Wermer syndrome; MEN I
Definition of Multiple endocrine neoplasia (MEN) I
Multiple endocrine neoplasia (MEN) type I is an inherited disorder in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include:
Causes, incidence, and risk factors
MEN I is caused by a defect in a gene that carries the code for a protein called menin. The condition causes tumors of various glands to appear in the same person, but not necessarily at the same time.
Symptoms vary from person to person, and depend on which gland is involved. They may include:
Signs and tests
Signs may include:
Surgery to remove the diseased gland is the treatment of choice. A medication called bromocriptine may be used instead of surgery for pituitary tumors that release the hormone prolactin.
Pituitary and parathyroid tumors are usually noncancerous (), but some pancreatic tumors may become cancerous (malignant) and spread to the liver. These can lower life expectancy.
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 3/2/2010