Pheochromocytoma and Paraganglioma: Types of Treatment

Approved by the Cancer.Net Editorial Board, 12/2018

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. To learn more about clinical trials, see the About Clinical Trials and Latest Research sections.

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, 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 tumors 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. 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. A combination of hormone-blocking drugs is also given to patients before and during treatments that can cause dangerous levels of catecholamines to be released. This kind of drug therapy is continued after other treatments if catecholamine-related symptoms are still occurring.

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 to remove the tumor. For patients with head and neck paragangliomas that do not produce catecholamines, “watchful waiting” may be recommended instead. This means 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 found in the body.

Laparoscopic surgery may be performed for pheochromocytomas and paragangliomas, 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.

Radiation therapy is generally recommended when a pheochromocytoma or paraganglioma has spread. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.

Side effects of radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about the basics of radiation therapy.

Therapies using medication

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).

The types of systemic therapies used for a pheochromocytoma or paraganglioma include:

  • Chemotherapy

  • Peptide receptor radionuclide therapy (PRRT)

Each of these types of therapies is discussed below in more detail. A person may receive only 1 type of systemic therapy at a time or a combination of systemic therapies given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.

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. Learn more about your prescriptions by using searchable drug databases.

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by ending those cells’ ability to grow and divide. 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 “Care for symptoms and side 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 catecholamine-producing tumors 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.

Learn more about the basics of chemotherapy.

Peptide receptor radionuclide 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. The U.S. Food and Drug Administration (FDA) approved a radiotherapy drug called iobenguane I131 (Azedra) for treatment of pheochromocytoma or paraganglioma that cannot be surgically removed and that has spread beyond the original tumor site. This systemic treatment helps reduce cardiovascular side effects associated with these tumors and reduces tumor size. Not all tumors respond to this type of treatment. A test is done before the treatment to see if the therapy should be used. This may also be called MIBG therapy.

A second form of PRRT, 177Lu-dotatate (Lutathera), is approved to treat NETs of the gastrointestinal tract. This drug may be used to treat pheochromocytoma or paraganglioma in clinical trials.

Care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatments intended to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process. People often receive treatment for the cancer at the same time that they receive treatment to ease side effects. In fact, people who receive both at the same time often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional support, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, or radiation therapy. Talk with your doctor about the goals of each treatment in your treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of the specific treatment plan and palliative care options. During and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care

Treatment options by 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, at times, difficult to bear. 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 does return 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 is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain 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 Trials. It 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.