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Neuroendocrine Tumors - Introduction

Approved by the Cancer.Net Editorial Board, 04/2019

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Neuroendocrine Tumors. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

About tumors

A tumor begins when the DNA of healthy cells is damaged, causing the cells to change and grow out of control, forming a mass. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body if it is not found early and treated. A benign tumor means the tumor can grow but will not spread. A benign tumor usually can be removed without it causing much harm.

About neuroendocrine tumors

A neuroendocrine tumor (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 are considered malignant tumors. Most NETs take years to develop and grow slowly. However, some NETs can be fast-growing. See the Grades section to learn more.

NETs can begin in any part of the body, including the:

  • Lung. The lung is the second most common location of NETs. About 30% of NETs occur in the bronchial system, which carries air to the lungs. Lung NETs used to be called carcinoid tumors. Learn more about NET of the lung.

  • Gastrointestinal (GI) tract. NETs develop most commonly in the GI tract, specifically in the small intestine (19%), appendix (4%), and large intestine (20%). The GI tract plays a central role in digesting foods and liquid and in processing waste. GI tract NETs also used to be called carcinoid tumors. Learn more about NET of the GI tract.

  • Pancreas. Approximately 7% of NETs can develop in the pancreas, a pear-shaped gland located in the abdomen between the stomach and the spine. Pancreas NETs used to be called islet cell tumors. Learn more about NET of the pancreas.

NETs can also begin in other organs. In about 15% of cases, a primary site cannot be found. Sometimes, NETs may develop in or on the adrenal glands. These rare types of NETs are called pheochromocytoma and paraganglioma, and they are discussed in another section of this website. Other types of tumors that begin in hormone-producing cells are also described in their own sections on Cancer.Net, including thyroid canceradrenal gland tumors, and pituitary gland tumors.

Classification of NETs

NETs may be classified, or described, in many different ways, including by where they are located, what the tumor cells look like under a microscope, and how fast the cells are growing and dividing (see Stages  and Grades sections of this guide). In addition, NETs are described as “functional” or “non-functional.” Functional NETs make and release hormones and other substances that cause symptoms. Carcinoid syndrome is 1 sign of a “functional” NET in which a substance called serotonin is released that can cause diarrhea and facial flushing (see Symptoms and Signs). Non-functional NETs either do not release substances or do not release enough substances to cause symptoms. About 60% of NETs are non-functional. Because of a lack of symptoms, NETs are usually diagnosed (see Diagnosis) at a more advanced stage.

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

Neuroendocrine Tumors - Statistics

Approved by the Cancer.Net Editorial Board, 04/2019

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

Overall, it is estimated that more than 12,000 people in the United States are diagnosed with a NET each year, and approximately 170,000 people are living with one. The number of people diagnosed with this type of tumor is increasing by more than 5% each year. This increase is thought to be mostly related to improvements in the way NETs are diagnosed, including better imaging tests and endoscopy, and increased awareness of these tumors.

The 5-year survival rate tells you what percent of people live at least 5 years after the cancer is found. Percent means how many out of 100. The 5-year survival rate for people with a NET varies and depends on several factors, including the type of NET, where the tumor is located, and whether the tumor can be removed using surgery. Visit the individual section for a specific type of NET for more information about survival statistics (see the Introduction for a list.)

It is important to remember that statistics on the survival rates for people with a NET are an estimate. The estimate comes from annual data based on the number of people with this type of tumor in the United States. Learn more about understanding statistics.

Statistics adapted from the Neuroendocrine Tumor Research Foundation and Dasari A, et al.: Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States. JAMA Oncol. 2017;3(10):1335–1342. doi:10.1001/jamaoncol.2017.0589.

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

Neuroendocrine Tumors - Risk Factors

Approved by the Cancer.Net Editorial Board, 04/2019

ON THIS PAGE: You will find out more about the factors that increase the chance of developing a NET. 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 cancer. 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 NETs is unknown, and no avoidable risk factors have been found. However, the following factors may raise a person’s risk for developing a NET:

  • Inherited syndromes. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition associated with certain types of NETs, including lung NETs, GI tract NETs, and pancreatic NETs.  Other hereditary conditions related to NETs include Von Hippel-Lindau syndrome, neurofibromatosis type 1, multiple endocrine neoplasia type 2 (MEN2), and tuberous sclerosis complex.

