Cushing syndrome - ectopic; Ectopic ACTH syndrome
Ectopic Cushing syndrome is a form of Cushing syndrome in which a tumor outside the pituitary gland produces a hormone called adrenocorticotropic hormone (ACTH).
Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the hormone cortisol. This hormone is made in the adrenal glands. Too much cortisol can be due to various problems. One such problem is called Cushing disease. It occurs when the pituitary gland makes too much of the hormone ACTH. ACTH then signals the adrenal glands to produce too much cortisol. This is much more common than ectopic Cushing syndrome.
Ectopic Cushing syndrome, on the other hand, occurs when ACTH is produced somewhere other than the pituitary gland. Ectopic means occurring in an abnormal place.
Ectopic Cushing syndrome is caused by tumors that release ACTH. Tumors that can, in rare cases, release ACTH include:
Ectopic Cushing syndrome tends to have fewer symptoms than classic Cushing syndrome. Most people with any type of Cushing syndrome have:
Skin changes that are often seen:
Muscle and bone changes include:
Body-wide (systemic) problems may include:
Women may have:
Men may have:
Other symptoms that may occur include:
The health care provider will perform a physical exam and ask about your symptoms.
Tests that may be done include:
The best treatment for ectopic Cushing syndrome is surgery to remove the tumor. Surgery is usually possible when the tumor is noncancerous (benign).
In some cases, the tumor is cancerous and spreads to other areas of the body before the doctor can discover the problem with cortisol production. Surgery may not be possible in these cases. But the doctor may prescribe medicines to block cortisol production.
Surgery to remove the tumor may lead to full recovery. But there is a chance that the tumor will come back.
The tumor may spread or return after surgery. A high cortisol level may continue.
Call your provider if you develop symptoms of Cushing syndrome.
Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable.
Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing's syndrome: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2015;100(8):2807-2831. PMID 26222757 www.ncbi.nlm.nih.gov/pubmed/26222757.
Stewart PM, Newell-Price JDC. The adrenal cortex. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 13th ed. Philadelphia, PA: Elsevier; 2016:chap 15.BACK TO TOP
Review Date: 5/7/2017
Reviewed By: Brent Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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