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Ectopic Cushing syndrome

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).

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 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:

  • Benign carcinoid tumors of the lung
  • Islet cell tumors of the pancreas
  • Medullary carcinoma of the thyroid
  • Small cell tumors of the lung
  • Tumors of the thymus gland

 

Symptoms

 

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:

  • Decreased or no desire for sex
  • Impotence

Other symptoms that may occur include:

  • Mental changes, such as depression, anxiety, or changes in behavior
  • Fatigue
  • Headache
  • 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:

  • 24-hour urine sample to measure cortisol and creatinine levels
  • Blood tests to check ACTH, cortisol, and potassium levels
  • Dexamethasone suppression test
  • Fasting glucose
  • MRI and high resolution CT scans to find the tumor

 

Treatment

 

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.

 

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.

 

 

References

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.

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        Review Date: 10/28/2015

        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.

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