  • Race/ethnicity. In general, NETs are more common in white people than black people. However, the location of where the NET begins varies considerably by race.

  • Gender. NETs are slightly more common in women than in men, but the reason why is not known.

  • Age. A person of any age can develop a NET. However, children rarely develop NETs.

  • Other medical conditions. Certain diseases can put people at greater risk of developing specific types of NETs. For example, people with diseases that damage the stomach and reduce acid production have a greater risk of developing a NET of the stomach.

  • Environment and diet. There are no known connections between the environment and what a person eats and the risk of developing a NET.

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

Neuroendocrine Tumors - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 04/2019

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

NETs can be discovered for many different reasons because there is no single set of common symptoms. In its early stages, a NET often causes no symptoms and may only be detected during an unrelated x-ray or surgery for another condition. If a person with a NET has symptoms, those symptoms may be associated with the size and/or location of the tumor or with the release of hormones, such as carcinoid syndrome.

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

Symptoms of a NET

Depending on the type of NET, a person may experience the following tumor-related symptoms or signs:

General cancer symptoms:

  • Fatigue

  • Loss of appetite

  • Unexplained weight loss

Symptoms related to the tumor size and/or location:

  • Persistent pain in a specific area

  • Thickening or a lump in any part of the body

  • Nausea or vomiting

    • A cough or hoarseness that does not go away

    • Changes in bowel or bladder habits

    • Jaundice, which is the yellowing of the skin and whites of the eyes

    • Unusual bleeding or discharge

Symptoms related to the release of hormones:

  • Diarrhea

  • Facial flushing, usually without sweating

  • Hyperglycemia, which is a high level of glucose in the blood. Glucose is a sugar that is converted into energy by the body. Hyperglycemia causes frequent urination, increased thirst, and increased hunger.

  • Hypoglycemia, which is a low level of glucose in the blood. It causes fatigue, nervousness and shakiness, dizziness or light-headedness, sweating, seizures, and fainting.

  • Gastric ulcer disease

  • Skin rash

  • Confusion

  • Anxiety

  • Some people also experience nutritional deficiencies before a diagnosis, such as niacin and protein deficiency. Others develop this symptom later.

For symptoms related to a specific type of NET, visit its individual section (see the Introduction for a list.)

Carcinoid syndrome

Carcinoid syndrome is the classic example of a sign of a functional NET. It occurs most commonly in people with small intestine and lung NETs that have spread to another part of the body, called metastatic. In carcinoid syndrome, serotonin is produced by the tumor and can cause 1 or more of the following symptoms or signs. Serotonin is most easily and reliably measured in the urine, when it gets converted into 5-hydroxyindoleacetic acid (5-HIAA), and it is measured with a 24-hour urine collection test. It is important to note that these symptoms alone are not enough to diagnose carcinoid syndrome. Blood or urine tests to measure for suspected hormones are also needed to make a diagnosis. Not all people with a NET develop carcinoid syndrome.

  • Facial flushing, which is redness and a warm feeling over the face

  • Diarrhea

  • Shortness of breath

  • Wheezing or asthma-like symptoms

  • Unexplained weight gain

  • Weakness

  • Fast heartbeat

  • Heart murmur

  • High blood pressure and significant fluctuations in blood pressure

  • Carcinoid heart disease, which is a scarring of the heart valves caused by serotonin.

Stress, strenuous exercise, and drinking alcohol may make these symptoms worse. Some foods may also trigger the symptoms of carcinoid syndrome, including foods high in:

  • Amines, such as aged cheeses, yeast extracts, tofu, sauerkraut, and smoked fish and meats

  • Serotonin, such as walnuts, pecans, plantains, bananas, and tomatoes

Carcinoid crisis

Carcinoid crisis is a term used when people experience severe, sudden symptoms of carcinoid syndrome, usually in times of extreme stress, such as surgery. Carcinoid crisis primarily includes serious fluctuations in blood pressure and heart rate. Carcinoid crisis is the most serious and life-threatening complication of carcinoid syndrome. It is generally recommended that patients receive intravenous octreotide (Sandostatin), a medication that helps control the production of hormones, before a planned surgery. Octreotide is also used to treat carcinoid crisis.

