Sipple syndrome; MEN II
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.
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.
The spread of cancerous cells is a possible complication.
Call your health care provider if you notice symptoms of MEN II or if someone in your family receives such a diagnosis.
Kronenberg HM. Polyglandular disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 250.
National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. National Comprehensive Cancer Network; 2009. Version 2.2009.
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