Banner image

Ectopic Cushing syndrome

Cushing syndrome - ectopic; Ectopic ACTH syndrome

Find

Learn More

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:

Definition

Ectopic Cushing syndrome is a form of Cushing syndrome in which a tumor outside the pituitary gland produces a hormone called adrenocorticotropic hormone (ACTH).

Exams and Tests

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

Tests that may be done include:

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.

Prevention

Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable.

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:

Other symptoms that may occur include:

  • Mental changes, such as depression, anxiety, or changes in behavior
  • Fatigue
  • Headache
  • Increased thirst and urination

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.

When to Contact a Medical Professional

Call your provider if you develop symptoms of 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.

ADAM QualityA.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

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 9-1-1 for all medical emergencies. Links to other sites are provided for information only—they do not constitute endorsements of those other sites. © 1997-2010 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.