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Pheochromocytoma and Paraganglioma Treatment (PDQ®)

Patient Version
Last Modified: 04/06/2012

General Information About Pheochromocytoma and Paraganglioma

Key Points for This Section


Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue.

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 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 symptoms of disease.

Certain inherited disorders and changes in certain genes increase the risk of pheochromocytoma or paraganglioma.

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:

Having Carney triad (paraganglioma, GIST, and pulmonary chondroma) is also a risk factor for paraganglioma.

Possible signs of pheochromocytoma and paraganglioma include high blood pressure and headache.

Some tumors do not make extra adrenaline or noradrenaline and do not cause symptoms. These tumors may be found when a lump forms in the neck or when a test or procedure is done for another reason. Symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other symptoms may be caused by pheochromocytoma and paraganglioma. Other conditions may cause the same symptoms. Talk to your doctor if you have any of the following problems:

  • High blood pressure.
  • Headache.
  • Heavy sweating for no known reason.
  • A strong, fast, or irregular heartbeat.
  • Being shaky.
  • Being extremely pale.

The most common symptom 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.

Symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events.

Symptoms of pheochromocytoma and paraganglioma may occur when one of the following events happens:

  • Hard physical activity.
  • A physical injury or having a lot of emotional stress.
  • Childbirth.
  • Going under anesthesia.
  • Surgery, including procedures to remove the tumor.
  • Eating foods high in tyramine (such as red wine, chocolate, and cheese).

Tests that examine the blood and urine are used to detect (find) and diagnose pheochromocytoma and paraganglioma.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as high blood pressure or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of catecholamines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts of certain catecholamines may be a sign of pheochromocytoma.
  • Blood catecholamine studies: A procedure in which a blood sample is checked to measure the amount of certain catecholamines released into the blood. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher than or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher than normal amounts of certain catecholamines may be a sign of pheochromocytoma.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the neck, chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma.

All patients who are diagnosed with pheochromocytoma or paraganglioma should have genetic counseling to find out their risk for having an inherited syndrome.

Genetic testing is recommended for patients who:

  • Have a personal or family history of traits linked with inherited pheochromocytoma or paraganglioma syndrome.
  • Have tumors in both adrenal glands.
  • Have more than one tumor in one adrenal gland.
  • Have symptoms of paraganglioma or malignant (cancerous) paraganglioma.
  • Are diagnosed before age 40.

Genetic testing is sometimes recommended for patients with pheochromocytoma who:

  • Are aged 40 to 50 years.
  • Have a tumor in one adrenal gland.
  • Do not have a personal or family history of an inherited syndrome.

Genetic testing is not recommended for patients older than 50 years.

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 symptoms.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • Whether the tumor is benign or malignant.
  • Whether the tumor is in one area only or has spread to other places in the body.
  • Whether there are symptoms caused by a higher than normal amount of catecholamines.
  • Whether the tumor has just been diagnosed or has recurred (come back).

Stages of Pheochromocytoma and Paraganglioma

Key Points for This Section


After pheochromocytoma and paraganglioma have been diagnosed, tests are done to find out if the tumor has spread to other parts of the body.

The following tests and procedures may be used to determine if the tumor has spread to other parts of the body:

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. The abdomen and pelvis are imaged to detect catecholamine -releasing tumors. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the neck, chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma. A very small amount of radioactive material called MIBG is injected into a vein and travels through the bloodstream. Pheochromocytoma cells take up the radioactive material and are detected by a radiation -measuring device.
  • Octreotide scan: A type of radionuclide scan used to find certain tumors, including tumors that release catecholamine. A small amount of radioactive octreotide (a hormone that attaches to certain tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body.
  • FDG-PET scan (fluorodeoxyglucose-positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of FDG, a type of radioactive glucose (sugar), is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, 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 and Paraganglioma

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.

