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Pheochromocytoma and Paraganglioma - Introduction

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will find some basic information about these diseases and the parts of the body they may affect. This is the first page of Cancer.Net’s Guide to Pheochromocytoma and Paraganglioma. Use the menu to see other pages. Think of that menu as a roadmap for this entire guide.

About endocrine and neuroendocrine tumors

A tumor begins when the DNA of healthy cells is damaged, causing the cells to change and grow with less control, forming a mass.

Pheochromocytomas and paragangliomas are considered a subset of a group of tumors called neuroendocrine tumors (NETs). A NET begins in the specialized cells of the body’s neuroendocrine system. These cells have traits of both hormone-producing endocrine cells and nerve cells. They are found throughout the body’s organs and help control many of the body’s functions. Hormones are chemical substances that are carried through the bloodstream to have a specific effect on the activity of other organs or cells in the body. All NETs have the potential to become metastatic, meaning the tumor can spread to other parts of the body. Most NETs take years to develop and grow slowly. However, some NETs can be fast-growing. See the Stages section of this guide to learn more.

It can be difficult to tell if a pheochromocytoma or paraganglioma will grow or spread, even after surgically removing the tumor and examining it under a microscope. The majority of these tumors will not spread.

About pheochromocytoma

A pheochromocytoma is a rare NET of the adrenal glands. The body has 2 of these small, yellowish glands. One is on top of each kidney. Adrenal glands have 2 main parts that function separately: the outer adrenal cortex and the inner adrenal medulla. Each part produces a different set of hormones.

A pheochromocytoma begins in the chromaffin cells of the adrenal medulla. The cells release hormones called catecholamines during times of stress. Adrenaline and noradrenaline, which increase blood pressure and heart rate, are 2 of those catecholamines. A pheochromocytoma can cause uncontrolled surges of extra adrenaline and noradrenaline into the blood. Even though an estimated 90% of pheochromocytomas remain localized to the area they began, these hormone surges can still lead to life-threatening health problems, such as a stroke, heart attack, hemorrhage, or sudden death.

Most people develop a pheochromocytoma in 1 adrenal gland. Some people develop a tumor in both glands. There can also be multiple tumors in a gland. Pheochromocytomas usually grow slowly.

A pheochromocytoma is called a primary adrenal gland tumor because it starts inside an adrenal gland. Read about other types of primary adrenal gland tumors.

About paraganglioma

A paraganglioma is a rare NET that develops from the same type of cells that pheochromocytomas do (see above). However, paragangliomas form outside the adrenal glands. This type of tumor may also be called an extra-adrenal paraganglioma. The majority of paragangliomas form in the abdomen. They can also form in other parts of the body, including near the carotid artery, along nerve pathways in the head and neck, and in the chest, heart, and abdomen. Paragangliomas are less common than pheochromocytomas. Depending on the tumor's location in the body and certain genetic mutations, about 20% to 40% of paragangliomas can metastasize, or spread (see Diagnosis). Paragangliomas are typically slow growing.

There are 2 types of paragangliomas:

  • Functional (sympathetic) paragangliomas. Like pheochromocytomas, sympathetic paragangliomas almost always produce catecholamines. These tumors develop in the sympathetic nervous system, which controls the body’s response to stress and activity.
  • Non-functional (parasympathetic) paragangliomas. These tumors are most often found in the head and neck. They usually do not release catecholamines, so they rarely cause hormone-related symptoms or signs of a problem. They develop in the parasympathetic nervous system, which controls the body during rest.

The next section in this guide is Statistics. It helps explain the number of people who are diagnosed with a pheochromocytoma or paraganglioma and general survival rates. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Statistics

Approved by the Cancer.Net Editorial Board, 03/2022

ON THIS PAGE: You will find information about the number of people who are diagnosed with a pheochromocytoma or paraganglioma each year. You will also read general information on surviving these diseases. Remember, survival rates depend on several factors. Use the menu to see other pages.

Pheochromocytomas and paragangliomas are rare. It is estimated that about 2 to 8 people per every 1 million people are diagnosed with these tumors each year. Around 15% of these cases are malignant. Paragangliomas are far less common than pheochromocytomas.

Pheochromocytomas affect men and women equally. The tumor can be diagnosed at any age, but it is most common between the ages of 30 and 50. Approximately 10% of these tumors are diagnosed in children. Age at diagnosis depends on whether the tumor is related to an inherited syndrome. People younger than 50 are more likely to have a hereditary pheochromocytoma (see Risk Factors).

The 5-year survival rate tells you what percent of people live at least 5 years after a tumor is found. Percent means how many out of 100. However, the survival rate depends on many factors, including the location of the tumor and its stage.

Localized pheochromocytomas have a 5-year survival rate of 95%. The 5-year survival rate for a pheochromocytoma that has spread or recurred is estimated to be between 34% and 60%.

