Gastrointestinal Stromal Tumor (GIST)

Overview

What are gastrointestinal stromal tumors (GISTs)?

Gastrointestinal stromal tumors (GISTs) are rare tumors that occur when cells become abnormal and grow out of control. GISTs can start anywhere in your digestive (gastrointestinal) tract, from the esophagus to the anus. More than half start in the stomach, and most others start in the small intestine.

Who do gastrointestinal stromal tumors (GISTs) affect?

GISTS are very rare in people under 40 years old. Some GISTs occur spontaneously, while others run in families. Talk to your healthcare provider if you have a family history of this condition.

How common are gastrointestinal stromal tumors (GISTs)?

The actual number of people with GIST is not known. However, the condition is rare. Healthcare providers estimate that there are between 4,000 and 6,000 cases per year in the U.S.

How does a gastrointestinal stromal tumor (GIST) affect my body?

GISTs are uncommon tumors that can grow anywhere in your digestive tract , from the esophagus to the anus. Some GISTs are small and not harmful, while others may be larger or cancerous. While some people may have no symptoms, others may feel unwell or have pain or bleeding.

Symptoms and Causes

What causes gastrointestinal stromal tumors (GISTs)?

All of your body's cells normally grow, divide, and then die to keep your body healthy and functioning properly. Sometimes this process gets out of control. Cells keep growing and dividing even when they are supposed to die. When the cells lining your digestive tract multiply uncontrolled, GISTs may ultimately develop.

Most GISTs involve changes (mutations) in the KIT oncogene. This gene tells cells to make a protein (KIT CD117), which causes them to grow faster and divide.

Is gastrointestinal stromal tumor (GIST) hereditary?

Most GISTs are the result of spontaneous changes in your cells and are not inherited. However, certain inherited tumor disorders can increase your risk. These include familial GIST syndrome, neurofibromatosis type 1 and Carney-Stratakis syndrome. Talk to your healthcare provider if these or any similar conditions run in your family.

What are the symptoms of gastrointestinal stromal tumors (GISTs)?

Some people have no symptoms of gastrointestinal stromal tumors (GISTs). Instead, their tumors are found accidentally during testing or surgery for another reason. Other individuals may experience the following symptoms:

Diagnosis and Tests

How are gastrointestinal stromal tumors (GISTs) diagnosed?

Your healthcare provider will first obtain a medical history, physical exam and blood work. If GIST is suspected, your provider will order imaging or endoscopy to determine if it is cancer or another condition.

What tests will be done to diagnose a gastrointestinal stromal tumor (GIST)?

There are several tests your provider can do to diagnose your gastrointestinal stromal tumor. These tests might include:

  • Upper endoscopy: This test may be ordered to locate the tumor and/or obtain a piece of the tumor (biopsy). In endoscopy, your doctor places a tube with a camera at the end into your mouth, through your food pipe (esophagus), and into your stomach. This allows your provider to look for a tumor.
  • Computed tomography (CT): A scan of the abdomen and pelvis may also be obtained to help your provider understand the location and size of the tumor. CT will also help your provider decide if the tumor can be removed.
  • Positron emission tomography (PET): A scan may also be performed to ensure your tumor is truly localized (in one place) and can be removed. If your surgeon can take out the whole thing, surgery will be performed. A pathologist will study your tumor under a microscope to find out if it is a GIST. They’ll also be able to test your tumor for genetic changes. Sometimes, the tumor is large or has spread and cannot be removed successfully. When this is the case, a biopsy is performed.
  • Biopsy: This procedure involves using a needle or surgical procedure to remove a part of the tumor. A pathologist then studies this tissue and can usually tell if it is a GIST and check for genetic changes.

GIST diagnosis and treatment are best accomplished by a team of experts working together with you. Each person needs to evaluate the advantages and limitations of each type of treatment. You’ll work with your team of physicians to develop the best approach.

What are the stages of gastrointestinal stromal tumor (GIST)?

After arriving at a cancer diagnosis, healthcare providers often try to figure out if your cancer has spread. Knowing how much cancer is in your body can help to determine how to treat it.

Cancer staging can be complicated. It’s a good idea to ask your provider to explain what it means for you.

For adults with GIST, providers use the American Joint Committee on Cancer (AJCC) TNM system. The AJCC TNM system is based on four factors:

  • The size of the primary tumor (T).
  • The spread to nearby lymph nodes (N).
  • The spread (metastasis) to other areas of the body, such as the liver, bones, lungs and the tissue that lines the abdomen (M).
  • How fast the cancer cells grow and divide (mitotic rate).

