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Gastrointestinal Stromal Tumor - GIST - Introduction

Approved by the Cancer.Net Editorial Board, 09/2018

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

About the gastrointestinal tract

The gastrointestinal (GI or digestive) tract includes the:

  • Esophagus

  • Stomach

  • Gallbladder and bile ducts

  • Liver

  • Pancreas

  • Small intestine

  • Colon

  • Rectum

  • Anus

  • Lining of the gut

The GI tract plays a central role in digesting food and liquid and in processing waste. When you swallow food, it is pushed down a muscular tube called the esophagus and enters the stomach. The muscles in the stomach mix the food and release gastric juices that help break down and digest the food. The food then moves into the small intestine, or small bowel, for further digestion before entering the large intestine. The large intestine helps remove waste from the body. The colon makes up the first 5 to 6 feet of the large intestine. The rectum makes up the last 6 inches, ending at the anus.

About gastrointestinal stromal tumor (GIST)

A tumor begins when healthy cells change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread. A tumor can start in any part of the GI tract. There are several different types of GI tumors, including gastrointestinal stromal tumor (GIST).

GISTs are different from more common types of GI tumors, like colon cancer or stomach cancer, because of the type of tissue in which they start. GISTs belong to a group of cancers called soft-tissue sarcomas. Soft-tissue sarcomas develop in the tissues that support and connect the body. The sarcoma cells resemble the cells that hold the body together, including fat cells, muscles, nerves, tendons, joints, blood vessels, and lymph vessels. Learn more about soft-tissue sarcoma in a different section of Cancer.Net.

Research shows that GIST begins in “pacemaker” cells found in the walls of the GI tract. These pacemaker cells are called interstitial cells of Cajal (ICCs), and they send signals to the GI tract to help move food and liquid through the digestive system.

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

Gastrointestinal Stromal Tumor - GIST - Statistics

Approved by the Cancer.Net Editorial Board, 01/2019

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

GISTs are rare, making up less than 1% of all gastrointestinal tumors. Each year, approximately 4,000 to 6,000 adults in the United States will be diagnosed with a GIST. About 60% of GISTs begin in the stomach, and around 35% develop in the small intestine. The remaining types of GISTs usually start in the rectum, colon, and esophagus. Most people diagnosed with a GIST are in their 60s. They rarely occur in people younger than 40.

The 5-year survival rate tells you what percent of people live at least 5 years after the cancer is found. Percent means how many out of 100. The 5-year survival rate for people with a GIST depends on several factors, including specific biologic characteristics of the tumor, the type of treatment, and the risk that it will come back after treatment.

The American Cancer Society’s most recent data available is from 2003 to 2009. This means that these survival rates do not show the results of better diagnosis or treatment available since then. Based on that data, the overall 5-year survival rate of people diagnosed with a malignant GIST is 76%. If the tumor has not spread from the organ where it started, the 5-year survival rate is 91%. If the cancer has spread to surrounding tissues or organs, the 5-year survival rate is 74%. If the cancer has spread to a distant part of the body when it was first diagnosed, the survival rate is 48%.

It is important to remember that statistics on the survival rates for people with a GIST are an estimate. The estimate comes from annual data based on the number of people with a GIST 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 American Cancer Society website (January 2019).

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

Gastrointestinal Stromal Tumor - GIST - Risk Factors

Approved by the Cancer.Net Editorial Board, 09/2018

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

The majority of GISTs develop sporadically, which means for no known reason. Doctors are rarely able to find a specific risk factor for patients diagnosed with a GIST, other than the hereditary syndromes discussed below. However, the following factors may raise a person’s risk for developing a GIST:

  • Age. GIST most often occurs in people older than 50.

  • Gender. GIST is slightly more common in men than in women.

  • Family history. GIST rarely runs in families, and having a family member with a GIST usually does not increase your risk of developing the disease. Hereditary syndromes that can increase the risk of GIST include neurofibromatosis type 1 (NF1) and Carney-Stratakis dyad.

Because no non-hereditary, preventable risk factors have been found, there is no good way to prevent GIST.

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

Gastrointestinal Stromal Tumor - GIST - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 09/2018

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.

People with GIST often do not experience any specific symptoms or signs. When symptoms do occur, they may be vague. Or, the cause of a symptom may be a different medical condition that is not a tumor.

  • Pain or discomfort in the abdomen

  • A mass in the abdomen that you can feel with your hand

  • Bowel obstruction

  • Nausea and vomiting

  • Vomiting blood

  • Blood in the stool

  • Fatigue due to anemia, which is a low red blood cell count and is often caused by bleeding in the GI tract

People often do not experience any particular symptoms with GIST. It is often discovered during evaluation of chronic anemia, abdominal pain, or gastrointestinal bleeding, or on imaging test done for another reason.

