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Cushing's Syndrome

Many of us do not have to worry about our body suffering from prolonged exposure to the stress hormone, cortisol. But what happens to us when we do? How do we know that we are suffering from Cushing's syndrome? And when should you get a urologist's opinion and advice? The following information should help answer these questions.

What happens under normal conditions?

The adrenal glands are endocrine glands that are located on the top of both kidneys. Each adrenal gland is composed of two layers, an inner layer known as the medulla and an outer layer known as the cortex. Normally, the adrenal cortex secretes a variety of steroid hormones including cortisol, which helps the body respond to stress and change. It also regulates salt and water levels that affect blood volume and blood pressure. The secretion of cortisol is regulated by the pituitary gland, which is located inside the head at the base of the brain. Chemical signals from the pituitary are carried by the bloodstream and act on the cells of the adrenal cortex to promote the secretion of cortisol. This chemical messenger is called corticotrophin or adrenocorticotropic hormone (ACTH). When needed, ACTH is secreted by the pituitary and, as a result, cortisol is then secreted into the circulation. When cortisol is no longer required, the high levels of cortisol in the blood are detected by the pituitary. This feedback causes the pituitary to reduce ACTH secretion so that cortisol levels are reduced. The overall system acts like a thermostat and is highly regulated.

What is Cushing's syndrome?

Cushing's syndrome (CS) occurs when there is excessive production of cortisol that is not controlled by the normal regulatory system. It can also be caused by excessive or prolonged use of steroids.

What causes Cushing's syndrome?

Cushing's syndrome (CS) is a rare disorder that can be caused by a number of different problems. The most common cause (65 to 70 percent) is a benign pituitary tumor called an adenoma. Cushing's syndrome due to a pituitary tumor is sometimes called Cushing's disease (named after Dr. Harvey Cushing, who provided the initial disease description). The second leading cause of CS is an adrenal tumor (20 to 30 percent), which may be benign or malignant. Lastly, CS can be produced by a variety of cancers originating in other organs due to over secretion of ACTH (10 to 15 percent). This is termed ectopic ACTH.

What are the symptoms of Cushing's syndrome?

The signs that signal the development of Cushing's syndrome appear slowly in time. The symptoms are subtle. Some patients may only display a few symptoms while others may have a variety of symptoms. The most noticeable symptom would be a change in physical appearance. The face becomes round, rosy and puffy (the Campbell's soup girl), there is abdominal weight gain and development of fatty deposits just below the back of the neck. Patients may experience thinning of the skin, with easy bruising and pink or purple stretch marks. Some patients may develop high blood pressure or diabetes. Since cortisol is secreted in response to the normal waking and sleep pattern, patients may experience sleep disturbance. Others may experience rapid emotional changes. Some patients experience a specific type of muscle weakness that makes it difficult to arise from a seated to standing position. Since the signs and symptoms may develop slowly over the course of years, serial photographs of a person's appearance are often a way to document the changes. Signs of virilization (masculinization) may occur in women.

How is Cushing's syndrome diagnosed?

Since the signs and symptoms of the disease are subtle and may overlap with other disease processes, a trip to the doctor may be necessary to clarify whether Cushing's syndrome (CS) is a possibility. The first step to treatment is making the diagnosis. Therefore, the evaluating doctor must recognize CS as the possible cause for the pattern of signs and symptoms. The most widely used test is measurement of the total amount of cortisol contained in a whole day's urine collection. Medically, this is known as the 24-hour urinary free cortisol test. Another important test is the dexamethasone suppression test where dexamethasone (steroid) pills are given by mouth then blood and urine are collected to assess the levels of cortisol the body. Under normal circumstances, the excessive dexamethasone will suppress the body's ability to secrete cortisol. In the body of a person with CS, the levels of cortisol will not be suppressed and will remain abnormally high.

Once the diagnosis of CS is made, then the cause must be identified. This requires the distinction between pituitary tumor, adrenal tumor or ectopic ACTH. A blood test for the determination of both cortitrophin and cortisol taken in the late afternoon is the next step.. When corticotrophin is very low or undetectable, it indicates an adrenal tumor. A CT scan or MRI usually confirms the diagnosis. Mildly elevated cortitrophin is associated with pituitary tumors. An MRI of the brain can usually confirm this diagnosis. More sophisticated testing may be needed in some cases when the pituitary tumor is so small that it cannot be seen by conventional imaging techniques. Very high levels of ACTH are associated with the ectopic ACTH syndrome. Testing to look for an underlying tumor should be performed.

How is Cushing's syndrome treated?

If Cushing's syndrome is a side effect of taking high doses of prescribed steroid hormones, withdrawing these medicines will allow the body to go back to normal. The ability to reduce or stop the steroids, however, depends on the type of disease being treated and the pattern of response.

Pituitary tumors can be treated with surgery. Usually, an operation can be done through the nose (transsphenoidal surgery). This is performed by a neurosurgeon.