Treatment Option Overview

Key Points for This Section


There are different types of treatment for patients with pheochromocytoma or paraganglioma.

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 treatment

Patients with pheochromocytoma and paraganglioma that cause symptoms are treated with drug therapy.

Drug therapy begins when pheochromocytoma or paraganglioma is diagnosed. This may include:

  • Drugs that keep the blood pressure normal. For example, one type of drug called alpha-blockers stops noradrenaline from making small blood vessels more narrow. Keeping the blood vessels open and relaxed improves blood flow and lowers blood pressure.
  • Drugs that keep the heart rate normal. For example, one type of drug called beta-blockers stops the effect of too much noradrenaline and slows the heart rate.
  • Drugs that block the effect of extra hormones made by the adrenal gland.

Drug therapy is often given for one to three weeks before surgery.

Five 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. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

The way the radiation therapy is given depends on the type of cancer being treated and whether it is localized, regional, metastatic, or recurrent.

Pheochromocytoma is sometimes treated with I131-MIBG, which carries radiation directly to tumor cells. I131-MIBG is a radioactive substance that collects in certain kinds of tumor cells, killing them with the radiation that is given off. The I131-MIBG is given by infusion. Not all pheochromocytomas take up I131-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 include:

  • Radiofrequency ablation: A procedure that uses radio waves to heat and destroy abnormal cells. The radio waves travel through electrodes (small devices that carry electricity). Radiofrequency ablation may be used to treat cancer and other conditions.
  • Cryoablation: A procedure in which tissue is frozen to destroy abnormal cells. Liquid nitrogen or liquid carbon dioxide is used to freeze the tissue.

Embolization therapy

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.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site 1.

Targeted therapy

Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitor (TKI) therapy is a type of targeted therapy that blocks signals needed for tumors to grow. Sunitinib is a type of TKI being studied to treat pheochromocytoma.

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, 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 or MRI should be done every year. An MIBG scan should be done to check if the tumor has come back. For patients with inherited pheochromocytoma, more screening tests are done on a regular basis 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 Options for Pheochromocytoma and Paraganglioma

A link to a list of current clinical trials is included for each treatment section. 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 Pheochromocytoma and Paraganglioma

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. The tumor is usually benign.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized benign pheochromocytoma 2. 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 3.

Inherited Pheochromocytoma

In patients with inherited pheochromocytoma linked to multiple endocrine neoplasia (MEN2) or von Hippel-Lindau disease (VHL), tumors often form in both adrenal glands. The tumors are usually benign.

  • Treatment for inherited pheochromocytoma that forms in one adrenal gland is surgery to completely remove the gland. This surgery may help patients avoid lifelong steroid hormone replacement therapy and acute adrenal insufficiency.
  • Treatment for inherited pheochromocytoma that forms in both adrenal glands or later forms in the remaining adrenal gland may be surgery to remove the tumor and as little normal tissue in the adrenal cortex as possible. This surgery may help patients avoid life-long hormone replacement therapy and health problems due to the loss of hormones made by the adrenal gland.
Regional Pheochromocytoma and Paraganglioma

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 for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with regional pheochromocytoma 4. 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 3.

Metastatic Pheochromocytoma and Paraganglioma

Treatment of metastatic pheochromocytoma or paraganglioma may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with metastatic pheochromocytoma 5. 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 3.

Recurrent Pheochromocytoma and Paraganglioma

Treatment of recurrent pheochromocytoma or paraganglioma may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent pheochromocytoma 6. 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 3.

Pheochromocytoma During Pregnancy

Key Points for This Section


Pregnant women with pheochromocytoma need special care.

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.

Symptoms of pheochromocytoma in pregnancy may include any of the following:

  • High blood pressure during the first 3 months of pregnancy.
  • Sudden periods of high blood pressure.
  • High blood pressure that is very hard to treat.