Managing symptoms

If a NET is diagnosed, relieving symptoms remains an important part of your care and treatment. This may be called palliative care or supportive care. It is often started soon after diagnosis and continued throughout treatment. Below is information on how some of the symptoms of a NET can be managed. Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

  • Facial flushing. Avoid stress. Ask your doctor about specific substances and foods, including alcohol, that can cause facial flushing so you can avoid them. Somatostatin analogs, like octreotide and lanreotide (Somatuline Depot), can help. Read more about somatostatin analogs in Types of Treatment.

  • Diarrhea. There can be many causes of diarrhea in people with a NET. If your diarrhea is caused by carcinoid syndrome, somatostatin analogs (octreotide and lanreotide) and telotristat ethyl (Xermelo) can help. In addition, there are other medications that can help control diarrhea, depending on what is causing it. Ask your doctor for specific recommendations.

  • Wheezing. Ask your doctor about the use of a bronchodilator, a medication that relaxes the muscles in the lungs to make breathing easier.

  • Heart valve problems. Tell your doctor immediately if you think you may have a problem with your heart. Signs of this problem include difficulty breathing and becoming easily tired during exercise.

Learn more about managing common cancer symptoms and treatment side effects.

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.

Neuroendocrine Tumors - Diagnosis

Approved by the Cancer.Net Editorial Board, 04/2019

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 NET. They also do tests to learn if cancer has spread to another part of the body from where it started. If this happens, 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.

For most types of tumors, a biopsy is the only sure way for the doctor to know if an area of the body has a tumor. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

This section describes options for diagnosing a NET. Not all tests listed below will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • The type of NET suspected

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

Most NETs are found unexpectedly when people have x-rays or a medical procedure done for reasons unrelated to the tumor. If a doctor suspects a NET, he or she will ask for a complete medical and family history and perform a thorough physical examination. In addition, the following tests may be used to diagnose a NET:

  • Biopsy. biopsy is the removal of a small amount of tissue for examination under a microscope and is required to make a diagnosis of NET. A pathologist then analyzes the sample(s) removed during the biopsy. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. It should be noted that tumors suspected to be a pheochromocytoma should not be biopsied, unless it is absolutely necessary to confirm a diagnosis. A biopsy of these tumors can be life-threatening. See the Pheochromocytoma and Paraganglioma section for more information on this type of NET.

  • Blood/urine tests. The doctor may need samples of your blood and urine to check for abnormal levels of hormones and other substances. Urine tests check the amount of 5-HIAA, a breakdown product of serotonin which is made by some NETs. Measurements of blood serotonin levels may also be taken, however these are less reliable due to fluctuations. A blood test to measure chromogranin A, a protein produced by tumor cells, is also sometimes performed after diagnosis to watch the tumor’s response to treatment. Chromogranin A is not part of diagnostic testing.

  • Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tumor to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests can help determine your treatment options. This testing is less helpful in NETs than in other cancers.

  • Endoscopy. An endoscopy allows the doctor to see inside the body with a thin, lighted, flexible tube called an endoscope. There are many different types of endoscopy, including upper endoscopy to examine the esophagus and stomach and colonoscopy to examine the rectum and colon. The person may be sedated as the tube is gently inserted into the body. Sedation is giving medication to become more relaxed, calm, or sleepy. If an abnormality is found, a biopsy will be performed (see above).

  • Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. Tumors generate different echoes of the sound waves than normal tissue. This means that when the waves are bounced back to a computer, creating images, the doctor can locate a mass inside the body. There are different types of ultrasounds used to examine different parts of the body. In some cases, a biopsy may be done during the procedure.

