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Multiple endocrine neoplasia (MEN) I
Definition
Multiple endocrine neoplasia (MEN) type I is disease passed down through families 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
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 (in women)
- Loss of appetite
- Loss of body or facial hair (in men)
- Mental changes or confusion
- Muscle pain
- Nausea and vomiting
- Sensitivity to the cold
- Unintentional weight loss
- Vision problems
- Weakness
Exams and Tests
The health care provider will perform a physical exam and ask questions about your medical history and symptoms. The following tests may be done:
- Blood cortisol level
- CT scan of abdomen
- CT scan of the head
- Fasting blood sugar
- Genetic testing
- 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
- 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.
Medicine is available to decrease 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.
Outlook (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.
Possible Complications
The tumors can keep coming back. Symptoms and complications depend on which glands are involved. Coma is possible.
When to Contact a Medical Professional
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. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 239.
National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. National Comprehensive Cancer Network; 2011. Version 1.2011.
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.