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.

If a GIST is diagnosed, relieving symptoms remains an important part of your medical care and treatment. This may be called palliative care or supportive care. Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

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

Gastrointestinal Stromal Tumor - GIST - Diagnosis

Approved by the Cancer.Net Editorial Board, 09/2018

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 tumor. 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

In addition to a physical examination, the following tests may be used to diagnose GIST or determine the best treatment. Not all tests listed below will be used for every person.

  • Computed tomography (CT or CAT) scan. A CT scan is often the best test to diagnose and monitor a GIST. 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 or help doctors determine whether the cancer has spread to the liver or gut lining. 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.

  • Endoscopy/endoscopic ultrasound. An endoscopy allows the doctor to see the inside of the stomach or large bowel. The patient may be sedated. Sedation is giving medication to make a patient more relaxed, calm, or sleepy. With upper endoscopy, the doctor inserts a thin, lighted tube called a gastroscope through the mouth, down the esophagus, and into the stomach and upper small bowel. With lower endoscopy, the scope is inserted through the anus to visually evaluate the rectum and colon. If abnormal areas are found, the doctor can remove a sample of tissue and check it for evidence of cancer (see "Biopsy" below). A special type of test called a capsule endoscopy involves swallowing a small camera. This allows very clear viewing of the small intestine. It is rarely used but can be useful when other diagnostic methods are unable to find the cause of GI bleeding.

    An endoscopic ultrasound is similar to an endoscopy, but the scope has a small ultrasound probe on the end. The probe uses sound waves to create an image of the stomach or rectum and nearby organs. The ultrasound image helps doctors determine if or how far the cancer has spread into nearby tissues.

  • 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.

If a tumor (mass or nodule) is found, the doctor will also do tests to learn if a tumor is cancerous. For most types of tumors, a biopsy is the only sure way for the doctor to know if an area of the body has cancer. 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.

  • Biopsy. Your doctor may recommend a biopsy if a mass suspected of being a GIST is found. A biopsy is the removal of a small amount of tissue for examination under a microscope. The type of biopsy performed will depend on the location of the tumor. Other tests can suggest that a tumor is present, but only a biopsy can make a definite diagnosis. A pathologist then analyzes the sample. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

    Based on the results of imaging scans, your doctor may recommend biopsy or surgery. That’s because a single biopsy does not always diagnose a GIST. A patient may need surgery to remove the entire tumor and biopsies done in several locations of the tumor to make a diagnosis. However, it is very important that you see a team of medical and surgical oncologists experienced in treating GIST before a big surgery.

  • Tumor pathology. A pathologist makes a diagnosis of GIST by looking at the shape and appearance of tumor cells, doing tests for a protein called KIT and other tumor markers, and finding the mitotic count (a measure of actively dividing cells; see Stages for more information).

  • Molecular testing of the tumor. Your doctor or pathologist may recommend running additional 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.

    GIST most often develops because of genetic mutations, or changes. This includes non-hereditary tumors. The 2 most common affected genes in GIST are called KIT and platelet-derived growth factor receptor alpha (PDGFRA). A tumor may have 1 of several different mutations to these genes, some of which are more harmful than others. Different mutations have different prognoses. Prognosis is the chance of recovery. When a tumor does not have a mutation in any gene that is tested for, it is called “wild type” (WT).

    Researchers are finding more mutations thought to cause GIST that may be used to help choose a patient’s treatment options. A protein called succinate dehydrogenase (SDH) is 1 of them. Another is BRAF, a gene occasionally changed in people with other types of cancer, such as melanoma or colorectal cancer.

    The results of mutation testing may help your doctor make treatment recommendations. This could include what dose of a drug called imatinib (Gleevec,  Glivec) to use, or whether no further treatment or a different drug is recommended.

  • Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan. However, you may hear your doctor refer to this procedure just as a PET scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body. Doctors often use PET scans to add to the information gathered from the CT scan, MRI, or physical examination. Doctors may also use the scans to show activation of the disease (called PET flare) or to measure how well treatment is working.

After your diagnostic tests, your doctor will review all of the results with you. If the diagnosis is GIST, some of these results also help the doctor describe the tumor. This is called staging.

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

Gastrointestinal Stromal Tumor - GIST - Stages

Approved by the Cancer.Net Editorial Board, 09/2018

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

Staging is a way of describing where the tumor is located, or if it has spread to another part of the body from where it started. If this happens, it is called metastasis. Doctors may also do tests to learn which treatments could work best.