It is important to remember that statistics on the survival rates for people with a pheochromocytoma or paraganglioma are an estimate. The estimate comes from annual data based on the number of people with these tumors in the United States. Also, experts measure the survival statistics every 5 years. This means the estimate may not reflect the results of advancements in how a pheochromocytoma or paraganglioma is diagnosed or treated from the last 5 years. Talk with your doctor if you have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the websites of the National Cancer Institute and the National Organization for Rare Disorders. (All sources accessed February 2022.)

The next section in this guide is Risk Factors. It describes the factors that may increase the chance of developing a pheochromocytoma or paraganglioma. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Risk Factors

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will find out more about the factors that increase the chance of developing a pheochromocytoma or paraganglioma. Use the menu to see other pages.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause it. Some people with several risk factors never develop a tumor, while others with no known risk factors do. Knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

What causes pheochromocytomas and paragangliomas is unknown, and no avoidable risk factors have been found. However, the following factors may raise a person's risk for these types of tumors:

  • Inherited syndromes and gene changes. Up to 40% of pheochromocytomas and paragangliomas are linked to hereditary syndromes. Changes to certain genes have also been associated with the tumors when they are not connected to known hereditary syndromes. Syndromes and gene changes that raise the risk of developing a pheochromocytoma or paraganglioma include:

    • Multiple endocrine neoplasia type 2 (MEN2). Approximately 50% of people with MEN 2 develop a pheochromocytoma.

    • Von Hippel-Lindau syndrome (VHL). Approximately 10% to 20% of people with VHL will develop a pheochromocytoma. They often occur in both adrenal glands.

    • Neurofibromatosis type 1 (NF1). Approximately 1% to 13% of people with NF1 are diagnosed with a pheochromocytoma or paraganglioma.

    • Succinate dehydrogenase (SDHx) syndromes. Germline, or inherited, mutations in the SDH family of genes have also been associated with an increased risk of pheochromocytoma and paraganglioma. The increased risk can range from very low up to 45%, depending on the specific mutation. The Carney-Stratakis dyad, for example, is linked with paragangliomas and gastrointestinal stromal tumor (GIST).

    • Other mutations. There are many other, less common genetic mutations that may increase the risk of pheochromocytoma or paraganglioma.

  • Age. A pheochromocytoma can occur at any age, but the tumor is most common in people between ages 30 and 50.

  • Gender. Men and women have an equal chance of developing a pheochromocytoma.

  • Race/ethnicity. People of all races can be diagnosed with a pheochromocytoma, but the tumor is less common in Black people.

  • Carney triad. This rare, non-hereditary condition can cause paragangliomas, GISTs, and tumors in the cartilage of the lungs to form. It almost exclusively affects women.

Researchers continue to look into what factors cause pheochromocytomas and paragangliomas and what people can do to lower their personal risk. Talk with your health care team if you have concerns about your personal risk of developing these types of tumors.

The next section in this guide is Symptoms and Signs. It explains what changes or medical problems a pheochromocytoma or paraganglioma can cause. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will find out more about changes and other things that can signal a problem that may need medical care. Use the menu to see other pages.

People with a pheochromocytoma or paraganglioma may experience the following symptoms or signs. Symptoms are changes that you can feel in your body. Signs are changes in something measured, like by taking your blood pressure or doing a lab test. Together, symptoms and signs can help describe a medical problem.

These symptoms and signs occur when the tumor releases too many catecholamines in the bloodstream (see Introduction). Some people may experience symptoms several times a day, while others may experience them weekly or every few months. Episodes can last minutes, several hours, or several days. Sometimes, people with these tumors do not have any of the symptoms or signs described below. Or, the cause of a symptom or sign may be a medical condition that is not a neuroendocrine tumor.

The most common symptom of a catecholamine-producing pheochromocytoma or paraganglioma is episodes of high blood pressure or persistent high blood pressure that can be hard to control. However, most people who have high blood pressure do not have these tumors. High blood pressure, along with headaches, rapid heart rate, and heavy sweating, strongly suggest a pheochromocytoma or paraganglioma.

Other possible symptoms include:

  • Anxiety attacks

  • Fever

  • Irregular heartbeat

  • Extreme paleness in the face

  • Shortness of breath

  • Tremors or shakiness

  • Nausea

  • Vomiting

  • Clammy skin

  • Constipation

  • Tingling fingers

  • Vision disturbances

  • Chest or stomach pain

  • Weight loss

  • High blood sugar levels

Symptom triggers

Episodes of symptoms caused by a pheochromocytoma or paraganglioma can occur at any time. They can also be triggered by:

  • Physical activity

  • Physical injury and pain

  • Stress or anxiety

  • Drinking coffee

  • Medical procedures, such as anesthesia or surgery

  • Eating foods high in tyramine, such as red wine, dried meats, chocolate, and cheese

  • Urination, in people with a paraganglioma in the bladder

  • Childbirth

If you are concerned about any changes you experience, please talk with your doctor. Your doctor will ask how long and how often you have been experiencing the symptom(s), in addition to other questions. This is to help figure out the cause of the problem, called a diagnosis.

