Pheochromocytoma and Paraganglioma: Types of Treatment

Approved by the Cancer.Net Editorial Board, 06/2020

ON THIS PAGE: You will learn about the different types of treatments doctors use for people with a pheochromocytoma or paraganglioma. Use the menu to see other pages.

This section explains the types of treatments that are the standard of care for pheochromocytomas and paragangliomas. “Standard of care” means the best treatments known. When making treatment plan decisions, you are encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option to consider for treatment and care for all stages of cancer. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections in this guide. 

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team and is especially important for people with NETs. Cancer care teams include a variety of other health care professionals, such as physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Approximately 25% to 35% of pheochromocytomas and paragangliomas may be linked to a genetic syndrome (see Risk Factors). When planning treatment, all patients diagnosed with these tumor types should meet with a cancer genetics team to learn whether the tumor is associated with a specific syndrome. Some syndromes are more likely to cause malignant tumors, multiple tumors, or recurrent disease. By identifying a hereditary syndrome, doctors can also screen for other tumors related to that syndrome. In addition, it is important to learn if any other members of your family may have had these tumors in the past. If so, the syndrome may affect other family members, and specific genetic tests may be recommended for them. Learn more about genetic testing.

Descriptions of the common types of treatments used for pheochromocytoma and paraganglioma are listed below. Your care plan also includes treatment for symptoms and side effects, an important part of cancer care.

Treatment options and recommendations depend on several factors, including if the tumor produces catecholamines, its location, if it is cancerous and the stage, possible side effects, and the patient’s preferences and overall health. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. These types of talks are called “shared decision making.” Shared decision making is when you and your doctors work together to choose treatments that fit the goals of your care. Shared decision making is particularly important for pheochromocytoma and paraganglioma because there are different treatment options. Learn more about making treatment decisions.

Treatments to control hormone levels

When pheochromocytoma or paraganglioma is diagnosed, often the first treatment is intended to control hormones and lower blood pressure. These treatments will normally be given for several days or weeks before surgery.

If a tumor is producing catecholamines, the doctor will prescribe medication to help control the symptoms they cause. For example, alpha-adrenergic blockers, such as phenoxybenzamine (Dibenzyline), are commonly used to lower blood pressure. Beta-blockers, such as propranolol (Inderal), may be used to control a fast or irregular pulse. Beta-blockers should be used with caution and under the direction of an expert in endocrinology. Certain treatments can cause dangerous levels of catecholamines to be released, so patients may be given a combination of hormone-blocking drugs before and during these treatments. This kind of drug therapy is continued after other treatments if catecholamine-related symptoms are still occurring. Metyrosine (Demser) is another medication that may be used to lower blood pressure and reduce other related symptoms.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. A surgical oncologist is a doctor who specializes in cancer surgery. When it is possible, completely removing the entire tumor is the standard first treatment. For people with a head and neck paraganglioma that does not produce catecholamines, “watchful waiting” may be recommended instead. This means active treatment only starts if symptoms develop.

Most localized pheochromocytomas and paragangliomas are successfully treated with surgery alone. The surgeon will usually remove some tissue surrounding the tumor, called a margin, in hopes of leaving no traces of cancer in the body.

Laparoscopic surgery may be performed for a pheochromocytoma or paraganglioma, unless the tumor is very large. Laparoscopic surgery is a less invasive type of surgery that uses 3 or 4 small incisions instead of 1 large incision. A thin, lighted tube called a laparoscope that is attached to a video camera is inserted through 1 opening to guide the surgeon. Surgical instruments are inserted through the other openings to perform the surgery. For pheochromocytoma, 1 or both adrenal glands, or part of a gland, may be removed.

When completely removing a tumor is not possible, debulking surgery is sometimes recommended. Debulking surgery removes as much of the tumor as possible and may provide some relief from symptoms, but it generally does not provide a cure. If cancer has spread to other organs, those organs may also be removed using surgery.

If a tumor is producing catecholamines and causing symptoms, medications are given 1 to 3 weeks before surgery as well as during surgery to control a patient’s blood pressure and heart (see “Treatments to control hormone levels,” above). After surgery, blood and urine tests are done to check the levels of catecholamines in the body. If the levels are normal, it is likely that all pheochromocytoma or paraganglioma cells were successfully removed. However, follow-up care is still needed for the rest of the person’s life.

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have and how the side effects will be managed. Learn more about the basics of cancer surgery.

If removing the tumor using surgery is not possible, it is called an “inoperable tumor.” In these situations, the doctor will recommend another type of treatment.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. Radiation therapy for a pheochromocytoma or paraganglioma is usually only recommended when cancer has spread and to help relieve pain or another symptom. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time. Learn more about the basics of radiation therapy.

Side effects of external-beam radiation therapy may include fatigue and mild skin reactions where radiation therapy was given. Most side effects go away soon after treatment is finished. 

