Paragangliomas form in nerve tissue in the adrenal glands and near certain blood vessels and nerves. Paragangliomas that form in the adrenal glands are called pheochromocytomas. Paragangliomas that form outside the adrenal glands are called extra-adrenal paragangliomas. In this summary, extra-adrenal paragangliomas are called paragangliomas.
Pheochromocytomas and paragangliomas may be benign (not cancer) or malignant (cancer).Pheochromocytoma is a rare tumor that forms in the adrenal medulla (the center of the adrenal gland).
Pheochromocytoma forms in the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla.
Pheochromocytoma is a rare tumor of the adrenal medulla. Usually, pheochromocytoma affects one adrenal gland, but it may affect both adrenal glands. Sometimes there is more than one tumor in one adrenal gland.
The adrenal glands make important hormones called catecholamines. Adrenaline (epinephrine) and noradrenaline (norepinephrine) are two types of catecholamines that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Sometimes a pheochromocytoma will release extra adrenaline and noradrenaline into the blood and cause signs or symptoms of disease.Paragangliomas form outside the adrenal gland.
Paragangliomas are rare tumors that form near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of these extra catecholamines into the blood may cause signs or symptoms of disease.
Paraganglioma of the head and neck. A rare tumor that often forms near the carotid artery. It may also form along nerve pathways in the head and neck and in other parts of the body.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor doesn't mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk to your doctor if you think you may be at risk.
The following inherited syndromes or gene changes increase the risk of pheochromocytoma or paraganglioma:
Some tumors do not make extra adrenaline or noradrenaline and do not cause signs and symptoms. These tumors are sometimes found when a lump forms in the neck or when a test or procedure is done for another reason. Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma and paraganglioma or by other conditions. Check with your doctor if you have any of the following:
The most common sign is high blood pressure. It may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death.Signs and symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events.
Signs and symptoms of pheochromocytoma and paraganglioma may occur when one of the following events happens:
The following tests and procedures may be used:
All patients who are diagnosed with pheochromocytoma or paraganglioma should have genetic counseling to find out their risk for having an inherited syndrome and other related cancers.
Genetic testing may be recommended by a genetic counselor for patients who:
Genetic testing is sometimes recommended for patients with pheochromocytoma who:
When certain gene changes are found during genetic testing, the testing is usually offered to family members who are at risk but do not have signs or symptoms.
Genetic testing is not recommended for patients older than 50 years.Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
The extent or spread of cancer is usually described as stage. It is important to know whether the cancer has spread in order to plan treatment. The following tests and procedures may be used to determine if the tumor has spread to other parts of the body:
Cancer can spread through tissue, the lymph system, and the blood:
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if pheochromocytoma spreads to the bone, the cancer cells in the bone are actually pheochromocytoma cells. The disease is metastatic pheochromocytoma, not bone cancer.There is no standard staging system for pheochromocytoma and paraganglioma.Pheochromocytoma and paraganglioma are described as localized, regional, or metastatic.Localized pheochromocytoma and paraganglioma
The tumor is found in one or both adrenal glands (pheochromocytoma) or in one area only (paraganglioma).Regional pheochromocytoma and paraganglioma
Cancer has spread to lymph nodes or other tissues near where the tumor began.Metastatic pheochromocytoma and paraganglioma
The cancer has spread to other parts of the body, such as the liver, lungs, bone, or distant lymph nodes.
Recurrent pheochromocytoma or paraganglioma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in another part of the body.
Different types of treatments are available for patients with pheochromocytoma or paraganglioma. 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 treatmentPatients with pheochromocytoma and paraganglioma that cause signs or symptoms are treated with drug therapy.
Drug therapy begins when pheochromocytoma or paraganglioma is diagnosed. This may include:
Drug therapy is often given for one to three weeks before surgery.Six types of standard treatment are used:Surgery
Surgery to remove pheochromocytoma is usually an adrenalectomy (removal of one or both adrenal glands). During this surgery, the tissues and lymph nodes inside the abdomen will be checked and if the tumor has spread, these tissues may also be removed. Drugs may be given before, during, and after surgery to keep blood pressure and heart rate normal.