If a pheochromocytoma or paraganglioma is diagnosed, relieving symptoms remains an important part of your medical care and treatment. Managing symptoms may also be called palliative care or supportive care. It is often started soon after diagnosis and continued throughout treatment. Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Diagnosis

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, a pheochromocytoma or paraganglioma. They also do tests to learn if cancer has spread to another part of the body from where it started. If the cancer has spread, it is called metastasis. For example, imaging tests can show if the cancer has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments could work best.

Some pheochromocytomas and paragangliomas are found unexpectedly when people have tests and procedures for reasons unrelated to the tumor, if a lump forms on the neck, or if the tumor presses on other organs and causes symptoms.

For most types of cancer, a biopsy is the only sure way for the doctor to know whether an area of the body has a tumor. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. However, a tumor suspected to be a pheochromocytoma or paraganglioma should not have a biopsy, unless it is absolutely necessary to confirm a diagnosis. A biopsy of these tumors can cause a serious and even life-threatening release of catecholamines. In addition, it can be difficult to tell if these tumors are benign or malignant under a microscope, even after surgically removing the tumor (learn more about treatments in Types of Treatment). The only sure way to know if a pheochromocytoma or paraganglioma is malignant is if it has metastasized or if it comes back after treatment, called a recurrence.

How a pheochromocytoma or paraganglioma is diagnosed

There are many tests used for diagnosing pheochromocytomas and paragangliomas. Not all tests described here will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • The type of tumor suspected

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

If a doctor suspects a pheochromocytoma or paraganglioma, they will ask for a complete medical and family history and perform a physical examination. In addition, the following tests may be used to diagnose these tumors:

  • Blood/urine tests. The doctor may collect blood and urine samples to check for abnormal levels of catecholamines and other substances. If these biochemical tests indicate a pheochromocytoma or paraganglioma, imaging tests (described below) are used to confirm the diagnosis and determine the location and size of the tumor. This is called localization.

    Urine tests check for increased levels of catecholamines in the body as well as metanephrines. When catecholamines break down, they turn into metanephrines. Large amounts can be a sign of a pheochromocytoma or a paraganglioma. These tests require a person to collect all of their urine for an entire 24-hour period.

  • X-ray. An x-ray is a way to create a picture of the structures inside of the body, using a small amount of radiation.

  • Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumor. A CT scan can be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill or liquid to swallow.

  • Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill or liquid to swallow.

  • Genetic testing. Genetic testing and genetic counseling are recommended for all people with a paraganglioma or pheochromocytoma. Your blood or saliva will be tested for certain gene mutations associated with these tumors.

  • Nuclear medicine imaging. During this test, a small amount of a radioactive drug, called a tracer, is injected into a patient’s vein. The body is then scanned to show where the radioactivity has built up in the body. However, the amount of radiation in the substance is too low to be harmful. There are different methods of nuclear medicine imaging for neuroendocrine tumors (NETs):

    • Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan (see above), called a PET or PET-CT scan. The 3 types of tracers used in a PET scan include gallium-68 (68Ga) DOTATATE, copper-64 (64Cu) DOTATATE, and (18F) fluorodeoxyglucose (FDG). The DOTATATE PET is the primary scan used to look at slow-growing NETs. An FDG PET scan is sometimes used for faster-growing NETs. The DOTATATE PET scan has replaced a different method of nuclear imaging called OctreoScan because it is more effective. A 64Cu DOTATATE PET scan is the latest tracer to be made available to locate NETs.

    • Metaiodobenzylguanidine (MIBG) scan. MIBG is a chemical similar to adrenaline that will collect in a pheochromocytoma or paraganglioma. The MIBG scan takes place over 2 days in a row. On the first day, an injection of MIBG is given in the arm. Several hours later, pictures are taken with a special camera that can show if or where in the body the MIBG has collected. The following morning, more pictures are taken, and the process may be repeated if needed. This scan may be used when a CT scan or MRI does not show a tumor that biochemical tests indicate is present.

After diagnostic tests are done, your doctor will review the results with you. If the diagnosis is cancer, these results also help the doctor describe the tumor. This is called staging.

The next section in this guide is Stages. It explains the system doctors use to describe the extent of the disease. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Stages

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will learn about how doctors describe a tumor's growth or spread. This is called the stage. Use the menu to see other pages.

What is staging?

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body.

Staging can be clinical or pathological. Doctors use diagnostic tests to find out the tumor’s stage, so clinical staging may not be complete until all of the tests are finished. If surgery is done on the primary tumor, the medical team will also do pathological staging. Knowing the stage helps the doctor recommend the best kind of treatment, and it can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of tumors.

This page provides detailed information about the systems used to find the stage of pheochromocytoma and paraganglioma and the stage groups for these tumors, such as stage II or stage IV.

General staging system for pheochromocytoma and paraganglioma

Pheochromocytoma or paraganglioma may be described using a general system or the TNM system (see below). In the general system, the tumor will be described as localized, regional, or metastatic:

  • Localized pheochromocytoma. The tumor is detected at an early stage in the adrenal medulla in 1 or both adrenal glands.

  • Localized paraganglioma. The tumor is detected at an early stage in 1 area of the body only.

