View All Pages

Neuroendocrine Tumor of the Pancreas - Introduction

Approved by the Cancer.Net Editorial Board, 06/2019

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 Tumor of the Pancreas. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

About neuroendocrine 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.

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 Stages and Grades section to learn more.

NETS can begin in any part of the body, including the pancreas. Pancreas NETs used to be called islet cell tumors. NETs are more commonly found in the gastrointestinal (GI) tract (learn more about NETs of the GI tract) or in the lungs (learn more about NETs of the lung). GI tract NETs and lung NETs used to be called carcinoid tumors. Rare types of NET that develop in or on the adrenal glands are called pheochromocytoma or paraganglioma. Learn more about NETs that develop in other parts of the body.

About pancreas NETs

Approximately 7% of NETs develop in the pancreas. The pancreas is a pear-shaped gland located in the abdomen between the stomach and the spine. It is about 6 inches long and is made up of 2 major components:

  • Exocrine component. This component is made up of ducts with small sacs called acini at the end. It produces specialized proteins called enzymes that are released into the small intestine to help the body digest and break down food, particularly fats.

  • Endocrine component. This part of the pancreas is made up of specialized cells clustered together in islands within the organ, called islets of Langerhans. These cells make hormones, specifically insulin. Insulin is the substance that helps control the amount of sugar in the blood.

Pancreas NETs develop in the islets of Langerhans in the endocrine component.

A pancreas NET is very different from pancreas adenocarcinoma. This type of pancreatic cancer begins in the exocrine component. You can read about adenocarcinoma of the pancreas in another section of this website.

Subtypes of pancreas NETs

A pancreas NET may be classified as either “functional” or “non-functional.” The cells in a functional pancreas NET make and release measurable hormones and other substances that cause symptoms. A non-functional pancreas NET either does not release substances or does not release enough substances to cause symptoms. Most pancreas NETs are non-functional. Because of a lack of symptoms, these tumors are usually diagnosed at a more advanced stage.

There are 5 major classifications of functional pancreas NETs. These types are based on the hormone that the cells normally make. However, some tumors can make 2 or more of these hormones at the same time.

  • Gastrinoma. A gastrinoma is a pancreas NET that makes too much gastrin, a hormone that causes acid production in the stomach. Too much stomach acid can cause severe ulcers, a condition called Zollinger-Ellison syndrome.

  • Insulinoma. This type of tumor makes too much insulin, causing hypoglycemia, also known as low blood sugar. An insulinoma is more likely to be noncancerous. Only 10% become cancerous.

  • Glucagonoma. A glucagonoma is a pancreas NET that makes too much of the hormone glucagon. Unlike an insulinoma, a glucagonoma causes hyperglycemia, a condition where there is too much sugar in the blood.

  • VIPoma. A VIPoma starts in the cells in the pancreas that make vasoactive intestinal peptide (VIP), a hormone that helps move water into the intestines. Too much VIP can cause chronic, watery diarrhea, which causes a condition called Verner-Morrison syndrome.

  • Somatostatinoma. A somatostatinoma usually develops in the head of the pancreas. A somatostatinoma may make a hormone called somatostatin. This hormone stops the production of several other hormones, such as growth hormone, insulin, and gastrin.

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

Neuroendocrine Tumor of the Pancreas - Statistics

Approved by the Cancer.Net Editorial Board, 06/2019

ON THIS PAGE: You will find information about the number of people who are diagnosed with a pancreas 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.

Pancreas NETs are uncommon, with about 1,000 people diagnosed each year in the United States. Pancreas NETs account for about 7% of all cancers in the pancreas. The number of all diagnosed NETs has been increasing by around 5% per year. This increase is thought to be mostly related to improvements in the way NETs are found, including imaging tests and endoscopy, and increased awareness of these tumors.

People are usually diagnosed with a pancreas NET between the ages of 30 and 60. However, when these types of tumors are the result of a genetic syndrome (see Risk Factors), the age of diagnosis is generally earlier, during childhood or young adulthood.

The 5-year survival rate tells you what percent of people live at least 5 years after the tumor is found. Percent means how many out of 100. In general, the 5-year survival rate for people with a pancreas NET is about 42%.

However, the survival rate depends on a variety of factors, including whether the tumor can be removed using surgery. If a tumor is localized and can be removed, the 5-year survival rate is about 55%. If a tumor cannot be removed, the 5-year survival rate is about 15%.

