Cushing's Syndrome

Treatment Overview

Treatment for Cushing's syndrome depends on the underlying cause.

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

Never stop taking corticosteroid medication 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 medication, 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 health professional will want to gradually reduce and then stop your medication.

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

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

Contact your health professional immediately if you and your health professional are trying to reduce the dosage of your medication and you become ill.

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

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

Surgical removal of the pituitary tumour offers the best chance for recovery, if you are well enough to have surgery. The surgery (transsphenoidal adenomectomy) requires great skill and should be performed at a major medical centre where teams of health professionals specialize in pituitary surgery.

Transsphenoidal adenomectomy is successful:¹

• About 85% of the time if the tumour is less that 1 cm (0.4 in.) in diameter.
• About 25% of the time if the tumour is 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 tumour 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 centres in the United States have gamma knife facilities. This treatment is not yet available in Canada.

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

For Cushing's syndrome caused by adrenal tumours

Health professionals almost always recommend surgery to remove benign adrenal tumours that are producing hormones. If a tumour 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 tumour is not cancerous. However, surgery is not as successful for a cancerous tumour if the cancer has spread to other parts of the body.²

If surgery is not possible, medication therapy may be tried to reduce the tumour's production of cortisol. This includes using ketoconazole and mitotane (Lysodren). These medications 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 tumours of the lungs and elsewhere

To successfully treat Cushing's syndrome caused by a non-cancerous (benign) or cancerous tumour, the tumour 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 tumour.

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:	Douglas Dana Robin Parks, MS	Last Updated: August 28, 2006
Medical Review:	Adam Husney, MD - Family Medicine Caroline S. Rhoads, MD - Internal Medicine David C.W. Lau, MD, PhD, FRCPC - Endocrinology & Metabolism
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