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Difference between revisions of "Gastrointestinal stromal tumour"
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Gastrointestinal stromal tumour (view source)
Revision as of 18:26, 4 March 2011
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==General== | ==General== | ||
===Definition=== | |||
*Mutation in the Kit gene or PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.<ref name=pmid17090188/> | |||
===Epidemiology=== | ===Epidemiology=== | ||
*Arise from ''Interstitial cells of Cajal''.<ref name=pmid17090188>{{cite journal |author=Miettinen M, Lasota J |title=Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=10 |pages=1466–78 |year=2006 |month=October |pmid=17090188 |doi= |url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=130&page=1466}}</ref> | *Arise from ''Interstitial cells of Cajal''.<ref name=pmid17090188>{{cite journal |author=Miettinen M, Lasota J |title=Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=10 |pages=1466–78 |year=2006 |month=October |pmid=17090188 |doi= |url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=130&page=1466}}</ref> | ||
May be familial/syndromic:<ref name=pmid20848108>{{cite journal |author=Agaimy A, Hartmann A |title=[Hereditary and non-hereditary syndromic gastointestinal stromal tumours] |language=German |journal=Pathologe |volume=31 |issue=6 |pages=430–7 |year=2010 |month=October |pmid=20848108 |doi=10.1007/s00292-010-1354-6 |url=}}</ref> | |||
*[[Neurofibromatosis|Neurofibromatosis 1]] (Recklinghausen). | |||
*[[Carney triad]]. | |||
*Others. | |||
===Treatment=== | |||
*[[Imatinib]] (Gleevec) - drug was developed for [[chronic myelogenous leukemia]]. | |||
**There are other similar drugs, e.g. ''nilotinib'' and ''dasatinib''. | |||
===Factors predictive of malignant behaviour=== | ===Factors predictive of malignant behaviour=== | ||
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Small intestinal GISTs have a worse prognosis than gastric ones.<ref name=pmid17090188/> | Small intestinal GISTs have a worse prognosis than gastric ones.<ref name=pmid17090188/> | ||
==Microscopic== | ==Microscopic== | ||
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*Classically, spindle cell morphology; however, may be epithelioid (round). | *Classically, spindle cell morphology; however, may be epithelioid (round). | ||
*+/-Cytoplasmic inclusions.<ref name=pmid7757951>{{cite journal |author=Pasquinelli G, Severi B, Martinelli GN, Santini D, Gelli MC, Tison V |title=Gastro-intestinal stromal tumors: an ultrastructural reinterpretation of the clear cell component |journal=J. Submicrosc. Cytol. Pathol. |volume=27 |issue=2 |pages=251–7 |year=1995 |month=April |pmid=7757951 |doi= |url=}}</ref> | *+/-Cytoplasmic inclusions.<ref name=pmid7757951>{{cite journal |author=Pasquinelli G, Severi B, Martinelli GN, Santini D, Gelli MC, Tison V |title=Gastro-intestinal stromal tumors: an ultrastructural reinterpretation of the clear cell component |journal=J. Submicrosc. Cytol. Pathol. |volume=27 |issue=2 |pages=251–7 |year=1995 |month=April |pmid=7757951 |doi= |url=}}</ref> | ||
*Classically splits the layers of the ''muscularis propria'' - as this is where the ''interstitial cells of Cajal'' are located.<ref name=pmid16402273>{{cite journal |author=Agaimy A, Wünsch PH |title=Gastrointestinal stromal tumours: a regular origin in the muscularis propria, but an extremely diverse gross presentation. A review of 200 cases to critically re-evaluate the concept of so-called extra-gastrointestinal stromal tumours |journal=Langenbecks Arch Surg |volume=391 |issue=4 |pages=322–9 |year=2006 |month=August |pmid=16402273 |doi=10.1007/s00423-005-0005-5 |url=}}</ref> | |||
==IHC== | ==IHC== | ||
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*Sequence Kit gene, PDGFRA gene. | *Sequence Kit gene, PDGFRA gene. | ||
**Kit gene sequencing is being done more frequently as of late-- if a mutation is found it suggest the drug ''imatinib'' will be effective. | **Kit gene sequencing is being done more frequently as of late-- if a mutation is found it suggest the drug ''imatinib'' will be effective. | ||
==See also== | ==See also== | ||