Adrenal tumors can be removed surgically by a urologist. When the adrenal tumor is small (less than six centimeters), the tumor is more likely to be benign. These small tumors can be removed using laparoscopic techniques. Several small cuts are made in the abdomen to pass instruments that dissect and remove the tumor. In less than 5% of operations, the laparoscopic approach may not be feasible and may be converted to an "open" operation. Tumors larger than six centimeters are more likely to be malignant. These tumors are often removed using a larger, open incision. During removal of a large adrenal tumor, adjacent organs such as the spleen, kidney or a portion of the colon may need to be removed to ensure complete tumor removal.

What can be expected after treatment for Cushing's Syndrome?

Recovery from laparoscopic surgery is rapid. Most patients are discharged within one to two hospital days. Full recovery back to normal activity is three to five weeks. There are usually no physical restrictions after recovery from surgery. It takes longer to recover from open surgery with hospital recuperation between five and 10 days and full recovery taking up to eight weeks.

An important distinction for adrenal tumors is whether or not they are malignant. This is important because the prognosis is very different for benign and malignant tumors. Small adrenal tumors are usually benign and are called adenomas. While size is an important criterion, careful pathologic review of the tumor is the only certain way to determine benign and malignant tumors.

The high levels of cortisol secretion from one adrenal gland suppress the function of the other gland. Postoperatively, patients require supplemental steroids to avoid steroid deficiency. The other adrenal usually recovers, but may take up to a year to do so. In 25 percent of patients, the other adrenal gland never recovers and there may be a need to take steroids as replacement treatment.

Malignant tumors are adrenal cancer. The three-year survival rate is 40 percent and the five-year survival rate is 30 percent. These tumors may recur in the abdominal cavity or spread to the liver, lymph nodes, lungs or bones. Therefore, follow-up CT scans and chest X-rays will be needed at regular intervals if this diagnosis is confirmed. If recurrent disease causes symptoms of Cushing's syndrome to return, there are drugs that may be used to control this.

Frequently asked questions:

If a person has been diagnosed with Cushing's syndrome, how normal can they expect their life to be?

The symptoms, disabilities and lifestyle of a person with Cushing's syndrome (CS) depend on the degree of cortisol excess, the duration of the disease, the basic health of the person and especially the type and curability of the syndrome. If it is cured, all of the features of the disease can subside, but this may take as long as two to 18 months. During that time, most people get annoyed and frustrated by the slow improvements in physical changes. The combination of CS and adrenal insufficiency signs and symptoms (e.g., dizziness, weakness, nausea and loss of appetite) decrease as replacement steroid hormones are tapered and adrenal hormone production slowly improves toward normal. Frequent calls and visits to a physician are necessary.

If the CS is incurable, or if medically-induced CS remains, these individuals will have to cope with persistent fatigue, muscle weakness, abdominal and facial weight gain, depression, mood swings and all the other signs and symptoms mentioned earlier. Drugs are available to moderate these residual symptoms. Regular visits to a physician for examinations, blood tests and treatments of infections and complications will be necessary and are often viewed as a severe burden.

What is the difference between Cushing's syndrome and Cushing's disease?

Any condition that causes the adrenal gland to produce excessive cortisol results in the disorder known as Cushing's syndrome (CS). CS is characterized by facial and torso obesity, high blood pressure, stretch marks on the belly, weakness, osteoporosis and facial hair growth in females.

CS has many possible causes, including tumors within the adrenal gland, ectopic cortitrophin produced from cancer such as lung cancer and cortotrophin excessively produced from a pituitary tumor within the brain. Corticotrophin is normally produced by the pituitary gland (located in the center of the brain) to stimulate the adrenal glands' natural production of cortisol, especially in times of stress.

When a pituitary tumor secretes excessive cortitrophin the disorder resulting from this specific form of CS is referred to as Cushing's disease.

As an aside, it should be noted that doctors sometimes describe certain patients with features identical to CS as having "Cushingoid" features. Typically, these features are occurring as side effects of cortisone-related medications, such as prednisone and prednisolone.

Can stress cause Cushing's syndrome?

Stress-related increased cortisol production by the adrenal glands is normal and physiological, and does not lead to Cushing's syndrome.

Links for additional information:

Society for Endocrinology

Reviewed January 2011

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Cushing's Syndrome Glossary
  • abdomen: Also referred to as the belly. It is the part of the body that contains all of the internal structures between the chest and the pelvis.

  • abdominal: in the abdomen, the cavity of this part of the body containing the stomach, intestines and bladder.

  • ACTH: Also known as adrenocorticotropic hormone or corticotrophin. Hormone produced by the pituitary gland that stimulates the adrenal cortex to produce steroid hormones.

  • adenoma: A benign (non-cancerous) tumor that arises in or resembles glandular tissue.

  • adrenal: Glands that reside above the kidneys composed of an inner medulla and an outer cortex.

  • adrenal cancer: Cancer of the adrenal glands.

  • adrenal cortex: The outer layer of the adrenal gland.

  • adrenal gland: One of a pair of small glands, each of which sits on top of one of the kidneys. These glands produce hormones that help control heart rate, blood pressure, the way the body uses food and other vital functions.

  • adrenal tumor: Abnormal mass of tissue or growth of cells in the adrenal glands.