The diagnosis of pheochromocytoma in pregnant women includes testing for catecholamine levels in blood and urine. See the General Information 7 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:

  • Surgery to completely remove the cancer during the second trimester (the fourth through the sixth month of pregnancy).
  • Surgery to completely remove the cancer combined with delivery of the fetus by cesarean section.

To Learn More About Pheochromocytoma and Paraganglioma

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:

Changes to This Summary (04/06/2012)

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 to this summary.

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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 23. 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.

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Glossary Terms

abdomen (AB-doh-men)
The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
adrenal cortex (uh-DREE-nul KOR-tex)
The outer part of the adrenal gland (a small organ on top of each kidney). The adrenal cortex makes androgen and corticosteroid hormones.
adrenal gland (uh-DREE-nul...)
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.
adrenal medulla (uh-DREE-nul meh-DOO-luh)
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.
adrenaline (uh-DREH-nuh-lin)
A hormone and neurotransmitter. Also called epinephrine.
anesthesia (A-nes-THEE-zhuh)
A loss of feeling or awareness caused by drugs or other substances. Anesthesia keeps patients from feeling pain during surgery or other procedures. Local anesthesia is a loss of feeling in one small area of the body. Regional anesthesia is a loss of feeling in a part of the body, such as an arm or leg. General anesthesia is a loss of feeling and a complete loss of awareness that feels like a very deep sleep.
benign (beh-NINE)
Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
blood (blud)
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.
blood pressure (blud PREH-sher)
The force of circulating blood on the walls of the arteries. Blood pressure is taken using two measurements: systolic (measured when the heart beats, when blood pressure is at its highest) and diastolic (measured between heart beats, when blood pressure is at its lowest). Blood pressure is written with the systolic blood pressure first, followed by the diastolic blood pressure (for example 120/80).
blood sugar (blud SHUH-ger)
Glucose (a type of sugar) found in the blood. Also called glycemia.
blood vessel (blud VEH-sel)
A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a 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. Also called malignancy.
Carney triad (KAR-nee TRY-ad)
A very rare disorder marked by tumors of the gastrointestinal tract (usually the stomach), tumors that form in embryonic nervous tissue in the head, neck, and torso, and tumors that form in cartilage in the lungs. Sometimes tumors also form in the adrenal glands and esophagus. Carney triad is most common in young females.
Carney-Stratakis dyad (KAR-nee-STRA-tuh-kis DY-ad)
A rare, inherited disorder marked by tumors of the gastrointestinal tract and tumors that form in embryonic nervous tissue in the head, neck, and torso. Also called Carney dyad and Carney-Stratakis syndrome.
carotid artery (kuh-RAH-tid AR-tuh-ree)
A major artery that carries blood from the heart to the head. There is a carotid artery on each side of the neck, and each one splits into two branches. The interior branch carries blood to the brain and eyes, and the exterior branch carries blood to the face, tongue, and outside parts of the head.
catecholamine (ka-teh-KOH-luh-meen)
A type of neurohormone (a chemical that is made by nerve cells and used to send signals to other cells). Catecholamines are important in stress responses. High levels cause high blood pressure which can lead to headaches, sweating, pounding of the heart, pain in the chest, and anxiety. Examples of catecholamines include dopamine, epinephrine (adrenaline), and norepinephrine (noradrenaline).
clinical trial (KLIH-nih-kul TRY-ul)
A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.
condition (kun-DIH-shun)
In medicine, a health problem with certain characteristics or symptoms.
CT scan (… skan)
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 CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
epinephrine (eh-pih-NEH-frin)
A hormone and neurotransmitter. Also called adrenaline.
family history (FA-mih-lee HIH-stuh-ree)
A record of the relationships among family members along with their medical histories. This includes current and past illnesses. A family history may show a pattern of certain diseases in a family. Also called family medical history.
gastrointestinal stromal tumor (GAS-troh-in-TES-tih-nul STROH-mul TOO-mer)
A type of tumor that usually begins in cells in the wall of the gastrointestinal tract. It can be benign or malignant. Also called GIST.
gene (jeen)
The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.
genetic counseling (jeh-NEH-tik KOWN-suh-ling)
A communication process between a specially trained health professional and a person concerned about the genetic risk of disease. The person's family and personal medical history may be discussed, and counseling may lead to genetic testing.
genetic testing (jeh-NEH-tik TES-ting)
Analyzing DNA to look for a genetic alteration that may indicate an increased risk for developing a specific disease or disorder.
high blood pressure (hy blud PREH-sher)
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.
hormone (HOR-mone)
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.
inherited (in-HAYR-ih-ted)
Transmitted through genes that have been passed from parents to their offspring (children).
injection (in-JEK-shun)
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
kidney (KID-nee)
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.
malignant (muh-LIG-nunt)
Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.
medical history (MEH-dih-kul HIH-stuh-ree)
