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.
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.
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
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Sarcoma Foundation of America 9899 Main Street Ste 204 Damascus, MD 20872 USA Tel: (301)253-8687 Fax: (301)253-8690 Email: firstname.lastname@example.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: email@example.com 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: firstname.lastname@example.org 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: email@example.com Internet: http://www.gistsupport.org
Friends of Cancer Research 1800 M Street NW Suite 1050 South Washington, DC 22202 Tel: (202)944-6700 Email: firstname.lastname@example.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: email@example.com 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: firstname.lastname@example.org Internet: http://www.beatsarcoma.org/
Northwest Sarcoma Foundation P.O. Box 91460 Portland, OR 97291 Tel: (503)954-5740 Email: email@example.com Internet: http://www.nwsarcoma.org
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Last Updated: 3/5/2014 Copyright 2004, 2014 National Organization for Rare Disorders, Inc.
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