Multiple Endocrine Neoplasia (MEN) II

Alternative names:

Sipple's syndrome

Definition:

A hereditary disorder in which 2 or more of the following glands develop an overgrowth of normal cells (hyperplasia) or of malignant cells (carcinoma): the thyroid, the adrenal medulla, or the parathyroid.

Causes, incidence, and risk factors:

The cause of multiple endocrine neoplasia II (MEN II) is genetic, but the mechanism is unknown. Tumors of various glands appear in the same person, but not necessarily at the same time. The disorder may occur at any age, and affects men and women equally. The adrenal tumor is a pheochromocytoma and the thyroid tumor is a medullary carcinoma of the thyroid. Risk factors are a family history of MEN II. The incidence is 3 out of 100,000 people.