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Neuroendocrine Tumor of the Gastrointestinal Tract - Introduction

Approved by the Cancer.Net Editorial Board, 08/2018

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 Gastrointestinal Tract. 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. All NETs are considered malignant tumors.

NETs develop most commonly in the gastrointestinal (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. NETs can also develop in the pancreas (learn more about NETs of the pancreas) and other locations throughout the body. A rare type of NET that develops in the adrenal gland is called pheochromocytoma. Learn more about NETs that develop in other parts of the body.

About GI tract NETs

NETs can develop in any part of the body. The most common places they form is the GI tract or digestive system, specifically the small intestine and appendix. The GI tract, which starts at the esophagus and ends at the anus, plays a central role in digesting food and liquid and in processing waste. Neuroendocrine cells perform specific functions in the GI tract, such as controlling how quickly food moves through it.

Here is a general overview of where GI tract NETs begin:

  • 39% occur in the small intestine.

  • 15% occur in the rectum.

  • 7% occur in the appendix.

  • 5% to 7% occur in the colon.

  • 2% to 4% occur in the stomach.

Because these tumors develop from neuroendocrine cells, they can make high levels of hormone-like substances called neuropeptides and amines. About 40% of NETs release hormones that lead to symptoms. These tumors are called “functional.” Carcinoid syndrome (see Symptoms and Signs) is a classic type of functional NET and is associated with the release of a substance called serotonin (see Diagnosis). In other tumors, the hormone-like substances may not be released in large enough amounts to cause symptoms, or the substances may be defective and not cause symptoms. These NETs are called “non-functional.”

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

Neuroendocrine Tumor of the Gastrointestinal Tract - Statistics

Approved by the Cancer.Net Editorial Board, 08/2018

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

As explained in the Introduction, NETs often develop in the GI tract. The number of diagnosed NETs has been increasing, but the reason why is unknown.

Each year, about 8,000 adults in the United States are diagnosed with a GI tract NET. The most common place in the GI tract for this type of tumor is the small intestine. People are most often diagnosed in their early 60s.

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. However, the survival rate depends on many factors, including the location of the tumor.

The 5-year survival rate for people with a GI tract NET that has not spread to other parts of the body ranges from 65% to 90%, depending on the stage and location of the tumor. If the tumor has spread to nearby tissue or the regional lymph nodes, the 5-year survival ranges from 46% to 78%. If the tumor has spread to distant areas of the body, survival rates range from 14% to 54%.

It is important to remember that statistics on the survival rates for people with a GI tract NET are an estimate. The estimate comes from annual data based on the number of people with this cancer 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. People should talk with their doctor if they have any questions about this information. Learn more about understanding statistics.

Source: American Cancer Society.

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

Neuroendocrine Tumor of the Gastrointestinal Tract - Medical Illustrations

Approved by the Cancer.Net Editorial Board, 08/2018

ON THIS PAGE: You will find a basic drawing of the main body parts affected by a GI tract NET. Use the menu to see other pages.

Bladder Cancer Illustration

This illustration shows an overview of the body’s internal organs, including the lungs, liver, stomach, small intestine, appendix, colon, and rectum. Copyright 2004 American Society of Clinical Oncology. Robert Morreale/Visual Explanations, LLC. 

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

Neuroendocrine Tumor of the Gastrointestinal Tract - Risk Factors

Approved by the Cancer.Net Editorial Board, 08/2018

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

  • Family history and inherited syndromes. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition that increases the risk of developing tumors in the pituitary gland, parathyroid gland, and pancreas. It is estimated that around 10% of GI tract NETs are associated with MEN1. In addition, some families have multiple relatives with GI tract NETs but no clear association with MEN1. This suggests that there may be other genetic risk factors not yet discovered.

    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 and gender. NETs are more common among white people than black people. They are slightly more common in women than in men.

  • Age. For GI tract NETs, the average age at diagnosis is 55 to 65. Children rarely develop NETs.

  • Other medical conditions. People with diseases that damage the stomach and reduce acid production have a greater risk of developing a NET of the stomach. In particular, people with pernicious anemia have a higher risk of developing a NET of the stomach. Pernicious anemia is a type of anemia in which a person has very large, malformed red blood cells.

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

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

Neuroendocrine Tumor of the Gastrointestinal Tract - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 08/2018

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 GI tract NET often causes no symptoms in its early stages. This type of tumor is usually found by a surgeon during an unrelated surgery or on x-rays for another condition. People with a GI tract NET may experience the following symptoms or signs. The signs and symptoms of carcinoid syndrome and carcinoid crisis, conditions that a GI tract NET can cause, are also described. However, the cause of a symptom may be a different medical condition that is not a GI tract NET.

