Pheochromocytoma and Paraganglioma Treatment
- Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue.
- Pheochromocytoma is a rare tumor that forms in the adrenal medulla (the center of the adrenal gland).
- Paragangliomas form outside the adrenal gland.
- Certain inherited disorders and changes in certain genes increase the risk of pheochromocytoma or paraganglioma.
- Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache.
- Signs and symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events.
- Tests that examine the blood and urine are used to detect (find) and diagnose pheochromocytoma and paraganglioma.
- Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma.
- Certain factors affect prognosis (chance of recovery) and treatment options.
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 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.
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:
- Multiple endocrine neoplasia 2 syndrome, types A and B (MEN2A and MEN2B).
- von Hippel-Lindau (VHL) syndrome.
- Neurofibromatosis type 1 (NF1).
- Hereditary paraganglioma syndrome.
- Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor [GIST]).
- Carney triad (paraganglioma, GIST, and pulmonary chondroma).
Screening and Detection
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 and other related cancers.
Genetic testing may be recommended by a genetic counselor 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 signs or symptoms of extra catecholamines being released into the blood 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.
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.
Signs and Symptoms
Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache.
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:
- High blood pressure.
- Heavy sweating for no known reason.
- A strong, fast, or irregular heartbeat.
- Being shaky.
- Being extremely pale.
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:
- Hard physical activity.
- A physical injury or having a lot of emotional stress.
- Going under anesthesia.
- Surgery, including procedures to remove the tumor.
- Eating foods high in tyramine (such as red wine, chocolate, and cheese).
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 signs or 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.
Six types of standard treatment are used:
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 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 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 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 is a treatment to remove or destroy a body part or tissue or its function. Ablation therapies used to help kill cancer cells 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 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 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.