  • Regional pheochromocytoma or paraganglioma. Cancer has spread from where the tumor began to nearby surrounding tissues and/or lymph nodes.

  • Metastatic pheochromocytoma or paraganglioma. Cancer has spread from where the tumor began to a distant part of the body.

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TNM staging system

One tool that doctors use to describe the stage is the TNM system. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many?

  • Metastasis (M): Has the cancer spread to other parts of the body? If so, where and how much?

The results are combined to determine the stage of the tumor for each person.

There are 4 stages: stages I through IV (1 through 4). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

Here are more details on each part of the TNM system for pheochromocytoma and paraganglioma:

Tumor (T)

Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Tumor size is measured in centimeters (cm). A centimeter is roughly equal to the width of a standard pen or pencil.

Stage may also be divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.

TX: The primary tumor cannot be assessed.

T1: The tumor is less than 5 cm in size, and it has not grown beyond the adrenal gland.

T2: The tumor is 5 cm or more in size, and it has not grown beyond the adrenal gland. Or, it is a paraganglioma that causes symptoms.

T3: The tumor is any size and invades nearby tissues, such as the liver, pancreas, spleen, or kidneys.

Node (N)

The “N” in the TNM staging system stands for lymph nodes. These small, bean-shaped organs help fight infection. Lymph nodes near where the cancer started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.

NX: The regional lymph nodes cannot be assessed.

N0 (N zero): The cancer has not spread to the regional lymph nodes.

N1: The cancer has spread to the regional lymph nodes.

Metastasis (M)

The “M” in the TNM system describes whether the cancer has spread to other parts of the body, called metastasis.

M0 (M zero): There is no metastasis.

M1a: There is metastasis only to the bone.

M1b: There is metastasis only to distant lymph nodes, the liver, or a lung.

M1c: There is metastasis to bone and other sites.

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Stage groups for pheochromocytoma and paraganglioma

Doctors combine the T, N, and M information to say what stage the tumor is.

Stage I: The tumor is smaller than 5 cm. It is located only in the adrenal gland. No other organs are involved (T1, N0, M0)

Stage II: The tumor is 5 cm or more in size, and it has not grown beyond the adrenal gland. Or, it is a paraganglioma that causes symptoms. No other organs are involved. (T2, N0, M0)

Stage III: Any of the following applies:

  • The tumor is smaller than 5 cm. It is located only in the adrenal gland. The cancer involves the regional lymph nodes. (T1, N1, M0)

  • The tumor is 5 cm or larger but does not grow beyond the adrenal gland. Or, it is a paraganglioma that causes symptoms. The cancer involves the regional lymph nodes. (T2, N1, M0)

  • The tumor is any size and invades nearby tissues, such as the liver, pancreas, spleen, or kidneys. (T3, any N, M0)

Stage IV: There is metastasis. (Any T, any N, M1)

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Recurrent pheochromocytoma and paraganglioma

Recurrent pheochromocytoma or paraganglioma is a tumor that has come back after treatment. If the tumor does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

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Used with permission of the American College of Surgeons, Chicago, Illinois. The original and primary source for this information is the AJCC Cancer Staging Manual, Eighth Edition (2017), published by Springer International Publishing.

Information about the tumor’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Types of Treatment

Approved by the Cancer.Net Editorial Board, 09/2021

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, also known as therapies, 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 discuss with your doctor whether clinical trials are an option. A clinical trial is a research study that tests a new approach to treatment. Doctors learn through clinical trials whether a 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 for all stages of a tumor. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.

How pheochromocytoma and paraganglioma are treated

In tumor 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 neuroendocrine tumors (NETs). Medical teams include a variety of other health care professionals, such as physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Up to 40% 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 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 possible. If so, the syndrome may affect other family members, and specific genetic tests may be recommended for them. Learn more about genetic testing.

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.

The common types of treatments used for pheochromocytoma and paraganglioma are described below. Your care plan also includes treatment for symptoms and side effects, an important part of medical care.

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, which is the area of medicine focusing on the body's hormone system. 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.

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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 surgical removal of a tumor. 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.

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.

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Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor 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 a metaiodobenzylguanidine (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 a DOTATATE PET scan. This drug may be used in clinical trials to treat pheochromocytoma or paraganglioma.

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Therapies using medication

The treatment plan may include medications to destroy tumor cells. Medication may be given through the bloodstream to reach tumor cells throughout the body. When a drug is given this way, it is called systemic therapy. Medication may also be given locally, which is when the medication is applied directly to the tumor or kept in a single part of the body.

This treatment is generally prescribed by a medical oncologist, a doctor who specializes in treating a tumor with medication.

Medications are often given through an intravenous (IV) tube placed into a vein using a needle or as a pill or capsule that is swallowed (orally). If you are given oral medications, be sure to ask your health care team about how to safely store and handle them.

The types of medications used for pheochromocytoma or paraganglioma include:

  • Chemotherapy

  • Targeted therapy

Each of these types of therapies is discussed below in more detail. A person may receive 1 type of medication at a time or a combination of medications 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 these tumors 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 these medications, causing unwanted side effects or reduced effectiveness. Learn more about your prescriptions by using searchable drug databases.

