Gastrointestinal stromal tumor causes: Difference between revisions
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{{Gastrointestinal stromal tumor}} | {{Gastrointestinal stromal tumor}} | ||
{{CMG}} | {{CMG}}{{AE}}{{PSD}} | ||
==Overview== | |||
==Causes== | |||
Most GISTs are sporadic. Less than 5% occur as part of [[genetic disorder|hereditary]] familial or idiopathic multitumor syndromes. These include, in descending order of frequency, [[neurofibromatosis]] Recklinghausen (NF-1), [[Carney's triad]] (gastric GIST, [[pulmonary]] [[chondroma]] and extra-[[adrenal]] [[paraganglioma]]), [[germline]] [[Gain-of-function mutation|gain-of-function]] [[mutation]]s in [[c-Kit]]/[[PDGFRA]], and the [[Carney's triad#Carney-Stratakis syndrome|Carney-Stratakis syndrome]].<ref name="Agaimy2010" /> The Carney-Stratakis syndrome is a [[Dyad (Greek philosophy)|dyad]] of hereditary GIST and paraganglioma, that is caused by germline mutations in the [[mitochondria]]l [[tumor suppressor gene]] pathway involving the [[succinate dehydrogenase]] (SDH) subunits [[SDHD]], [[Succinate dehydrogenase complex subunit C|SDHC]] and [[SDHB]].<ref name="Stratakis2009" /> Carney's triad does not feature SDH mutations.<ref name="Stratakis2009" /> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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[[Category:Gastroenterology]] | [[Category:Gastroenterology]] | ||
[[Category:Types of cancer]] | [[Category:Types of cancer]] | ||
[[Category:Disease]] | [[Category:Disease]] | ||
Revision as of 14:34, 31 August 2015
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Gastrointestinal stromal tumor Microchapters |
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Differentiating Gastrointestinal stromal tumor from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Gastrointestinal stromal tumor causes On the Web |
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American Roentgen Ray Society Images of Gastrointestinal stromal tumor causes |
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Directions to Hospitals Treating Gastrointestinal stromal tumor |
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Risk calculators and risk factors for Gastrointestinal stromal tumor causes |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
Causes
Most GISTs are sporadic. Less than 5% occur as part of hereditary familial or idiopathic multitumor syndromes. These include, in descending order of frequency, neurofibromatosis Recklinghausen (NF-1), Carney's triad (gastric GIST, pulmonary chondroma and extra-adrenal paraganglioma), germline gain-of-function mutations in c-Kit/PDGFRA, and the Carney-Stratakis syndrome.[1] The Carney-Stratakis syndrome is a dyad of hereditary GIST and paraganglioma, that is caused by germline mutations in the mitochondrial tumor suppressor gene pathway involving the succinate dehydrogenase (SDH) subunits SDHD, SDHC and SDHB.[2] Carney's triad does not feature SDH mutations.[2]
References