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 GI tract NET

GI tract NETs may cause tumor-related symptoms. GI tract NETs are also the type of NET most likely to cause carcinoid syndrome (see below), which has its own set of symptoms.

People with a GI tract NET may experience the following tumor-related symptoms or signs:

  • Abdominal pain caused by blockage of the intestines

  • Diarrhea, especially in people who have carcinoid syndrome, had part of their intestines removed, or had their gallbladder removed.

  • Rash

  • Bright, red blood in the stool or dark, tarry stool. This is a sign of intestinal bleeding.

  • Scale-like skin sores, which can be a sign of pellagra, a severe deficiency of vitamin B3

  • Mental disturbances, another sign of pellagra

  • Constipation

  • Nausea and vomiting

  • Weight loss that cannot be explained

  • Jaundice, which is when the skin and whites of the eyes turn yellow

  • Fatigue

Carcinoid syndrome

Carcinoid syndrome occurs when a NET releases so many hormones into the bloodstream that a group of symptoms occurs. This syndrome is more common in people with metastatic NETs, which is when a NET has spread to another part of the body, especially to the liver. In this situation, hormone-like substances produced by the tumor can travel throughout the body and cause carcinoid syndrome.  A GI tract NET is far more likely to cause carcinoid syndrome than a NET of the lung. Not all people with a GI tract NET develop carcinoid syndrome.

People with carcinoid syndrome may experience 1 or more of the following symptoms or signs. 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.

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

  • Sweating

  • 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

Carcinoid syndrome may damage the heart, so reducing its symptoms is important. 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 patients 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. A carcinoid crisis may be prevented and successfully treated with octreotide (Sandostatin), a medication that helps control the production of hormones, or lanreotide (Somatuline Depot).

Managing symptoms

If a GI tract NET is diagnosed, relieving symptoms remains an important part of cancer care and treatment. This may be called palliative care or supportive care. Below is information on how some of the symptoms of a GI tract 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.

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

  • Diarrhea. There can be many causes of diarrhea in people with NETs. If your diarrhea is caused by carcinoid syndrome, somatostatin analogs and telotristat ethyl (Xermelo) can help. If the diarrhea is caused by bile acid malabsorption, which occurs after removal of the gallbladder, ursodiol (Actigall, URSO 250, URSO Forte) can help. If a lack of pancreatic digestive enzymes has caused the diarrhea, replacement enzymes can help. Ask your doctor for specific recommendations.

  • Heart problems. Tell your doctor immediately if you think you may have a problem with your heart and ask about the use of diuretics. Diuretics are drugs that lower blood pressure by helping the body get rid of water and sodium.

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 Gastrointestinal Tract - Diagnosis

Approved by the Cancer.Net Editorial Board, 08/2018

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

Doctors use many tests to find, or diagnose, a GI tract 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 cancer, 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 list describes options for diagnosing a GI tract 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 cancer suspected

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

Most GI tract NETs are found unexpectedly when people have x-rays or a medical procedure done for reasons unrelated to the tumor. For example, many NETs of the appendix are found during surgery to remove the appendix, called an appendectomy. NETs in the stomach and duodenum, the top of the small intestine, are usually found during an endoscopy (see below).

If a doctor suspects a GI tract 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 GI tract 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 product of serotonin breakdown. Serotonin, a neurotransmitter involved in behavior and depression, is made by some NETs. Measurements of serotonin levels may also be taken. A doctor may be able to diagnose a NET from a urine test alone. A blood test to measure chromogranin-A, a protein produced by tumor cells, may be needed since the serum serotonin level often changes and may not be as useful as a chromogranin-A test.

  • Biopsy. biopsy is the removal of a small amount of tissue for examination under a microscope. 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 will help decide whether your treatment options include a type of treatment called targeted therapy (see Types of Treatments).

  • Endoscopy. An endoscopy allows the doctor to see the lining of the upper digestive system with a thin, lighted, flexible tube called an endoscope. The person may be sedated as the tube is inserted through the mouth, down the esophagus, and into the stomach and small bowel. Sedation is giving medication to become more relaxed, calm, or sleepy. If an abnormality is found, a biopsy will be performed.

    colonoscope is a type of endoscope that is inserted through the anus and into the colon. It can be used to diagnose a tumor in the lower section of the digestive tract.