  • X-ray. An x-ray is a way to create a picture of the structures inside the body using a small amount of radiation. Sometimes, a NET may not show up on an x-ray because of its size or location, so the doctor may also recommend other types of scans. If the doctor suspects a GI tract NET, a barium x-ray may be done. A person swallows a liquid containing barium that coats the lining of the esophagus, stomach, and intestines. Then a series of x-rays is taken.

  • 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 tumors. A CT scan can be used to measure the tumor’s size. Additionally, a CT scan is used to see if a tumor has spread to the liver. 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.

  • 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. There are a few available methods of nuclear medicine imaging in NETs, including OctreoScan and 2 types of PET-CT scans.

  • OctreoScan. This imaging test uses octreotide linked with indium-111 as the tracer. This procedure is useful for identifying where a NET has spread, especially if it has spread to the liver. OctreoScan is slowly being replaced by the newer and more sensitive gallium-68 DOTATATE PET scan (see below).

  • 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 2 types of tracers used in a PET scan include gallium-68 ( 68Ga) DOTATATE and (18F) fluorodeoxyglucose (FDG). The 68Ga DOTATATE PET is the primary scan used to look at slow-growing NETs (grades 1 and 2, see Grades). An FDG PET scan is sometimes used for faster-growing NETs (grade 3).

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is a NET, 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.

Neuroendocrine Tumors - Stages

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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.

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. Stage and grade may be discussed at the same time when NETs are being discussed, but they are very different elements in understanding the risk a tumor poses to a person. This section covers staging for NETs.

Doctors use diagnostic tests to find out the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis, which is the chance of recovery. There are different stage descriptions for different types of NETs

Staging systems for NETs

NETs of the GI tract and pancreas have their own staging systems based on the location and characteristics of the tumor. Some NETs use the staging system for other cancers. For example, the staging of a lung NET is the same as the staging of non-small cell lung cancer. These staging systems are created by the American Joint Committee on Cancer (AJCC).

Detailed information about the staging for a specific type of NET can be found in each specific NET section. See the Introduction for a list of those NETs.

In general, a lower number stage is linked with a better outcome. However, no doctor can predict how long a patient will live with a NET based only on the stage of disease.

Recurrent NETs

Recurrent cancer is cancer that has come back after treatment. If the NET 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.

The next section in this guide is Grades. It explains the system doctors use to describe how fast the tumor cells are growing and dividing. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumors - Grades

Approved by the Cancer.Net Editorial Board, 04/2019

ON THIS PAGE: You will learn about how doctors describe how fast the tumor cells are growing and dividing, called the grade or degree of differentiation. Use the menu to see other pages.

Doctors may describe a NET by its World Health Organization (WHO) grade (G). Knowing the grade helps the doctor create a treatment plan for the NET. Grade and stage may be discussed at the same time when NETs are being discussed, but they are very different elements in understanding the risk a tumor poses to a person. This section covers grading for NETs. The WHO grades for NETs include:

  • Grade 1 (low-grade tumor): These cells divide at a low rate and therefore grow slowly.

  • Grade 2 (intermediate-grade tumor): These cells divide at an intermediate rate.

  • Grade 3 (high-grade tumor): These cells divide at a fast rate and therefore grow quickly.

For many types of cancer, the grade also describes how much cancer cells look like healthy cells when viewed under a microscope, called degree of differentiation.

  • Well differentiated: The cells are well differentiated, which means they look more like healthy cells.

  • Poorly differentiated: The cells are poorly differentiated, which means they look less like healthy cells.

Mitosis is the process by which tumor cells grow and divide. Grading includes measurements of how fast mitosis is happening. To measure mitosis, the doctor counts the number of dividing cells seen in 10 high-power fields under a microscope. This is called the mitotic count. The doctor also measures the Ki-67 index. Ki-67 is a protein in cells that increases as they prepare to divide. If there is a high percentage of cells in an area with Ki-67, it means that the cells are dividing rapidly. The Ki-67 index is an indicator of how quickly the tumor cells are multiplying. 

The tumor’s grade and degree of differentiation may help the doctor predict how quickly the NET will grow and spread. In general, the lower the tumor’s grade and degree of differentiation, the better a person’s prognosis.

