BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuEctopic Cushing syndromeCushing syndrome - ectopic; Ectopic ACTH syndromeEctopic Cushing syndrome is a form of Cushing syndrome in which a tumor outside the pituitary gland produces a hormone called adrenocorticotropic hormone (ACTH).Cushing syndromeCushing syndrome is a disorder that occurs when your body has a high level of the hormone cortisol.ImageRead Article Now Book Mark Article Causes 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 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.Adrenal glandsThe adrenal glands are two small triangle-shaped glands. One gland is located on top of each kidney.ImageRead Article Now Book Mark Article Ectopic Cushing syndrome is caused by tumors that release ACTH. Tumors that can, in rare cases, release ACTH include:TumorsA tumor is an abnormal growth of body tissue. Tumors can be cancerous (malignant) or noncancerous (benign).Read Article Now Book Mark Article Benign carcinoid tumors of the lung Islet cell tumors of the pancreas Islet cell tumorsA pancreatic islet cell tumor is a rare tumor of the pancreas that starts from a type of cell called the islet cell.ImageRead Article Now Book Mark Article Medullary carcinoma of the thyroid Medullary carcinoma of the thyroidMedullary carcinoma of the thyroid is cancer of the thyroid gland that starts in cells that release a hormone called calcitonin. These cells are cal...ImageRead Article Now Book Mark Article Small cell tumors of the lung Small cell tumors of the lungSmall cell lung cancer (SCLC) is a fast-growing type of lung cancer. It spreads much more quickly than non-small cell lung cancer. There are two typ...ImageRead Article Now Book Mark Article Tumors of the thymus gland Symptoms Ectopic 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:Round, red, and full face (moon face) Moon faceFacial swelling is the buildup of fluid in the tissues of the face. Swelling may also affect the neck and upper arms.ImageRead Article Now Book Mark Article 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 stretch marks (1/2 inch 1 centimeter or more wide) called striae on the skin of the abdomen, thighs, upper arms, and breasts StriaeStretch marks are irregular areas of skin that look like bands, stripes, or lines. Stretch marks are seen when a person grows or gains weight rapidl...ImageRead Article Now Book Mark Article 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 Thinning of the bonesOsteoporosis is a disease in which bones become fragile and more likely to break (fracture).ImageRead Article Now Book Mark Article Weak muscles, especially of the hips and shoulders Body-wide (systemic) problems may include:Type 2 diabetes mellitus High blood pressure High cholesterol and triglyceridesWomen may have:Excess hair growth on the face, neck, chest, abdomen, and thighs Periods that become irregular or stop Men may have: Decreased or no desire for sex ImpotenceImpotenceAn erection problem occurs when a man cannot get or keep an erection that is firm enough for intercourse. You may not be able to get an erection at ...ImageRead Article Now Book Mark Article Other symptoms that may occur include: Mental changes, such as depression, anxiety, or changes in behavior Fatigue FatigueFatigue is a feeling of weariness, tiredness, or lack of energy.Read Article Now Book Mark Article Headache Increased thirst and urinationThirstExcessive thirst is an abnormal feeling of always needing to drink fluids.ImageRead Article Now Book Mark Article UrinationExcessive amount of urination means that your body makes larger than normal amounts of urine each day.ImageRead Article Now Book Mark Article Exams and Tests The health care provider will perform a physical exam and ask about your symptoms. Tests that may be done include:24-hour urine sample to measure cortisol and creatinine levels CreatinineThe creatinine blood test measures the level of creatinine in the blood. This test is done to see how well your kidneys are working. Creatinine can ...ImageRead Article Now Book Mark Article Blood tests to check ACTH, cortisol, and potassium levels (often very low in ectopic Cushing syndrome) CortisolThe cortisol blood test measures the level of cortisol in the blood. Cortisol is a steroid (glucocorticoid or corticosteroid) hormone produced by th...Read Article Now Book Mark Article PotassiumThis test measures the amount of potassium in the fluid portion (serum) of the blood. Potassium (K+) helps nerves and muscles communicate. It also ...ImageRead Article Now Book Mark Article Dexamethasone suppression test (both high and low dose) Dexamethasone suppression testDexamethasone suppression test measures whether adrenocorticotrophic hormone (ACTH) secretion by the pituitary can be suppressed.Read Article Now Book Mark Article Inferior petrosal sinus sampling (a special test that measures ACTH from veins near the brain and in the chest) Fasting glucose Fasting glucoseA blood sugar test measures the amount of a sugar called glucose in a sample of your blood. Glucose is a major source of energy for most cells of the...ImageRead Article Now Book Mark Article MRI and high resolution CT scans to find the tumor (sometimes nuclear medicine scans may be needed)MRIA magnetic resonance imaging (MRI) scan is an imaging test that uses powerful magnets and radio waves to create pictures of the body. It does not us...ImageRead Article Now Book Mark Article CT scansA computed tomography (CT) scan is an imaging method that uses x-rays to create pictures of cross-sections of the body. Related tests include:Abdomin...ImageRead Article Now Book Mark Article Treatment The best treatment for ectopic Cushing syndrome is surgery to remove the tumor. Surgery is usually possible when the tumor is noncancerous (benign).BenignBenign refers to a condition, tumor, or growth that is not cancerous. This means that it does not spread to other parts of the body. It does not in...ImageRead Article Now Book Mark Article 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.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 Complications 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. Prevention Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable.Open ReferencesReferencesNewell-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/.AllVideoImagesTogEndocrine glands - illustration Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).Endocrine glandsillustrationEndocrine glands - illustration Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).Endocrine glandsillustration Tests for Ectopic Cushing syndrome Dexamethasone suppression testRelated Information ACTH blood test(Medical Test)Tumor(Condition)Adrenal glands(Special Topic)Cushing syndrome(Condition)Rheumatoid arthritis(Condition)Asthma(Condition)Pancreatic islet cell tumor(Condition)Thyroid cancer - medullary carcinoma(Condition)Pheochromocytoma(Condition)Rheumatoid arthritis(In-Depth)Asthma in adults(In-Depth) Review Date: 5/13/2021 Reviewed By: Brent Wisse, MD, Board Certified in Metabolism/Endocrinology, 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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