Chemotherapy

Chemotherapy is the medication commonly used for a pheochromocytoma or paraganglioma. Chemotherapy destroys 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 a tumor 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) with or without capecitabine (Xeloda). 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, possible 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.

Learn more about the basics of chemotherapy.

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Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells and limits damage to healthy cells.

Not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.

Targeted therapy for pheochromocytoma and paraganglioma includes a type of targeted therapy called a tyrosine kinase inhibitor or TKI. These medications are usually only given in clinical trials. The TKIs being studied for the treatment of these tumors include cabozantinib (Cometriq), sunitinib (Sutent), and axitinib (Inlyta). Learn more about clinical trials in this guide's Latest Research section.

Talk with your doctor about possible side effects for a specific medication and how they can be managed.

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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 recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

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.

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Treatment by stage of pheochromocytoma or paraganglioma

Different treatments may be recommended for each stage of pheochromocytoma or paraganglioma. The general options by stage are described below. For more detailed descriptions, see “How pheochromocytoma and paraganglioma are treated,” above. Your doctor will work with you to develop a specific treatment plan based on your specific diagnosis and needs. 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 specialized 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 many 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 your health care team. It may also be helpful to talk with other patients, such as through a support group or other peer support program.

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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.

If a recurrence happens, a new cycle of testing will begin again to learn as much as possible about it. 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 recurrent pheochromocytoma or paraganglioma. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with recurrent cancer sometimes 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.

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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 some 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 may live for many years. However, eventually they may be expected to live 6 months or less. If that is the case, you and your doctor may want to discuss and 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.

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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.

Pheochromocytoma and Paraganglioma - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are studied to see how well they work. Use the menu to see other pages.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for people with neuroendocrine tumors (NETs), including pheochromocytoma or paraganglioma. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. Every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Clinical trials are used for all types and stages of pheochromocytoma and paraganglioma. Many focus on new treatments to learn if a new treatment is safe, effective, and possibly better than the existing treatments. These types of studies evaluate new drugs, different combinations of treatments, new approaches to radiation therapy or surgery, and new methods of treatment.

People who participate in clinical trials can be some of the first to get a treatment before it is available to the public. However, there are some risks with a clinical trial, including possible side effects and the chance that the new treatment may not work. People are encouraged to talk with their health care team about the pros and cons of joining a specific study.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects.

Deciding to join a clinical trial

People decide to participate in clinical trials for many reasons. For some, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Others volunteer for clinical trials because they know that these studies are a way to contribute to the progress in treating pheochromocytomas and paragangliomas. Even if they do not benefit directly from the clinical trial, their participation may benefit future people with these types of tumors.

Insurance coverage and the costs of clinical trials differ by location and by study. In some programs, some of the expenses from participating in the clinical trial are reimbursed. In others, they are not. It is important to talk with the research team and your insurance company first to learn if and how your treatment in a clinical trial will be covered. Learn more about health insurance coverage of clinical trials.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” When used, placebos are usually combined with standard treatment in most cancer clinical trials. Study participants will always be told when a placebo is used in a study. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

To join a clinical trial, people must participate in a process known as informed consent. During informed consent, the doctor should:

  • Describe all of the treatment options so that the person understands how the new treatment differs from the standard treatment.

  • List all of the risks of the new treatment, which may or may not be different from the risks of standard treatment.

  • Explain what will be required of each person in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

  • Describe the purposes of the clinical trial and what researchers are trying to learn.

Clinical trials also have certain rules called “eligibility criteria” that help structure the research and keep patients safe. You and the research team will carefully review these criteria together. You will need to meet all of the eligibility criteria in order to participate in a clinical trial. Learn more about eligibility criteria in clinical trials.

People who participate in a clinical trial may stop participating at any time for personal or medical reasons. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that people participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if they choose to leave the clinical trial before it ends.

Finding a clinical trial

Research through clinical trials is ongoing for all types of NETs. For specific topics being studied for pheochromocytoma and paraganglioma, learn more in the Latest Research section.

Cancer.Net offers more information about clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of tumor.

PRE-ACT, Preparatory Education About Clinical Trials

In addition, you can find a free video-based educational program about clinical trials located in another section of this website.

The next section in this guide is Latest Research. It explains areas of scientific research for pheochromocytoma and paraganglioma. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Latest Research

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will read about the scientific research being done to learn more about pheochromocytomas and paragangliomas and how to treat them. Use the menu to see other pages.

Doctors are working to learn more about pheochromocytoma and paraganglioma, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with these types of neuroendocrine tumors (NETs). The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the best diagnostic and treatment options for you.

  • Targeted therapy. Targeted therapy is a treatment that targets the tumor’s specific genes, proteins, or the tissue environment that contributes to tumor growth and survival. This type of treatment blocks the growth and spread of tumor cells and limits damage to healthy cells. Several targeted therapies are being studied for the treatment of malignant pheochromocytomas and paragangliomas, including a class of drugs called tyrosine kinase inhibitors (TKIs), which includes cabozantinib (Cometriq), sunitinib (Sutent), and axitinib (Inlyta). Learn more about the basics of targeted therapy.