More detailed information about grading for a specific type of NET can be found in each specific NET section. See the Introduction for a list of those NETs.

Information about the tumor’s stage and grade 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.

Neuroendocrine Tumors - Types of Treatment

Approved by the Cancer.Net Editorial Board, 04/2019

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

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

Treatment overview

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

Treatment options and recommendations depend on several factors, including:

  • The type of NET

  • Stage and grade

  • Possible side effects

  • 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 a NET because there are different treatment options. Learn more about making treatment decisions.

Descriptions of the common types of treatments used for a NET are listed below. Your care plan also includes treatment for symptoms and side effects, an important part of cancer care. Visit the individual section for a specific type of NET for more information about treatment options (see the Introduction for a complete list).

Active surveillance

Active surveillance, which is also called watchful waiting or watch-and-wait, may be recommended sometimes. This approach is used most often for a person with a low-grade NET that may grow slowly and not spread or cause problems for many months or years. Active treatment usually begins if the tumor shows signs of growing or spreading. With this approach, the tumor is closely monitored with regular tests, which may include:

  • Imaging tests, usually CT scans or sometimes MRI scans (see Diagnosis)

  • Blood tests

  • Physical examinations

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. Completely removing the entire tumor is the standard treatment, when possible. Most localized NETs are successfully treated with surgery alone. The surgeon will usually remove some tissue surrounding the tumor, called a margin, in an effort to leave no traces of cancer in the body.

When completely removing the 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 cure a NET.

People who have developed carcinoid syndrome are at risk of experiencing a carcinoid crisis during surgery (see Symptoms and Signs). To avoid major complications from a carcinoid crisis, the anesthesiology team must be fully aware of this risk before surgery, so they can have treatment on hand to control the symptoms. Intravenous octreotide is usually given before surgery to prevent carcinoid crisis.

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

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

Therapies using medication

Systemic therapy is the use of medication to destroy cancer cells. Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle, in a pill or capsule that is swallowed (orally), and intramuscular (IM) injections. This type of medication can reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

Depending on the type of NET, systemic therapies that may be used include:

  • Somatostatin analogs

  • Chemotherapy

  • Targeted therapy

  • Immunotherapy

  • Peptide receptor radionuclide therapy (PRRT)

Each of these types of therapies are discussed below in more detail. A person may receive 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 NETs 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 medications for NET. Learn more about your prescriptions by using searchable drug databases.

Somatostatin analogs

Somatostatin is a hormone in the body that controls the release of several other hormones, such as insulin and glucagon. Somatostatin analogs are drugs that are similar to somatostatin and are used to control the symptoms created by the hormones-like substances released by a NET. They may also slow the growth of a NET, although they do not generally shrink the tumors.

There are 2 somatostatin analogs used to treat NETs, octreotide (Sandostatin) and lanreotide (Somatuline Depot). Octreotide is available in 2 forms: short-acting is given under the skin (subcutaneously) and long-acting is given as an intramuscular (IM) injection. Lanreotide is given as a long-acting subcutaneous injection. The most common side effects are high blood sugars, the development of gallstones, and mild digestive system upset, such as bloating and nausea. Talk with your health care team about what you can expect and how side effects will be managed.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells.

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. Chemotherapy for a NET may be used when the tumor has spread from where it started to other organs, if the tumor is causing severe symptoms, or if hormonal or targeted therapies are no longer working. Chemotherapy may be used alone or in combination with other treatments.

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.

Targeted therapy

Targeted therapy is a treatment that targets the tumor’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 tumor cells while limiting 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. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Everolimus (Afinitor) is a targeted therapy approved by the U.S. Food and Drug Administration (FDA) for the treatment of advanced NETs of the GI tract, lung, and pancreas. This drug can help slow down the growth of these tumors in some patients, but it does not usually shrink tumors. Side effects include mouth sores, lowering of blood counts, and fatigue.

Another targeted therapy, sunitinib (Sutent), which targets a protein called VEGF, is also approved by the FDA for the treatment of advanced pancreatic NETs.

