Learn more about specialized surgery techniques available for pheochromocytoma at UPMC Cancer Centers
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.
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:
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.
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 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).
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.
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.
Treatment may be one of the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with regional pheochromocytoma.
Treatment may be one of the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic pheochromocytoma.
Treatment may be one of the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent pheochromocytoma.
For more information from the National Cancer Institute about pheochromocytoma, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
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.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
Search the NCI Web site
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.
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.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
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.
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:
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