  • Immunotherapy. Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight a tumor. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. The immunotherapy pembrolizumab (Keytruda) is being researched as a treatment for pheochromocytomas and paragangliomas in clinical trials. Learn more about the basics of immunotherapy.

  • Genetics. Researchers continue to study the role of genes in the development of pheochromocytomas and paragangliomas. The focus of these studies is to help fine-tune the diagnosis of these tumors and help predict treatment results. Learn more about the role of genetics in cancer.

  • Palliative care/supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current pheochromocytoma and paraganglioma treatments to improve comfort and quality of life for patients.

Looking for More About the Latest Research?

If you would like more information about the latest areas of research in pheochromocytoma and paraganglioma, explore these related items that will take you outside of this guide:

The next section in this guide is Coping with Treatment. It offers some guidance on how to cope with the physical, emotional, social, and financial changes that pheochromocytoma or paraganglioma and its treatment can bring. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of a tumor and its treatment. Use the menu to see other pages.

Every treatment can cause side effects or changes to your body and how you feel. For many reasons, people do not experience the same side effects even when they are given the same treatment for the same type of tumor. This can make it hard to predict how you will feel during treatment.

As you prepare to start treatment, it is normal to fear treatment-related side effects. It may help to know that your health care team will work to prevent and relieve side effects. This part of treatment is called palliative care or supportive care. It is an important part of your treatment plan, regardless of your age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for pheochromocytoma and paraganglioma are described in the Types of Treatment section. Learn more about side effects, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the tumor’s stage, the length and dose of treatment, and your general health.

Talk with your health care team regularly about how you are feeling. It is important to let them know about any new side effects or changes in existing side effects. If they know how you are feeling, they can find ways to relieve or manage your side effects to help you feel more comfortable and potentially keep any side effects from worsening.

You may find it helpful to keep track of your side effects so it is easier to talk about any changes with your health care team. Learn more about why tracking side effects is helpful.

Sometimes, side effects can last after treatment ends. Doctors call these long-term side effects. Side effects that occur months or years after treatment are called late effects. Treating long-term side effects and late effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

Coping with emotional and social effects

You can have emotional and social effects after your diagnosis. This may include dealing with a variety of emotions, such as sadness, anxiety, or anger, or managing your stress level. Sometimes, people find it difficult to express how they feel to their loved ones. Some have found that talking to an oncology social worker, counselor, or member of the clergy can help them develop more effective ways of coping and talking about their diagnosis and treatment.

You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your needs.

Coping with the costs of care

Treatment for a pheochromocytoma or paraganglioma can be expensive. Costs may be a source of stress and anxiety for patients and their families. In addition to treatment costs, many people find they have extra, unplanned expenses related to their care. For some people, the high cost of medical care stops them from following or completing their treatment plan. This can put their health at risk and may lead to higher costs in the future. Patients and their families are encouraged to talk about financial concerns with a member of their health care team. Learn more about managing financial considerations in a separate part of this website.

Coping with barriers to care

Some groups of people experience different rates of new cancer cases and experience different outcomes from their cancer diagnosis. These differences are called “cancer disparities.” Disparities are caused in part by real-world barriers to quality medical care and social determinants of health, such as where a person lives and whether they have access to food and health care. Cancer disparities more often negatively affect racial and ethnic minorities, people with fewer financial resources, sexual and gender minorities (LGBTQ+), adolescent and young adult populations, older adults, and people who live in rural areas or other underserved communities.

If you are having difficulty getting the care you need, talk with a member of your health care team or explore other resources that help support medically underserved people.

Talking with your health care team about side effects

Before starting treatment, talk with your doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they likely to happen?

  • What can we do to prevent or relieve them?

  • When and who should we call about side effects?

Be sure to tell your health care team about any side effects that happen during treatment and afterward, too. Tell them even if you do not think the side effects are serious. This discussion should include physical, emotional, social, and financial effects.

Caring for a loved one with a tumor

Family members and friends often play an important role in taking care of a person with a pheochromocytoma or paraganglioma. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away. Being a caregiver can also be stressful and emotionally challenging. One of the most important tasks for caregivers is caring for themselves.

Caregivers may have a range of responsibilities on a daily or as-needed basis, including:

  • Providing support and encouragement

  • Talking with the health care team

  • Giving medications

  • Helping manage symptoms and side effects

  • Coordinating medical appointments

  • Providing a ride to and from appointments

  • Assisting with meals

  • Helping with household chores

  • Handling insurance and billing issues

A caregiving plan can help caregivers stay organized and help identify opportunities to delegate tasks to others. It may be helpful to ask the health care team how much care will be needed at home and with daily tasks during and after treatment. Use this 1-page fact sheet to help make a caregiving action plan. This free fact sheet is available as a PDF, so it is easy to print.

