Ectopic Cushing syndrome

  • 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).

  • Alternative Names
    • Cushing syndrome - ectopic; Ectopic ACTH syndrome

  • 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:

  • 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 1 centimeter 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:

      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
  • 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

      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.