Difference between revisions of "Gastrointestinal stromal tumour"

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The '''gastrointestinal stromal tumour''', abbreviated '''GIST''', is uncommon tumour of the GI tract.   
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Gastrointestinal_stromal_tumour_-_very_low_mag.jpg
| Width      =
| Caption    = Gastrointestinal stromal tumour. [[H&E stain]].
| Micro      = spindle ''or'' epithelioid ''or'' mixed morphology, usu. centred on the muscularis propria
| Subtypes  =
| LMDDx      = [[schwannoma]], [[leiomyoma]], [[leiomyosarcoma]], [[neurofibroma]], [[desmoid-type fibromatosis]]
| Stains    =
| IHC        = CD117 +ve, [[DOG1]] +ve, CD34 +ve, S-100 -ve
| EM        =
| Molecular  = mutation in KIT gene ''or'' PDGFRA gene
| IF        =
| Gross      =
| Grossing  =
| Staging    = [[gastrointestinal stromal tumour staging]]
| Site      = [[stomach]], [[small intestine]], other sites
| Assdx      =
| Syndromes  = [[Neurofibromatosis type 1]], [[Carney triad]], [[Carney-Stratakis syndrome]]
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good to poor - dependent on size, site & mitotic rate
| Other      =
| ClinDDx    =
}}
The '''gastrointestinal stromal tumour''', abbreviated '''GIST''', is an uncommon tumour of the [[gastrointestinal tract pathology|gastrointestinal tract]].   


==General==
==General==
Epi.
===Definition===
*arises 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>
*Tumour resulting from a mutation in the KIT gene ''or'' PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.<ref name=pmid17090188/>
*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>
*Cases wild-type for KIT or PDFGRA may harbour defects in the [[succinate dehydrogenase]] complex, NF-1, BRAF, or extremely rarely KRAS.
 
===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>
 
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]] (von Recklinghausen's disease).
*[[Carney triad]].
*[[Carney-Stratakis syndrome]] - GISTs and [[paraganglioma]] - due to mutation in the genes for [[succinate dehydrogenase]].<ref name=pmid22997454>{{Cite journal  | last1 = Blay | first1 = JY. | last2 = Blomqvist | first2 = C. | last3 = Bonvalot | first3 = S. | last4 = Boukovinas | first4 = I. | last5 = Casali | first5 = PG. | last6 = De Alava | first6 = E. | last7 = Dei Tos | first7 = AP. | last8 = Dirksen | first8 = U. | last9 = Duffaud | first9 = F. | title = Gastrointestinal stromal tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. | journal = Ann Oncol | volume = 23 Suppl 7 | issue =  | pages = vii49-55 | month = Oct | year = 2012 | doi = 10.1093/annonc/mds252 | PMID = 22997454 | url = http://annonc.oxfordjournals.org/content/23/suppl_7/vii49.full }}</ref>
 
===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<ref name=pmid17090188/>
===Factors predictive of malignant behaviour===
Features suggesting a bad prognosis:<ref name=pmid17090188/>
*Large size.
*Large size.
**Often benign if small size.
**Often benign if small size.
*High mitotic rate (for area 5mm^2).
*High mitotic rate (for area 5mm<sup>2</sup>).
*Site - small intestine GISTs worse than stomach GISTs.
*Site - small intestine GISTs worse than stomach GISTs.


Small intestine bad prognosis:<ref name=pmid17090188/>
Small intestine bad prognosis:<ref name=pmid17090188/>
* >5 mitoses/5 mm^2 ''or'' size >10 cm.
* >5 mitoses/5 mm<sup>2</sup> ''or'' size >10 cm.


Stomach bad prognosis:<ref name=pmid17090188/>
Stomach bad prognosis:<ref name=pmid17090188/>
* >5 mitoses/5 mm^2 ''and'' size >5 cm.
* >5 mitoses/5 mm<sup>2</sup> ''and'' size >5 cm.


===Location===
===Location===
Line 24: Line 67:
*5% elsewhere.
*5% elsewhere.


