Gastrointestinal Stromal Tumors
Gastrointestinal Stromal Tumors
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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
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
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.
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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.
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