Radiation therapy can also be given as an infusion. The U.S. Food and Drug Administration (FDA) has approved a radiotherapy drug called iobenguane 131I (Azedra) for treatment of pheochromocytoma or paraganglioma that cannot be surgically removed and is bright and easily seen on an MIBG scan (see Diagnosis). This treatment helps reduce side effects that may affect the heart and blood vessels, which are associated with these tumors. This treatment may also help shrink tumors. Not all tumors respond to this treatment. A test is done beforehand to see if this treatment should be used. This is also called MIBG therapy.

Another form of radiation therapy given by infusion is peptide receptor radiation therapy (PRRT). In PRRT, a radioactive drug binds to a specific cell receptor that may be present on certain tumors. After binding to the receptor, the drug enters the cell, allowing radiation to damage the tumor cells. A PRRT drug called 177Lu-dotatate (Lutathera) is approved to treat NETs of the gastrointestinal tract that are bright and easily seen on 68Ga DOTATATE PET scan. This drug may be used to treat pheochromocytoma or paraganglioma.

Chemotherapy

Systemic therapy is the use of medication to destroy cancer cells. This type of medication is given through the bloodstream to reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

Chemotherapy is the systemic therapy used for a pheochromocytoma or paraganglioma. Chemotherapy is the use of drugs to destroy tumor cells, usually by keeping the tumor cells from growing, dividing, and making more cells.

People with a fast-growing, metastatic pheochromocytoma or paraganglioma may be treated with chemotherapy. It is most often used as part of supportive care to relieve symptoms, such as pain, caused by cancer that has spread to the bone and other areas of the body (see “Physical, emotional, and social effects” below).

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. The chemotherapy combination used most often for a pheochromocytoma or paraganglioma is called CVD: cyclophosphamide (Cytoxan), vincristine (Vincasar), and dacarbazine (DTIC-Dome). Another chemotherapy used is temozolomide (Temodar). Dangerous levels of catecholamines may be released during chemotherapy, particularly in the first 24 hours, so patients with a catecholamine-producing tumor need to be closely monitored.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished.

The medications used to treat pheochromocytomas and paragangliomas are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with cancer medications. Learn more about your prescriptions by using searchable drug databases.

Learn more about the basics of chemotherapy.

Physical, emotional, and social effects 

A tumor and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative care or supportive care. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the tumor.

Palliative care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of the tumor, may receive this type of care. And it often works best when it is started right after the diagnosis. People who receive palliative care along with treatment for the tumor often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. You may also receive palliative treatments similar to those meant to get rid of the tumor, such as chemotherapy, surgery, or radiation therapy.

Before treatment begins, talk with your doctor about the goals of each treatment in the treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative care options.

During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative care in a separate section of this website.

Treatment by stage of pheochromocytoma or paraganglioma

Different treatments may be recommended for each stage of pheochromocytoma or paraganglioma. Your doctor will recommend a specific treatment plan for you based on the tumor’s stage and other factors. Detailed descriptions of each type of treatment are provided earlier in this page. Clinical trials may also be a treatment option for each stage.

Localized

Localized pheochromocytoma or paraganglioma is usually treated with surgery to remove the tumor. If the tumor is in an adrenal gland, then the whole gland may be removed.

Regional

Regional pheochromocytoma or paraganglioma has spread to nearby organs and/or lymph nodes. Surgery will be used to remove the tumor. If nearby organs are affected, then those organs may also have to be removed with surgery.

Metastatic

If a cancerous pheochromocytoma or paraganglioma spreads to another part in the body from where it started, doctors call it metastatic cancer. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Clinical trials might also be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.

Pheochromocytomas and paragangliomas spread most often to the bone, lung, lymph nodes, and liver. Your treatment plan may include a combination of the treatments described above, including surgery, chemotherapy, radiation therapy, and systemic radiation therapy with PRRT. Targeted therapies may also be available through clinical trials (see Latest Research). Participation in clinical trials is encouraged. Palliative care will also be important to help relieve symptoms and side effects.

For most people, a diagnosis of metastatic cancer is very stressful and difficult. You and your family are encouraged to talk about how you feel with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer returns after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence). Approximately half of all pheochromocytoma and paraganglioma recurrences are distant recurrences.

When this occurs, a new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments described above, such as surgery, radiation therapy, and chemotherapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat the type of recurrent cancer that you have. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with recurrent cancer often experience emotions such as disbelief or fear. You are encouraged to talk with your health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.

If treatment does not work

Recovery from a pheochromocytoma or paraganglioma is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.

This diagnosis is stressful, and for many people, advanced cancer is difficult to discuss. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.

People who have advanced cancer and who are expected to live less than 6 months may want to consider hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

The next section in this guide is About Clinical TrialsIt offers more information about research studies that are focused on finding better ways to care for people with a pheochromocytoma or paraganglioma. Use the menu to choose a different section to read in this guide.