Multiple endocrine neoplasia 1

Overview

What is multiple endocrine neoplasia type 1?

Multiple endocrine neoplasia Type 1 (MEN1) is a inherited cancer syndrome found in men and women that increases the chance for tumors in the hormone producing organs of the endocrine system. The tumors may occur in many different locations and may be benign (non-cancerous) or malignant (cancerous). The most frequently developed tumors in MEN1 occur in the parathyroid glands which are found in 90% of individuals with MEN1 between ages 20 and 25 years. Since the parathyroid glands produce hormones that control the calcium levels in the body's blood and bones, often the first sign of a tumor is high levels of calcium in the blood (hypercalcemia). Other organs that may often exhibit tumors in MEN1 include the:

Pituitary gland- a gland produces hormones that controls the function of most other endocrine glands such as the adrenal glands to secrete hormones that regulates the body's growth, function, and composition.

Adrenal cortical gland- a part of the adrenal gland that produces hormones that helps regulate metabolism, the body's respond to stress, and helps control blood pressure

Pancreas- an organ that helps in digestion and regulates blood sugar.

Gastrointestinal tract- the organs that are involved in the digestion of food

Additional non-endocrine system tumors seen in MEN1 include:

  • Angiofibromas- small, red bumps on the face, especially on the nose and cheeks called
  • Collagenomas- multiple, flesh-colored asymptomatic thickened skin nodules appearing symmetrically on the trunk and upper arms
  • Lipomas- slow-growing, fatty lumps that are most often found between the skin and the underlying muscle layer
  • Meningiomas- tumors found in the membranes that surround the brain and spinal cord called the meninges
  • Ependymomas- cancerous tumors that can be found in the brain or spine
  • Leiomyomas- benign (non cancerous) smooth muscle tumors that can occur in any organ, but the most common forms occur in the uterus, small bowel, and the esophagus.

MEN1 can run in families or can occur as the result of a new disease causing gene change in the individual.

SOURCE: Emory University - Department of Human Genetics in collaboration with ThinkGenetic • https://www.thinkgenetic.com/diseases/multiple-endocrine-neoplasia-109954/overview/68260 • DATE UPDATED: 2020-01-08

References

Genetics Home Reference, Multiple Endocrine Neoplasia, published 1/7/2020. Accessed 07JAN2020. https://ghr.nlm.nih.gov/condition/multiple-endocrine-neoplasia# .

Giusti F, Marini F, Brandi ML. Multiple Endocrine Neoplasia Type 1. 2005 Aug 31 [Updated 2017 Dec 14]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1538/

National Cancer Institute. Cancer Terms Dictionary. https://www.cancer.gov/publications/dictionaries/cancer-terms/ Accessed 03DEC19.

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