Management and Treatment

How are gastrointestinal stromal tumors (GISTs) treated?

The type of treatment your doctor recommends will depend on the size and location of your GIST, the results of lab tests and the stage of your disease. Your doctor will usually consider your age and general health, as well as your feelings about the treatment options. Treatment for GIST may include surgery and/or targeted drug therapy. Other therapies, such as chemotherapy and radiation therapy, are used less often.

What treatments and medications are used for gastrointestinal stromal tumors (GISTs)?

Some GISTs can be cured by surgery. However, some tumors are too large or have spread to other areas where surgery alone would not be curative. In these situations, a targeted drug therapy called imatinib may be prescribed. This is a pill taken once or twice daily by mouth. It works by attaching to the KIT protein (associated with the growth of most GISTs) and blocking its ability to let cancer cells grow. This medication can shrink the tumor or keep it from growing in about 85% of patients. Sometimes, if your tumor shrinks enough, surgery may become a treatment option. Unfortunately, your tumor may become resistant to imatinib and may start to grow again. If this happens to you, other targeted drugs may work to shrink the GIST. These include sunitinib, regorafenib and ripretinib.

Whether you receive surgery or medication, your doctor will likely monitor your condition with CT scans every three to six months. Sometimes, PET scans are done to check your response to treatment.

What are the side effects of treatment for gastrointestinal stromal tumors (GISTs)?

The most common side effects of targeted therapy include:

Your cancer team will monitor you closely during treatment. It’s important to tell your provider if you have changes in your health during or after cancer treatment.

Prevention

Who is at risk for gastrointestinal stromal tumors (GISTs)?

There are few known risk factors for GIST. Some GISTs develop because of spontaneous changes in cells. Known risk factors for GISTs are being 50 years old and older, and specific tumor disorders, including:

  • Familial GIST syndrome: Rarely, abnormal KIT genes may be passed on from parent to child. This can increase your chances of developing GISTs. If you have familial GIST syndrome, you may develop GIST at a young age. You may have more than one GIST.
  • Neurofibromatosis type 1: This disorder is caused by abnormal increases in cell growth. This results in the formation of tumors all over the body. These tumors can grow on nerves, causing problems with the skin, bones, eyes and elsewhere. If you have neurofibromatosis type 1, you are at higher risk of cancer, including GISTs.
  • Carney-Stratakis syndrome: People with this rare, inherited condition are at higher risk of developing GISTs. They are also at increased risk for other nerve tumors. If you have Carney-Stratakis syndrome, you may develop GIST at a young age. You may have more than one GIST.

How can I prevent gastrointestinal stromal tumors (GISTs)?

While the risk for some cancers can be lowered by healthy lifestyle changes (such as cutting back on alcohol, quitting smoking, or maintaining a healthy weight), no known lifestyle factors contribute to your risk of GIST. Talk to your healthcare provider if you have any of the known tumor disorders that can increase your risk of GISTs.

Outlook / Prognosis

What is the survival rate if I’m diagnosed with gastrointestinal stromal tumors (GISTs)?

The National Cancer Institute reports that the five-year relative survival rate for GIST is 83%. That means 83% of people diagnosed with GIST are alive five years later. The rate is even higher when the GIST is localized and hasn’t spread to other areas of the body. Then the survival rate reaches as high as 93%. However, if the GIST has spread to distant locations in the body, the five-year survival rate is 55%. Treatments continue to improve, along with survival rates.

Can gastrointestinal stromal tumor (GIST) be cured?

The outlook for people with GIST has improved dramatically in the last 20 years. In some patients, surgery may be curable. In others, targeted drug therapy with Tyrosine kinase inhibitors (a therapy that blocks signals needed for tumors to grow) may be necessary to shrink the tumor so it can be removed in surgery, slow its growth or help prevent it from spreading.

Living With

What questions should I ask my doctor about gastrointestinal stromal tumors (GISTs)?

It’s normal to have questions about your health, especially if you have a cancer diagnosis. It can be helpful to write down your questions and take the list with you when you meet your doctor. Talk about any concerns you have about your health, cancer surgery or treatment. Common questions include:

  • What stage is my GIST? What does that mean for me?
  • What symptoms should I watch out for?
  • How long will it take for me to get better and feel more like myself?
  • What long-term health issues might I have as a result of my cancer treatment?
  • What is the chance that my cancer will return?
  • What records do I need to keep about my treatment?
  • What can I do to take care of myself and stay as healthy as possible?
  • Can you suggest a support group that might help me?