Other targeted therapies for NETs are being researched in clinical trials. They include drugs that interfere with new blood vessel formation or with specific survival pathways of cancer cells.

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the tumor. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Interferon alfa-2b (Intron A) is a type of immunotherapy that has been used to treat NETs. Interferon helps the body’s immune system work better and can lessen diarrhea and flushing. It may also shrink tumors.

Different types of immunotherapy can cause different side effects. Interferon alfa-2b may cause flu-like symptoms, fatigue, and low blood counts. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy.

Peptide receptor radionuclide therapy (PRRT)

In 2018, the FDA approved a treatment called 177Lu-dotatate (Lutathera) for advanced GI tract NETs and pancreas NETs. GI tract and pancreas NETs are sometimes referred to as GEP NETs. This treatment is being studied for the treatment of other NETs. It is a radioactive drug that works by binding to a cell’s somatostatin receptor, which may be present on certain tumors. After binding to the receptor, the drug enters the cell, allowing radiation to damage the tumor cells. The broader term to describe this treatment is peptide receptor radionuclide therapy (PRRT).

Common side effects include low levels of white blood cells, high enzyme levels in some organs, high blood sugar levels, low level of potassium, and nausea and vomiting. Talk with your doctor about ways these can be avoided or managed.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy tumor cells. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.

The most common type of radiation treatment for a NET is called external-beam radiation therapy, which is radiation given from a machine outside the body. It is most often used as part of supportive care to relieve symptoms, such as pain, caused by cancer that has spread to the bone and other areas of the body (see “Physical, emotional, and social effects of cancer” below).

Patients receiving radiation therapy may experience fatigue during treatment, and the treated area may become red and dry. Radiation therapy to the chest or neck may cause a dry, sore throat or a dry cough. Some patients have shortness of breath during radiation therapy. Most side effects go away after the treatment is finished. Learn more about the basics of radiation therapy.

Liver-directed treatment

If cancer has spread to the liver, the treatments below may be used. These procedures are usually performed by an experienced interventional radiologist and may require an overnight hospital stay. Side effects include pain around the liver, fever, and higher levels of liver enzymes as measured by blood tests for a few days or weeks after treatment.

  • Radiofrequency ablation (RFA).  RFA destroys a tumor by heating it with an electric current. It is usually used for small liver metastases and does not work well on larger tumors.

  • Hepatic artery embolization. This procedure blocks the tumor’s blood supply by sealing off the blood vessels leading to the tumor. If embolization is done by itself, it is called bland embolization. When combined with chemotherapy, it is called chemoembolization. It is not yet clear if 1 treatment is more effective than the other. These treatments are usually used for people with metastatic disease that mostly affects the liver, in particular those who have symptoms caused by the size of the tumor or by hormones produced by the tumor.

  • Radioembolization. This form of radiation therapy involves the use of tiny beads made of glass or resin that contain low levels of a radioactive material called yttrium-90. The beads are put into the blood vessel that sends blood to the tumor in the liver. The beads then become stuck in the liver and deliver the radiation directly to the tumor. This treatment avoids exposing other areas of the body to radiation. There are 2 FDA-approved methods of radioembolization: SIR-Spheres and TheraSphere.

Physical, emotional, and social effects of a NET

A NET 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 tumor, may receive this type of care. And it often works best when it is started right after a diagnosis. People who receive palliative care along with treatment for the tumor 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 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.

For some people, such as those with lung disease, heart disease, or other specific medical conditions, surgery cannot successfully treat the tumor. In these cases, palliative surgery to relieve symptoms may be helpful. Palliative surgery is often used together with radiation therapy.

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

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

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

Metastatic NETs

If a NET 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.

Your treatment plan may include a combination of the types of treatment described above. Surgery is used to relieve symptoms rather than eliminate the cancer at this stage. If distant metastases are not causing symptoms, surgery may not be needed. If the person develops carcinoid syndrome, surgery to remove as much cancer as possible is often recommended. Chemotherapy and radiation therapy may also be offered to help relieve symptoms.

