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:
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:
Symptoms vary from person to person, and may include:
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:
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.
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.
Recurrent tumors may develop.
Calling your health care provider:
Call your health care provider if you notice symptoms of MEN I.
Screening close relatives of people affected with this disorder is recommended.
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.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997-
A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.