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:
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.
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.
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.
National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. National Comprehensive Cancer Network; 2011. Version 1. 2011.
Review Date:
3/14/2012
Reviewed By:
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.