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 a NET 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 tumor will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the NET returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the tumor does return. Learn more about coping with the fear of recurrence.

If the NET returns after the original treatment, it is called recurrent NET. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

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, chemotherapy, and radiation therapy, 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 this type of recurrent tumor. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

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

If treatment does not work

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

This diagnosis is stressful, and for many people, advanced NET 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 disease 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 NET. Use the menu to choose a different section to read in this guide.

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ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested 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 a NET. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the FDA was tested in clinical trials.

Clinical trials are used for all types and stages of NETs. 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 people, a clinical trial is the best treatment option available. Because standard treatments are not perfect, people are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other people volunteer for clinical trials because they know that these studies are a way to contribute to the progress in treating a NET. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with a NET.

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. When a placebo is used in a study, it is done with the full knowledge of the participants. 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.

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.

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 cancer. For specific topics being studied for NETs, learn more in the Latest Research section.

Cancer.Net offers a lot of 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 cancer clinical trials in another section of this website.

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

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

Doctors are working to learn more about NETs, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with this type of tumor. 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.

  • Immunotherapy. As described in Types of Treatment, immunotherapy, also called biologic therapy, is designed to boost the body’s natural defenses to fight a tumor. Researchers are testing new cancer vaccines on NETs. Another specific area of immunotherapy research is looking at drugs that block a protein called PD-1. PD-1 is found on the surface of T-cells, which are a type of white blood cell that helps the body’s immune system fight disease. PD-1 stops the immune system from destroying tumor cells, so stopping PD-1 from working allows the immune system to better eliminate the disease.

    The FDA has approved a PD-L1 inhibitor, atezolizumab (Tecentriq), in combination with carboplatin and etoposide chemotherapy (both available as generic drugs) for the treatment of metastatic small cell lung cancer. NETs of the lung are a very different disease, but the effectiveness of atezolizumab shows the need to study immunotherapy specifically in those with a lung NET.

    Also, chimeric antigen receptor therapy (CAR T-cell therapy) combined with antibody-drug conjugates, a type of targeted therapy, is being explored. In CAR T-cell therapy, a patient's T lymphocytes (T cells), which are part of their immune system, are removed from the patient and then reprogrammed to target cancer cells in the patient. Then the T cells are returned to the patient’s body.

  • Targeted therapy and combined treatments. As noted in Types of Treatment, research continues on targeted therapy. Monoclonal antibodies are a type of targeted therapy that blocks tumor growth in different ways. Some block the ability of tumor cells to grow and spread. Others find tumor cells and help destroy them or carry tumor-destroying substances to them. 

  • Chemotherapy. Because NETs have unique properties that healthy cells do not, researchers are trying to develop “smart” chemotherapy that only attacks tumor cells and not all cells.

  • Genetics. As described in Risk Factors, family history of MEN1 may play a role in an individual’s risk for developing a NET. Researchers are investigating MEN1 to learn more about this and other hereditary risk factors, as well as the role mutations, or changes, in genes plays in NETs.

  • PRRT. Research is underway on how to improve PRRT (See Types of Treatment) for NETs. Studies are looking at ways to improve the ability of tumor cells to attach to radiopeptides, how to use PRRT to eliminate very small traces of a NET, and ways to reduce other organs being exposed to radiation.

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

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding NETs, explore these related items that take you outside of this guide:

  • To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now.

  • Visit the Cancer.Net Blog to review research announced at recent scientific meetings or in ASCO’s peer-reviewed journals.

  • Visit the website of ASCO’s Conquer Cancer Foundation to find out how to help support cancer research. Please note that this link takes you to a separate ASCO website.

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

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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 for NET 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 cancer. 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. Doctors call this part of cancer treatment “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 NETs are listed in the Types of Treatment section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the tumor’s stage and grade, 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 explain any changes with your health care team. Learn more about why tracking side effects is helpful.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment 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 as well as physical effects after a diagnosis of NET. This may include dealing with difficult 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 the diagnosis.

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 financial effects

Treatment for a NET can be expensive. Costs are often a big 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.