Small intestinal GISTs have a worse prognosis than gastric ones.<ref name=pmid17090188/>  
Notes:
*Small intestinal GISTs have a worse prognosis than gastric ones.<ref name=pmid17090188/>
*GISTs almost never metastasize to the [[lymph node]]s (except for SDH-B deficient epithelioid GISTs)
**Most common [[metastasis]] locations: [[liver]], abdominal soft tissue.


===Definition===
==Microscopic==
*Mutation in the Kit gene or PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.<ref name=pmid17090188/>
Features:
*Classically, spindle cell morphology ~ 50% of tumours.<ref name=pmid15613856>{{Cite journal  | last1 = Miettinen | first1 = M. | last2 = Sobin | first2 = LH. | last3 = Lasota | first3 = J. | title = Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up. | journal = Am J Surg Pathol | volume = 29 | issue = 1 | pages = 52-68 | month = Jan | year = 2005 | doi =  | PMID = 15613856 }}</ref>
** May be epithelioid (round) ~40% of tumours.
** Mixed epithelioid and spindle cell tumours ~10% tumours.
*+/-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> - perinuclear.<ref>{{Cite journal  | last1 = Boşoteanu | first1 = M. | last2 = Boşoteanu | first2 = C. | last3 = Deacu | first3 = M. | last4 = Aşchie | first4 = M. | title = Differential diagnosis of a gastric stromal tumor: case report and literature review. | journal = Rom J Morphol Embryol | volume = 52 | issue = 4 | pages = 1361-8 | month =  | year = 2011 | doi =  | PMID = 22203947 }}</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.
**Not seen in gastric GISTs.<ref name=pmid12692202/>
**High [[specificity]] for GIST.
 
===DDx===
*[[Leiomyosarcoma]].
*[[Leiomyoma]] - esp. in the [[esophagus]].
*Neural tumours.
**[[Neurofibroma]].
**[[Schwannoma]] (GFAP +ve).
***GFAP uniformly neg. in GISTs.<ref name=pmid17090188/>
*[[Desmoid-type fibromatosis]].
*[[Epstein-Barr virus-associated smooth muscle tumour]] - very uncommon, in immunoincompetent individuals.<ref name=pmid16330945>{{Cite journal  | last1 = Deyrup | first1 = AT. | last2 = Lee | first2 = VK. | last3 = Hill | first3 = CE. | last4 = Cheuk | first4 = W. | last5 = Toh | first5 = HC. | last6 = Kesavan | first6 = S. | last7 = Chan | first7 = EW. | last8 = Weiss | first8 = SW. | title = Epstein-Barr virus-associated smooth muscle tumors are distinctive mesenchymal tumors reflecting multiple infection events: a clinicopathologic and molecular analysis of 29 tumors from 19 patients. | journal = Am J Surg Pathol | volume = 30 | issue = 1 | pages = 75-82 | month = Jan | year = 2006 | doi =  | PMID = 16330945 }}</ref>
 
===Images===
*www:
**[http://radiographics.rsna.org/content/25/2/455/F67.expansion.html GIST (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://radiographics.rsna.org/content/25/2/455/F68.expansion.html GIST with skenoid fibres (radiographics.rsna.org)].<ref name=pmid15798063/>
**[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>


==Micro.==
<gallery>
*Classically, spindle cell morphology; however, may be epithelioid (round).
Image:Gastric_GIST_%282%29.jpg | GIST - low mag. (WC/KGH)
*+/-Cytoplasmic inclusions.<ref name=pmid775795>PMID 775795</ref>
Image:Gastric_GIST_%281%29.jpg | GIST - high mag. (WC/KGH)
</gallery>
<gallery>
Image:Gastrointestinal_stromal_tumour_-_very_low_mag.jpg | Intestinal spindle cell GIST - very low mag. (WC/Nephron)
Image:Gastrointestinal_stromal_tumour_-_low_mag.jpg | Intestinal spindle cell GIST - low mag. (WC/Nephron)
Image:Gastrointestinal_stromal_tumour_-_intermed_mag.jpg | Intestinal spindle cell GIST - intermed. mag. (WC/Nephron)
Image:Gastrointestinal_stromal_tumour_-_high_mag.jpg | Intestinal spindle cell GIST - high mag. (WC/Nephron)
Image:Gastrointestinal_stromal_tumour_-_very_high_mag.jpg | Intestinal spindle cell GIST - very high mag. (WC/Nephron)
Image:Gastrointestinal_stromal_tumour_-_superf_-_intermed_mag.jpg | Epithelioid GIST - intermed. mag. (WC/Nephron)
</gallery>