Doctors use diagnostic tests to find out the tumor's stage, so staging may not be complete until all the tests are finished. Tests that may be used to find the stage include CT scan, MRI, or PET-CT scan (see Diagnosis). Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis. There are different stage descriptions for different types of cancer.

For other types of GI tumors, the most common staging tool used is called the TNM system. TNM is an abbreviation for tumor (T), node (N), and metastasis (M). However, the TNM system is not required to stage GIST. Instead, doctors look at different factors to determine a patient’s prognosis and the specific risk of how aggressive an individual tumor will be. Specifically, doctors want to figure out how quickly the GIST may grow and the chance the tumor will come back, or recur, after surgery.

Doctors commonly use the factors listed below to determine how aggressive a GIST that has been removed could be:

  • The size of the tumor

  • The mitotic count, which describes the number of dividing cells

  • The location where the tumor started

A GIST that has spread to distant areas of the body is considered higher risk because it has a greater likelihood of coming back, called recurrent GIST.

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

Gastrointestinal Stromal Tumor - GIST - Types of Treatment

Approved by the Cancer.Net Editorial Board, 09/2018

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

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

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. 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 GIST are listed below. Your care plan also includes treatment for symptoms and side effects, an important part of cancer care.

Treatment options and recommendations depend on several factors, including the type and stage of tumor, possible side effects, the patient’s preferences, and the patient’s overall health. The doctor will look at many factors to determine the best treatment, including the tumor’s:

  • Size

  • Number of dividing cells

  • Genetic makeup

  • Primary location

  • Whether it has already spread

  • Whether the tumor ruptured, either on its own or as a result of surgery

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. For patients with a localized GIST, surgery is the standard treatment and should be performed whenever possible. Often the tumor can be completely removed through surgery. A surgical oncologist is a doctor who specializes in treating cancer by surgery.

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

If the tumor is large or has spread to nearby organs, you may first receive imatinib (see "Tyrosine kinase inhibitors" below). The purpose of this approach is to shrink the tumor so it is easier to remove with surgery. The surgeon will eventually try to remove the entire tumor to reduce the risk that the tumor will recur or block the GI tract.

To remove the entire tumor, it is possible that the surgeon may have to remove parts of nearby organs, depending on the tumor’s location. The removal of lymph nodes is not generally needed because GISTs do not often spread to the lymph nodes. Lymph nodes are tiny, bean-shaped organs that help fight infection. A tumor that cannot be removed using surgery is called "unresectable." The doctor will recommend a different type of treatment for an unresectable tumor.

GIST has the ability to come back after being surgically removed and can spread to other organs. Some GISTs have a higher risk of returning or spreading than others. After surgery, patients who may have a high risk of recurrence often receive imatinib for at least 3 years. This type of treatment after surgery is called "adjuvant therapy."

Therapies using medication

Systemic therapy is the use of medication to destroy tumor cells. This type of medication is given through the bloodstream to reach tumor cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

Systemic therapy used for GIST includes includes targeted therapy.

Different targeted 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.

Targeted therapy (updated 01/2019)

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. Because some of these genetic changes in the tumor may mean the drugs described below may not work well, 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.

Tyrosine kinase inhibitors (TKIs) are drugs that target specific proteins called kinases that contribute to the tumor’s growth and survival. This type of targeted treatment blocks the growth and spread of tumor cells while limiting damage to healthy cells. TKIs are often used to treat GIST.

The drugs described in this section are listed in alphabetical order, not in the order that they are used in treatment. Learn more about the basics of targeted treatments.

Imatinib (Gleevec)

This was the first targeted therapy approved for GIST by the U.S. Food and Drug Administration (FDA). It is often the first drug used to treat GIST. Since this drug became available, the prognosis for patients with a GIST improved a lot.

Research is being done to find out how long imatinib should be given after surgery to help delay or prevent a recurrence, and imatinib is the only drug approved for this use. If a GIST has spread to other parts of the body, imatinib is taken for the rest of the patient’s life to help control the tumor.

The usual dose of imatinib is 400 milligrams (mg) daily. For some patients, the dose may be raised to 800 mg daily, especially for those who have an exon 9 genetic mutation in the KIT gene.

Imatinib does have some common side effects that are often successfully managed. The dose can be adjusted to reduce side effects, or other medications can be added to the treatment plan to manage side effects. The most common side effects of imatinib are fluid accumulation, rash, nausea, diarrhea, fatigue, and minor muscle aches. Serious but relatively rare side effects include bleeding and inflammation of the liver. Many of these side effects can be managed with the help of the health care team. Also, some side effects get better over time. People who have serious side effects may take a lower dose of imatinib and still benefit from it. If you want to stop treatment because of the side effects, talk with your health care team to learn whether this is the best option.

