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.

When is MEN1 diagnosed?

Although the disorder can affect all age groups, the first symptoms are typically linked to overactive parathyroid glands and often appear in people in their early 20s. Most people are diagnosed as having MEN1 in their 40s, when the disorder has started to affect other endocrine glands.

How do I know if I have MEN1?

Symptoms include tiredness, depression, stomach ulcers, abdominal pain and non-specific aches and pains, and if left untreated, it can result in thinning of bones (osteoporosis) and kidney stones. Very rarely, patients with MEN1 may have a parathyroid cancer.

What are the characteristics of multiple endocrine neoplasia type 1?

Clinical characteristics: Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of more than 20 endocrine and non-endocrine tumors. Endocrine tumors become evident either by overproduction of hormones by the tumor or by growth of the tumor itself.

Is there genetic testing for multiple endocrine neoplasia?

Yes, genetic testing is available for MEN1, the gene known to cause multiple endocrine neoplasia, type 1 (MEN1). [2] Carrier testing for at-risk relatives and prenatal testing are possible if the disease-causing mutation in the family is known. The Genetic Testing Registry (GTR) is a centralized online resource for information about genetic tests.

What to do with pancreatic endocrine neoplasia type 1?

Because the type of pancreatic endocrine cancer associated with MEN1 can be difficult to recognize, difficult to treat, and slow to progress, doctors have different views about the value of surgery in managing these tumors. One approach is to “watch and wait,” using medical, or nonsurgical, treatments.

Which is the most common endocrine neoplasia in men?

The most common affected endocrine glands in MEN type 1 are parathyroids, both as tissue hyperplasia and/or adenomas. More than 90 percent of individuals with MEN type 1 develop, by age 50, primary hyperparathyroidism (PHPT).