Ectopic Cushing 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 - ectopic; Ectopic ACTH syndrome
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:
- Round, red, and full face (moon face)
- Slow growth rate in children
- Weight gain with fat accumulation on the trunk, but fat loss from the arms, legs, and buttocks (central obesity)
Skin changes that are often seen:
- Skin infections
- Purple marks (1/2 inch or more wide) called striae on the skin of the abdomen, thighs, and breasts
- Thin skin with easy bruising
Muscle and bone changes include:
- Backache, which occurs with routine activities
- Bone pain or tenderness
- Collection of fat between the shoulders and above the collar bone
- Rib and spine fractures caused by thinning of the bones
- Weak muscles, especially of the hips and shoulders
Women may have:
- Excess hair growth on the face, neck, chest, abdomen, and thighs
- Periods that become irregular or stop
Men may have:
Other symptoms that may occur include:
Mental changes, such as depression, anxiety, or changes in behavior
- Increased thirst and urination
Exams and Tests
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.
When to Contact a Medical Professional
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.
Ferri FF. Cushing's disease and syndrome. In: Ferri FF, ed. Ferri's Clinical Advisor 2016. Philadelphia, PA: Elsevier Mosby; 2016:385-386.
Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2015;100:2807-2831. 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 Saunders; 2016:chap 15.
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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency
or for the diagnosis or treatment of any medical condition. A licensed medical professional
should be consulted for diagnosis and treatment of any and all medical conditions. Call 911
for all medical emergencies. Links to other sites are provided for information only -- they
do not constitute endorsements of those other sites. © 1997-A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.
Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.