Multiple endocrine neoplasia (MEN) I
Multiple endocrine neoplasia (MEN) type I is a disease in which one or more of the endocrine glands are overactive or forms a tumor. It is passed down through families.
Endocrine glands most commonly involved include:
- Pancreas
- Parathyroid
- Pituitary
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
Abdominal pain
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
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- Black, tarry stools
Black, tarry stools
Black or tarry stools with a foul smell are a sign of a problem in the upper digestive tract. It most often indicates that there is bleeding in the ...
ImageRead Article Now Book Mark Article - 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 appetite
A decreased appetite is when your desire to eat is reduced. The medical term for a loss of appetite is anorexia.
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- 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 the abdomen
CT scan of the abdomen
An abdominal CT scan is an imaging test that uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomography....
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- Fasting blood sugar
- Genetic testing
- Insulin test
- MRI of the abdomen
MRI of the abdomen
An abdominal magnetic resonance imaging scan is an imaging test that uses powerful magnets and radio waves. The waves create pictures of the inside ...
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MRI of the head
A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...
ImageRead Article Now Book Mark Article - 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 often the treatment of choice. Medicines such as cabergoline and 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, so a total parathyroid removal is not done first in most cases.
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.
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.
Benign
Benign refers to a condition, tumor, or growth that is not cancerous. This means that it does not spread to other parts of the body. It does not in...
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 provider are essential.
When to Contact a Medical Professional
Contact your 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.
Reviewed By
Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
National Comprehensive Cancer Network website. Clinical practice guidelines in oncology (NCCN guidelines): neuroendocrine and adrenal tumors. Version 1.2023. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated August 2, 2023. Accessed May 7, 2024.
Newey PJ, Thakker RV. Multiple endocrine neoplasia. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 42.
Newey PJ, Thakker RV. Multiple endocrine neoplasia type 1. In: Robertson RP, ed. DeGroot's Endocrinology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 133.
Nieman LK, Spiegel AM. Polyglandular disorders. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 212.