Sunitinib (Sutent)

Sunitinib is a TKI that works in many different ways to slow tumor growth. Sunitinib also blocks both KIT and the process of making new blood vessels, which is called angiogenesis. The FDA approved sunitinib for treating GIST when the tumor continues to grow even after treatment with imatinib. Sunitinib may also be used when the side effects of imatinib are too serious, even at lower doses.

The side effects of sunitinib include fatigue, diarrhea, nausea and vomiting, heartburn, changes in taste, high blood pressure, low blood counts, and changing skin color.

Regorafenib (Stivarga)

Regorafenib (Stivarga) is a TKI that works in many different ways to slow tumor growth, including blockade of the KIT molecule and blocking blood vessel growth into tumors. It was approved in 2013 for people with later-stage GIST that cannot be surgically removed and when both imatinib and sunitinib did not work or caused severe side effects.

The side effects of regorafenib include fatigue, nausea, hand-foot syndrome, high blood pressure, diarrhea, and inflammation of the liver.

Larotrectinib (Vitrakvi)

A small percentage of sarcomas, less than 1%, have a mutation in the neurotrophic receptor tyrosine kinase (NTRK) gene. Larotrectinib (Vitrakvi) is an NTRK inhibitor that is now approved for any cancer that has a specific mutation in the NTRK gene. These NTRK mutations are not found in the most common types of GIST, including those with KIT, PDGFR, SDH, and RAF mutations. The most common side effects include fatigue, nausea, dizziness, vomiting, increased liver enzymes, cough, constipation, and diarrhea.

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

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by ending the cancer cells’ ability to grow and divide. However, standard chemotherapy is not effective for treating GIST and should not be used.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy.

A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.

Radiation therapy is not often used for to treat people with GIST. However, it may be used as a palliative treatment to relieve pain, such as bone pain, or to stop bleeding. Radiation therapy may damage healthy cells as well as tumor cells. Side effects from radiation therapy include tiredness, mild skin reactions, upset stomach, and loose bowel movements.

Most side effects go away soon after treatment is finished. Learn more about the basics of radiation therapy.

Care for symptoms and side effects

GIST and its treatment often cause side effects. In addition to treatment intended to slow, stop, or eliminate the tumor, an important part of care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care. Palliative care 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 tumor, may receive palliative care. It works best when palliative care is started as early as needed in the treatment process. People often receive treatment for the tumor 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 tumor, such as drug therapy, surgery, or radiation therapy. Talk with your doctor about the goals of each treatment in your treatment plan.

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

Learn more about palliative care.

Metastatic GIST

If a GIST has spread to another part in the body from where it started, doctors call it metastatic. 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. In many instances, people with metastatic GIST are able to live normal lives for several years, being treated only with pills and regular hospital visits.

Depending on previous treatment, your treatment plan may include surgery, a higher dose of a TKI, or treatment through a clinical trial. Your doctor may continue to recommend imatinib, regorafenib, or sunitinib, even if they are not working perfectly. He or she may also recommend going back to 1 of these drugs (usually imatinib) after the others have been tried and/or after they have stopped working. In general, some treatment with targeted therapy for GIST is better than no treatment at all. Palliative care will also be important to help relieve symptoms and side effects.

For most people, a diagnosis of metastatic tumor 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 the tumor cannot be detected in the body and there are no symptoms. This is also 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 the tumor does return 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 targeted therapy. However, 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 or metastatic GIST often experience emotions such as disbelief or fear. You are encouraged to talk with the health care team about these feelings and ask about support services to help you cope. Learn more about dealing with a recurrence.

If treatment does not work

Recovery from a GIST is not always possible. If the growth of the tumor cannot be controlled, you may not ever recover from the disease. This condition is called 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 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 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 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 GIST. Use the menu to choose a different section to read in this guide.

Gastrointestinal Stromal Tumor - GIST - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 09/2018

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 GIST. 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, new approaches to existing treatments, 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 will 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 GIST. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with GIST.

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 must:

  • 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 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 GIST, 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 this type of tumor. You may use the menu to choose a different section to read in this guide.

Gastrointestinal Stromal Tumor - GIST - Latest Research

Approved by the Cancer.Net Editorial Board, 09/2018

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of tumor and how to treat it. Use the menu to see other pages.

