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Multiple endocrine neoplasia type 1
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Multiple endocrine neoplasia type 1, also called Wermer syndrome, is a rare syndrome in which tumors develop in several endocrine glands, the ones that make hormones. The ones most often involved are the parathyroid glands, the anterior pituitary, and the neuroendocrine tissue of the digestive tract and the pancreas.
What ties them together is a gene. The cause is an inactivating germline mutation in the MEN1 gene, also called the menin gene, which sits on chromosome eleven. Germline means the person is born with it in every one of their cells, not that it shows up later in a single organ. And it is inherited in an autosomal dominant pattern, meaning that inheriting the altered copy from one of the two parents is enough. One faulty gene in every cell, and several different glands starting to grow tumors.
Not all of them are affected equally. The most common manifestation is hyperparathyroidism, meaning parathyroid glands that work too much, and it shows up in ninety percent of cases.
In brief
Multiple endocrine neoplasia type 1 is a rare endocrine tumor syndrome that mainly involves the parathyroid glands, the anterior pituitary, and gastroenteropancreatic neuroendocrine tissue.
It is caused by an inactivating germline mutation in the MEN1 gene, or menin gene, on chromosome eleven, and it is inherited in an autosomal dominant pattern.
One faulty gene in every cell, and several different glands starting to grow tumors.
Hyperparathyroidism is the most common manifestation and occurs in ninety percent of cases.
Sources (1)
- National Center for Biotechnology Information, StatPearls PublishingMultiple Endocrine Neoplasia Type 1, StatPearlsPublished
This page explains the topic in general terms; it does not tell anyone what treatment to follow.
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