Gastrointestinal Stromal Tumor (GIST)

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk.

Risk factors for gastrointestinal stromal tumors include the following disorders:

• Retinoblastoma
• Neurofibromatosis type 1 (NF1, von Recklinghausen disease)
• Tuberous sclerosis (Bourneville disease)
• Familial adenomatous polyposis (FAP, Gardner syndrome)
• Li-Fraumeni syndrome
• Werner syndrome (adult progeria)
• Nevoid basal cell carcinoma syndrome (Gorlin syndrome)
• Carney-Stratakis syndrome
• Familial history of GIST

Gastrointestinal stromal tumors may cause the following symptoms:

• Abdominal discomfort or pain
• Blood in stools
• Vomiting
• Feeling full after eating only a small amount
• Loss of appetite or weight loss
• Fatigue due to anemia

Sometimes gastrointestinal stromal tumors are found by chance or seen on an exam for another problem. If you are having symptoms of GIST, your surgeon will ask about your health history, your family’s history of cancer and risk factors. Diagnostic tests may include:

• Computed tomography scan (CT-scan) 
• Magnetic resonance imaging (MRI)
• Ultrasound
• Colonoscopy
• Lower GI (gastrointestinal) series (also called barium enema)
• Biopsy to look for KIT protein (also called CD117)

Learn more about diagnostic tests for GIST.

Samples will be taken from the primary tumor, lymph nodes, and other suspicious areas. A pathologist views the tissue under a microscope to look for cancer cells and to find out the grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade tumors usually grow and spread more quickly than low-grade tumors.

Pathologists at DF/BWCC are among the best and most experienced in the world with special expertise in providing an accurate diagnosis for complex cases. In many instances when asked to review a case to provide a second opinion, the diagnosis and treatment plan is changed.

After GIST has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. This process is called staging.

Learn more about the stages of gastrointestinal stromal tumors.

Surgical Treatment

Surgery—which removes the tumor from your body—is the primary treatment for a gastrointestinal stromal tumor (GIST) that hasn’t spread. Surgical oncologists at BWH are surgical specialists who are on the faculty at Harvard Medical School. They are among the national leaders in GIST surgery with special expertise in metastatic GIST surgery. The oncology surgeons at BWH are well known for having perfected many progressive surgical procedures including:

• Laparoscopic surgery for small GISTS. Your surgeon makes several small incisions and inserts a thin lighted tube (with a tiny video camera) called a laparoscope through one of the incisions. He or she then uses long, thin surgical tools to remove the tumor through the other incisions.
• If the tumor is large or growing into other organs, your surgeon could still remove it entirely. He or she might have to remove parts of organs (such as sections of the intestines) and GISTs that have spread elsewhere in the abdomen, such as the liver.

Non-surgical Treatment

• Targeted therapy drugs
  • Gleevec (imatinib) is used for patients with advanced-stage GIST, and may help patients with earlier-stage tumors, either before or after surgery.
  • Sutent (sunitinib) targets the KIT and PDGFRA proteins.
• Radiation therapy is used to shrink the tumor before surgery or kill cancer cells that are left after surgery. It can sometimes be the main treatment if surgery isn’t an option.
• Chemotherapy drugs can also be used with or instead of surgery and are often the first treatment when the cancer has spread.

You will receive a thorough diagnostic evaluation to determine your course of treatment. Careful monitoring and the involvement of an experienced surgical oncologist are important to the successful outcome for patients with GIST.

If you are having surgery or a procedure, you will likely be scheduled for a visit to the Weiner Center for Preoperative Evaluation for pre-operative information and tests.

The day of surgery, you will be cared for in the operating room by surgeons, anesthesiologists and nurses who specialize in surgery for patients with GIST. After surgery, you will recover in the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff.

The Center for Sarcoma and Bone Cancer at Dana-Farber/Brigham and Women’s Cancer Center provides advanced and innovative multidisciplinary care for patients with sarcoma, including gastrointestinal stromal tumors. Our treatment team includes surgical oncologists, pathologists, medical and radiation oncologists, plastic surgeons, nutritionists and anesthesiologists. In addition, patients have full access to BWH’s world-renowned academic medical community with its diverse specialists and state-of-the-art facilities.

Learn more about gastrointestinal stromal tumor (GIST) in our health library.

Learn more about treatment for GIST at DF/BWCC.

Visit the Kessler Health Education Library in the Bretholtz Center for Patients and Families to access computers and knowledgeable staff.

Visit the Weiner Center for Preoperative Evaluation.

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