  • Endoscopic ultrasound. An ultrasound uses sound waves to create a picture of internal organs. This procedure is often done at the same time as an upper endoscopy. During an endoscopic ultrasound, a machine that produces the sound waves, called a transducer, is inserted into the upper digestive tract through the mouth. The endoscopic ultrasound can show enlarged lymph nodes, which may help the doctor find a tumor or figure out the stage of the disease.

  • X-ray. An x-ray is a way to create a picture of the structures inside the body. Sometimes, a GI tract 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.

  • Barium x-rays. During a barium swallow, a person swallows a liquid containing barium that coats the lining of the esophagus, stomach, and intestines. Then a series of x-rays are taken. The barium makes abnormalities easier to see on the x-ray. If there is an abnormality, an endoscopic biopsy can help make a diagnosis of cancer.

    A barium enema may be given before x-rays are taken to show the inner surface of the large intestine. During this test, a barium solution is given through the anus and flows throughout the colon. Then the x-rays are 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 images 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 and to find a NET in the retroperitoneal (the area behind the abdomen) lymph nodes. 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 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 cancer, these results also help the doctor describe the tumor. This is called staging.

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 Gastrointestinal Tract - Stages and Grades

Approved by the Cancer.Net Editorial Board, 08/2018

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 cancer 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 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 it can help predict a patient's prognosis, which is the chance of recovery. 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 answer these questions:

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

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

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

The results are combined to determine the stage of cancer for each person. For a GI tract 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. Staging for a GI tract NET is based on the location of the primary tumor. The TNM system described in this section covers GI tract NETs found in these locations:

  • Stomach

  • Duodenum and ampulla of Vater (parts of the small intestine)

  • Jejunum and ileum (parts of the small intestine)

  • Appendix

  • Colon and rectum

Here are more details on each part of the TNM system for a GI tract NET:

Tumor (T)

Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. 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.

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

Stomach

TX: The tumor cannot be evaluated.

T0 (T plus zero): There is no evidence of a tumor.

T1: The tumor is 1 cm or smaller and has grown into a deeper layer of cells in the stomach, the lamina propria or submucosa.

T2:  The tumor is larger than 1 cm in size, or it has grown into the muscle layer of the stomach, called the muscularis propria.

T3: The tumor has grown through the muscularis propria and into the subserosa layer behind it.

T4: The tumor has grown into the layer of tissue on the outside of the stomach, called the serosa or peritoneum, or it has spread into nearby organs or structures.

Duodenum and ampulla of Vater (small intestine)

The duodenum is the first part of the small intestine after the stomach. The ampulla of Vater is a small bump in the duodenum where liquids from the bile ducts and pancreas enter the small intestine. 

TX: The tumor cannot be evaluated.

T1: The tumor is 1 cm or smaller and only involves the top layer of mucus membrane or connective tissue on top of the muscle layer of the duodenum or it only involves the sphincter of Oddi. The sphincter of Oddi is a muscle that controls the flow of digestive juices into the duodenum through the ampulla of Vater.

T2:  The tumor is larger than 1 cm in size. Or it has grown into the muscle layer of the duodenum, called the muscularis propria, or through the sphincter of Oddi and into the duodenum.

T3: The tumor has grown into the pancreas or the tissue surrounding the pancreas.

T4: The tumor has grown into the peritoneum or other organs.

Jejunum and ileum (small intestine)

The jejunum and ileum are the parts of the small intestine that connect the duodenum to the large intestine. 

TX: The tumor cannot be evaluated.

T0 (T plus zero): There is no evidence of a tumor.

T1: The tumor is 1 cm or smaller and only involves the top layers of tissue in the small intestine.

T2:  The tumor is larger than 1 cm in size, or it has grown into the muscle layer of the small intestine.

T3: The tumor has grown through the muscle layer and into the tissues beyond, but it has not grown outside of the small intestine.

T4: The tumor has grown past the outside of the small intestine and into the peritoneum or other organs.

Appendix

TX: The tumor cannot be evaluated.

T0 (T plus zero): There is no evidence of a tumor.

T1: The tumor is 2 cm or smaller.

T2:  The tumor is more than 2 cm but less than or equal to 4 cm in size.

T3: The tumor is larger than 4 cm, or it has grown into the layers of tissue on the outside of the appendix.

T4: The tumor has grown through the peritoneum or has invaded nearby organs or structures.

Colon and rectum

TX: The tumor cannot be evaluated.

