Cushing syndrome due to adrenal tumor

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 pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

Also known as: Adrenal tumor - Cushing syndrome

Definition

Cushing syndrome due to adrenal tumor is an endocrine system disorder that occurs when there is a tumor of the adrenal gland releasing excess amounts of the hormone cortisol.

Causes, incidence, and risk factors

Cushing syndrome is caused by constant, high levels of the steroid hormone cortisol. A tumor on one of the adrenal glands causes about 15% of all cases of Cushing syndrome. Adrenal tumors release cortisol.

Adrenal tumors may be noncancerous (benign) or cancerous (malignant).

Noncancerous tumors that may cause Cushing syndrome include:

Adrenal adenomas
Micronodular hyperplasia

Cancerous tumors that may cause Cushing syndrome include:

Adrenal carcinomas

Adrenal tumors are rare. They may develop in anyone at any age, but are much more common in adults. They are found in women more often than men.

Symptoms

Symptoms usually include:

Upper body obesity (above the waist) and thin arms and legs
Round, red, full face (moon face)
Slow growth rate in children

Skin changes that are often seen:

Acne or 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 (buffalo hump)
Thinning of the bones, which leads to rib and spine fractures
Weak muscles

Women often have:

Excess hair growth on the face, neck, chest, abdomen, and thighs
Menstrual cycle becomes irregular or stops

Men may have:

Decreased fertility
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
High blood pressure
Increased thirst and urination

Signs and tests

Tests to confirm Cushing syndrome:

24-hour urine sample to measure the following:
- Urine cortisol
- Urine creatinine
Dexamethasone suppression test
Serum cortisol levels

Tests to determine cause:

Abdominal CT
ACTH
High-dose dexamethasone suppression test

Other findings may include:

High cholesterol
High fasting glucose
Low bone density, seen by dual x-ray absorptiometry (DEXA)
Low serum potassium

Treatment

Cushing syndrome due to an adrenal tumor is treated with surgery to remove the tumor and often the entire adrenal gland.

Glucocorticoid replacement treatment is usually needed until the other adrenal gland recovers from surgery. You may need this treatment for 9 - 12 months.

If surgery is not possible (such as in cases of adrenal cancer), medicines can be used to stop the release of cortisol. These include:

Aminoglutethimide
Ketoconazole
Mifepristone
Mitotane

Radiation therapy usually does not work for cancerous adrenal tumors and is not appropriate for noncancerous tumors.

Expectations (prognosis)

Patients with an adrenal tumor who have surgery have an excellent outlook. Surgery success rates are very high with this type of tumor.

For adrenal cancer, surgery is sometimes not possible. When surgery is performed, it does not always cure the cancer.

Complications

Cancerous adrenal tumors can spread to the liver or lungs.

Calling your health care provider

Call your health care provider if you develop any symptoms of Cushing syndrome.

Prevention

Appropriate treatment of adrenal tumors may reduce the risk of complications in some patients with tumor-related Cushing syndrome.

References

Stewart PM. The adrenal cortex. In: Kronenberg HM, Melmed S, Polonsky KS, Larsen PR. Williams Textbook of Endocrinology. 11th ed. Philadelphia, PA: Elsevier; 2008:chap 14.

Review date:: November 23, 2009
Reviewed by:: Ari S. Eckman, MD, Division of Endocrinology and Metabolism, Johns Hopkins School of Medicine, Baltimore, MD. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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