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Multiple Endocrine Neoplasia India Type 1 Treatment India

Multiple Endocrine Neoplasia India Type 1 Treatment India

Multiple Endocrine Neoplasia India Type 1 Treatment India

Multiple Endocrine Neoplasia India Type 1 Treatment India

Multiple Endocrine Neoplasia India Type 1 Treatment India

Multiple Endocrine Neoplasia India Type 1 Treatment India



Multiple Endocrine Neoplasia India Type 1 Treatment India

Multiple Endocrine Neoplasia Type 1

Overview

What is multiple endocrine neoplasia type 1 (MEN1)?

MEN1 is an inherited disorder that causes tumors in the endocrine glands and the duodenum, the first part of the small intestine. MEN1 is sometimes called multiple endocrine adenomatosis or Wermer’s syndrome, after one of the first doctors to recognize it. MEN1 is rare, occurring in about one in 30,000 people.1 The disorder affects both sexes equally and shows no geographical, racial, or ethnic preferences.

Endocrine glands release hormones into the bloodstream. Hormones are powerful chemicals that travel through the blood, controlling and instructing the functions of various organs. Normally, the hormones released by endocrine glands are carefully balanced to meet the body’s needs.

 

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How does MEN1 affect the endocrine glands and the duodenum? The Parathyroid Glands

The parathyroids are the endocrine glands earliest and most often affected by MEN1. The body normally has four parathyroid glands, which are located close to the thyroid gland in the front of the neck. The parathyroids release into the bloodstream a chemical called parathyroid hormone (PTH), which helps maintain a normal supply of calcium in the blood, bones, and urine.


Hyperparathyroidism

In MEN1, all four parathyroid glands tend to be overactive, causing hyperparathyroidism. The parathyroid glands form tumors that release too much PTH, leading to excess calcium in the blood. High blood calcium, known as hypercalcemia, can exist for many years before it is found by accident or through screening for MEN1. Unrecognized hypercalcemia can cause excess calcium to spill into the urine, leading to kidney stones or kidney damage.

Also, the bones may lose calcium and weaken.
Nearly everyone who inherits a susceptibility to MEN1 will develop hyperparathyroidism by age 50, but the disorder can often be detected before age 20. Hyperparathyroidism may cause no problems for many years, or it may cause tiredness, weakness, muscle or bone pain, constipation, indigestion, kidney stones, or thinning of bones.
Doctors must decide whether hyperparathyroidism in MEN1 is severe enough to need treatment, especially in a person who has no symptoms. The usual treatment is an operation to remove most or all of the parathyroid glands. One option is to remove the three largest glands and all but a small part of the fourth. Another is to remove all four glands and at the same time transplant a small part of one gland into the forearm. By maintaining a portion of one gland, the parathyroid transplant continues to release PTH into the bloodstream to do its job.

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The Pituitary Gland

The pituitary, a small gland located at the base of the brain, produces many important hormones that regulate basic body functions. The normal major pituitary hormones are

  • prolactin—controls the formation of breast milk and influences fertility and bone strength
  • growth hormone—regulates body growth, especially during adolescence
  • ACTH—stimulates the adrenal glands to produce cortisol
  • thyrotropin—stimulates the thyroid gland to produce thyroid hormones, which regulate metabolism
  • luteinizing hormone—stimulates the ovaries or testes to produce sex hormones
  • FSH—regulates fertility

Prolactinomas

The pituitary gland becomes overactive in about one in four people with MEN1.4 This overactivity can usually be traced to a small tumor in the gland that releases too much prolactin, called a prolactinoma. High prolactin levels can cause excessive production of breast milk or interfere with fertility in women or with sex drive and fertility in men.
Treatment may not be needed for prolactinomas. If treatment is needed, a medicine known as a dopamine agonist can effectively shrink the tumor and lower the production of prolactin. Occasionally, prolactinomas do not respond well to this medication. In such cases, surgery, radiation, or both may be needed.

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Treatment of Pancreatic Endocrine Cancer in MEN1

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. According to this school of thought, pancreatic surgery has serious complications, so it should not be attempted unless it will cure a tumor or cure a hormone excess state.

Another school advocates early surgery, perhaps when a tumor grows to a certain size, to prevent or treat pancreatic endocrine cancer—even if the tumor does not over secrete a hormone—before the cancer spreads. No clear evidence exists, however, that surgery to prevent pancreatic endocrine cancer from spreading actually leads to longer survival for patients with MEN1.

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