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Multiple endocrine neoplasia (MEN) II - Overview

Alternative Names

Sipple's syndrome

Definition of Multiple endocrine neoplasia (MEN) II:

Multiple endocrine neoplasia II (MEN II) is an uncommon condition passed down through families (inherited), in which the thyroid, adrenal, and parathyroid glands are overactive.

Causes, incidence, and risk factors:

The cause of MEN II is a defect in a gene called RET. This defect causes many tumors to appear in the same person, but not necessarily at the same time. The adrenal tumor is called a pheochromocytoma, and the thyroid tumor is a medullary carcinoma of the thyroid.

The disorder may occur at any age, and affects men and women equally. The main risk factor is a family history of MEN II.

There are two similar subtypes of MEN II -- MEN IIa and IIb. MEN IIb is less common.

Endocrine glands

Reviewed last on: 9/4/2008
Sean O. Stitham, MD, private practice in Internal Medicine, Seattle, Washington; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Kronenberg HM. Plyglandular disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 250.

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