Difference between revisions of "Gastrointestinal stromal tumour"
Jump to navigation
Jump to search
m (→Epidemiology) |
(→Microscopic: more +skenoid fibers) |
||
| Line 47: | Line 47: | ||
*+/-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> | *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> | ||
*Skenoid fibres - extracellular collagen bundles<ref name=pmid15798063/> ~ 2-5 x 60 micrometers. | |||
**Uncommon finding. | |||
**High [[specificity]] for GIST. | |||
===DDx=== | ===DDx=== | ||
| Line 56: | Line 59: | ||
***GFAP uniformly neg. in GISTs.<ref name=pmid17090188/> | ***GFAP uniformly neg. in GISTs.<ref name=pmid17090188/> | ||
*[[Desmoid-type fibromatosis]]. | *[[Desmoid-type fibromatosis]]. | ||
Image: | |||
*[http://radiographics.rsna.org/content/25/2/455/F67.expansion.html GIST with skenoid fibres (radiographics.rsna.org)].<ref name=pmid15798063>{{Cite journal | last1 = Levy | first1 = AD. | last2 = Patel | first2 = N. | last3 = Dow | first3 = N. | last4 = Abbott | first4 = RM. | last5 = Miettinen | first5 = M. | last6 = Sobin | first6 = LH. | title = From the archives of the AFIP: abdominal neoplasms in patients with neurofibromatosis type 1: radiologic-pathologic correlation. | journal = Radiographics | volume = 25 | issue = 2 | pages = 455-80 | month = | year = | doi = 10.1148/rg.252045176 | PMID = 15798063 |URL = http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=15798063}}</ref> | |||
*[http://www.nature.com/modpathol/journal/v16/n4/fig_tab/3880774f6.html GIST with skenoid fibres (nature.com)].<ref name=pmid12692202>{{Cite journal | last1 = Greenson | first1 = JK. | title = Gastrointestinal stromal tumors and other mesenchymal lesions of the gut. | journal = Mod Pathol | volume = 16 | issue = 4 | pages = 366-75 | month = Apr | year = 2003 | doi = 10.1097/01.MP.0000062860.60390.C7 | PMID = 12692202 | URL = http://www.nature.com/modpathol/journal/v16/n4/full/3880774a.html}}</ref> | |||
==IHC== | ==IHC== | ||
Revision as of 22:10, 9 April 2012
The gastrointestinal stromal tumour, abbreviated GIST, is an uncommon tumour of the gastrointestinal tract.
General
Definition
- Mutation in the Kit gene or PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.[1]
Epidemiology
- Arise from Interstitial cells of Cajal.[1]
May be familial/syndromic:[2]
- Neurofibromatosis 1 (Recklinghausen).
- Carney triad.
- Familial GIST syndrome (several).
- Others.
Treatment
- Imatinib (Gleevec) - drug was developed for chronic myelogenous leukemia.
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.
Notes:
- Small intestinal GISTs have a worse prognosis than gastric ones.[1]
- GISTs almost never metastasize to the lymph nodes.
- Most common metastasis locations: liver, abdominal soft tissue.
Microscopic
Features:
- Classically, spindle cell morphology; however, may be epithelioid (round).
- +/-Cytoplasmic inclusions.[3]
- Classically splits the layers of the muscularis propria - as this is where the interstitial cells of Cajal are located.[4]
- Skenoid fibres - extracellular collagen bundles[5] ~ 2-5 x 60 micrometers.
- Uncommon finding.
- High specificity for GIST.
DDx
- Leiomyosarcoma.
- Leiomyoma - esp. in the esophagus.
- Neural tumours.
- Neurofibroma.
- Schwannoma (GFAP +ve).
- GFAP uniformly neg. in GISTs.[1]
- Desmoid-type fibromatosis.
Image:
IHC
- CD34 +ve in 70%.[1]
- CD117 +ve in 95%.[1]
- Mast cells are the internal positive control.
ICH Work-up panel
- S-100 (neural tumours, rarely +ve in GISTs[1]).
- CD34, CD117 (GIST).
- Desmin (muscle tumours).
Molecular 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.
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, Hartmann A (October 2010). "[Hereditary and non-hereditary syndromic gastointestinal stromal tumours]" (in German). Pathologe 31 (6): 430–7. doi:10.1007/s00292-010-1354-6. PMID 20848108.
- ↑ Pasquinelli G, Severi B, Martinelli GN, Santini D, Gelli MC, Tison V (April 1995). "Gastro-intestinal stromal tumors: an ultrastructural reinterpretation of the clear cell component". J. Submicrosc. Cytol. Pathol. 27 (2): 251–7. PMID 7757951.
- ↑ 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.
- ↑ 5.0 5.1 Levy, AD.; Patel, N.; Dow, N.; Abbott, RM.; Miettinen, M.; Sobin, LH.. "From the archives of the AFIP: abdominal neoplasms in patients with neurofibromatosis type 1: radiologic-pathologic correlation.". Radiographics 25 (2): 455-80. doi:10.1148/rg.252045176. PMID 15798063.
- ↑ Greenson, JK. (Apr 2003). "Gastrointestinal stromal tumors and other mesenchymal lesions of the gut.". Mod Pathol 16 (4): 366-75. doi:10.1097/01.MP.0000062860.60390.C7. PMID 12692202.
- ↑ Liegl, B.; Hornick, JL.; Corless, CL.; Fletcher, CD. (Mar 2009). "Monoclonal antibody DOG1.1 shows higher sensitivity than KIT in the diagnosis of gastrointestinal stromal tumors, including unusual subtypes.". Am J Surg Pathol 33 (3): 437-46. doi:10.1097/PAS.0b013e318186b158. PMID 19011564.