Multiple Endocrine Neoplasia (MEN) I is a hereditary disorder in which one, or often two or more of the following glands develop hyperplasia or adenoma ( tumor ): the parathyroid, the pancreas, the pituitary, and rarely, the adrenals and thyroid gland.

Alternative Names

Wermer's syndrome

Causes, incidence, and risk factors

The cause of multiple endocrine neoplasia 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 .

Signs and tests

Diagnostic tests are used to evaluate the function of each endocrine gland. Pancreatic evaluation may reveal the following signs:

An

MRI of abdomen shows a pancreatic tumor .

A

CT of abdomen shows a pancreatic tumor.

An

insulin test may show increased levels.

Fasting blood sugar

may be low.

Serum glucagon

may be elevated.

Serum gastrin may be elevated.

Parathyroid evaluation may reveal the following signs:

The

serum calcium level is elevated, and the serum parathyroid hormone is increased.

A

parathyroid biopsy shows tumor or hyperplasia Pituitary evaluation may reveal the following signs:

A

cranial CT scan or MRI of head may reveal a pituitary tumor . The following hormone levels may also be measured to evaluate the pituitary:

Serum thyroid stimulating hormone

(rarely used)

Cortisol level

Serum adrenocorticotropic hormone

level

Serum luteinizing hormone

Serum follicle stimulating hormone

Additional testing may be indicated.

Treatment

Surgical removal of the affected gland is the treatment of choice, although therapy with a medication called bromocriptine can also be used for pituitary tumors which secrete prolactin. Hormonal replacement therapy is indicated when glands are removed or secretion is inadequate.

Expectations (prognosis)

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.

Complications

Recurrent tumors may develop.

Calling your health care provider

Call your health care provider you notice symptoms of MEN I.

Prevention

Screening of close relatives of people affected with this disorder is recommended.