Gastrointestinal Stromal Tumors

Synonyms

• GIST

Disorder Subdivisions

• None

General Discussion

Gastrointestinal stromal tumors (GIST) 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 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. GIST results from a change 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 are sporadic and not inherited.

Treatment is with surgery. Patients who have disease that has spread are treated with surgery when possible and with imatinib mesylate (Gleevec, Glivec), a tyrosine kinase inhibitor that inhibits the KIT or PDGFR responsible for tumor growth. Ongoing studies are testing to see if imatinib mesylate can delay or prevent recurrence of GIST after the tumor has been removed (resection). Standard chemotherapy is not effective for this type of sarcoma, with a less than 5% response rate. The role of imatinib mesylate in pediatric GIST is being studied at this time.

Resources

American Cancer Society, Inc.
1599 Clifton Road NE
Atlanta, GA 30329
USA
Tel: 4043203333
Tel: 8002272345
Internet: http://www.cancer.org

National Cancer Institute
6116 Executive Blvd, MSC 8322, Room 3036A
Bethesda, MD 20892-8322
USA
Tel: 3014353848
Tel: 8004226237
TDD: 8003328615
Internet: http://www.cancer.gov

National Coalition for Cancer Survivorship
1010 Wayne Avenue
Suite770
Silver Spring, MD 20910-5600
Tel: (301)650-9127
Fax: (301)565-9670
Tel: (877)622-7937
Email: infor@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: 2153495445
Fax: 2153495445
Email: editors@oncolink.upenn.edu
Internet: http://www.oncolink.upenn.edu

Sarcoma Foundation of America
PO Box 458
9884 Main Street
Damascus, MD 20872
USA
Tel: 3012538690
Fax: 3012538690
Email: info@curesarcoma.org
Internet: http://www.curesarcoma.org

Sarcoma Alliance
775 E. Blithedale #334
Mill Valley, CA 94941
USA
Tel: 4153817236
Fax: 4153817235
Email: info@sarcomaalliance.org
Internet: http://www.sarcomaalliance.org

Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
USA
Tel: 5206255495
Fax: 6155264921
Email: sharon.lane@rare-cancer.org
Internet: http://www.rare-cancer.org

Life Raft Group
40 Galesi Dr
Wayne, NJ 07470
USA
Tel: 9728379092
Fax: 9738379095
Email: LifeRaft@liferaftgroup.org
Internet: http://www.LifeRaftGroup.org

GIST Cancer Research Fund
55 Saw Mill Road
New City, NY 10956
USA
Tel: 8456346060
Email: tania5kids@aol.com
Internet: http://www.gistinfo.org

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
2231 Crystal Drive
Suite 200
Arlington, VA 22202
Tel: (703)302-1503
Fax: (703)302-1568
Email: info@focr.org
Internet: http://www.focr.org

People Living With Cancer
1900 Duke Street
Suite 200
Alexandria, VA 22314
Tel: (703)299-0150
Fax: (703)684-8618
Tel: (888)651-3038
Email: contactus@plwc.org
Internet: http://www.plwc.org

Wellness Community
919 18th Street N.W.
Suite 54
Washington, DC 20006
Tel: (202)659-9709
Fax: (202)659-9301
Tel: (888)793-9355
Email: help@thewellnesscommunity.org
Internet: http://www.thewellnesscommunity.org

For a Complete Report

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Last Updated: 8/26/2005
Copyright 2004 National Organization for Rare Disorders, Inc.