T0 (T plus zero): There is no evidence of a tumor.

T1: The tumor is 2 cm or smaller and has grown past the top layer of cells and into the layers beneath, such as the lamina propria or submucosa.

  • T1a: The tumor is less than 1 cm in size.

  • T1b: The tumor is between 1 cm and 2 cm in size.

T2:  The tumor has grown into the muscle (muscularis propria). Or it is more than 2 cm in size and has invaded the lamina propria or submucosa.

T3: The tumor has spread through the muscle and into the subserosal tissue behind it.

T4: The tumor has spread to the peritoneum or has invaded nearby organs or structures.

Node (N)—for all parts of the GI tract

The “N” in the TNM staging 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: The lymph nodes cannot be evaluated.

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

N1: The cancer has spread to the regional lymph nodes. Jejunum and ileum only: The cancer has spread to fewer than 12 lymph nodes. 

N2 (jejunum and ileum only): The cancer has spread to 12 or more lymph nodes and/or the masses are larger than 2 cm.

Metastasis (M)—for all parts of the GI tract

The “M” in the TNM system indicates 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 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.

Stomach

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

Stage II: The tumor is larger and has grown as far as the subserosa, which is behind the layer of muscle in the stomach. The cancer has not spread to the lymph nodes or elsewhere in the body (T2 or T3, N0, M0).

Stage III: The tumor is any size, and the cancer has spread to regional lymph nodes (any T, N1, M0). Or, the tumor has spread to the outside of the stomach, but the cancer has not spread to the lymph nodes or elsewhere (T4, N0, M0).

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

Duodenum and ampulla of Vater (small intestine)

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

Stage II: The tumor is larger than 1 cm, or it has grown as far as the pancreas. The cancer has not spread to the lymph nodes or elsewhere in the body (T2 or T3, N0, M0).

Stage III: The tumor is any size, and the cancer has spread to regional lymph nodes (any T, N1, M0). Or, the tumor has spread to the peritoneum or other organs, but the cancer has not spread to the lymph nodes or elsewhere (T4, N0, M0).

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

Jejunum and ileum (small intestine)

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

Stage II: The tumor is larger than 1 cm and has grown as far as the subserosa, which is behind the layer of muscle in the small intestine. The cancer has not spread to the lymph nodes or elsewhere in the body (T2 or T3, N0, M0).

Stage III: The tumor is any size, and the cancer has spread to regional lymph nodes (any T, N1 or N2, M0). Or, the tumor has spread to the peritoneum or to other organs or structures, but the cancer has not spread to the lymph nodes or elsewhere (T4, N0, M0).

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

Appendix

Stage I: There is a tumor of 2 cm or less, and it has not spread elsewhere (T1, N0, M0).

Stage II: The tumor is larger than 2 cm and has grown as far as the membrane that connects the appendix to the abdomen wall. The cancer has not spread to the lymph nodes or elsewhere in the body (T2 or T3, N0, M0).

Stage III: The tumor is any size, and the cancer has spread to regional lymph nodes (any T, N1, M0). Or, the tumor has spread to the peritoneum or beyond, but the cancer has not spread to the lymph nodes or elsewhere (T4, N0, M0).

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

Colon and rectum

Stage I: There is a tumor of 2 cm or less, and it has not spread elsewhere (T1, N0, M0).

Stage IIA: The tumor has grown into the muscle. Or it is more than 2 cm in size and has invaded the lamina propria or submucosa. The cancer has not spread (T2, N0, M0).

Stage IIB: The tumor has spread through the muscle and into the subserosal tissue behind it. The cancer has not spread to the lymph nodes or elsewhere in the body (T3, N0, M0).

Stage IIIA: The tumor has spread to the peritoneum or has invaded nearby organs or structures, but the cancer has not spread to the lymph nodes or elsewhere (T4, N0, M0).

Stage IIIB: The tumor is any size, and the cancer has spread to regional lymph nodes (any T, N1, M0).

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

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

Grade (G)

Doctors may also describe a GI tract NET by its grade (G). For many types of cancer, the grade describes how much cancer cells look like healthy cells when viewed under a microscope, called pleomorphism. This type of evaluation is less useful for NETs. Instead, the grade for a NET describes how fast the tumor cells are growing and dividing, a process called mitosis.

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 cancer’s grade may help the doctor predict how quickly the NET will grow and spread. In general, the lower the tumor’s grade, the better the prognosis. The grade scale below applies to all types of GI tract NETs.

GX: Grade cannot be evaluated.

