Healthcare Library

Spanish Version
Print-Friendly
Bookmarks
bookmarks-menu

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. This is much more common than ectopic Cushing syndrome.

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:

Symptoms

Ectopic Cushing syndrome tends to have fewer symptoms than classic Cushing syndrome. Most people with any type of 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 stretch marks (1/2 inch 1 centimeter or more wide) called striae on the skin of the abdomen, thighs, upper arms, 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

Body-wide (systemic) problems may include:

  • Type 2 diabetes mellitus
  • High blood pressure
  • High cholesterol and triglycerides

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:

Exams and Tests

The health care provider will perform a physical exam and ask about your symptoms.

Tests that may be done include:

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

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 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; 2016:chap 15.

    • Endocrine glands

      Endocrine 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 glands

      illustration

      • Endocrine glands

        Endocrine 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 glands

        illustration

      Tests for Ectopic Cushing syndrome

       

      Review Date: 5/7/2017

      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, 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. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
      adam.com

       
       
       

       

       

      A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.
      Content is best viewed in IE9 or above, Firefox and Google Chrome browser.

      ways to give

      view all

      news room

      view all

      healthcare library

      view all
      Event Calendar

      Hunterdon Healthcare offers an array of educational events, including childbirth, healthy living and fitness classes.

      LEARN MORE
      Health and Wellness Centers

      Where health and fitness meet to help you stay healthy at every age.

      LEARN MORE
      Heart & Vascular

      Heart and Vascular Services Department brings world-class cardiovascular care to our community.

      LEARN MORE