Learn more about caregiving or read the ASCO Answers Guide to Caring for a Loved One With Cancer in English or Spanish.

Looking for More on How to Track Side Effects?

Cancer.Net offers several resources to help you keep track of your symptoms and side effects. Please note that these links will take you to other sections of Cancer.Net:

  • Cancer.Net Mobile: The free Cancer.Net mobile app allows you to securely record the time and severity of symptoms and side effects.

Cancer.Net Mobile app symptom tracker

  • ASCO Answers Managing Pain: Get this 32-page booklet about the importance of pain relief that includes a pain tracking sheet to help patients record how pain affects them. The free booklet is available as a PDF, so it is easy to print out.

  • ASCO Answers Fact Sheets: Read 1-page fact sheets on anxiety and depression, constipationdiarrhea and rash that provide a tracking sheet to record details about the side effect. These free fact sheets are available as a PDF, so they are easy to print, fill out, and give to your health care team.

The next section in this guide is Follow-up Care. It explains the importance of checkups after you finish treatment. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Follow-up Care

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will read about your medical care after treatment for a pheochromocytoma or paraganglioma is completed and why this follow-up care is important. Use the menu to see other pages.

Care for people diagnosed with a pheochromocytoma or paraganglioma does not end when active treatment has finished. Your health care team will continue to check that the tumor has not come back, manage any side effects, and monitor your overall health. This is called follow-up care.

People treated for a pheochromocytoma or paraganglioma need lifelong follow-up care. This is important because pheochromocytomas and paragangliomas can recur years after initial treatment. This care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead.

Generally, people treated for a catecholamine-producing pheochromocytoma or paraganglioma should have their blood and urine tested for increased levels of adrenaline and noradrenaline annually. For people with a tumor that does not produce catecholamines, imaging tests such as a computed tomography (CT) scan, magnetic resonance imaging (MRI), or metaiodobenzylguanidine (MIBG) scan, should be done annually for at least 10 years. If a tumor is linked to a hereditary syndrome, scans should be done for the rest of the patient's life, blood and urine tests should be done regularly, and there may be screening tests for other tumors linked to the inherited syndrome.

If both adrenal glands have been removed during surgery for pheochromocytoma, long-term medication will be needed to replace the hormones that are normally produced by these glands. You should also wear a medical alert bracelet. If you ever become sick or fall unconscious, the bracelet can inform health care professionals of your condition.

Rehabilitation may be recommended, and this could mean any of a wide range of services, such as physical therapy, occupational therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent as possible. Learn more about rehabilitation.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence, which means that the cancer has come back. A tumor recurs because small areas of tumor cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms.

During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will ask specific questions about your health. People may have tests done as part of regular follow-up care, but testing recommendations depend on several factors, including the type, location, and stage of tumor first diagnosed and the types of treatment given. During this time, it is also important to tell your doctor about any new signs or symptoms as soon as you notice them.

The anticipation before having a follow-up test or waiting for test results may add stress to you or a family member. This is sometimes called “scanxiety.” Learn more about how to cope with this type of stress.

Managing long-term and late side effects

Most people expect to have side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years after treatment has ended. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on your diagnosis, location of the tumor, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to discuss any concerns you have about your future physical or emotional health. ASCO offers forms to help track of the treatment you received and develop a survivorship care plan after treatment is completed.

This is also a good time to talk with your doctor about who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the care of their primary care doctor or another health care professional. This decision depends on several factors, including the type and stage of neuroendocrine tumor (NET), side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your medical care will lead your follow-up care, be sure to share your treatment summary and survivorship care plan forms with them and with all future health care providers. Details about your treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a diagnosis of a tumor. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Survivorship

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will read about how to cope with challenges in everyday life after being diagnosed with a tumor. Use the menu to see other pages.

What is survivorship?

The word “survivorship” is complicated because it means different things to different people. Common definitions include:

  • Having no signs of the tumor after finishing treatment.

  • Living with, through, and beyond the diagnosis. According to this definition,  survivorship begins at diagnosis and continues during treatment and through the rest of a person's life.

For some, even the term “survivorship” does not feel right, and they prefer to use different language to describe and define their experience. Sometimes extended treatment will be used for months or years to manage or control a tumor. Living with a tumor indefinitely is not easy, and the health care team can help you manage the challenges that come with it. Everyone has to find their own path to name and navigate the changes and challenges that are the results of their diagnosis and treatment.

Survivors may experience a mixture of feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after their diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain about coping with everyday life. Feelings of fear and anxiety may still occur as time passes, but these emotions should not be a constant part of your daily life. If they persist, be sure to talk with a member of your health care team.

Survivors may feel some stress when their frequent visits to the health care team end after completing treatment. Often, relationships built with the medical team provide a sense of security during treatment, and people miss this source of support. This may be especially true when new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexual health and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing

  • Thinking through solutions

  • Asking for and allowing the support of others

  • Feeling comfortable with the course of action you choose

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the place where you received treatment.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make lifestyle changes.

