Multiple Endocrine Neoplasia (MEN) I is a hereditary disorder in which one or more of the following glands develop excess normal tissue (hyperplasia) or adenoma (tumor): the parathyroid, the pancreas, the pituitary, and (rarely) the adrenals and thyroid gland.
These are all endocrine glands, which produce and secrete hormones into the blood or lymph systems.
Alternative Names: Wermer's syndrome
Causes, incidence, and risk factors:
The cause of MEN I is genetic. Tumors of various glands appear in the same person, but not necessarily at the same time. The disorder is inherited, may occur at any age, and affects men and women equally.
Most people affected with this syndrome seek medical treatment because of one of the following: peptic ulcer disease, symptoms related to low blood sugar, symptoms related to high serum calcium levels or kidney stones, or symptoms related to pituitary problems (such as headache).
Risk factors are a family history of this disorder, a previous pituitary tumor, and a history of Zollinger-Ellison syndrome.
Symptoms vary greatly from person to person and may be related to peptic ulcer disease, hypoglycemia, hypercalcemia, or pituitary dysfunction.
Signs and tests:
Diagnostic tests are used to evaluate the function of each endocrine gland.
Pancreatic evaluation may reveal the following signs:
Parathyroid evaluation may reveal the following signs:
Pituitary evaluation may reveal the following signs:
The following hormone levels may also be measured to evaluate the pituitary:
Additional testing may be needed.
Surgical removal of the affected gland is the treatment of choice, although therapy with a medication called bromocriptine may also be used for pituitary tumors that secrete prolactin. Hormonal replacement therapy is indicated when glands are removed or secretion is inadequate.
Pituitary and parathyroid tumors are usually benign, but some tumors may become malignant (cancerous), accounting for overall decreased life expectancy. The symptoms of peptic ulcer disease, hypoglycemia, hypercalcemia, or pituitary dysfunction should respond to treatment.
Recurrent tumors may develop.
Calling your health care provider:
Call your health care provider you notice symptoms of MEN I.
Screening of close relatives of people affected with this disorder is recommended.