Navigate Up

Neurology Center - A-Z Index

#
J
Q
X
Y
Z

Print This Page

Multiple endocrine neoplasia (MEN) II

Multiple endocrine neoplasia, type II (MEN II) is disorder passed down through families in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include:

  • Adrenal gland (about half the time)
  • Parathyroid gland  (20% of the time)
  • Thyroid gland (almost all of the time)

See also: MEN I

Alternative Names

Sipple syndrome; MEN II

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.

A tumor in the adrenal gland is called a pheochromocytoma .

A tumor in the thyroid gland is a medullary carcinoma of the thyroid .

Tumors in the thyroid, adrenal, or parathyroid glands may occur years apart.

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 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. Additional tests are done to determine which hormones are being overproduced.

A physical examination may reveal:

Imaging tests used to identify tumors may include:

Blood tests are used to see how well certain glands in the body are working. They may include:

Other tests or procedures that may be done include:

Treatment

Surgery is needed to remove 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.

If a child is known to carry the RET gene mutation, surgery to remove the thyroid before it becomes cancerous is considered. This should be discussed with a physician who is very familiar with this condition. It would be done at an early age (before age 5) in people with known MEN IIa, and before age 6 months in people with MEN IIb.

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 possible complication.

Calling your health care provider

Call your health care provider if you notice symptoms of MEN II or if someone in your family receives such a diagnosis.

Prevention

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

References

Kronenberg HM. Polyglandular disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 239.

National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. National Comprehensive Cancer Network; 2011. Version 1. 2011.

Updated: 3/14/2012

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. Health Solutions, Ebix, Inc.


©  UPMC | Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by A.D.A.M. Health Solutions. All rights reserved.

For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.

UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.

Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.

For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.

© UPMC
Pittsburgh, PA, USA UPMC.com