Multiple endocrine neoplasia (patient information)
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Multiple endocrine neoplasia |
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Where to find medical care for Multiple endocrine neoplasia? |
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Multiple endocrine neoplasia On the Web |
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Directions to Hospitals Treating Multiple endocrine neoplasia |
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Risk calculators and risk factors for Multiple endocrine neoplasia |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Jinhui Wu, M.D.
Overview
Multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. Multiple endocrine neoplasia may be classified according to tumor characteristics into 3 subtypes: multiple endocrine neoplasia type 1, multiple endocrine neoplasia type 2 and multiple endocrine neoplasia type 4. Symptoms vary from person to person, and depend on which gland is involved. Genetic mutation is the cause of multiple endocrine neoplasia. The most potent risk factor in the development of multiple endocrine neoplasia is family history of multiple endocrine neoplasia. Surgery to remove the diseased gland is the treatment of choice for multiple endocrine neoplasia.
What are the symptoms of multiple endocrine neoplasia?
- 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
What causes multiple endocrine neoplasia?
- 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.
Who is at highest risk?
The most potent risk factor in the development of multiple endocrine neoplasia is family history of multiple endocrine neoplasia.
Diagnosis
- 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 the 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
When to seek urgent medical care?
Call your health care provider if you notice symptoms of MEN I or have a family history of this condition.
Treatment options
- 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 (gastrinomas), and to reduce the risk of ulcers.
- Hormone replacement therapy is given when entire glands are removed or do not produce enough hormones.
Where to find medical care for multiple endocrine neoplasia?
Directions to Hospitals Treating Multiple endocrine neoplasia
Prevention of multiple endocrine neoplasia
Screening close relatives of people affected with this disorder is recommended.
What to expect (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 appropriate treatment.
Possible complications
- The tumors can keep coming back. Symptoms and complications depend on which glands are involved. Regular check-ups by your health care provider are essential.