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> | ||
| Line 30: | Line 30: | ||
*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=== | ||
| Line 51: | Line 51: | ||
*Neural tumours. | *Neural tumours. | ||
**Neurofibroma. | **Neurofibroma. | ||
**Schwannoma (GFAP+ | **Schwannoma (GFAP +ve). | ||
***GFAP uniformly neg. in GISTs.<ref name=pmid17090188/> | |||
==Special tests== | ==Special tests== | ||
Revision as of 20:09, 26 May 2010
The gastrointestinal stromal tumour, abbreviated GIST, is uncommon tumour of the GI tract.
General
Epidemiology
- Arise from Interstitial cells of Cajal.[1]
- Classically splits the layers of the muscularis propria - as this is where the interstitial cells of Cajal are located.[2]
Factors predictive of malignant behaviour
Features suggesting a bad prognosis:[1]
- Large size.
- Often benign if small size.
- High mitotic rate (for area 5mm^2).
- Site - small intestine GISTs worse than stomach GISTs.
Small intestine bad prognosis:[1]
- >5 mitoses/5 mm^2 or size >10 cm.
Stomach bad prognosis:[1]
- >5 mitoses/5 mm^2 and size >5 cm.
Location
Most common locations in order:[1]
- 60% in stomach.
- 35% in small intestine.
- 5% elsewhere.
Small intestinal GISTs have a worse prognosis than gastric ones.[1]
Definition
- Mutation in the Kit gene or PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.[1]
Microscopic
- Classically, spindle cell morphology; however, may be epithelioid (round).
- +/-Cytoplasmic inclusions.[3]
IHC
- Desmin +ve in 5%.[1]
ICH Work-up panel
- S-100 (neural tumours, rarely +ve in GISTs[1]).
- CD34, CD117 (GIST).
- Desmin (muscle tumours).
DDx
- Leiomyosarcoma.
- Leiomyoma.
- Neural tumours.
- Neurofibroma.
- Schwannoma (GFAP +ve).
- GFAP uniformly neg. in GISTs.[1]
Special tests
- 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.
Treatment
- Imatinib (Gleevec) - drug was developed for chronic myelogenous leukemia.
- There are other similar drugs, e.g. nilotinib and dasatinib.
See also
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 Miettinen M, Lasota J (October 2006). "Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis". Arch. Pathol. Lab. Med. 130 (10): 1466–78. PMID 17090188. http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=130&page=1466.
- ↑ Agaimy A, Wünsch PH (August 2006). "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". Langenbecks Arch Surg 391 (4): 322–9. doi:10.1007/s00423-005-0005-5. PMID 16402273.
- ↑ PMID 775795