Cushing's syndrome is a condition that leads to excessive levels of cortisol, a type of hormone, in the blood. It is sometimes called hypercortisolism. The condition is not very common and affects only 10 to 15 out of every million Americans each year. Most patients are between 20 and 50. It is more common in women than in men.

The most common cause of Cushing's syndrome is a benign tumor in the pituitary gland (a pituitary adenoma). This form of the syndrome is known as Cushing's disease. The pituitary gland is the master gland of the body. It is responsible for signaling the production of a number of important hormones (like thyroid hormone, cortisol and sex hormones). The pituitary adenoma secretes increased amounts of adrenocorticotropin (ACTH), which causes another set of glands, the adrenal glands, to release excess levels of cortisol into the blood.

As cortisol levels increase, patients begin to notice a variety of symptoms. A key sign of the condition is excessive weight gain, particularly around the midsection of the body, rounded face, and fat deposits on the back of the neck (this is sometimes referred to a buffalo hump). The skin may become fragile and thin and develop purplish stretch marks. Patients bruise easily. Some patients develop bone weakening and may be prone to fractures. Severe fatigue, muscle weakness, high blood pressure, diabetes, depression, and a decrease in both sex drive and fertility may also occur. Women may experience menstrual irregularities and excessive hair growth on the face, neck, chest, abdomen and thighs.

Treating Cushing's Disease

Once the cause is found, the first line of treatment is usually surgery. The procedure is called a transsphenoidal adenomectomy. Doctors make a tiny incision through the nostril (sometimes they go through the upper lip) to access the pituitary gland. The tumor is located and removed.

William Ludlam, M.D., Ph.D., Endocrinologist with Oregon Health & Science University (OHSU), says that once the source of the cortisol overproduction is removed, levels of the hormone may drop to normal. However, the body becomes used to the high levels of the hormone. So patients must take decreasing replacement doses of the hormone until they reach "normal" production levels.

Transsphenoidal adenomectomy is a very delicate procedure and should only be done by doctors with expertise in the surgery. In experienced hands, about 80 percent of patients are successfully cured. If the surgery doesn't completely help, doctors may attempt a second transsphenoidal adenomectomy in case any tumor tissue was missed during the first surgery. Some patients require removal of the adrenal gland. Another option is radiation therapy to the pituitary gland. About 40 to 50 percent of adults who receive radiation for pituitary adenomas see an improvement in symptoms.

Living with Cushing's Disease

Patients with Cushing's disease often have a hard time getting a diagnosis. The symptoms are often vague and can be associated with other problems or decreasing activity and older age. Ludlam says most patients seek help for five years before they receive a correct diagnosis.

In Portland, OR, a group of women with Cushing's disease get together from time to time. They call these occasions "Cushie parties." The women all know the struggles with diagnosis and treatment and have learned to support one another and share their friendships. The women have developed a website to help others around the country. It can be accessed at http://www.cushings-help.com.

The "Cushie party" women have developed a website to help others around the country. It can be accessed at http://www.cushings-help.com.

For general information on Cushing's Syndrome:

National Institute of Diabetes and Digestive and Kidney Diseases

Pituitary Network Association

National Institute of Neurological Disorders and Stroke