Multiple endocrine neoplasia II (MEN II) is an uncommon condition passed down through families (inherited) marked by overactivity of the thyroid, adrenal, and parathyroid glands.
Alternative Names:
Sipple's syndrome
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 type of adrenal tumor is 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.
The symptoms may vary, but are consistent with those of pheochromocytoma, medullary carcinoma of the thyroid, or sometimes hyperparathyroidism.
Signs and tests:
Diagnosis depends on identification of a mutation in the RET gene. This can be done with a blood test.
A physical examination may reveal enlarged cervical (neck) lymph nodes. An examination of the thyroid may reveal one or more thyroid nodules. The patient may have high blood pressure (continual or sporadic), rapid heart rate and fever.
Patients with MEN IIb may have benign tumors (neuromas) of the mucosa, as well as puffy lips and a prominent jaw.
Diagnostic tests are used to evaluate the function of each endocrine gland. The following tests help confirm the diagnosis:
Review Date: 10/25/2006
Reviewed By: Robert Hurd, M.D., Department of Biology, College of Arts and Sciences, Xavier University, Cincinnati, OH. Review provided by VeriMed Healthcare Network.
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