Gastrointestinal Stromal Tumor

(GIST; Tumor, Gastrointestinal Stromal; Gastrointestinal Stromal Sarcoma; Gastric Myosarcoma; Gastric Myoblastoma; Gastrointestinal Leiomyosarcoma; Gastrointestinal Smooth Muscle Tumor)
  • Definition

    Gastrointestinal stromal tumors (GISTs) are a type of tumor that occurs in the digestive system. The digestive system includes the esophagus, liver, stomach, gall bladder, large and small intestines, and rectum. The digestive system processes food to fuel the body and gets rid of waste. About half of GISTs occur in the stomach, but they can occur anywhere in the digestive system.
    GISTs are rare. All GISTs are now considered potentially cancerous.
    Digestive System
    Digestive tract
    GIST may occur anywhere in the digestive system.
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  • Causes

    The cause of GISTs is not well understood.
    Many people with GIST have a defect in a gene called c-kit. Usually, this gene is not active. However, in people with GIST, the c-kit gene often has a defect and is active, which enables the cells to grow and divide uncontrollably. This may explain why a GIST forms.
    Theories about GISTs say that they begin in special cells of the digestive system. These cells are called interstitial cells of Cajal (ICCs). They send signals that tell the digestive system to contract, which moves food and liquid through the digestive system.
  • Risk Factors

    GIST is most common in people over age 50. It is rare in children. Risk factors for GIST are mostly unknown. Possible risk factors include:
    • Neurofibromatosis—a genetic disorder of the nervous system
    • Family history of GIST—rare
  • Symptoms

    GISTs may not cause any symptoms until they grow to a certain size. Symptoms may include:
    • Tiredness and weakness
    • Fever; sweating at night
    • Weight loss
    • Feeling full after eating a small amount of food
    • Abdominal pain
    • Painless lump in the abdomen
    • Nausea and/or vomiting
    • Blood in stool or vomit
    • Problems swallowing
  • Diagnosis

    Your doctor will ask about your symptoms and medical history. A physical exam will also be done.
    Your bodily structures will need to be viewed to determine the location and size of the tumor. This can be done with:
    Your bodily tissues may be tested. This can be done with a fine-needle biopsy.
  • Treatment

    Talk with your doctor about the best treatment plan for you. Treatment options include:
    Surgery
    Surgery is used to treat a GIST that has not spread. Surgery to treat GIST should be done by an experienced cancer surgeon. GISTs are delicate tumors. There may be an increased risk of cancer spreading if the sac surrounding the GIST is opened during surgery.
    You may be given medications to shrink the tumor before surgery. For small tumors, laparoscopic surgery may be used.
    Even if the tumor is large or growing into other organs, your doctor may use surgery to remove some or the entire tumor. This reduces the chance that the tumor will grow large enough to block the digestive system.
    Medication
    You may be given a medication called a tyrosine kinase inhibitor. It may make the tumor shrink or stop growing.
  • Prevention

    GISTs cannot be prevented.
  • RESOURCES

    American Cancer Society http://www.cancer.org

    American Gastroenterological Association http://www.gastro.org

    CANADIAN RESOURCES

    Canadian Association of Gastroenterology http://www.cag-acg.org

    Canadian Cancer Society http://www.cancer.ca

    References

    Basu S, Balaji S, et al. Gastrointestional stromal tumors (GIST) and laparoscopic resection. Surg Endosc. 2007;21:1685-1689.

    Huang HY, Li CF, et al. A modification of NIH consensus criteria to better distinguish the highly lethal subset of primary localized gastrointestinal stromal tumors: a subdivision of the original high-risk group on the basis of outcome. Surgery. 2007;141(6):748

    Demetri GD, von Mehren M, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med. 2002;347(7):472.

    Duffaud F, Blay J. Gastrointestinal stromal tumors: biology and treatment. Oncology. 2003;65:187-197.

    Gastrointestinal stromal tumor. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated December 12, 2012. Accessed June 20, 2014.

    Gastrointestinal stromal tumor. Ped Surg Update. 2005;24(6):3.

    Gastrointestinal stromal tumor (GIST). American Cancer Society website. Available at: http://www.cancer.org/cancer/gastrointestinalstromaltumorgist/detailedguide/gastrointestinal-stromal-tumor-what-is-gist. Updated February 26, 2013. Accessed August 14, 2013.

    Gastrointestinal stromal tumors (GISTs). Macmillan Cancer Support website. Available at: h http://www.macmillan.org.uk/Cancerinformation/Cancertypes/Softtissuesarcomas/Typesofsofttissuesarcomas/GIST.aspx. Updated January 1, 2013. Accessed August 14, 2013.

    Rubin BP, Heinrich MC, et al. Gastrointestinal stromal tumour. Lancet. 2007;369(9574):1731-1741.

    Shinomura Y, Kinoshita K, et al. Pathophysiology, diagnosis, and treatment of gastrointestinal stromal tumors. J Gastroenterol. 2005;40:775-780.

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