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

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

G3: Mitotic count is more than 20, and 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.

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 Gastrointestinal Tract - Types of Treatment

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

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

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team and is especially important for people with 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.

Descriptions of the common types of treatments used for a GI tract NET are listed below, followed by an outline of treatment options by stage. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care.

Treatment options and recommendations depend on several factors, including the stage and grade of cancer, possible side effects, and the patient’s preferences and overall health. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. Learn more about making treatment decisions.

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 GI tract 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.

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.

Surgical options for a GI tract NET include:

  • Local excision. During this operation, the surgeon removes the tumor and some surrounding healthy tissue. Most localized tumors can be surgically removed through a skin incision, but a rectal neuroendocrine tumor may be removed through the anus. Other GI tract NETs can sometimes be removed using an endoscope (see Diagnosis).

  • Partial gastrectomy. This surgery removes part of the stomach.

  • Esophagectomy. This procedure is the removal of all or part of the esophagus. The esophagus is the tube that connects the throat to the stomach.

  • Small bowel resection. The small bowel is another term for the small intestine. This surgery removes parts of the small intestine.

  • Appendectomy. Appendectomy is surgery to remove the appendix.

  • Segmental colon resection or hemicolectomy. This surgery removes one-third to one-half of the colon, including nearby blood vessels and lymph nodes.

  • Low anterior resection. During this surgery, a portion of the upper part of the rectum is removed.

  • Abdominoperineal resection. This surgery is used for a large tumor located in the lower part of the rectum. During the operation, the surgeon removes the anus, rectum, and part of the colon. After surgery, a colostomy may be created to carry waste out of the body. A colostomy is an opening from the colon to the outside of the body.

  • Liver resection. This operation removes cancer that has spread to the liver. The goal of this surgery is not to eliminate the cancer, but it often helps relieve or reduce the symptoms of carcinoid syndrome.

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 or in a pill or capsule that is swallowed (orally). 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 types of systemic therapies used for a GI tract NET 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 only 1 type of systemic therapy at a time or a combination of systemic therapies given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.

The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

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 GI tract 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 and lanreotide.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by ending the cancer cells’ ability to grow and divide. Chemotherapy for a GI tract NET is most often used when the tumor has spread to other organs or is causing severe symptoms.

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 drugs for a GI tract NET include:

  • 5-fluorouracil (5-FU)

  • Capecitabine (Xeloda)

  • Carboplatin

  • Cisplatin

  • Dacarbazine (DTIC)

  • Etoposide

  • Oxaliplatin (Eloxatin)

  • Streptozocin (Zanosar)

  • Temozolomide (Temodar)

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 cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells 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.

A U.S. Food and Drug Administration (FDA)-approved targeted therapy for the treatment of advanced GI tract NETs is everolimus (Afinitor). This treatment can help slow down the growth of these tumors in some patients, but it does not usually shrink tumors.

Other targeted therapies for GI tract 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.

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. 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 January 2018, the FDA approved a treatment called 177Lu-dotatate (Lutathera) for advanced GI tract NETs. This 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).

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer 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 GI tract 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 “Getting care for symptoms and side effects” 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 following treatments may be used:

  • 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.

Care for symptoms and side effects

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

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

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

For some people, such as those with lung disease, heart disease, or other specific medical conditions, surgery cannot successfully treat the cancer. 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 health care team about the possible side effects of the specific treatment plan and palliative care options. During and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care.

Treatment of a GI tract NET by stage

Stages I, II, and III

  • Stomach. A localized NET of the stomach can often be completely removed through an endoscope. A tumor larger than 2 cm is removed with a margin of surrounding stomach tissue through an incision, or cut, in the abdomen.

  • Small intestine. Surgery to remove the tumor and surrounding tissue is the most common treatment for a NET smaller than 1 cm located in the small intestine. Surgery for a larger tumor involves removing more surrounding tissue and some surrounding blood vessels and lymph nodes.

  • Large intestine/colon. The most common treatment for a tumor smaller than 2 cm is the surgical removal of the tumor and surrounding tissue, often done through a colonoscope (see Diagnosis). If the tumor is larger than 2 cm, surgery most often involves an incision through the skin.

  • Appendix. An appendectomy is usually the only treatment needed for a NET smaller than 1.5 cm. For a tumor larger than 2 cm, the removal of about one-third of the colon next to the appendix and nearby blood vessels and lymph nodes is often needed.

