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Endocrine glands
Endocrine glands


Definition:

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.

See also: MEN I



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 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.



Symptoms:

The symptoms may vary. However, they are similar to those of:



Signs and tests:

To diagnose this condition, the health care provider looks for a mutation in the RET gene. This can be done with a blood test.

A physical examination may reveal:

Diagnostic tests are used to evaluate the function of each endocrine gland. The following tests may be performed:



Treatment:

Surgery is needed to remove both the medullary carcinoma of the thyroid and the pheochromocytoma. For medullary carcinoma of the thyroid, the thyroid gland and surrounding lymph nodes must be totally removed. Thyroid hormone replacement therapy is given after surgery.

Surgery to remove the thyroid before it becomes cancerous is often being performed at an early age (before age 5) in people with known MEN IIa, and before age 6 months in people with MEN IIb.

Family members should be screened for the RET gene mutation.



Support Groups:



Expectations (prognosis):

Pheochromocytoma is usually not cancerous (benign). Medullary carcinoma of the thyroid is a very aggressive and potentially fatal cancer. However, early diagnosis and surgery can often lead to a cure.



Complications:

The spread of cancerous cells is a complication.



Calling your health care provider:

Call your health care provider if you notice symptoms of MEN II.



Prevention:

Screening close relatives of people with MEN II may lead to early detection of the syndrome and related cancers.



References:

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




Review Date: 9/4/2008
Reviewed By: 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.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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