It is important to remember that statistics on the survival rates for people with a pancreas 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. Also, experts measure the survival statistics every 5 years. So the estimate may not show the results of better diagnosis or treatment available for less than 5 years. Talk with your doctor if you have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the National Cancer Institute, the National Organization for Rare Disorders, and the Neuroendocrine Tumor Research Foundation (January 2019).

The next section in this guide is Medical Illustrations. It offers drawings of body parts often affected by a pancreas NET. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Pancreas - Risk Factors

Approved by the Cancer.Net Editorial Board, 06/2019

ON THIS PAGE: You will find out more about the factors that increase the chance of developing a pancreas 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 the tumor. 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.

  • Inherited genetic syndromes. Researchers have found that 3 genetic syndromes may increase a person’s risk of developing a pancreas NET: neurofibromatosis type 1 (NF1), multiple endocrine neoplasia type 1 (MEN1), and Von Hippel-Lindau syndrome (VHL). These syndromes can be passed from generation to generation in a family.

  • Other potential risk factors. There are other potential risk factors but the scientific evidence supporting the connection to a pancreas NET is weaker. These include smoking, alcohol, family history of cancer, diabetes, and chronic pancreatitis, which is inflammation of the pancreas.

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

Neuroendocrine Tumor of the Pancreas - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 06/2019

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.

A pancreas NET often causes no symptoms in its early stages. People with a pancreas NET may experience the following symptoms or signs. Sometimes, people with a pancreas NET do not have any of these changes. Or, the cause of a symptom may be a different medical condition that is not a tumor.

Symptoms of a gastrinoma

  • Ulcers, caused by too much stomach acid

  • Diarrhea

Symptoms of an insulinoma

  • Hypoglycemia, which causes fatigue, nervousness and shakiness, dizziness or lightheadedness, seizures, and fainting episodes

  • Confusion

Symptoms of a glucagonoma

  • Hyperglycemia, which causes frequent urination, increased thirst, and increased hunger

  • Rash that spreads on the face, abdomen, or lower extremities

Symptoms of a VIPoma

  • Watery diarrhea

  • Too little potassium in the blood, which can cause an irregular heartbeat, muscle cramping and weakness, and decreased reflexes

  • Too little acid in the stomach, which can cause digestive problems and poor absorption of vitamins and nutrients

  • Flushing or redness of the face, neck, or chest

  • Fatigue

  • Nausea

Symptoms of a somatostatinoma

  • Type 2 diabetes

  • Gallstones

  • Steatorrhea, a condition where the body cannot absorb fat, causing oily and loose stools with a particularly bad odor
  • Diarrhea

  • Weight loss

  • Too little hydrochloric acid in the stomach, which can cause digestive problems and poor absorption of vitamins and nutrients

Other symptoms

  • Abdominal pain

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

  • Vomiting blood

  • Sweating

  • Rapid heart rate

  • Anxiety

  • Headache

  • Convulsions

  • Loss of consciousness

  • Clouding of vision

  • Unexplained weight gain or loss

  • Inflamed mouth and tongue

  • A mass or lump in the abdomen

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 tumor 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. Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

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 Tumor of the Pancreas - Diagnosis

Approved by the Cancer.Net Editorial Board, 06/2019

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 pancreas 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 pancreas 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 tumor suspected

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

Most pancreas NETs are found unexpectedly when people have x-rays or medical procedures done for reasons unrelated to the tumor. If a doctor suspects this type of tumor, 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 pancreas NET:

  • Blood/urine tests. The doctor may take samples of blood and urine to check for abnormal levels of hormones, glucose, and other substances.

  • Biopsy. A 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.

  • Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tumor sample 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.

  • 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.

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

    There are different types of ultrasounds. During a standard transabdominal ultrasound, the probe is placed on the outside of the abdomen. However, these images are often not clear enough to diagnose small changes in the pancreas and other organs.

    During an endoscopic ultrasound, the probe is connected to the end of a thin, flexible, lighted tube called an endoscope. This tube is carefully slid down the esophagus into the stomach and duodenum, the top part of the small intestine. This type of ultrasound gives a clearer picture of the pancreas and other organs. Learn more about endoscopic techniques.

  • 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. Sometimes, a pancreas 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. In some cases, the patient will be asked to swallow barium, which coats the mouth and throat, to provide better detail on the x-ray. This is called a barium 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. Pancreas NETs are sometimes best shown using an MRI, especially if they have spread to the liver.

  • 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 Stages and 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 pancreas NET, these results also help the doctor describe the tumor. This is called staging and grading.