  • Rectum. A rectal NET smaller than 1 cm is treated with RFA (see “Surgery” above). A tumor larger than 2 cm is more likely to grow and spread quickly. Larger tumors are removed using the same procedure that is used for rectal cancer. This involves removing some healthy colorectal tissue and some of the nearby lymph nodes.

Stage IV

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

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

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

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 recurrent cancer 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 cancer recurrence.

If treatment does not work

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

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

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

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

The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with a GI tract NET. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Gastrointestinal Tract - About Clinical Trials

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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 GI tract 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.

Many clinical trials focus on new treatments. Researchers want to learn if a new treatment is safe, effective, and possibly better than the treatment doctors use now. These types of studies evaluate new drugs, different combinations of existing 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 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. There are also clinical trials studying ways to prevent cancer.

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 GI tract NET. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with this type of tumor.

Insurance coverage of clinical trials costs differs 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.” 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 any personal or medical reason. 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 GI tract NETs, learn more in the Latest Research section.

Cancer.Net offers a lot of information about cancer 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 cancer.

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 currently going on for a GI tract NET. 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 now to learn more about GI tract NETs and how to treat them. Use the menu to see other pages.

Doctors are working to learn more about GI tract 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.

  • New treatment approaches. Currently, many clinical trials for GI tract NETs are studying new drugs and different drug combinations. New approaches to drug treatment include targeted therapy, as described in Types of Treatment.

  • Genetics. As described in Risk Factors, family history of MEN1 may play a role in an individual’s risk for developing a GI tract NET. Researchers are investigating MEN1 to learn more about this hereditary risk factor.

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

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding GI tract 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, and social changes that a GI tract 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. This page includes several links outside of this guide to other sections of this website. Use the menu to see other pages.

Every cancer 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 cancer. This can make it hard to predict how you will feel during treatment.

As you prepare to start cancer 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 GI tract 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 cancer’s stage, the length and dose of treatment, and your general health.

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 cancer diagnosis. This may include dealing with difficult emotions, such as sadness, anxiety, or anger, or managing your stress level. Sometimes, patients have problems expressing how they feel to their loved ones, or people do not know what to say in response.

Patients and their families are encouraged to share their feelings with a member of their health care team. 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 GI tract NET can be expensive. Costs are often a big source of stress and anxiety for people with cancer 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 cancer 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 cancer

Family members and friends often play an important role in taking care of a person with a GI tract 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. Below are some of the responsibilities caregivers take care of:

  • 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 cancer.

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.

The next section in this guide is Follow-up Care. It explains the importance of checkups after treatment has finished. 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 GI tract NET is completed and why this follow-up care is important. Use the menu to see other pages.

Care for people diagnosed with a GI tract NET does not end when active treatment has finished. Your health care team will continue to check to make sure the tumor has not returned, 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 GI tract NET can recur even several years after treatment.

Patients and families should be aware that a GI tract NET grows 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. 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.

The most common place for a NET recurrence to develop is in the GI tract. 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 cancer 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 cancer, 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 after treatment is completed.

This is also a good time to decide 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 cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with him or her and with all future health care providers. Details about your cancer 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 cancer 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 cancerous 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 cancer after finishing treatment.

  • Living with, through, and beyond cancer. According to this definition, cancer 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 cancer. 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 cancer 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 cancer 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 cancer, 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 cancer 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 positive lifestyle changes.

People recovering from a GI tract 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 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 cancer care team. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Gastrointestinal Tract - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 08/2018

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 your 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.

Questions to ask after getting a diagnosis

  • Where in my GI tract is the neuroendocrine tumor 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?

  • What are my treatment options?

Questions to ask about choosing a treatment and managing side effects

  • 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 cancer, help me feel better, or both?

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

  • What medications will reduce these side effects?

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

  • Who will lead my overall treatment?

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

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

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

  • If I’m worried about managing the costs of cancer care, who can help me?

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

  • Whom should I call with questions or problems?

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

Questions to ask about having surgery

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

  • What type of surgery do you recommend?

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

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?

  • 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?

  • 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 cancer will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the cancer 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 beyond this guide that may be helpful to you. Use the menu to choose a different section to read in this guide.

Neuroendocrine Tumor of the Gastrointestinal Tract - Additional Resources

Approved by the Cancer.Net Editorial Board, 08/2018

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 GI tract NET. Use the menu to go back and review other pages.

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

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

This is the end of Cancer.Net’s Guide to Neuroendocrine Tumor of the Gastrointestinal Tract. Use the menu to choose a different section to read in this guide.