Helpful guidelines

What is multiple endocrine neoplasia syndrome type 1?

What is multiple endocrine neoplasia syndrome type 1?

Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition associated with tumors of the endocrine (hormone producing) glands. MEN1 was originally known as Wermer syndrome. The most common tumors seen in MEN1 involve the parathyroid gland, islet cells of the pancreas, and pituitary gland.

How do you remember multiple endocrine neoplasia?

PiParPanc (mnemonic) PPP (mnemonic for MEN1)…Mnemonics to remember the classic triad of multiple endocrine neoplasia type 1 (MEN1) are:

  1. PPP.
  2. PiParPanc.
  3. ParaPanPit.

What organs does multiple endocrine neoplasia type 1 affect?

In individuals with MEN type 1, tumors develop in multiple endocrine glands, principally the parathyroids, gastro-entero-pancreatic tract and pituitary gland. These affected glands secrete excessive amounts of hormones into the bloodstream, which can result in a variety of symptoms and related syndromes.

How do you get tested for MEN1?

To diagnose multiple endocrine neoplasia, type 1 (MEN 1), your doctor will perform a physical exam and review your medical history and family history. You may have a blood test and imaging tests, including the following: Magnetic resonance imaging (MRI) Computerized tomography (CT) scan.

What does the MEN1 gene do?

The MEN1 gene provides instructions for making a protein called menin. This protein acts as a tumor suppressor, which means that it keeps cells from growing and dividing too fast or in an uncontrolled way. Although the exact function of menin is unclear, it is likely involved in several important cell functions.

How do you know if you have multiple endocrine neoplasia?

Multiple endocrine neoplasia, type 1 (MEN1) is diagnosed based on the presence of two or more endocrine tumors in one person. The results of genetic testing can help confirm the diagnosis, and can help identify people at risk for MEN1 who have not yet developed tumors.

Is MEN1 curable?

Although MEN1 can’t be cured, most people with the disorder lead long and productive lives. Your doctor will monitor your health and provide treatment as needed.

What is multiple endocrine neoplasia type 1?

Multiple endocrine neoplasia type 1 (MEN1), also known as Wermer syndrome, is an autosomal dominant genetic disease that results in proliferative lesions in multiple endocrine organs, particularly the pituitary gland, islet cells of the pancreas and parathyroid glands.

What are the other endocrine neoplasia syndromes associated with MEN1?

There are other multiple endocrine neoplasia syndromes and these are discussed separately. In addition to the characteristic lesions involving the pituitary, parathyroid, and pancreas, numerous other lesions are encountered with greater frequency in patients with MEN1.

How common are carcinoid tumors in people with MEN1?

Eventually, about half of people with MEN1 will develop a cancerous pancreatic or carcinoid tumor. A carcinoid is a slow-growing endocrine tumor inside the chest or stomach of a person with MEN1. Although carcinoids arise from endocrine cells, which are present in many parts of the body,…

Can MEN1 cause multiple tumors?

Previously called Wermer’s syndrome, MEN1 causes tumors to develop in the People with MEN1 may also develop tumors—usually benign (not cancerous)—in other endocrine glands and body tissues, including the skin. Multiple tumors often develop at the same time in different tissues. How common is MEN1?