Cushing's Syndrome

Treatment Overview

Treatment for Cushing's syndrome depends on the cause.

For Cushing's syndrome caused by long-term corticosteroid medicine use

Never stop taking corticosteroid medicine on your own because it might lead to a life-threatening adrenal crisis. When you take steroids, your adrenal glands stop making cortisol. If you suddenly stop taking your medicine, your adrenal glands may not be able to start making cortisol quickly enough. This can lead to an adrenal crisis and a severe drop in blood pressure. To avoid this, your doctor will want to gradually reduce and then stop your medicine.

Your doctor may change your corticosteroid medicine from a longer-acting steroid (such as prednisone) to a shorter-acting one (such as hydrocortisone). Sometimes corticosteroid medicines can be taken every other day. Either way, the body's normal production of cortisol returns gradually.

If you must continue taking corticosteroid medicine to control another condition, the dosage can sometimes be lowered to reduce symptoms and the risk of complications.

If your doctor and you are trying to reduce the dosage of your medicine and you become ill, contact your doctor immediately.

If reducing the dosage does not make Cushing's syndrome go away, your doctor will perform more tests to look for another cause of your condition.

For Cushing's syndrome caused by pituitary tumors (Cushing's disease)

If you are well enough to have surgery, surgical removal of the pituitary tumor offers the best chance for recovery. The surgery (transsphenoidal adenomectomy) requires great skill and should be performed at a major medical center where teams of doctors specialize in pituitary surgery.

Transsphenoidal adenomectomy is successful:¹

• About 85% of the time if the tumor is less than 1 cm (0.4 in) in diameter.
• About 25% of the time if the tumor is 1 cm (0.4 in) in diameter or larger.

Cushing's disease returns in about 2% of adults and up to 40% of children who have the operation.² Surgery can be repeated, often with good results.

Gamma knife radiosurgery has recently been introduced in the United States. In this technique, many small beams of radiation are focused on the tumor to shrink and destroy it. It does not involve a surgical incision (there is no "knife" involved), and there is minimal damage to surrounding tissue. It can be done as an outpatient and with local anesthesia. Few centers in the United States have gamma knife facilities.

Medicine therapies may be tried if surgery is not possible or has failed.

For Cushing's syndrome caused by adrenal tumors

Doctors almost always recommend surgery to remove benign adrenal tumors that are producing hormones. If a tumor is cancerous, the affected adrenal gland is removed. Although chemotherapy is usually advised, there is no proven long-term treatment for adrenal cancer. On rare occasions, both adrenal glands must be removed. In this case, you would take daily long-term hormone replacement.

Surgery is usually successful if the tumor is not cancerous. But surgery is not as successful for a cancerous tumor if the cancer has spread to other parts of the body.²

If surgery is not possible, medicine therapy may be tried to reduce the tumor's production of cortisol. This includes using ketoconazole (Nizoral), mitotane (Lysodren), and aminoglutethimide (Cytadren). These medicines also are sometimes used before surgery in people with severe Cushing's syndrome. Pregnant women with Cushing's disease can take aminoglutethimide.

For Cushing's syndrome caused by tumors of the lungs and elsewhere

To successfully treat Cushing's syndrome caused by a noncancerous (benign) or cancerous tumor, the tumor tissue that is secreting adrenocorticotropic hormone (ACTH) must be destroyed or removed. Surgery, chemotherapy, radiation therapy, immunotherapy, or a combination of treatments may be recommended to treat the tumor.

If left untreated for a long time, Cushing's syndrome may cause serious problems, including complications from high blood pressure (such as a heart attack or stroke), osteoporosis, or diabetes. The condition also can cause death. Because of these risks, treatment usually begins as soon as possible.

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Author:	Caroline Rea, RN, BS, MS	Last Updated: April 29, 2008
Medical Review:	Caroline S. Rhoads, MD - Internal Medicine David C.W. Lau, MD, PhD, FRCPC - Endocrinology & Metabolism
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