Multiple Endocrine Neoplasia (MEN) I

Endocrine glands
Definition:
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:
Symptoms vary greatly from person to person and may be related to peptic ulcer disease,
hypoglycemia, hypercalcemia, or pituitary dysfunction.

Abdominal pain
Burning, aching, or hunger discomfort in the upper abdomen or lower chest that is
relieved by antacids, milk, or food

Black, tarry stools

Nausea and vomiting

Bloated feeling after meals

Weakness

Headache

Vision problems

Loss of coordination

Anxiety

Mental changes or confusion

Coma if hypoglycemia is untreated

Loss of appetite

Muscle pain

Fatigue

Sensitivity to the cold

Unintentional weight loss

Low blood pressure

Loss of axillary or pubic hair

In women, cessation of menses, infertility, or failure to lactate

In men, decreased sexual interest, loss of body or facial hair

Signs and tests:
Diagnostic tests are used to evaluate the function of each endocrine gland.

Pancreatic evaluation may reveal the following signs:

MRI of abdomen shows a pancreatic tumor.

CT of abdomen shows a pancreatic tumor.

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:

Serum calcium level is elevated, and the serum parathyroid hormone is increased.
Parathyroid biopsy shows tumor or hyperplasia
Pituitary evaluation may reveal the following signs:

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

Serum adrenocorticotropic hormone

Serum luteinizing hormone

Serum follicle stimulating hormone

Additional testing may be needed.

Treatment:
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.
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.
Review Date: 11/9/2004
Reviewed By: Marcia S. Brose, M.D., Ph.D., Assistant Professor, Hematology/Oncology, The
University of Pennsylvania Cancer Center, Philadelphia, PA. Review provided by VeriMed
Healthcare Network.