A record of information about a person’s health. A personal medical history may include information about allergies, illnesses, surgeries, immunizations, and results of physical exams and tests. It may also include information about medicines taken and health habits, such as diet and exercise. A family medical history includes health information about a person’s close family members (parents, grandparents, children, brothers, and sisters). This includes their current and past illnesses. A family medical history may show a pattern of certain diseases in a family.
MRI
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, NMRI, and nuclear magnetic resonance imaging.
multiple endocrine neoplasia type 2A syndrome (MUL-tih-pul EN-doh-krin NEE-oh-PLAY-zhuh ... SIN-drome)
A rare, genetic disorder that affects the endocrine glands and causes a type of thyroid cancer called medullary thyroid cancer, pheochromocytoma, and parathyroid gland cancer. It may also cause benign (noncancerous) tumors in the parathyroid glands and adrenal glands. The affected endocrine glands may make high levels of hormones, which can lead to other medical problems such as high blood pressure and kidney stones. An itchy skin condition may also occur. Multiple endocrine neoplasia type 2A syndrome is caused by a mutation (change) in a gene called RET. Also called MEN2A, MEN2A syndrome, multiple endocrine adenomatosis type 2A, and Sipple syndrome.
multiple endocrine neoplasia type 2B syndrome (MUL-tih-pul EN-doh-krin NEE-oh-PLAY-zhuh ... SIN-drome)
A rare, genetic disorder that affects the endocrine glands and causes a type of thyroid cancer called medullary thyroid cancer, pheochromocytoma, and parathyroid gland cancer. It may also cause benign (noncancerous) tumors in the adrenal glands and growths around the nerves in the lips, tongue, lining of the mouth, and eyelids. Gastrointestinal symptoms and trouble with the spine or bones in the feet and thighs may also occur. Multiple endocrine neoplasia type 2B syndrome is caused by a mutation (change) in a gene called RET. Also called MEN2B, MEN2B syndrome, and multiple endocrine adenomatosis type 2B.
National Cancer Institute (NA-shuh-nul KAN-ser IN-stih-TOOT)
The National Cancer Institute, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. The National Cancer Institute conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the National Cancer Institute Web site at http://www.cancer.gov. Also called NCI.
nerve (nerv)
A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.
neurofibromatosis type 1 (NOOR-oh-FY-broh-muh-TOH-sis ...)
A rare genetic condition that causes brown spots and tumors on the skin, freckling in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system, muscles, bone, and skin. Also called NF1.
noradrenaline (NOR-uh-dreh-nuh-lin)
A chemical made by some nerve cells and in the adrenal gland. It can act as both a neurotransmitter (a chemical messenger used by nerve cells) and a hormone (a chemical that travels in the blood and controls the actions of other cells or organs). Noradrenaline is released from the adrenal gland in response to stress and low blood pressure. Also called norepinephrine.
norepinephrine (NOR-eh-pih-NEH-frin)
A chemical made by some nerve cells and in the adrenal gland. It can act as both a neurotransmitter (a chemical messenger used by nerve cells) and a hormone (a chemical that travels in the blood and controls the actions of other cells or organs). Norepinephrine is released from the adrenal gland in response to stress and low blood pressure. Also called noradrenaline.
organ (OR-gun)
A part of the body that performs a specific function. For example, the heart is an organ.
paraganglioma (PAYR-uh-GANG-glee-OH-muh)
A rare, usually benign tumor that develops from cells of the paraganglia. Paraganglia are a collection of cells that came from embryonic nervous tissue, and are found near the adrenal glands and some blood vessels and nerves. Paragangliomas that develop in the adrenal gland are called pheochromocytomas. Those that develop outside of the adrenal glands near blood vessels or nerves are called glomus tumors or chemodectomas.
pelvis (PEL-vus)
The lower part of the abdomen, located between the hip bones.
pheochromocytoma (FEE-oh-KROH-moh-sy-TOH-muh)
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 (not cancer) but can cause high blood pressure, pounding headaches, heart palpitations, flushing of the face, nausea, and vomiting.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
pulmonary (PUL-muh-NAYR-ee)
Having to do with the lungs.
recover (ree-KUH-ver)
To become well and healthy again.
recur (ree-KER)
To come back or to return.
recurrent cancer (ree-KER-ent KAN-ser)
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.
risk factor (... FAK-ter)
Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.
stage (stayj)
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.
stress (stres)
The response of the body to physical, mental, or emotional pressure. This may make a person feel frustrated, angry, or anxious, and may cause unhealthy chemical changes in the body. Untreated, long-term stress may lead to many types of mental and physical health problems.
stroke (stroke)
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.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom (SIMP-tum)
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
syndrome (SIN-drome)
A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.
tissue (TIH-shoo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
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 cancer), or malignant (cancer). Also called neoplasm.
urine (YOOR-in)
Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
von Hippel-Lindau syndrome (von HIH-pul-LIN-dow SIN-drome)
A rare inherited disorder in which blood vessels grow abnormally in the eyes, brain, spinal cord, adrenal glands, or other parts of the body. People with von Hippel-Lindau syndrome have a higher risk of developing some types of cancer. Also called VHL syndrome.
x-ray (EX-ray)
A type of radiation used in the diagnosis and treatment of cancer and other diseases. 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.

