The word “endocrine” refers to glands in the body that secrets hormones that are needed for the normal functioning of the body.
Multiple Endocrine Neoplasia (MEN) Syndrome is a disorder that causes either malignant or benign–cancerous or noncancerous–tumors to form and grow in several endocrine glands. The condition is caused by mutations in the genes of an individual and is a genetic/hereditary disease.
Multiple endocrine neoplasia syndrome is a rare disorder and consists of 3 types: Type1, Type2; 2A and 2B, and Type4. Type 1 and 2 are the most common types of MEN.
In this article, we will be discussing Type 1 of the MEN syndrome.
What Is Multiple Endocrine Neoplasia Type1 (MEN1)?
Also called “Wermer Syndrome”, MEN1 is a hereditary disorder that is characterized by the presence of tumors in the endocrine gland, which includes the parathyroid gland, islet cells of the pancreas, and pituitary gland.
Sometimes, it can be seen in the adrenal gland called carcinoid tumor and in some part of the digestive tract like some part of the small intestine, and stomach. These tumors produce hormones in excess amount than what is needed in the body, resulting In a wide range of symptoms.
Such hormones that increase in production include:
However, these tumors are usually benign, although research has shown approximately 1 in 3 cases of pancreatic neuroendocrine tumors and mediastinal neuroendocrine tumors turning cancerous.
It is estimated that about 1 in 30,000 people has MEN1. About 10% of people with MEN1 do not have a family history of the condition; they have a de novo (new) mutation in the MEN1 gene.
The Primary Endocrine Tumours Which Makes Up MEN1 Includes:
Causes of Multiple Endocrine Neoplasia Type1 (MEN1)
The causes of MEN1 includes:
Mutation in the MEN1 gene. Recent studies have also shown that more than 90% of individuals with MEN1 mutation stand the risk of developing 1 or more signs of MEN1.
Symptoms of MEN1
The symptoms of MEN1 varies but some predominant signs include:
Complications associated with MEN1
There are certain conditions associated with MEN1. They include:
When the excess hormone is secreted into the bloodstream, it could lead to the following complications:
Diagnosis of MEN1
It can be diagnosed if one is experiencing at least two of these tumors namely:
Also, it can be diagnosed through:
Treatment of MEN1
Since MEN1 involves tumors, steps are taken either to remove it or suppress its growth and these steps are applied based on the tumor or tumors present. Such treatment for MEN1 includes the following:
What happens when MEN1 is left untreated?
When MEN1 is left untreated, could result in osteoporosis–broken bones and kidney stones. Although, people with MEN1 can develop parathyroid cancer.
How common is multiple endocrine neoplasia type 1?
MEN 1 is a rare genetic condition that has been estimated to affect between 1 in 10,000 to 1 in 30,000 people; both men and women are affected. Although, the age at which people with MEN1 begin developing tumors varies and hence, not predictable just based on family history only.
What are the side-effects of the treatment?
After the surgery e.g parathyroid, calcium levels in the blood will reduce (hypocalcemia), causing tingling sensations in the hands and the face. Also, muscular spasms may be severe. This is managed using life-long supplements of activated vitamin D and calcium to aid boost normal healthy blood calcium levels.
Are there other tumors associated with MEN1 that are not Endocrine?
Non-endocrine tumors seen in MEN1 include:
Is MEN1 cancerous?
Most tumors are benign-small and not cancerous, although some risk of it leading to cancer is present, it is negligible.