  • adrenocorticotropic hormone: Also known as ACTH. Hormone produced by the pituitary gland that stimulates the adrenal cortex to produce steroid hormones.

  • benign: Not malignant; not cancerous.

  • cancer: An abnormal growth that can invade nearby structures and spread to other parts of the body and may be a threat to life.

  • colon: Large intestine.

  • cortex: The outer layer of an organ.

  • cortisol: Primary stress hormone.

  • cortisone: A hormone secreted by the adrenal gland and used to treat rheumatoid arthritis and allergies.

  • CT scan: Also known as computerized tomography, computerized axial tomography or CT scan. A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images of the body. Shows detailed images of any part of the body, including bones, muscles, fat and organs. CT scans are more detailed than general X-rays.

  • depression: A disorder characterized by feelings of extreme sadness, guilt, helplessness and hopelessness, and thoughts of death.

  • diabetes: A medical disorder of increased blood sugar levels that can cause bladder and kidney problems.

  • diagnosis: The process by which a doctor determines what disease or condition a patient has by studying the patient's symptoms and medical history, and analyzing any tests performed (e.g., blood tets, urine tests, brain scans, etc.).

  • ectopic: Used to describe an organ or body part occurring in a position or form that is not usual.

  • ectopic ACTH: When ACTH is produced outside the pituitary gland usually by a malignant tumor.

  • endocrine: Relating to glands that secrete hormones internally directly into the lymph nodes or bloodstream.

  • endocrine glands: Any gland of the body that secretes hormones directly into the lymph nodes or bloodstream.

  • gland: A mass of cells or an organ that removes substances from the bloodstream and excretes them or secretes them back into the blood with a specific physiological purpose.

  • high blood pressure: Medical term is hypertension.

  • hormone: A natural chemical produced in one part of the body and released into the blood to trigger or regulate particular functions of the body. Antidiuretic hormone tells the kidneys to slow down urine production.

  • incision: Surgical cut for entering the body to perform an operation.

  • infection: A condition resulting from the presence of bacteria or other microorganisms.

  • ions: Electrically charged atoms.

  • kidney: One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.

  • kidneys: One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.

  • laparoscopic: Using an instrument in the shape of a tube that is inserted through the abdominal wall to give an examining doctor a view of the internal organs.

  • liver: A large, vital organ that secretes bile, stores and filters blood, and takes part in many metabolic functions, for example, the conversion of sugars into glycogen. The liver is reddish-brown, multilobed, and in humans is located in the upper right part of the abdominal cavity.

  • lymph: Fluid containing white cells. It can transport bacteria, viruses and cancer cells.

  • lymph nodes: Small rounded masses of tissue distributed along the lymphatic system most prominently in the armpit, neck and groin areas. Lymph nodes produce special cells that help fight off foreign agents invading the body. Lymph nodes also act as traps for infectious agents.

  • malignant: A cancerous growth that is likely to grow and spread which can cause serious disablement or death.

  • malignant tumor: Refers to cancer. A new growth of tissue (tumor) in which the cells multiply uncontrollably, with a potential for spreading from one organ to other parts of the body and unless neutralized will eventually result in death.

  • medulla: The innermost area of an organ.

  • MRI: Also referred to a magnetic resonance imaging. A diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.

  • neurosurgeon: Surgeon who operates on any part of the nervous system.

  • osteoporosis: A disease occurring among women after menopause or in men on hormonal therapy for prostate cancer in which the bones become very porous, break easily and heal slowly. Found in patients with Cushing's syndrome.

  • pathologic: Relating to disease or arising from disease.

  • pituitary: Relating to or produced by the pituitary gland.

  • pituitary gland: The main endocrine gland. It is a small oval shaped structure in the head and it regulates growth, sexual maturing and metabolism.

  • pituitary tumor: Abnormal mass in the pituitary gland.

  • renal: Pertaining to the kidneys.

  • secretion: Process of producing a substance from the cells and fluids within a gland or organ and discharging it.

  • spleen: An organ in the left upper abdomen of humans and other vertebrates that helps to destroy old red blood cells, form lymphocytes and store blood.

  • stent: With regard to treating ureteral stones, a tube inserted through the urethra and bladder and into the ureter. Stents are used to aid treatment in various ways, such as preventing stone fragments from blocking the flow of urine.

  • steroid: An organic fat-soluble compound.

  • transsphenoidal: Surgical procedure performed through a nostril or an opening made below the upper lip.

  • tumor: An abnormal mass of tissue or growth of cells.

  • urge: Strong desire to urinate.

  • urinary: Relating to urine.

  • urine: Liquid waste product filtered from the blood by the kidneys, stored in the bladder and expelled from the body through the urethra by the act of urinating (voiding). About 96 percent of which is water and the rest waste products.

  • urologist: A doctor who specializes in diseases of the male and female urinary systems and the male reproductive system. Click here to learn more about urologists. (Download the free Acrobat reader.)

  • urology: Branch of medicine concerned with the urinary tract in males and females and with the genital tract and reproductive system of males.

  • void: To urinate, empty the bladder.

Cushing's Syndrome Anatomical Drawings

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