Difference between revisions of "Gastrointestinal stromal tumour"
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==General== | ==General== | ||
===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> | ||
*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> | *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> | ||
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*Mutation in the Kit gene or PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.<ref name=pmid17090188/> | *Mutation in the Kit gene or PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.<ref name=pmid17090188/> | ||
== | ==Microscopic== | ||
*Classically, spindle cell morphology; however, may be epithelioid (round). | *Classically, spindle cell morphology; however, may be epithelioid (round). | ||
*+/-Cytoplasmic inclusions.<ref name=pmid775795>PMID 775795</ref> | *+/-Cytoplasmic inclusions.<ref name=pmid775795>PMID 775795</ref> | ||
==IHC== | ==IHC== | ||
*CD34+ in 70%.<ref name=pmid17090188/> | *CD34 +ve in 70%.<ref name=pmid17090188/> | ||
*CD117+ in 95%.<ref name=pmid17090188/> | *CD117 +ve in 95%.<ref name=pmid17090188/> | ||
**Mast cells are the internal positive control. | **Mast cells are the internal positive control. | ||
*Desmin+ in 5%.<ref name=pmid17090188/> | *Desmin +ve in 5%.<ref name=pmid17090188/> | ||
===ICH Work-up panel=== | ===ICH Work-up panel=== | ||
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*Neural tumours. | *Neural tumours. | ||
**Neurofibroma. | **Neurofibroma. | ||
**Schwannoma (GFAP+ | **Schwannoma (GFAP +ve). | ||
***GFAP uniformly neg. in GISTs.<ref name=pmid17090188/> | |||
==Special tests== | ==Special tests== | ||