==IHC==
==IHC==
*CD34+ in 70%.<ref name=pmid17090188/>
*CD117 +ve in 95%.<ref name=pmid17090188/>
*CD117+ in 95%.<ref name=pmid17090188/>
**[[Mast cell]]s are the internal positive control.
**Mast cells are the internal positive control.
*[[DOG1]] +ve.<ref name=pmid19011564>{{Cite journal  | last1 = Liegl | first1 = B. | last2 = Hornick | first2 = JL. | last3 = Corless | first3 = CL. | last4 = Fletcher | first4 = CD. | title = Monoclonal antibody DOG1.1 shows higher sensitivity than KIT in the diagnosis of gastrointestinal stromal tumors, including unusual subtypes. | journal = Am J Surg Pathol | volume = 33 | issue = 3 | pages = 437-46 | month = Mar | year = 2009 | doi = 10.1097/PAS.0b013e318186b158 | PMID = 19011564 }}</ref>


*Desmin+ in 5%.<ref name=pmid17090188/>
Others:
 
*CD34 +ve in 70%.<ref name=pmid17090188/>
===ICH Work-up panel===
*Desmin +ve in 5%.<ref name=pmid17090188/>
*WT1 +ve -- cytoplasmic (28/28 cases<ref name=pmid18528287>{{Cite journal  | last1 = Bing | first1 = Z. | last2 = Pasha | first2 = TL. | last3 = Acs | first3 = G. | last4 = Zhang | first4 = PJ. | title = Cytoplasmic overexpression of WT-1 in gastrointestinal stromal tumor and other soft tissue tumors. | journal = Appl Immunohistochem Mol Morphol | volume = 16 | issue = 4 | pages = 316-21 | month = Jul | year = 2008 | doi = 10.1097/PAI.0b013e31815c2e02 | PMID = 18528287 }}</ref>).
===IHC work-up panel===
*S-100 (neural tumours, rarely +ve in GISTs<ref name=pmid17090188/>).
*S-100 (neural tumours, rarely +ve in GISTs<ref name=pmid17090188/>).
*CD34, CD117 (GIST).
*CD34, CD117 (GIST).
*Desmin (muscle tumours).
*Desmin (muscle tumours).


==DDx==
==Molecular tests==
*Leiomyosarcoma.
:See ''[[Molecular_pathology_tests#Other]]''.
*Leiomyoma.
*Sequence Kit gene, PDGFRA gene.
*Neural tumours.
**Kit gene sequencing is being done more frequently as of late-- if a mutation is found it suggest the drug ''[[imatinib]]'' will be effective.
**Neurofibroma.
**Exon 11 mutation associated with malignant behaviour.<ref name=pmid9916918>{{Cite journal  | last1 = Lasota | first1 = J. | last2 = Jasinski | first2 = M. | last3 = Sarlomo-Rikala | first3 = M. | last4 = Miettinen | first4 = M. | title = Mutations in exon 11 of c-Kit occur preferentially in malignant versus benign gastrointestinal stromal tumors and do not occur in leiomyomas or leiomyosarcomas. | journal = Am J Pathol | volume = 154 | issue = 1 | pages = 53-60 | month = Jan | year = 1999 | doi = 10.1016/S0002-9440(10)65250-9 | PMID = 9916918 }}</ref>
**Schwannoma (GFAP+ --uniformly neg. in GISTs).<ref name=pmid17090188/>
**Secondary mutations of c-kit lead to imatinib resistance,<ref name=pmid26779618>{{Cite journal  | last1 = Wada | first1 = N. | last2 = Kurokawa | first2 = Y. | last3 = Takahashi | first3 = T. | last4 = Hamakawa | first4 = T. | last5 = Hirota | first5 = S. | last6 = Naka | first6 = T. | last7 = Miyazaki | first7 = Y. | last8 = Makino | first8 = T. | last9 = Yamasaki | first9 = M. | title = Detecting Secondary C-KIT Mutations in the Peripheral Blood of Patients with Imatinib-Resistant Gastrointestinal Stromal Tumor. | journal = Oncology | volume = 90 | issue = 2 | pages = 112-7 | month =  | year = 2016 | doi = 10.1159/000442948 | PMID = 26779618 }}</ref> and resistance to other similar inhibitors.
 
