Skip to content

    Cancer Health Center

    Font Size
    A
    A
    A

    Gastrointestinal Stromal Tumors

    Important
    It is possible that the main title of the report Gastrointestinal Stromal Tumors is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

    Synonyms

    • GIST

    Disorder Subdivisions

    • None

    General Discussion

    Gastrointestinal stromal tumors (GISTs) belong to a group of cancers known as soft tissue sarcomas. The number of new cases in the United States annually has been estimated to be 5,000-6,000. Tumors usually arise from the intestinal tract with the most common site being the stomach, followed by the small intestine, and then the colon/rectum with rare cases arising in the esophagus. There are also tumors that appear to arise in the membranous tissue lining the wall of the stomach (peritoneum) or in a fold of such membranous tissue (the omentum). There are also case reports of tumors arising in the appendix and/or pancreas. These tumors most commonly present with abdominal pain, bleeding or signs of intestinal obstruction. They spread most commonly to sites within the abdominal cavity and to the liver, although there are rare cases of spread to the lungs and bone. Some GISTs are noncancerous (benign) and do not spread (indolent); others are aggressive with extensive local invasion as well as distant metastases. Most cases result from a change (mutation) in one of two genes, KIT or PDGFR, which leads to continued growth and division of tumor cells. There are a few reported cases of families in which a gene mutation is inherited; however, the majority of tumors occur randomly for no apparent reason (sporadically) and not inherited (acquired mutation). Most cases arise in older adults.

    Approximately 10-15% of cases of GIST in adults and 85% of cases in children are not associated with mutations in either the KIT or PDGFR genes. These cases are known as wild type GISTs and are sometimes grouped together under the umbrella term pediatric-like GIST. They may be associated with other genes or have no identifiable gene mutation.
    Introduction

    GISTs were initially believed to be a single entity, but recent research has shown that there are several molecular subtypes with different characteristics including different prognoses, clinical symptoms, and different associated genes. that respond differently to various treatment options. Broadly, GISTs are classified as a soft tissue sarcoma. Sarcomas are malignant tumors that arise from the connective tissue, which connects, supports, and surrounds various structures and organs in the body. Soft tissue includes fat, muscle, nerves, tendons, and blood and lymph vessels.

    Resources

    American Cancer Society, Inc.
    250 Williams NW St
    Ste 6000
    Atlanta, GA 30303
    USA
    Tel: (404)320-3333
    Tel: (800)227-2345
    TDD: (866)228-4327
    Internet: http://www.cancer.org

    National Cancer Institute
    6116 Executive Blvd Suite 300
    Bethesda, MD 20892-8322
    USA
    Tel: (301)435-3848
    Tel: (800)422-6237
    TDD: (800)332-8615
    Email: cancergovstaff@mail.nih.gov
    Internet: http://www.cancer.gov

    National Coalition for Cancer Survivorship
    1010 Wayne Avenue
    7th Floor
    Silver Spring, MD 20910
    Tel: (301)650-9127
    Fax: (301)565-9670
    Tel: (888)650-9127
    Email: info@canceradvocacy.org
    Internet: http://www.canceradvocacy.org/

    OncoLink: The University of Pennsylvania Cancer Center Resource
    3400 Spruce Street
    2 Donner
    Philadelphia, PA 19104-4283
    USA
    Tel: (215)349-8895
    Fax: (215)349-5445
    Email: hampshire@uphs.upenn.edu
    Internet: http://www.oncolink.upenn.edu

    Sarcoma Foundation of America
    9899 Main Street Ste 204
    Damascus, MD 20872
    USA
    Tel: (301)253-8687
    Fax: (301)253-8690
    Email: info@curesarcoma.org
    Internet: http://www.curesarcoma.org

    Sarcoma Alliance
    775 East Blithedale 334
    Mill Valley, CA 94941
    USA
    Tel: (415)381-7236
    Fax: (415)381-7235
    Email: info@sarcomaalliance.org
    Internet: http://www.sarcomaalliance.org

    Rare Cancer Alliance
    1649 North Pacana Way
    Green Valley, AZ 85614
    USA
    Internet: http://www.rare-cancer.org

    Life Raft Group
    155 Route 46 West, Suite 202
    Wayne, NJ 07470
    USA
    Tel: (972)837-9092
    Fax: (973)837-9095
    Email: LifeRaft@liferaftgroup.org
    Internet: http://www.LifeRaftGroup.org

    GIST Cancer Research Fund
    55 Saw Mill Road
    New City, NY 10956
    USA
    Tel: (845)634-6060
    Fax: (845)634-1174
    Email: tania5kids@aol.com
    Internet: http://www.gistinfo.org

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    GIST Support International
    12 Bomaca Drive
    Doylestown, PA 18901
    Tel: (215)340-9374
    Fax: (215)340-1630
    Email: gsi@gistsupport.org
    Internet: http://www.gistsupport.org

    Friends of Cancer Research
    1800 M Street NW
    Suite 1050 South
    Washington, DC 22202
    Tel: (202)944-6700
    Email: info@focr.org
    Internet: http://www.focr.org

    Cancer.Net
    American Society of Clinical Oncology
    2318 Mill Road Suite 800
    Alexandria, VA 22314
    Tel: (571)483-1780
    Fax: (571)366-9537
    Tel: (888)651-3038
    Email: contactus@cancer.net
    Internet: http://www.cancer.net/

    Cancer Support Community
    1050 17th St NW Suite 500
    Washington, DC 20036
    Tel: (202)659-9709
    Fax: (202)974-7999
    Tel: (888)793-9355
    Internet: http://www.cancersupportcommunity.org/

    BeatSarcoma
    76 Ellsworth Street
    San Francisco, CA 94110
    Tel: (415)826-0474
    Email: info@beatsarcoma.org
    Internet: http://www.beatsarcoma.org/

    Northwest Sarcoma Foundation
    P.O. Box 91460
    Portland, OR 97291
    Tel: (503)954-5740
    Email: melissa@nwsarcoma.org
    Internet: http://www.nwsarcoma.org

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 3/5/2014
    Copyright 2004, 2014 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

    Today on WebMD

    man holding lung xray
    What you need to know.
    stem cells
    How they work for blood cancers.
     
    woman wearing pink ribbon
    Separate fact from fiction.
    Colorectal cancer cells
    Symptoms, screening tests, and more.
     
    Jennifer Goodman Linn self-portrait
    Blog
    what is your cancer risk
    HEALTH CHECK
     
    colorectal cancer treatment advances
    Video
    breast cancer overview slideshow
    SLIDESHOW
     
    prostate cancer overview
    SLIDESHOW
    lung cancer overview slideshow
    SLIDESHOW
     
    ovarian cancer overview slideshow
    SLIDESHOW
    Actor Michael Douglas
    Article