Description

What is pheochromocytoma?

Stage Explanation

Stages of pheochromocytoma Localized benign pheochromocytoma Regional pheochromocytoma Metastatic pheochromocytoma Recurrent pheochromocytoma

Treatment Option Overview

How pheochromocytoma is treated Treatment by stage

Localized Benign Pheochromocytoma
Regional Pheochromocytoma
Metastatic Pheochromocytoma
Recurrent Pheochromocytoma
Get More Information From NCI
Changes to This Summary (07/20/2007)
About PDQ

Description

What is pheochromocytoma?

Pheochromocytoma is a rare disease in which tumors form in chromaffin cells of the body. Most pheochromocytomas start inside the adrenal gland (the adrenal medulla) where most chromaffin cells are located. There are two adrenal glands, one above each kidney in the back of the upper abdomen. Cells in the adrenal glands make important hormones that help the body work properly. Usually pheochromocytoma affects only one adrenal gland. Pheochromocytoma may also start in other parts of the body, such as the area around the heart or bladder.

Most tumors that start in the chromaffin cells do not spread to other parts of the body and are not cancer. These are called benign tumors. If a tumor is found, the doctor will need to determine whether it is cancer or benign.

Pheochromocytomas often cause the adrenal glands to make too many hormones called catecholamines. The extra catecholamines cause high blood pressure (hypertension), which can cause headaches, sweating, pounding of the heart, pain in the chest, and a feeling of anxiety. High blood pressure that goes on for a long time without treatment can lead to heart disease, stroke, and other major health problems.

If there are symptoms, a doctor may order blood and urine tests to see if there are extra hormones in the body. A patient may also have a special nuclear medicine scan. A CT scan, an x-ray that uses a computer to make a picture of the inside of a part of the body or an MRI scan, which uses magnetic waves to make a picture of the abdomen, may also be done.

Pheochromocytoma is sometimes part of a condition called multiple endocrine neoplasia syndrome (MEN). People with MEN often have other cancers (such as thyroid cancer) and other hormonal problems.

The chance of recovery (prognosis) depends on how far the cancer has spread, and the patient’s age and general health.

Stage Explanation

Stages of pheochromocytoma

Once pheochromocytoma is found, more tests will be done to see if the tumor has spread. This is called staging. A doctor needs to know the stage of the disease to plan treatment. The following stages are used for pheochromocytoma:

Tumor is found in only one area and has not spread to other tissues. Most pheochromocytomas do not spread to other parts of the body and are not cancer.

Cancer has spread to lymph nodes in the area or to other tissues around the original cancer. (Lymph nodes are small bean-shaped structures that are found throughout the body. They produce and store infection -fighting cells.)

The cancer has spread to other parts of the body.

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the area where it started or in another part of the body.

Treatment Option Overview

How pheochromocytoma is treated

There are treatments for all patients with pheochromocytoma. Three kinds of treatment are used:

• Surgery (taking out the cancer).
• Radiation therapy (using high- dose x-rays or other high-energy rays to kill cancer cells).
• Chemotherapy (using drugs to kill cancer cells).

Surgery is the most common treatment of pheochromocytoma. A doctor may remove one or both adrenal glands in an operation called adrenalectomy. The doctor will look inside the abdomen to make sure all the cancer is removed. If the cancer has spread, lymph nodes or other tissues may also be taken out.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in the vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body.

Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation comes from a machine outside the body (external radiation therapy).

Treatments for pheochromocytoma depend on the stage of the disease, and the patient’s age and overall health. For more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Localized Benign Pheochromocytoma

Treatment will probably be surgery to remove one or both adrenal glands (adrenalectomy). After surgery the doctor will order blood and urine tests to make sure hormone levels return to normal.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with localized benign pheochromocytoma ¹.

Regional Pheochromocytoma

Treatment may be one of the following:

1. Surgery to remove one or both adrenal glands (adrenalectomy) and as much of the cancer as possible. If cancer remains after surgery, drugs will be given to control high blood pressure.
2. External radiation therapy to relieve symptoms (in rare cases).
3. Chemotherapy.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with regional pheochromocytoma ².