==Gastrointestinal stromal tumour staging==
{{Main|Gastrointestinal stromal tumour staging}}
GIST has its own staging.
 
==Sign out==
<pre>
STOMACH (MASS), LESSER CURVE, WEDGE RESECTION:
- GASTROINTESTINAL STROMAL TUMOUR (GIST).
-- MARGINS NEGATIVE FOR GIST.
 
COMMENT:
The tumour stains as follows:
POSITIVE: CD117, CD34.
NEGATIVE: Desmin, S-100.
</pre>
 
<pre>
SMALL BOWEL (ILEUM), RESECTION:
- GASTROINTESTINAL STROMAL TUMOUR (GIST), LOW-GRADE, NO RISK OF
  PROGRESSIVE DISEASE.
-- MARGINS NEGATIVE FOR GIST.
-- PLEASE SEE TUMOUR SUMMARY.
- THREE BENIGN LYMPH NODES.
 
COMMENT:
The tumour stains as follows:
POSITIVE: CD117, CD34.
NEGATIVE: Desmin, S-100.
PROLIFERATION (Ki-67): <1%.
</pre>
 
====Incidental GIST====
<pre>
Partial Stomach, Sleeve Gastrectomy:
- Stomach wall with incidental GASTROINTESTINAL STROMAL TUMOUR (GIST), 2 mm in maximal dimension.
-- Margin clear.
- Gastric mucosa within normal limits.
 
Comment:
The tumour stains as follows:
POSITIVE: DOG1, CD117, CD34.
NEGATIVE: desmin, S-100.
PROLIFERATION (Ki-67): <2%.
</pre>


==Special tests==
===Staging===
*Sequence Kit gene, PDGFRA gene.
*The stage is primarily determined by the tumour size and mitotic grade.
**Kit gene sequencing is being done more frequently as of late-- if a mutation is found it suggest the drug ''imatinib'' will be effective.
**In the stomach, the mitotic grade determines whether a given tumour is Stage I or Stage III.<ref>{{Cite journal  | last1 = Coccolini | first1 = F. | last2 = Catena | first2 = F. | last3 = Ansaloni | first3 = L. | last4 = Pinna | first4 = AD. | title = Gastrointestinal stromal tumor and mitosis, pay attention. | journal = World J Gastroenterol | volume = 18 | issue = 6 | pages = 587-8 | month = Feb | year = 2012 | doi = 10.3748/wjg.v18.i6.587 | PMID = 22363128 }}</ref>


==Treatment==
===Micro===
*[[Imatinib]] (Gleevec) - drug was developed for [[chronic myelogenous leukemia]].  
The sections show a spindle cell lesion that is well-circumscribed and without significant
**There are other similar drugs, e.g. ''nilotinib'' and ''dasatinib''.
nuclear pleomorphism. No lymphocytic cuff is surrounding the lesion.  The lesion is focally
seen at the inked soft tissue margin. Three mitoses are seen in 5 mm*mm.


==See also==
==See also==
*[[Stomach]]
*[[Stomach]].
*[[Small bowel]]
*[[Small bowel]].
*[[Gastrointestinal pathology]]
*[[Gastrointestinal pathology]].