After surgery to remove the tumor, catecholamine levels in the blood or urine are checked. Normal catecholamine levels are a sign that all the pheochromocytoma cells were removed.
If both adrenal glands are removed, life-long hormone therapy to replace hormones made by the adrenal glands is needed.Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:
The way the radiation therapy is given depends on the type of cancer being treated and whether it is localized, regional, metastatic, or recurrent. External radiation therapy and 131I-MIBG therapy are used to treat pheochromocytoma.
Pheochromocytoma is sometimes treated with 131I-MIBG, which carries radiation directly to tumor cells. 131I-MIBG is a radioactive substance that collects in certain kinds of tumor cells, killing them with the radiation that is given off. The 131I-MIBG is given by infusion. Not all pheochromocytomas take up 131I-MIBG, so a test is done first to check for this before treatment begins.Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type of cancer being treated and whether it is localized, regional, metastatic, or recurrent.Ablation therapy
Ablation is a treatment to remove or destroy a body part or tissue or its function. Ablation therapies used to help kill cancer cells include:
Embolization therapy is a treatment to block the artery leading to the adrenal gland. Blocking the flow of blood to the adrenal glands helps kill cancer cells growing there.Targeted therapy
Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Targeted therapies are used to treat metastatic and recurrent pheochromocytoma.
Sunitinib (a type of tyrosine kinase inhibitor) is a new treatment being studied for metastatic pheochromocytoma. Tyrosine kinase inhibitor therapy is a type of targeted therapy that blocks signals needed for tumors to grow.New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.Patients may want to think about taking part 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.Patients can enter clinical trials before, during, or after starting their cancer treatment.
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. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.Follow-up tests will be needed.
Some of the tests that were done to diagnose the cancer or to find out the extent of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment will be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests.
For patients with pheochromocytoma or paraganglioma that causes symptoms, catecholamine levels in the blood and urine will be checked on a regular basis. Catecholamine levels that are higher than normal can be a sign that the cancer has come back.
For patients with paraganglioma that does not cause symptoms, follow-up tests such as CT, MRI, or MIBG scan should be done every year.
For patients with inherited pheochromocytoma, catecholamine levels in the blood and urine will be checked on a regular basis. Other screening tests will be done to check for other tumors that are linked to the inherited syndrome.
Talk to your doctor about which tests should be done and how often. Patients with pheochromocytoma or paraganglioma need lifelong follow-up.
Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor. If the tumor is in the adrenal gland, the entire adrenal gland is removed.
Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
In patients with inherited pheochromocytoma linked to multiple endocrine neoplasia (MEN2) or von Hippel-Lindau (VHL) syndrome, tumors often form in both adrenal glands. The tumors are usually benign.
Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. Nearby organs that the cancer has spread to, such as the kidney, liver, part of a major blood vessel, and lymph nodes, may also be removed.
Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Treatment of metastatic pheochromocytoma or paraganglioma may include the following:
Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Treatment of recurrent pheochromocytoma or paraganglioma may include the following:
Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
Although it is rarely diagnosed during pregnancy, pheochromocytoma can be very serious for the mother and the newborn. Women who have an increased risk of pheochromocytoma should have prenatal testing. Pregnant women with pheochromocytoma should be treated by a team of doctors who are experts in this type of care.
Signs of pheochromocytoma in pregnancy may include any of the following:
The diagnosis of pheochromocytoma in pregnant women includes testing for catecholamine levels in blood and urine. See the General Information section for a description of these tests and procedures. An MRI can be done to safely find tumors in pregnant women because it does not expose the fetus to radiation.Treatment of pregnant women with pheochromocytoma may include surgery.
Treatment of pheochromocytoma during pregnancy may include the following:
For more information from the National Cancer Institute about pheochromocytoma and paraganglioma, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
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PDQ® Adult Treatment Editorial Board. PDQ Pheochromocytoma and Paraganglioma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389499]
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