Multiple endocrine neoplasia (MEN) I
Wermer syndrome; MEN IMultiple 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 ...
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- Burning, aching, or hunger discomfort in the upper abdomen or lower chest that is relieved by antacids, milk, or food
Hunger
Increased appetite means you have an excess desire for food.
Read Article Now Book Mark Article - Decreased sexual interest
- Fatigue
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
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- 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.
Read Article Now Book Mark Article - Loss of body or facial hair (in men)
- Mental changes or confusion
Mental changes
Confusion is the inability to think as clearly or quickly as you normally do. You may feel disoriented and have difficulty paying attention, remembe...
Read Article Now Book Mark Article - Muscle pain
Muscle pain
Muscle aches and pains are common and can involve more than one muscle. Muscle pain also can involve ligaments, tendons, and fascia. Fascias are th...
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- Sensitivity to the cold
Sensitivity to the cold
Cold intolerance is an abnormal sensitivity to a cold environment or cold temperatures.
Read Article Now Book Mark Article - 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
Blood cortisol level
The cortisol blood test measures the level of cortisol in the blood. Cortisol is a steroid (glucocorticoid or corticosteroid) hormone produced by th...
Read Article Now Book Mark Article - CT scan of the abdomen
CT scan of the abdomen
An abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...
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- Fasting blood sugar
Fasting blood sugar
A blood sugar test measures the amount of a sugar called glucose in a sample of your blood. Glucose is a major source of energy for most cells of the...
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- 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...
Read Article Now Book Mark Article - Serum adrenocorticotropic hormone
Serum adrenocorticotropic hormone
The ACTH test measures the level of adrenocorticotropic hormone (ACTH) in the blood. ACTH is a hormone released from the pituitary gland at the base...
Read Article Now Book Mark Article - Serum calcium
Serum calcium
The calcium blood test measures the level of calcium in the blood. This article discusses the test to measure the total amount of calcium in your blo...
Read Article Now Book Mark Article - Serum follicle stimulating hormone
Serum follicle stimulating hormone
The follicle stimulating hormone (FSH) blood test measures the level of FSH in blood. FSH is a hormone released by the pituitary gland, located on t...
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- Serum glucagon
Serum glucagon
A glucagon blood test measures the amount of a hormone called glucagon in your blood. Glucagon is produced by specific cells in the pancreas. It he...
Read Article Now Book Mark Article - Serum luteinizing hormone
Serum luteinizing hormone
The LH blood test measures the amount of luteinizing hormone (LH) in blood. LH is a hormone released by the pituitary gland, located on the undersid...
Read Article Now Book Mark Article - Serum parathyroid hormone
Serum parathyroid hormone
The PTH test measures the level of parathyroid hormone in the blood. PTH stands for parathyroid hormone. It is a protein hormone released by the par...
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- Serum thyroid stimulating hormone
Serum thyroid stimulating hormone
A TSH test measures the amount of thyroid stimulating hormone (TSH) in your blood. TSH is produced by the pituitary gland. It prompts the thyroid g...
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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...
Read Article Now Book Mark ArticleThe symptoms of peptic ulcer disease, low blood sugar, excess calcium in the blood, and pituitary dysfunction usually respond well to appropriate treatment.
Peptic ulcer
A peptic ulcer is an open sore or raw area in the lining of the stomach or intestine. There are two types of peptic ulcers:Gastric ulcer -- occurs in...
Read Article Now Book Mark ArticlePossible 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.
References
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.
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
Review Date: 3/31/2024
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.