The next section in this guide is Stages and Grades. 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 Tumor of the Pancreas - Stages and Grades

Approved by the Cancer.Net Editorial Board, 06/2019

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. In addition, you can read about how doctors describe how fast the tumor cells are growing and dividing, called the grade. 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.

Doctors use diagnostic tests to find out the tumor'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 it can help predict a patient's prognosis, which is the chance of recovery. For example, people with a smaller tumor may not need surgery, while many people with a larger tumor do. This is because a pancreas NET that is smaller than 2 centimeters (cm) has a lower risk of spreading. There are different stage descriptions for different types of cancer.

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 answers 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 cancer for each person.

For a pancreas NET, there are 4 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 a pancreas NET.

Tumor (T)

Using the TNM system, the "T" plus a letter or number (1 to 4) is used to describe the size and location of the tumor. If the T is followed by a number in parentheses or the letter “m,” it indicates the number of tumors found or that multiple tumors are found. For example, a T2(3) would mean that the largest tumor found is T2 size and that there are 3 tumors. Tumor size is measured in centimeters (cm). A centimeter is roughly equal to the width of a standard pen or pencil.

Stages 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 tumor cannot be evaluated.

T1: The tumor is limited to the pancreas and is less than 2 cm in size.

T2: The tumor is limited to the pancreas and is 2 cm to 4 cm in size.

T3: The tumor is limited to the pancreas and is greater than 4 cm in size, or the tumor is invading the duodenum or bile duct.

T4: The tumor has grown into the stomach, spleen, colon, or adrenal gland, or the wall of the celiac axis or the superior mesenteric artery, which are large vessels.

Node (N)

The "N" in the TNM system stands for lymph nodes. These tiny, 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: Regional lymph nodes cannot be evaluated.

N0 (N plus 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 distant metastasis.

M0 (M plus zero): There is no distant spread.

M1: There is distant metastasis.

  • M1a: The cancer has spread only to the liver.

  • M1b: The cancer has spread to at least 1 area beyond the liver, such as a lung, ovary, a nonregional lymph node, the peritoneum, or a bone.

  • M1c: The cancer has spread to both nearby and distant parts of the body.

Cancer stage grouping

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

Stage I: There is a small tumor in the pancreas and it has not spread elsewhere in the body (T1, N0, M0).

Stage II: The tumor is larger and has not spread to the regional lymph nodes or elsewhere in the body (T2 or T3, N0, M0).

Stage III: The tumor is any size and has spread to the regional lymph nodes (any T, N1, M0). Or, the tumor has spread to the stomach, spleen, colon, or adrenal gland, or the wall of the celiac axis or the superior mesenteric artery, but the cancer has not spread to the regional lymph nodes or anywhere else (T4, N0, M0).

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

Grade (G) and degree of differentiation

Doctors also describe a pancreas NET by its grade (G) and degree of differentiation, both features that are determined when the tumor cells are viewed under the microscope.

For NETs, the grade describes how fast the tumor cells are growing and dividing, measured in 2 ways: mitosis and Ki-67. 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 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 may help the doctor predict how quickly the cancer will spread. In general, the lower the tumor’s grade, the better the prognosis.

GX: Grade cannot be evaluated.

G1: Mitotic index is less than 2, or Ki-67 index is less than 3.

G2: Mitotic index is between 2 and 20, or Ki-67 index is 3 to 20.

G3: Mitotic index is more than 20, or Ki-67 index is more than 20.

Grade 1 and grade 2 NETs tend to grow slowly. Grade 3 NETs tend to grow fast and are more aggressive. The final grade is based on whichever index (mitotic index or Ki-67) places the tumor in the highest grade category.

Degree of differentiation is also determined by looking at cells under a microscope. Differentiation refers to how much a cancer cell looks like a healthy cell. Well-differentiated tumors look more like healthy cells and grow more slowly. Poorly differentiated cells look less like healthy cells and grow more quickly. Poorly differentiated tumors are usually referred to as neuroendocrine carcinomas (NECs) and are divided into large-cell and small-cell types.

In the latest version of the World Health Organization (WHO) guidelines, both grade and degree of differentiation are used to place NETs into categories. There is a new category of well-differentiated, grade 3 pancreatic NET, which shows that sometimes a cancer cell can look more like a healthy cell but still multiply quickly.

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.

