Multiple Endocrine Neoplasia Type 1 (MEN1)

What is multiple endocrine neoplasia type 1 (MEN1)?

Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited disorder that causes (noncancerous) tumors to form in the endocrine glands and the duodenum, which is the first part of the small intestine. In people with MEN1, these tumors in the multiple endocrine glands, such as the parathyroids, pancreas, or pituitary, cause them to become overactive and secrete excessive amounts of hormones into the bloodstream.

What causes multiple endocrine neoplasia type 1 (MEN1)?

MEN1 is caused by a defect in the gene MEN1 gene that carries the code for a protein called menin. Although the exact role of menin is not fully understood, the MEN1 gene is thought to be a tumor suppressor gene. This mutation may be inherited as an autosomal dominant trait or spontaneously occur as a new gene mutation in the affected person.

What are the symptoms of multiple endocrine neoplasia type 1 (MEN1)?

The symptoms of MEN1 vary from person to person, and they largely depend on which particular gland is involved. They may include:

Abdominal pain
Anxiety or other mental changes or confusion
Black, tarry stools
Nausea and vomiting
Sensitivity to the cold
Loss of appetite and unintentional weight loss
Vision problems
Fatigue or weakness

How is multiple endocrine neoplasia type 1 (MEN1) diagnosed?

A diagnosis of MEN type 1 can be made following a thorough clinical evaluation, a detailed patient and family history, and at least two of the three characteristic endocrine tumors associated with the disorder. Molecular genetic testing can confirm a MEN type 1 diagnosis.

What are the available treatments for multiple endocrine neoplasia type 1 (MEN1)?

The treatment of choice for MEN1 is surgery to remove the diseased gland. Chemotherpay or radiation therapy can also help shrink or destroy tumors. However, instead of surgery, a medicine called bromocriptine or hormone replacement therapy may also be used to counteract the effects of excess hormones.

Where can I find more information on multiple endocrine neoplasia type 1 (MEN1)?