Multiple Endocrine Neoplasia Type II (MEN II)

Multiple endocrine neoplasia type II is a rare inherited disease that yields three major endocrine problems. The three endocrine glands involved in MEN II are the parathyroid glands, the adrenal glands and the thyroid glands. It is a disease in which almost all patients with the disease develop medullary thyroid cancer at some point in their lives. There can be an overactive adrenal gland or glands and you can develop a pheochromocytoma (too much adrenaline produced in the adrenal gland). The parathyroid glands, located in the neck, can become overactive as well and can produce too much parathyroid hormone. Only the thyroid gland portion of the disease is actually cancerous.

Multiple endocrine neoplasia type II is very rare. It happens in about 1 in every 20,000 individuals who carry the gene for the disease. It is an autosomal dominant disorder, inherited to both men and women in all races. Half of all children of those with the disease have the potential to get the disease.

MEN II increases the activity of the parathyroid, thyroid and adrenal glands. They increase the number of certain hormones in the body so that the body does not act properly. The glands tend to enlarge as well as producing too much hormone.

Most people inherit the disease at birth but don't get symptoms of the disease until they reach the age of ten years or more. The endocrine glands tend to go bad at different times of the patient's life. This makes the diagnosis of MEN II somewhat complicated. It can be perceived that the person has two or more different endocrine diseases without the linkage made to the cohesive diagnosis of MEN II. The likelihood of getting the various endocrine disorders associated with MEN II increases with age. Most people have at least one overactive gland by the age of 30 with symptoms holding off until they are older than 30. If one gland is affected, doctors should check the other glands for abnormalities, even if there are no symptoms.

Patients who have the MEN II gene, which can be assessed at any age, should have their thyroid gland surgically removed while they are in their early to mid-teen years because of the high incidence of thyroid cancer. Those who should be tested for the MEN II gene are those with a parent with the disease. It doesn't certainly need to be tested for in everyone because it is so rare, even if the person has one gland affected by an adenoma. MEN I and MEN II are completely different disorders with different genes involved.

There is no cure for MEN II; however, with removal of the thyroid gland early in life, the risk of cancer of the thyroid can be eliminated. Because MEN II is hereditary, it can only be prevented in future generations by not having children if you have the disease. In the future, genetic enhancements can be done to change the way the MEN II gene works but research is a long way from that.

The diagnosis of MEN II is twofold. First you need a diagnosis to see if the person has the MEN II gene. Then you need tests for hyperactivity of the affected glands. The test for the MEN II gene is complex but can be done at specialized laboratories. Those with a strong family history of MEN II should be genetically tested for the disease.

If you are positive for the MEN II gene, you should have your parathyroid hormone level checked to make sure you don't have overactive parathyroid glands. If the hormone level is high, you need to consider having the parathyroid glands removed. They can also be removed at the time of the removal of the thyroid gland, because they are imbedded in the thyroid gland. You will have to have hormone replacement after that. The same is true of thyroid hormone, which will need to be removed in the early teens. Thyroid hormone replacement is cheap and easy to take and will normalize the thyroid hormone in the system. The test for a pheochromocytoma or overactive adrenal gland is a test for epinephrine or its metabolites in the urine. If positive, the adrenal glands may need to be removed.