Metastatic Pheochromocytoma

Treatment may be one of the following:

1. Surgery to remove as much of the cancer as possible. If cancer remains after surgery, drugs will be given to control high blood pressure.
2. External radiation therapy to relieve symptoms.
3. Chemotherapy.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic pheochromocytoma ³.

Recurrent Pheochromocytoma

Treatment may be one of the following:

1. Surgery to remove as much of the cancer as possible. If cancer remains after surgery, drugs will be given to control high blood pressure.
2. External radiation therapy to relieve symptoms.
3. Chemotherapy.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent pheochromocytoma ⁴.

Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

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There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

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Changes to This Summary (07/20/2007)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made and glossary links were added to this summary.

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site ⁶. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site ⁸. Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

A small gland that makes steroid hormones, adrenaline, and noradrenaline. These hormones help control heart rate, blood pressure, and other important body functions. There are two adrenal glands, one on top of each kidney. Also called suprarenal gland.

The inner part of the adrenal gland (a small organ on top of each kidney). The adrenal medulla makes chemicals such as epinephrine (adrenaline) and norepinephrine (noradrenaline) which are involved in sending nerve signals.

Surgery to remove one or both adrenal glands (a small organ on top of each kidney).

Feelings of fear, dread, and uneasiness that may occur as a reaction to stress. A person with anxiety may sweat, feel restless and tense, and have a rapid heart beat. Extreme anxiety that happens often over time may be a sign of an anxiety disorder.

A noncancerous growth that does not invade nearby tissue or spread to other parts of the body.

The organ that stores urine.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

CIS. The Cancer Information Service is the National Cancer Institute's link to the public, interpreting and explaining research findings in a clear and understandable manner, and providing personalized responses to specific questions about cancer. Access the CIS by calling 1-800-4-CANCER (1-800-422-6237), or by using the LiveHelp instant-messaging service at https://cissecure.nci.nih.gov/livehelp/welcome.asp. Also called CIS.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

Computed tomography scan. A series of detailed pictures of areas inside the body taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computed tomography scan, computerized tomography, computerized axial tomography scan, and CAT scan.

The amount of medicine taken, or radiation given, at one time.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

A type of radiation therapy that uses a machine to aim high-energy rays at the cancer from outside of the body. Also called external-beam radiation therapy.

A blood pressure of 140/90 or higher. High blood pressure usually has no symptoms. It can harm the arteries and cause an increase in the risk of stroke, heart attack, kidney failure, and blindness. Also called hypertension.

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, nuclear magnetic resonance imaging, and NMRI.

An inherited condition that may result in the development of cancers of the endocrine system. There are several types of multiple endocrine neoplasia syndrome, and patients with each type may develop different types of cancer. The altered genes that cause each type can be detected with a blood test. Also called MEN syndrome.

A method of diagnostic imaging that uses very small amounts of radioactive material. The patient is injected with a liquid that contains the radioactive substance, which collects in the part of the body to be imaged. Sophisticated instruments detect the radioactive substance in the body and process that information into an image.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

Tumor that forms in the center of the adrenal gland (gland located above the kidney) that causes it to make too much adrenaline. Pheochromocytomas are usually benign (noncancerous) but can cause high blood pressure, pounding headaches, heart palpitations, flushing of the face, nausea, and vomiting.

The likely outcome or course of a disease; the chance of recovery or recurrence.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. It is important to know the stage of the disease in order to plan the best treatment.

In medicine, a loss of blood flow to part of the brain, which damages brain tissue. Strokes are caused by blood clots and broken blood vessels in the brain. Symptoms include dizziness, numbness, weakness on one side of the body, and problems with talking, writing, or understanding language. The risk of stroke is increased by high blood pressure, older age, smoking, diabetes, high cholesterol, heart disease, atherosclerosis (a build-up of fatty material and plaque inside the coronary arteries), and a family history of stroke.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

Treatment using substances that travel through the bloodstream, reaching and affecting cells all over the body.

Cancer that forms in the thyroid gland (an organ at the base of the throat that makes hormones that help control heart rate, blood pressure, body temperature, and weight). Four main types of thyroid cancer are papillary, follicular, medullary, and anaplastic thyroid cancer. The four types are based on how the cancer cells look under a microscope.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.