Doctors are working to learn more about how to best treat a GIST and provide the best care to people diagnosed with GIST. 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 drugs. Research continues to find new drugs for the treatment of GIST, particularly new targeted therapies. Several different drugs that target proteins that help tumor cells survive are being researched. Drugs that affect the tumor's blood supply are also being studied to treat GIST. Treatments working through the immune system, called immunotherapy, are also of interest in GIST research.

  • Post-operative treatment length. The length of time to continue imatinib treatment after surgery is based on the risk of recurrence. People with a high risk of recurrence should be considered for 3 years of after-surgery imatinib if the tumor has a mutation that is targeted by imatinib. Doctors are currently evaluating the best length of treatment.

  • Palliative care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current GIST treatments 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 GISTs, 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 cancer and its treatment can bring. Use the menu to choose a different section to read in this guide.

Gastrointestinal Stromal Tumor - GIST - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 09/2018

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of GIST 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 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 GIST are listed in the Types of Treatment section. Learn more about side effects of a tumor 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.

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

Medical treatment can be expensive. It is 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 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 GIST

Family members and friends often play an important role in taking care of a person with a GIST. 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. Many side effects from GIST drugs may be managed effectively, either directly or through changing the dose. Tell your health care team about side effects even if you do not think they 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 is finished. Use the menu to choose a different section to read in this guide.

Gastrointestinal Stromal Tumor - GIST - Follow-Up Care

Approved by the Cancer.Net Editorial Board, 09/2018

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

Care for people diagnosed with a GIST does not end when active treatment has finished. Your health care team will continue to check to make sure the cancer 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. A person who received treatment for a GIST is typically seen by the doctor every 3 to 6 months for up to 5 years and then once a year after that. This follow-up care is important because there is always a risk that the tumor could come back, even many years after surgery. For people with metastatic GIST, lifelong treatment is often needed to help control tumor growth.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. Cancer recurs because small areas of cancer 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. Testing recommendations depend on several factors, including the type and stage of cancer originally diagnosed and the types of treatment given.

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 tumor, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

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

Gastrointestinal Stromal Tumor - GIST - Survivorship

Approved by the Cancer.Net Editorial Board, 09/2018

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a cancer diagnosis. 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 tumor after finishing treatment.

  • Living with, through, and beyond cancer. According to this definition, survivorship begins at diagnosis and includes people who continue to have treatment over the long term. The treatment is used 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, talking with a member of your health care team, individual counseling, and 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 GIST 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. This could mean any of a wide range of services such as:

  • Physical therapy

  • Career counseling

  • Pain management

  • Nutritional planning

  • 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.

Gastrointestinal Stromal Tumor - GIST - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 09/2018

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 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 cancer 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 is the tumor located?

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

  • Should my tumor be tested for genetic changes?

  • How aggressive is the tumor?

  • Can you recommend a GIST specialist?

Questions to ask about choosing a treatment and managing side effects

  • What are my treatment options?

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

  • What treatment plan do you recommend? Why?

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

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

  • 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 be leading my overall treatment?

  • How will this treatment affect my daily life? Will I be able to work, exercise, or 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 medical care, who can help me?

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

  • Whom should I call with questions or problems?

Questions to ask about having surgery

  • What type of surgery will I have? Will anything besides the tumor be removed?

  • What is the goal of surgery? Will it remove the entire tumor?

  • How long will the operation take?

  • How long will I be in the hospital?

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

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

Questions about targeted therapy/TKIs

  • If I have surgery, should I take imatinib before surgery? What dosage? For how long? How much and how long after surgery?

  • What factors determine how effective imatinib is likely to be?

  • What are the possible side effects? How will they be treated?

  • What can be done if imatinib stops working? Can I receive sunitinib if that happens?

  • What can be done if sunitinib stops working? Can I receive regorafenib if that happens?

  • Can I take the generic form of the prescribed drug?

Questions to ask about having radiation therapy

  • What is the goal of this treatment?

  • How often will I receive radiation therapy?

  • What side effects can I expect during treatment?

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

  • What can be done to relieve the side effects?

  • What will I experience when I receive radiation therapy? Will it hurt or cause me discomfort?

  • What can I do to get ready for this treatment? Are there recommendations on what clothes to wear or leave behind?

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 be leading 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.

Gastrointestinal Stromal Tumor - GIST - Additional Resources

Approved by the Cancer.Net Editorial Board, 09/2018

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about care and treatment of a person diagnosed with cancer. This is the final page of Cancer.Net’s Guide to Gastrointestinal Stromal Tumors. 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 cancer 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 Gastrointestinal Stromal Tumors. Use the menu to choose a different section to read in this guide.