Cushing’s Disease

About Cushing’s disease

Cushing’s disease is a rare, but serious, endocrine disorder caused by a noncancerous tumor on the pituitary gland at the base of the brain that ultimately leads to the production of excess cortisol in the body. The onset of Cushing’s disease symptoms is gradual and often mistaken for other medical conditions, and as a result, the average time to diagnosis is 6 years.

Cortisol is a vital hormone that regulates metabolism and cardiovascular function and helps the body respond to stress. Excess levels of cortisol can lead to severe physical and physiological changes in the body, including uncontrollable weight gain, central obesity (abdomen that sticks out with thin arms and legs), a round, red full face, buffalo hump (collection of fat on the back of the neck), thin skin and easy bruising, excess body and facial hair, interrupted menstrual cycle, and purple stretch marks (striae) on the abdomen, breasts, and thighs.

Elevated cortisol levels may also put patients at risk for serious health complications, including osteoporosis and bone fractures, diabetes and hyperglycemia, cardiovascular disease, hypertension (high blood pressure), kidney stones, and blood clotting disorders.

Treating Cushing’s disease

The main goal of treatment is to control excess cortisol production from the pituitary gland. Treatment depends on the specific reason for excess cortisol and can include the following options:

• Surgery: The goal of surgery is to remove the pituitary tumor and, consequently, control cortisol production. For many patients, surgery improves cortisol levels, but does not always return them to normal, so additional treatment may be required. Additionally, many patients who achieve biochemical control following surgery can experience recurrence and may require further treatment.
• Medical therapy: There are different medical treatments available for patients with Cushing’s disease, which can reduce cortisol production.
• Radiation therapy: Radiation therapy is commonly used following surgery in cases where parts of the tumor are still present and medications are not controlling the disease. Patients might not receive the full effects of this therapy for many years.
• Bilateral adrenalectomy: In some cases, if other treatments fail, surgical removal of the adrenal glands, called bilateral adrenalectomy, may replace drug therapy. 

Patients should work closely with their doctors to determine which treatment approach is the most appropriate for them. It is also critical for Cushing’s disease patients to regularly monitor their hormone levels to identify recurrence in a timely manner, as even after surgery, up to 35% of patients will experience disease recurrence. Therefore, it is important to monitor and test hormone levels every 6 months, as even those who do not notice signs or symptoms of recurrence may still be affected.

Questions to ask your doctor

If you have recently been diagnosed with Cushing’s disease, be sure to speak with your doctor about questions you may have about managing the disease:

• What are my treatment options?
• Am I a candidate for surgery?
• Will I need additional treatment after surgery?
• What are the advantages and disadvantages of each treatment option?
• Do I need treatment right away?
• If left untreated, what is my risk of developing serious complications such as diabetes and heart disease?
• What is the likelihood that the treatment you recommend will:
• Normalize my cortisol levels?
• Control the tumor mass without harming normal pituitary function?
• Relieve the signs and symptoms?
• Improve my life expectancy?
• How quickly will I experience relief of my symptoms with each treatment?
• How is each treatment administered?
• How will each treatment affect my daily life?
• How will you be monitoring my condition (ie, pituitary tumor size, cortisol levels, and symptoms)?
• Where can I go for more information?