==References==
==References==
Line 69: Line 196:


[[Category:Gastrointestinal pathology]]
[[Category:Gastrointestinal pathology]]
[[Category:Diagnosis]]

Latest revision as of 17:28, 15 November 2020

Gastrointestinal stromal tumour
Diagnosis in short

Gastrointestinal stromal tumour. H&E stain.
LM spindle or epithelioid or mixed morphology, usu. centred on the muscularis propria
LM DDx schwannoma, leiomyoma, leiomyosarcoma, neurofibroma, desmoid-type fibromatosis
IHC CD117 +ve, DOG1 +ve, CD34 +ve, S-100 -ve
Molecular mutation in KIT gene or PDGFRA gene
Staging gastrointestinal stromal tumour staging
Site stomach, small intestine, other sites
Syndromes Neurofibromatosis type 1, Carney triad, Carney-Stratakis syndrome
Prognosis good to poor - dependent on size, site & mitotic rate

The gastrointestinal stromal tumour, abbreviated GIST, is an uncommon tumour of the gastrointestinal tract.

General

Definition

  • Tumour resulting from a mutation in the KIT gene or PDGFRA (Platelet-derived growth factor receptor, alpha polypeptide) gene.[1]
  • Cases wild-type for KIT or PDFGRA may harbour defects in the succinate dehydrogenase complex, NF-1, BRAF, or extremely rarely KRAS.

Epidemiology

  • Arise from Interstitial cells of Cajal.[1]

May be familial/syndromic:[2]

Treatment

Factors predictive of malignant behaviour

Features suggesting a bad prognosis:[1]

  • Large size.
    • Often benign if small size.
  • High mitotic rate (for area 5mm2).
  • Site - small intestine GISTs worse than stomach GISTs.

Small intestine bad prognosis:[1]

  • >5 mitoses/5 mm2 or size >10 cm.

Stomach bad prognosis:[1]

  • >5 mitoses/5 mm2 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 (except for SDH-B deficient epithelioid GISTs)

Microscopic

Features:

  • Classically, spindle cell morphology ~ 50% of tumours.[4]
    • May be epithelioid (round) ~40% of tumours.
    • Mixed epithelioid and spindle cell tumours ~10% tumours.
  • +/-Cytoplasmic inclusions[5] - perinuclear.[6]
  • Classically splits the layers of the muscularis propria - as this is where the interstitial cells of Cajal are located.[7]
  • +/-Skenoid fibres - extracellular collagen bundles[8] ~ 2-5 x 60 micrometers - uncommon finding.

DDx

Images

  • GIST - low mag. (WC/KGH)

  • GIST - high mag. (WC/KGH)

  • Intestinal spindle cell GIST - very low mag. (WC/Nephron)

  • Intestinal spindle cell GIST - low mag. (WC/Nephron)

  • Intestinal spindle cell GIST - intermed. mag. (WC/Nephron)

  • Intestinal spindle cell GIST - high mag. (WC/Nephron)

  • Intestinal spindle cell GIST - very high mag. (WC/Nephron)

  • Epithelioid GIST - intermed. mag. (WC/Nephron)

IHC

Others:

  • CD34 +ve in 70%.[1]
  • Desmin +ve in 5%.[1]
  • WT1 +ve -- cytoplasmic (28/28 cases[12]).

IHC work-up panel

  • S-100 (neural tumours, rarely +ve in GISTs[1]).
  • CD34, CD117 (GIST).
  • Desmin (muscle tumours).

Molecular tests

See Molecular_pathology_tests#Other.
  • 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.
    • Exon 11 mutation associated with malignant behaviour.[13]
    • Secondary mutations of c-kit lead to imatinib resistance,[14] and resistance to other similar inhibitors.

Gastrointestinal stromal tumour staging

Main article: Gastrointestinal stromal tumour staging

GIST has its own staging.

Sign out

STOMACH (MASS), LESSER CURVE, WEDGE RESECTION:
- GASTROINTESTINAL STROMAL TUMOUR (GIST).
-- MARGINS NEGATIVE FOR GIST.