People recovering from a pheochromocytoma or paraganglioma are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, exercising regularly, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

It is important to have recommended medical checkups and tests (see Follow-up Care) to take care of your health.

Talk with your health care team to develop a survivorship care plan that is best for your needs.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with a tumor, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving.

Looking for More Survivorship Resources?

For more information about survivorship, explore these related items. Please note that these links will take you to other sections of Cancer.Net:

  • ASCO Answers Guide to Cancer Survivorship: Get this 48-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The free booklet is available as a PDF, so it is easy to print.

  • Survivorship Resources: Cancer.Net offers information and resources to help survivors cope, including specific sections for children, teens and young adults, and people over age 65. There is also a main section on survivorship for people of all ages.

The next section offers Questions to Ask the Health Care Team to help start conversations with your medical team. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team to help you better understand your diagnosis, treatment plan, and overall care. Use the menu to see other pages.

Talking often with your health care team is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for a digital list and other interactive tools to manage your care. It may also be helpful to ask a family member or friend to come with you to appointments to help take notes.Cancer.Net Mobile app question tracker

Questions to ask after getting a diagnosis

  • Where in my body is the tumor located?

  • Are there other tumors elsewhere? 

  • Is there a genetic cause for my tumor(s)? When will I be tested?

  • Is the tumor producing catecholamines?

  • What is the stage of the tumor? What does this mean?

  • Should I talk with a genetic counselor before I start treatment?

  • How much experience do you have treating this type of tumor?

  • Where can I get more information about my diagnosis and treatment?

Questions to ask about choosing a treatment and managing side effects

  • What are my treatment options?

  • What clinical trials are available for me? Where are they located, and how do I find out more about them?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment? Is it to eliminate the tumor, help me feel better, or both?

  • What are the possible side effects of this treatment, both in the short term and the long term?

  • What medications will reduce these side effects?

  • Could any of these side effects be permanent?

  • Who will be part of my health care team, and what does each member do?

  • Who will lead my overall treatment?

  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

  • Could this treatment affect my sex life? If so, how and for how long?

  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before treatment begins?

  • If I am worried about managing the costs of medical care, who can help me?

  • What support services are available to me? To my family?

  • If I have questions or problems, who should I call?

Questions to ask about having surgery

  • Can surgery remove the entire tumor? Why or why not?

  • If not, can surgery be done to debulk the tumor? What is the benefit of this?

  • What type of surgery do you recommend?

  • How long will the operation take?

  • How experienced is the surgeon and anesthesiologist with this type of surgery?

  • What medications will I be given before, during, and after surgery?

  • How long will I need to be in the hospital?

  • Can you describe what my recovery from surgery will be like?

  • Who should I contact about any side effects I experience? And how soon?

  • What are the possible long-term effects of having this surgery?

Questions to ask about having radiation therapy

  • What medications will I be given before, during, and after radiation therapy?

  • What type of radiation therapy do you recommend?

  • What is the goal of radiation therapy?

  • How many treatments will I need, and how long will each one take?

  • What side effects can I expect during treatment?

  • Who should I contact about any side effects I experience? And how soon?

  • What are the possible long-term or late effects of having radiation therapy?

  • What can be done to prevent or manage these side effects?

Questions to ask about having therapies using medications

  • What medications will I be given before, during, and after treatment?

  • Which drug(s) do you recommend?

  • What is the goal of this treatment?

  • How will the treatment be given? Through an IV, orally, or injected?

  • How often will I receive this treatment? For how long?

  • Will I receive this treatment at a hospital or clinic? Or will I take it at home?

  • What side effects can I expect during treatment?

  • Who should I contact about any side effects I experience? And how soon?

  • What are the possible long-term or late effects of this treatment?

  • What can be done to prevent or manage these side effects?

Questions to ask about other treatments

  • What other type(s) of treatment do you recommend?

  • What is the goal of this treatment?

  • What medications will I be given before, during, and after treatment?

  • How long will it take to give this treatment?

  • What side effects can I expect during treatment?

  • What are the possible long-term or late effects of having this treatment?

  • What can be done to prevent or manage the side effects?

Questions to ask about planning follow-up care

  • What is the chance that the tumor will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the treatment(s) I received?

  • What follow-up tests will I need, and how often will those tests be needed?

  • Will I need to have hormone replacement therapy if I was treated for pheochromocytoma? For how long?

  • How do I get a treatment summary and survivorship care plan to keep in my personal records?

  • Who will lead my follow-up care?

  • What survivorship support services are available to me? To my family?

The next section in this guide is Additional ResourcesIt offers more resources on this website that may be helpful to you. Use the menu to choose a different section to read in this guide.

Pheochromocytoma and Paraganglioma - Additional Resources

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about medical care and treatment. This is the final page of Cancer.Net’s Guide to Pheochromocytoma and Paraganglioma. Use the menu to go back and review other pages.

Cancer.Net includes many other sections about the medical and emotional aspects of being diagnosed with a tumor for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Pheochromocytoma and Paraganglioma. Use the menu to choose a different section to read in this guide.