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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).


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).
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.
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.
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.
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.