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Ectopic Cushing syndrome

Ectopic Cushing syndrome is a condition in which a tumor outside the pituitary or adrenal glands produces a hormone called adrenocorticotropic hormone (ACTH).

Alternative Names

Cushing syndrome - ectopic

Causes, incidence, and risk factors

Cushing syndrome is the result of too much glucocorticoid hormones being released over a long period of time. About 15% of Cushing syndrome cases are due to ectopic Cushing syndrome.

Ectopic Cushing syndrome is caused by tumors that release adrenocorticotropic hormone (ACTH). Other causes of Cushing syndrome are:

  • Excess release of ACTH by the pituitary gland
  • Long-term use of corticosteroid drugs (commonly used to treat conditions such as rheumatoid arthritis and asthma )
  • Tumor of the adrenal gland

Tumors that can, in rare cases, release ACTH include:

Symptoms

Ectopic Cushing syndrome tends to have fewer symptoms than classic Cushing syndrome. Weight loss may occur due to cancer.

Symptoms of Cushing syndrome are:

Signs and tests

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 health care provider can discover the problem with cortisol production. Surgery may not be possible in these cases, but the doctor may prescribe drugs to block cortisol production.

Expectations (prognosis)

Surgery to remove the tumor may lead to full recovery, but there is a chance that the tumor will come back.

Complications

The tumor may spread or return after surgery. High cortisol levels may continue.

Calling your health care provider

Call your health care 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

Stewart PM, Krone NP. The adrenal cortex. In: Kronenberg HM, Melmed S, Polonsky KS, Larsen PR. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 15.

Updated: 12/11/2011

Nancy J. Rennert, MD, Chief of Endocrinology & Diabetes, Norwalk Hospital, Associate Clinical Professor of Medicine, Yale University School of Medicine, New Haven, CT. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.


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