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 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 Tumor of the Pancreas - Types of Treatment

Approved by the Cancer.Net Editorial Board, 06/2019

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

This section explains the types of treatments that are the standard of care for a pancreas 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 pancreas NET. Health care teams include a variety of other health care professionals, such as physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

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

Treatment options and recommendations depend on several factors, including:

  • The type, grade, and stage of the tumor

  • 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 pancreas NETs because there are different treatment options. Learn more about making treatment decisions.

Active surveillance

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

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

  • Blood tests

  • Physical examinations

Active treatment usually only begins if the tumor shows signs of growing or spreading.

Surgery

Surgery is the most common treatment for a pancreas NET that is localized, meaning it has not spread outside the pancreas. Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. A surgical oncologist is a doctor who specializes in treating cancer using surgery. During surgery, the doctor may need to remove most or part of the pancreas, depending on the location and size of the tumor. 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. If no surgery is possible, it is called an “inoperable” tumor. In these situations, the doctor will recommend another type of treatment.

Common surgical procedures that may be used to treat a pancreas NET include:

  • Enucleation. During this surgery, only the tumor is removed.

  • Whipple procedure. During this procedure, the surgeon removes the head of the pancreas and part of the small intestine, bile duct, and stomach. The digestive tract and biliary system is then reconnected.

  • Distal pancreatectomy. If the tumor is located in the tail of the pancreas, the most common operation is a distal pancreatectomy. During this operation, the surgeon removes the tail and body of the pancreas, as well as the spleen.

  • Splenectomy. This is the removal of the spleen.

  • Gastrectomy. For a gastrinoma, it may also be necessary to take out the stomach to remove ulcers, a procedure called a gastrectomy.

Side effects of surgery include weakness, fatigue, and pain for the first few days following the procedure. The doctor may prescribe medication to help manage these side effects. The patient will need to stay in the hospital for several days and will probably need to rest at home for about 1 month.

If all or part of the pancreas was removed during surgery, it may be difficult to digest food. A special diet and medicine may help. The doctor can also prescribe hormones and enzymes to replace those lost by the removal of the pancreas. Diabetes is another side effect of pancreas removal because the body no longer produces insulin. For this, the doctor can prescribe insulin.

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.

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.

The type of systemic therapies used for a pancreas NET include:

  • Somatostatin analogs

  • Targeted therapy

  • Chemotherapy

  • 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 cancer medications. 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 released by a pancreas 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). Octreotide is available in 2 forms, short-acting is given under the skin (subcutaneously) and long-acting is given as an 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.

These hormonal therapy drugs are primarily used for people with a low-grade or intermediate-grade pancreas NET.

Targeted therapy

Targeted therapy is a treatment that targets the tumor’s specific genes, proteins, or the tissue environment that contributes to a tumor's 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.

There are 2 targeted treatments approved by the U.S. Food and Drug Administration (FDA) for locally advanced or metastatic pancreas NETs. These treatments have been shown to significantly slow the growth of pancreas NETs. Both drugs are taken by mouth each day. A specific gene mutation does not have to be present in the tumor cells to receive these treatments.

  • Everolimus (Afinitor). This drug targets a protein called mTOR that is important for cell growth and survival. Common side effects associated with everolimus include mouth sores, fatigue, diarrhea, and rash.

  • Sunitinib (Sutent). This drug targets a protein called VEGF. VEGF is important in angiogenesis, which is the process of making new blood vessels. Because a tumor needs the nutrients delivered by blood vessels to grow and spread, the goal of anti-angiogenesis therapies like sunitinib is to “starve” the tumor. Common side effects associated with this drug include diarrhea, nausea, vomiting, fatigue, and high blood pressure.

Other targeted therapies for pancreas 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.

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

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by keeping the tumor cells from growing, dividing, and making more cells. Generally, chemotherapy is used for higher grade (grade 3) pancreas NETs, large pancreas NETs, or if hormonal or targeted therapies are no longer working.

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.

Common drug combinations for pancreas NETs include:

  • Capecitabine (Xeloda) and temozolomide (Temodar), which are given orally

  • Carboplatin or cisplatin combined with etoposide, all available as generic drugs, given intravenously. This regimen is usually used only for grade 3 disease.

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.

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 pancreas NETs and advanced GI 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. 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. The broader term to describe this treatment is peptide receptor radionuclide therapy (PRRT).

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 pancreas NET

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 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.

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 pancreas NETs

If a tumor spreads to another part of 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. As noted above, hepatic artery occlusion or embolization may be used if cancer has spread to the liver. 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. 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 tumor 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 tumor 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 a pancreas NET returns after the original treatment, it is called a recurrent tumor. 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 and therapy using medications, 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 recurrence.