How can I stay healthy during treatment for gastrointestinal stromal tumors (GISTs)?

Quit smoking and using other tobacco products

Smoking can increase the chances of getting cancer at the same site or a different site.

Cut down on how much alcohol you drink

Drinking alcohol increases the risk of certain cancers.

Maintain a healthy weight

Eating well and staying active can help you reach a healthy weight and stay there. A healthy and balanced diet is important for overall wellness. This includes eating fruits, vegetables, whole grains and protein. Talk with your healthcare provider or a dietitian to find out about any special dietary needs that you may have. You could also ask if you should talk to a nutritionist for guidance on eating a healthy diet.

Exercise and stay active

Research suggests that staying active after cancer may help lower the risk of recurrence and lead to longer survival. In addition, moderate exercise (walking, biking, swimming) for about 30 minutes every — or almost every — day can:

  • Reduce anxiety and depression.
  • Improve mood and boost self-esteem.
  • Reduce fatigue, nausea, pain and diarrhea.

It’s important to start an exercise program slowly and increase activity over time. Some people may need to take special care in exercising. Talk with your provider before you begin any exercise program, and work with your provider or a specialist (such as a physical therapist) if needed. If you need to stay in bed during your recovery, even doing small activities can help. Stretching or moving your arms or legs can help you stay flexible and relieve muscle tension.

What happens after my treatment for gastrointestinal stromal tumors (GISTs) ends?

After cancer treatment ends, all cancer survivors should have follow-up care. Follow-up care for GIST means seeing a healthcare provider for regular medical checkups once you're finished with treatment. These checkups may include bloodwork and other tests and procedures that look for any changes in your health or any problems that may occur due to your cancer treatment.

These visits are also a time to check for physical and emotional problems that may develop months or years after treatment ends. Your follow-up care plan, along with a summary of your cancer treatment, is part of what is called a survivorship care plan. This plan will have all the information for you and your doctor to discuss to ensure that you get regular and thorough care after your treatment ends. Please note that it’s important that you continue to receive your routine care from your primary care provider in addition to follow-up cancer care.

A note from Cleveland Clinic

GISTs are rare tumors that can start anywhere in the intestinal tract. Some GISTs may cause bleeding, stomach pain or bloating. Other GISTs cause no symptoms and are detected accidentally during a procedure for another condition. Some GISTs are cancerous, but with treatment, the outlook is promising. It’s important to pay close attention to your body and let your doctor know if anything unusual happens.

If you are diagnosed with GIST, remember that you are not alone. You have your family and friends, and there are support groups for nearly every type of cancer. Ask your doctor for information about these groups. You can also contact your local chapter of the American Cancer Society for more information. In addition, your doctor can refer you to a social worker or a mental health professional, both of whom can help you deal with the emotional aspects of your diagnosis. The social worker can also help you with the practical and financial issues related to the disease.

Last reviewed by a Cleveland Clinic medical professional on 08/23/2021.

References

  • American Cancer Society. Gastrointestinal Stromal Tumor (GIST). (https://www.cancer.org/cancer/gastrointestinal-stromal-tumor.html) Accessed 9/23/2021.
  • Joensuu H, Hohenberger P, Corless CL. Gastrointestinal stromal tumor. (https://pubmed.ncbi.nlm.nih.gov/23623056/) Lancet. 2013 Sep;382(9896):973-983. Accessed Accessed 9/23/2021.
  • Miettinen M, Lassota J. Gastrointestinal stromal tumors – definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. (https://pubmed.ncbi.nlm.nih.gov/11213830/) Virchows Arch. 2001 Jan;438(1):1-12. AAccessed 9/23/2021.
  • Soreide K, Sandvik OM, Soreide JA et al. Global epidemiology of gastrointestinal stromal tumours (GIST): a systematic review of population-based cohort studies. (https://pubmed.ncbi.nlm.nih.gov/26618334/) Cancer Epidemiol. 2016 Feb;40:39-46. Accessed 9/23/2021.
  • Stratakis CA, Carney JA. The triad of paragangliomas, gastric stromal tumours and pulmonary chondromas (Carney triad), and the dyad of paragangliomas and gastric stromal carcomas (Carney-Stratakis syndrome): molecular genetics and clinical implications. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3129547/) J Intern Med. 2009 Jul;266(1):43-52. Accessed 9/23/2021.

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