Cushing syndrome – ectopic; Ectopic ACTH syndrome
Ectopic Cushing syndrome is a form of Cushing syndrome in which a tumor outside the pituitary or adrenal glands produces a hormone called adrenocorticotropic hormone (ACTH).
Other forms of Cushing syndrome include:
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.
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 Cushing syndrome have:
Skin changes that are often seen:
Muscle and bone changes 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 health care provider if you develop symptoms of Cushing syndrome.
Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable.
McGee S. Cushing syndrome. In: Evidence-Based Physical Diagnosis. 3rd ed. Philadelphia, PA: Elsevier Saunders. 2012:chap 13.
Stewart PM, Krone NP. The adrenal cortex. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 15.
Susmeeta TS, Nieman LK. Cushing's syndrome: all variants, detection, and treatment. Endocrinol Metab Clin N Am. 2011;40:379-91. PMID: 21565673 www.ncbi.nlm.nih.gov/pubmed/21565673.BACK TO TOP
Review Date: 11/7/2013
Reviewed By: Brent Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
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