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Pheochromocytoma Treatment (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. Treatments for pheochromocytoma depend on the stage of the disease and the patient?s age and general health. The following stages are used for pheochromocytoma:

Localized benign 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.

Regional pheochromocytoma

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.)

Metastatic pheochromocytoma

The cancer has spread to other parts of the body.

Recurrent pheochromocytoma

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

Different types of treatment are available for patients with pheochromocytoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. 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.

Three kinds of standard treatment are used:

  • Surgery (taking out the cancer).

  • Radiation therapy (using high-dosex-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).

Treatment in a clinical trial

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. In the following lists of treatments for the different stages, a link to search results for current clinical trials is included for each section. These have been retrieved from NCI's clinical trials database. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

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 U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with localized benign pheochromocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

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 U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with regional pheochromocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

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 U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic pheochromocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

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 U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent pheochromocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

To Learn More About Pheochromocytoma

For more information from the National Cancer Institute about pheochromocytoma, see the following:

  • Pheochromocytoma Home Page

  • Understanding Cancer Series: Gene Testing

For general cancer information and other resources from the National Cancer Institute, see the following:

  • What You Need to Know About? Cancer - An Overview

  • Understanding Cancer Series: Cancer

  • Staging: Questions and Answers

  • Chemotherapy and You: Support for People With Cancer

  • Radiation Therapy and You: Support for People With Cancer

  • Coping with Cancer

  • Support and Resources

  • Cancer Library

  • Information For Survivors/Caregivers/Advocates

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Changes to This Summary (06/18/2008)

The PDQcancer 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.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

  • Information about patients taking part in clinical trials was added to the Treatment Option Overview section.

  • Links to ongoing clinical trials listed in NCI's PDQ Cancer Clinical Trials Registry were added to the treatment sections.

  • A new section called "To Learn More" was added. It includes links to more information about this type of cancer and about cancer in general.

  • The "Get More Information from NCI" section (originally called "To Learn More") was revised.

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. 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. 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).