polyglandular disorders. in: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 250.Update Date: 9/4/2008 Updated 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.