Cushing syndrome - ectopic; Ectopic ACTH syndrome; Ectopic Cushing's syndrome DefinitionEctopic Cushing syndrome is a form of Cushing syndrome in which a tumor outside the pituitary gland produces a hormone called adrenocorticotropic hormone (ACTH). CausesCushing 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 of the causes is if there is too much of the hormone ACTH in the blood. ACTH is usually made by the pituitary in small amounts and then signals the adrenal glands to produce cortisol. Sometimes other cells outside the pituitary can make large amounts of ACTH. This is called ectopic Cushing syndrome. Ectopic means that something is occurring in an abnormal place in the body. Ectopic Cushing syndrome is caused by tumors that release ACTH. Tumors that can, in rare cases, release ACTH include:
SymptomsEctopic Cushing syndrome can cause a lot of different symptoms. Some people have many symptoms while others have only a few. 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:
Exams and TestsThe health care provider will perform a physical exam and ask about your symptoms. Tests that may be done include:
TreatmentThe best treatment for ectopic Cushing syndrome is surgery to remove the tumor that is producing too much ACTH. 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 your provider can discover the problem with cortisol production. Surgery may not be possible in these cases. But your provider may prescribe medicines to block cortisol production. Sometimes removal of both adrenal glands is needed if the tumor cannot be found and medicines do not fully block cortisol production. Outlook (Prognosis)Surgery to remove the tumor may lead to full recovery. But there is a chance that the tumor will come back. Possible ComplicationsThe tumor may spread or return after surgery. A high cortisol level may continue. When to Contact a Medical ProfessionalContact your provider if you develop symptoms of Cushing syndrome. PreventionPrompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable. ReferencesNewell-Price JDC, Auchus RJ. The adrenal cortex. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 15. 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 pubmed.ncbi.nlm.nih.gov/26222757/. | |
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Review Date: 5/12/2023 Reviewed By: Sandeep K. Dhaliwal, MD, board-certified in Diabetes, Endocrinology, and Metabolism, Springfield, VA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, 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. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. | |
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Endocrine glands
