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Gastrointestinal Stromal Tumors

National Organization for Rare Disorders, Inc.

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 (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.

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 E. 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/



Lance Armstrong Foundation

2201 E. Sixth Street

Austin, TX 78702

Tel: (512)236-8820

Fax: (512)236-8482

Tel: (877)236-8820

Email: media@livestrong.org

Internet: http://www.livestrong.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:  2/15/2012

Copyright  2004 National Organization for Rare Disorders, Inc.

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