Cushing Syndrome

What is Cushing syndrome?

Cushing syndrome is also called hypercortisolism (hi-per-KOR-ti-sol-izm). It is where your body has too much of a hormone called cortisol. Cortisol is produced in the outer part of the adrenal glands in the body. The adrenal glands are located above each kidney. The amount of cortisol produced is controlled by another hormone called adrenocorticotrophic hormone or ACTH. ACTH is made in the pituitary gland, which lies just below the brain. Cortisol helps your body respond to stress and keeps blood sugar and blood pressure levels normal.

Normally, the adrenal or pituitary glands produce the correct amount of cortisol and ACTH in your body. In Cushing syndrome, too much cortisol is released in the body for long periods of time. This condition usually affects women more than men. It usually affects people between the ages of 20 and 50 years old. If the cause of Cushing syndrome is discovered, and treatment begins as soon as possible, Cushing syndrome can be cured in some people.

What causes Cushing syndrome?

Adrenal gland diseases:

Adrenal glands that make too many cells and grow larger than normal because of a disease may cause Cushing syndrome. Another cause for Cushing syndrome is a cancer tumor on an adrenal gland.

Cancers:

Lung cancer may also make ACTH, causing the adrenal gland to make too much cortisol. This condition is known as ectopic ACTH syndrome.

Pituitary gland tumors:

These are small, benign (non-cancerous) tumors in the pituitary gland which make too much ACTH, leading to too much cortisol. Cushing syndrome caused by a benign pituitary tumor is called Cushing's Disease.

Steroid medicine:

Long-term use of steroid medicine such as prednisone may cause Cushing syndrome.

What are the signs and symptoms of Cushing syndrome?

Cushing syndrome includes all of these four signs and symptoms:

Increased fat around the neck and collar areas.

Puffiness of face. Your face may look rounded and often redder than usual.

Extra fat around your trunk area and thinning of your arms and legs.

Thin skin with purplish-pink stretch marks on your abdomen (stomach), thighs, buttocks, arms, and breasts.

You may also have any of the following:

Fatigue (more tired than usual) and muscle weakness.

High blood sugar or high blood pressure.

Excess hair growth on the face, neck, chest, abdomen, and thighs of women. A woman's monthly period may become irregular or stop.

Mood swings, such as being irritable, depressed, or anxious.

Little to no interest in having sexual intercourse (sex). Men may have a problem getting or keeping an erection during sex.

How is Cushing syndrome diagnosed?

You may have one or more of the following tests:

24-hour urinary free cortisol level test:

This measures the amount of cortisol in your urine over 24 hours. Ask your caregiver for more information if you need to have this test.

Blood tests:

Many different tests will be done on your blood.

Imaging tests:

Computerized tomography scan:

This is also called a CT scan. A special x-ray machine uses a computer to take pictures of your head, chest, abdomen, or hip area. Before taking the pictures, you may be given dye through an IV in your vein. The dye helps cancer show up better in the pictures. People who are allergic to shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your caregiver if you are allergic to any of these.

Magnetic resonance imaging test:

This test is also called an MRI. During the MRI, computerized pictures are taken of your body using radio waves. Caregivers use these pictures to look for cancers of your lungs or pituitary or adrenal glands. You will need to lie still during a MRI. Never enter the MRI room with an oxygen tank, watch, or any other metal objects. Doing this can cause serious injury. Tell your caregiver if you have any metal implants in your body.

Late night salivary cortisol test:

This test is done at 11 PM on two separate nights to measure the amount of cortisol in your saliva. Glands in your mouth make saliva. Saliva is the liquid (spit) that you regularly swallow.

How is Cushing syndrome treated?

Treatment depends on why you have too much cortisol in your body. Caregivers may change your steroid dose if the cause is long-term steroid use to treat another condition. You may also have one or more of the following:

Surgery:

Surgery to remove the tumor that causes increased cortisol is the main treatment for Cushing syndrome.

Chemotherapy:

This medicine, often called chemo, is used to treat cancer. It works by killing tumor cells. Chemotherapy may also be used to shrink lymph nodes that have cancer in them. Once the tumor is smaller, you may need surgery to cut out the rest of the cancer.

Many different chemotherapy medicines are used to treat cancer. You may need blood tests often. These blood tests show how your body is doing and how much chemotherapy is needed. Chemotherapy can have many side effects. Caregivers will watch you closely and will work with you to decrease side effects. Chemotherapy can cure some cancers. Even if the chemotherapy does not cure your cancer, it may help you feel better or live longer.

Cortisol-inhibiting medicines:

Adrenal-acting inhibitors:

These medicines block the adrenal glands from making cortisol. The effects of cortisol can be changed with this medicine.

Glucocorticoid antagonist:

These medicines decrease the amount of cortisol made by your body.

Radiation:

This is a treatment using x-rays or gamma rays to treat cancer. Radiation kills cancer and keeps the cancer from spreading. It also keeps cancer cells from dividing into new cells, which is one way cancer spreads. Lymph nodes with cancer are also treated with radiation. In some cancers, radiation may be given after surgery to kill cancer cells that were not removed. It may also be given with chemotherapy. Radiation may help decrease pain, control bleeding, and shrink the tumor.

Where can I get support and more information?

Cushing syndrome is a life-changing condition for you and your family. Accepting that you have Cushing syndrome is hard. You and those close to you may feel scared, angry, or sad. These are normal feelings. Talk to your caregivers, family, or friends about your feelings. You may also want to join a support group. This is a group of people who also have Cushing syndrome. Contact the following for more information:

National Digestive Diseases Information Clearinghouse (NDDIC)
2 Information Way
Bethesda, MD 20892-3570
800-891-5389
www.digestive.niddk.nih.gov

Cushing's Support and Research Foundation
65 E India Row, Suite 22B
Boston, MA 02110
617-723-3674
http://csrf.net/