Gastrointestinal stromal tumor causes: Difference between revisions

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Revision as of 14:22, 3 September 2015

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

There are no established causes for gastrointestinal stromal tumor.

Most GISTs are sporadic. Less than 5% occur as part of hereditary familial or idiopathic multitumor syndromes.

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 __NOTOC__
 {{Gastrointestinal stromal tumor}}
 {{CMG}}{{AE}}{{PSD}}
 ==Overview==
+There are no established causes for gastrointestinal stromal tumor.
 ==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]].
+Most GISTs are sporadic. Less than 5% occur as part of [[genetic disorder|hereditary]] familial or idiopathic multitumor syndromes.
-<ref name="Agaimy2010">Agaimy A, Hartmann A. [Hereditary and non-hereditary syndromic gastointestinal stromal tumours]. [in German] Pathologe. 2010 Oct;31(6):430-7. doi: 10.1007/s00292-010-1354-6. PMID 20848108</ref> 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]]. Carney's triad does not feature SDH mutations.
 ==References==
 {{reflist|2}}