What is the definition of Multiple Endocrine Neoplasia Type 1?

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

What are the alternative names for Multiple Endocrine Neoplasia Type 1?

Wermer syndrome; MEN I

What are the causes for Multiple Endocrine Neoplasia Type 1?

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.

What are the symptoms for Multiple Endocrine Neoplasia Type 1?

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 are the current treatments for Multiple Endocrine Neoplasia Type 1?

Surgery to remove the diseased gland is often the treatment of choice. A medicine 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, 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.

What is the outlook (prognosis) for Multiple Endocrine Neoplasia Type 1?

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.

What are the possible complications for Multiple Endocrine Neoplasia Type 1?

The tumors can keep coming back. Symptoms and complications depend on which glands are involved. Regular check-ups by your provider are essential.

When should I contact a medical professional for Multiple Endocrine Neoplasia Type 1?

Call your provider if you notice symptoms of MEN I or have a family history of this condition.

How do I prevent Multiple Endocrine Neoplasia Type 1?

Screening close relatives of people affected with this disorder is recommended.

Endocrine

REFERENCES

National Comprehensive Cancer Network website. Clinical practice guidelines in oncology (NCCN guideines): neuroendocrine tumors. Version 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated March 5, 2019. Accessed March 8, 2020.

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.

Nieman LK, Spiegel AM. Polyglandular disorders. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 218.

Thakker RV. Multiple endocrine neoplasia type 1. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 148.

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