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Multiple Endocrine Neoplasia (MEN)

Alternative names: Wermer's Syndrome, Familial Endocrine Adenomatosis, Multiple Endocrine Adenomatosis

What is multiple endocrine neoplasia (MEN)?
What are the signs of multiple endocrine neoplasia (MEN)?
What causes multiple endocrine neoplasia (MEN)?
How does my doctor tell if I have multiple endocrine neoplasia (MEN)?
How is multiple endocrine neoplasia (MEN) treated?

What is multiple endocrine neoplasia (MEN)?

Multiple Endocrine Neoplasia (MEN) is the name of three rare, inherited disorders that cause extra tissue (hyperplasia) or adenomas (tumors) to grow on the endocrine glands. This can cause several endocrine glands to become overactive – produce too many hormones – at the same time.

The three kinds of MEN are Type 1 (the most common), Type 2A, and Type 2B. The glands most often affected are the parathyroid, the pancreas, and the pituitary.

What are the signs of multiple endocrine neoplasia (MEN)?

MEN can cause several conditions, including:

  • Hyperparathyroidism, or too many parathyroid hormones. This is often the earliest sign of MEN Type 1.
  • Thyroid cancer (medullary thyroid cancer). This is the most common sign of MEN Type 2A and Type 2B.
  • Pancreatic tumors (gastrinomas)
  • Pituitary tumors
  • Tumors of the adrenal gland (pheochromocytomas)

Tumors can cause endocrine glands to become overactive and create too many hormones. This can cause many other symptoms, including tiredness, weakness, muscle or bone pain, constipation, indigestion, nausea, loss of body hair, and loss of regular menstruation.

Pancreatic tumors can cause the stomach to release extra gastric acid, causing stomach ulcers. Pancreatic tumors can also produce too much insulin, and cause low blood sugar (hypoglycemia).

Most people with MEN seek medical treatment because of ulcers, low blood sugar, kidney stones, or other conditions caused by overactive endocrine glands.

What causes multiple endocrine neoplasia (MEN)?

The three kinds of MEN are inherited conditions that are caused by a genetic mutation. A person with a family history of MEN cannot pass the condition to his or her children unless he or she has MEN.

How does my doctor tell if I have multiple endocrine neoplasia (MEN)?

Hyperparathyroidism, and other symptoms of overactive endocrine glands, can be diagnosed through blood and/or urine tests.

A doctor can also use blood tests to check for the development of thyroid cancer, caused by MEN Type 2A or 2B. Such tests measure the levels of the thyroid hormone, calcitonin.

Doctors now offer a genetic test for MEN to close relatives of people with the condition. This test can see if the relative has inherited the mutated MEN gene. A laboratory analyzes the DNA of a single blood sample. A negative genetic test result means that a person doesn't have MEN, and can't pass it on to his or her children.

People with a positive MEN genetic test should have regular blood tests to make sure the endocrine glands haven't become overactive. A doctor may also recommend magnetic resonance imaging (MRI), computed tomography (CT), or other scans to check on the patient's endocrine glands.

How is multiple endocrine neoplasia (MEN) treated?

There is no cure for MEN, but most of the health problems the conditions cause can be treated.

Surgical removal of the affected parathyroid glands is the only known cure for hyperparathyroidism. Your doctor will want to monitor your loss of bone density, to see if surgery is needed. Other treatments include estrogen therapy, for postmenopausal women, and drugs that are used to treat osteoporosis, such as bisphosphonates.

Treatment for pituitary tumors depends on the type and size of the tumor. Although a small adenoma may not need treatment, your doctor will monitor its size over time. For most patients with functioning (hormone-producing) tumors, doctors recommend removing the adenoma through surgery. This is called a transsphenoidal hypophysectomy.

A prolactin-producing pituitary tumor is often treated with medications called long-acting dopamine agonists, which lower blood levels of prolactin and may also shrink the size of the pituitary tumor. These medications include bromocriptine (Parlodel), pergolide (Permax), and cabergoline (Dostinex).

Gastrinomas, or pancreatic tumors that cause the stomach to produce excess acid, can be treated with medications called acid pump inhibitors. These medicines keep the stomach from releasing acid.

In a patient with MEN Type 2 – which often leads to thyroid cancer – a doctor may recommend removing the thyroid gland before it becomes cancerous. The patient will need to take replacement thyroid hormones for the rest of his or her life.

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