If treatment does not work

Recovery from a pancreas 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 disease 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 pancreas NET. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Pancreas - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 06/2019

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 pancreas 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 a pancreas NET. 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 pancreas NET. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with a pancreas 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 a pancreas NET, 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, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

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

Neuroendocrine Tumor of the Pancreas - Latest Research

Approved by the Cancer.Net Editorial Board, 06/2019

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

Doctors are working to learn more about pancreas 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.

  • Anti-angiogenesis therapy. As discussed in the Types of Treatment section, anti-angiogenesis therapies are a type of targeted therapy used to treat advanced pancreas NETs. A number of new anti-angiogenesis therapies similar to sunitinib are being tested in clinical trials for pancreas NETs. These drugs are all taken orally.

    Another anti-angiogenic drug is bevacizumab (Avastin), which is a monoclonal antibody given through an intravenous (IV) tube placed into a vein using a needle. It is already approved to treat several other cancers. A recent study showed that adding bevacizumab to everolimus plus octreotide increased the chances that a pancreas NET would shrink and kept the tumor from growing or spreading for a longer time. However, people who took bevacizumab did not live longer than those who did not. Treatment with bevacizumab also caused more side effects than the standard treatment.

  • Immunotherapy. There is much interest in studying immunotherapy, as described in Types of Treatment, to treat many types of tumors, including pancreas NETs. Certain immunotherapies, such as PD-1 and CTLA-4 antibodies (also known as immune checkpoint inhibitors) have already been approved for other types of cancer. However, they are not currently approved for routine treatment of pancreas NETs.

  • Palliative care/supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current pancreas NET treatments 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 pancreas NETs, explore these related items that take you outside of this guide:

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 pancreas NET and its treatment can bring. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Pancreas - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 06/2019

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. Doctors call this part of 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 a pancreas NET 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, 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. 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 their 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 pancreas NET can be expensive. Costs are often a big source of stress and anxiety for people with a tumor 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 pancreas NET

Family members and friends often play an important role in taking care of a person with a pancreas 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 pancreas 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 cancer treatment. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Pancreas - Follow-Up Care

Approved by the Cancer.Net Editorial Board, 06/2019

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

Care for people diagnosed with a pancreas 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 pancreas NET can recur even several years after treatment.

Patients and families should be aware that a pancreas NET usually grows slowly and may be similar to a chronic illness. This means that 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 tumor 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 tumor 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.

There are no established guidelines for a specific schedule of follow-up care after treatment for a pancreas NET. In general, people who have had surgery should see their doctor 3 months after their operation for a physical examination, blood tests, and CT scan. After that, follow-up care should include a physical examination and blood tests about every 6 to 12 months, with additional imaging tests, such as x-rays, done as needed.

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 cancer, your individual treatment plan, and your overall health. For example, some people experience late effects after being treated with octreotide, such as thyroid or 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 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 and stage of tumor, 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 him or her 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. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Pancreas - Survivorship

Approved by the Cancer.Net Editorial Board, 06/2019

ON THIS PAGE: You will read about how to with challenges in everyday life after being diagnosed with a tumor. 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 a pancreatic neuroendocrine tumor after finishing treatment. 

  • Living with, through, and beyond the 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 this diagnosis. 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 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 health 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, sexuality 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 in this article.

A new perspective on your health

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

People recovering from a pancreatic neuroendocrine tumor 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. Rehabilitation services 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 doctor 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 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 next 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. You may use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Pancreas - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 06/2019

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 your appointments to help take notes.

Questions to ask after getting a diagnosis

  • Is the tumor functional or non-functional? What does this mean?

  • Has the tumor spread outside the pancreas?

  • 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 of the tumor and degree of differentiation? What does this mean?

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

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?

  • Do I need to start treatment right away?

  • 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 questions or problems, who should I call?

Questions to ask about having surgery

  • What type of surgery do you recommend?

  • 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?

  • Can you explain what will happen during the surgery?

  • 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?

  • Will this surgery affect my ability to digest food? How? For how long?

  • Will I need to take medicine, hormones, or enzymes after this surgery? For how long?

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

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

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

Questions to ask about having therapies using medication

  • What 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?

  • How can I best prepare myself for this treatment?

  • 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 taking this medication?

  • 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 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 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 Tumor of the Pancreas - Additional Resources

Approved by the Cancer.Net Editorial Board, 06/2019

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 Pancreas 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 Pancreas Neuroendocrine Tumor. Use the menu to choose a different section to read in this guide.