Multiple Endocrine Neoplasia Type 1

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Endocrine diseases affect the hormone-producing glands that help control basic bodily functions, including everything from metabolism to growth and even libido. These diseases may be debilitating and can cause a range of symptoms, because disorders like multiple endocrine neoplasia, type 1 (MEN1) disrupt many of the bodily functions we often take for granted.

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What Is Multiple Endocrine Neoplasia Type 1?

Multiple endocrine neoplasia (MEN) type 1 is a rare, inherited disorder occurring in only 1 out of every 30,000 people, according to the National Institute of Diabetes and Digestive and Kidney Diseases. Sometimes called multiple endocrine adenomatosis, or Wermer’s syndrome, MEN1 causes tumor growth along the body’s network of hormone-producing glands, called the endocrine system. Because this condition oftencauses tumors to grow on the parathyroid and pituitary glands, as well as the pancreas, they cause the glands to secrete excess hormones, causing an array of health problems.

MEN1 is harmful to the body because of how it affects the network of glands secreting hormones through the bloodstream to reach the target organs. These glands make up the endocrine system and regulate chemical processes influencing the various functions of the body’s organs. Hormones affect virtually every bodily system, including the regulation of heart rate, body temperature, and blood pressure.

MEN1 tumors can be benign or malignant, and those that are cancerous can spread to other organs. However, the majority of MEN1 tumors are benign (noncancerous).

The most common tumors seen in MEN1 involve these endocrine glands:

  • Parathyroid gland
  • Islet cells of the pancreas
  • Pituitary gland
  • Adrenal cortical tumors
  • Pheochromocytomas
  • Tumors in other parts of the digestive tract

Signs and Symptoms of Multiple Endocrine Neoplasia Type 1

Symptoms of MEN1 vary greatly from one person to the next, and depend mainly on where the tumors are located. The symptoms may also be related to secondary conditions caused by MEN1.

Overproduction of the following hormones results in the symptoms listed below:

  • Prolactin: This causes abnormal milk production of the breasts, ends menstruation in women, and lowers testosterone production in men.
  • Growth hormone: If this is affected, it causes excessive jaw growth and other soft tissues.
  • Gastrin: This can cause stomach ulcers.
  • Glucagon: Too much glucose can cause Type 2 diabetes and skin rash.
  • Vasoactive intestinal peptide: This is produced by a pancreatic neuroendocrine tumor and causes watery diarrhea.
  • Parathyroid hormone: This causes hypercalcemia (high blood calcium) and kidney stones.

Other common symptoms include: abdominal pain, nausea, vomiting, bloating, weakness, headache, blurry vision, difficulty with coordination, personality changes, confusion, loss of appetite, muscle pain, fatigue, unexplained weight loss, loss of axillary or pubic hair, and more.

Diagnosis of Multiple Endocrine Neoplasia Type 1

MEN1 is suspected when a person has at least 2 of these tumors: parathyroid tumor, pancreatic neuroendocrine tumor, or a pituitary gland tumor. Genetic testing for mutations in the MEN1 gene are also possible, as about 90% of individuals diagnosed with the disorder have a genetic marker, whereas the other 10% have a rare genetic mutation causing MEN1. Children of parents who have MEN1 should be screened for the disorder.

Multiple Endocrine Neoplasia Type 1 Treatment

People diagnosed with MEN1 need regular screenings to allow for treatment of tumors and recurring tumors. Usually, these screenings begin in early childhood and continue on a regular basis throughout the patient’s life.

MEN1 is often treated through surgical removal of the affected gland, although medication therapy may also be used for prolactin-secreting pituitary tumors. When glands are removed or secretion is inadequate, the patient may be prescribed hormone replacement therapy.

Tumors may recur and need to be removed or treated again, and cancerous tumors can spread. Treatment for cancerous tumors depends on which endocrine gland is affected and whether the growths are cancerous or benign.

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