Caring for a loved one with a NET

Family members and friends often play an important role in taking care of a person with a NET. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away.

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

Learn more about caregiving.

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?

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, and social effects of a NET.

Also, ask how much care you may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan. Create a caregiving plan with this 1-page fact sheet that includes an action plan to help make caregiving a team effort. This free fact sheet is available as a PDF, so it is easy to print out.

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.

  • ASCO Answers Managing Pain: Get this 36-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 diarrhea and rash that provide a tracking sheet to record the timing and severity of the side effect. These free fact sheets are available as a PDF, so they are easy to print out.

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

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

Care for people diagnosed with a NET 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.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. This is important because a NET can recur even several years after treatment. While there are no standard guidelines for follow-up care after treatment of a NET, people who have had surgery should be seen by their doctor 3 months after their operation for a physical examination, blood tests, and a CT scan. After that, follow-up care should include a physical examination and blood tests about every 6 to 12 months, with additional imaging studies such as x-rays, as needed. 

Patients and families should be aware that many NETs tend to grow slowly and may be similar to a chronic illness. This means the patient will receive treatment and follow-up care in cycles on an ongoing basis. Learn more about living with chronic cancer and 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 NET 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. Some people may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors, including the type and stage of the NET originally diagnosed and the types of treatment given. During this time, it is also important to tell your doctor about any new symptoms as soon as you notice them, such as fatigue, breathing problems, or pain in any part of the body.

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

Managing long-term and late side effects

Most people expect to experience 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 afterwards. 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 the type of NET, your individual treatment plan, and your overall health. For example, some patients experience late effects after being treated with octreotide, such as thyroid and gallbladder problems. 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 keep track of the cancer treatment you received and develop a survivorship care plan when 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 family doctor or another health care professional. This decision depends on several factors, including the type, stage, and grade of the tumor, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your care for the NET will lead your follow-up care, be sure to share your treatment summary and survivorship care plan forms with him or her and with all future health care providers. Details about your treatment for the NET 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 NET diagnosis. Use the menu to choose a different section to read in this guide.

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

What is survivorship?

The word “survivorship” means different things to different people. Common definitions include:

  • Having no signs of tumor after finishing treatment.

  • Living with, through, and beyond this diagnosis. According to this definition, survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

Survivorship is one of the most complicated parts of having a tumor. This is because it is different for everyone.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a tumor diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain about coping with everyday life.

Survivors may feel some stress when their frequent visits to the health care team end after completing treatment. Often, relationships built with the medical care 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.

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.

A new perspective on your health

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

People recovering from a NET are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, 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. Cancer rehabilitation may be recommended, and this could mean any of a wide range of services such as physical 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 and productive as possible.

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

Looking for More Survivorship Resources?

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

  • ASCO Answers Cancer Survivorship Guide: Get this 44-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 out.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including those in different age groups.

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

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ON THIS PAGE: You will find some questions to ask your doctor or other members of the 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 the 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.

Questions to ask after getting a diagnosis

  • Where is the NET located?

  • Can you explain my pathology report (laboratory test results) to me?

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

  • What is the grade? What does this mean?

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

  • Do I have carcinoid syndrome? What does this mean?

  • Is my heart affected by the tumor? How will you monitor my heart’s health and performance?

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 each treatment, both in the short term and the long term?

  • What medications will reduce these side effects?

  • 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?

  • Are there medications that can help control symptoms my tumor causes?

  • 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’m 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 problems or questions, 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 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 therapies using medication

  • 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 therapy? For how long?

  • Will I receive it 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 effects of this treatment?

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

Questions to ask about having 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 effects of having radiation therapy?

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

Questions to ask about planning follow-up care

  • What is the chance that the NET 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 I received?

  • What follow-up tests will I need, and how often will I need them?

  • 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 Resources. It offers some more resources on this website that may be helpful to you. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumors - Additional Resources

Approved by the Cancer.Net Editorial Board, 04/2019

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 Neuroendocrine Tumor. Use the menu to go back and see 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 Neuroendocrine Tumors. Use the menu to choose a different section to read in this guide.