Table of Links

1http://cancer.gov/clinicaltrials
2http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=40116&tt=1&a
mp;format=1&cn=1
3http://www.cancer.gov/clinicaltrials
4http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=40117&tt=1&a
mp;format=1&cn=1
5http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=40118&tt=1&a
mp;format=1&cn=1
6http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=40119&tt=1&a
mp;format=1&cn=1
7http://www.cancer.gov/cancertopics/pdq/treatment/pheochromocytoma/Patient/#Sect
ion_19
8http://www.cancer.gov/cancertopics/types/pheochromocytoma
9http://www.cancer.gov/cancertopics/UnderstandingCancer/genetesting
10http://www.cancer.gov/cancertopics/factsheet/Therapy/cryosurgery
11http://www.cancer.gov/cancertopics/understandingcancer/targetedtherapies
12http://www.cancer.gov/cancertopics/factsheet/Therapy/targeted
13http://www.cancer.gov/cancertopics/wyntk/overview
14http://www.cancer.gov/cancertopics/understandingcancer/cancer
15http://www.cancer.gov/cancertopics/factsheet/Detection/staging
16http://www.cancer.gov/cancertopics/chemotherapy-and-you
17http://www.cancer.gov/cancertopics/radiation-therapy-and-you
18http://www.cancer.gov/cancertopics/coping
19http://www.cancer.gov/cancertopics/cancerlibrary/questions
20http://www.cancer.gov/cancertopics/cancerlibrary
21http://dccps.cancer.gov/ocs/resources.html
22https://livehelp.cancer.gov
23http://cancer.gov
24https://pubs.cancer.gov/ncipl