COMMENT:
The tumour stains as follows:
POSITIVE: CD117, CD34.
NEGATIVE: Desmin, S-100.

SMALL BOWEL (ILEUM), RESECTION:
- GASTROINTESTINAL STROMAL TUMOUR (GIST), LOW-GRADE, NO RISK OF 
  PROGRESSIVE DISEASE.
-- MARGINS NEGATIVE FOR GIST.
-- PLEASE SEE TUMOUR SUMMARY.
- THREE BENIGN LYMPH NODES.

COMMENT:
The tumour stains as follows:
POSITIVE: CD117, CD34.
NEGATIVE: Desmin, S-100.
PROLIFERATION (Ki-67): <1%.

Incidental GIST

Partial Stomach, Sleeve Gastrectomy:
	- Stomach wall with incidental GASTROINTESTINAL STROMAL TUMOUR (GIST), 2 mm in maximal dimension.
	-- Margin clear.
	- Gastric mucosa within normal limits.

Comment:
The tumour stains as follows:
POSITIVE: DOG1, CD117, CD34.
NEGATIVE: desmin, S-100.
PROLIFERATION (Ki-67): <2%.

Staging

  • The stage is primarily determined by the tumour size and mitotic grade.
    • In the stomach, the mitotic grade determines whether a given tumour is Stage I or Stage III.[15]

Micro

The sections show a spindle cell lesion that is well-circumscribed and without significant nuclear pleomorphism. No lymphocytic cuff is surrounding the lesion. The lesion is focally seen at the inked soft tissue margin. Three mitoses are seen in 5 mm*mm.

See also

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ Blay, JY.; Blomqvist, C.; Bonvalot, S.; Boukovinas, I.; Casali, PG.; De Alava, E.; Dei Tos, AP.; Dirksen, U. et al. (Oct 2012). "Gastrointestinal stromal tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.". Ann Oncol 23 Suppl 7: vii49-55. doi:10.1093/annonc/mds252. PMID 22997454. http://annonc.oxfordjournals.org/content/23/suppl_7/vii49.full.
  4. ↑ Miettinen, M.; Sobin, LH.; Lasota, J. (Jan 2005). "Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up.". Am J Surg Pathol 29 (1): 52-68. PMID 15613856.
  5. ↑ 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.
  6. ↑ Boşoteanu, M.; Boşoteanu, C.; Deacu, M.; Aşchie, M. (2011). "Differential diagnosis of a gastric stromal tumor: case report and literature review.". Rom J Morphol Embryol 52 (4): 1361-8. PMID 22203947.
  7. ↑ 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.
  8. ↑ 8.0 8.1 8.2 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.
  9. ↑ 9.0 9.1 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.
  10. ↑ Deyrup, AT.; Lee, VK.; Hill, CE.; Cheuk, W.; Toh, HC.; Kesavan, S.; Chan, EW.; Weiss, SW. (Jan 2006). "Epstein-Barr virus-associated smooth muscle tumors are distinctive mesenchymal tumors reflecting multiple infection events: a clinicopathologic and molecular analysis of 29 tumors from 19 patients.". Am J Surg Pathol 30 (1): 75-82. PMID 16330945.
  11. ↑ 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.
  12. ↑ Bing, Z.; Pasha, TL.; Acs, G.; Zhang, PJ. (Jul 2008). "Cytoplasmic overexpression of WT-1 in gastrointestinal stromal tumor and other soft tissue tumors.". Appl Immunohistochem Mol Morphol 16 (4): 316-21. doi:10.1097/PAI.0b013e31815c2e02. PMID 18528287.
  13. ↑ Lasota, J.; Jasinski, M.; Sarlomo-Rikala, M.; Miettinen, M. (Jan 1999). "Mutations in exon 11 of c-Kit occur preferentially in malignant versus benign gastrointestinal stromal tumors and do not occur in leiomyomas or leiomyosarcomas.". Am J Pathol 154 (1): 53-60. doi:10.1016/S0002-9440(10)65250-9. PMID 9916918.
  14. ↑ Wada, N.; Kurokawa, Y.; Takahashi, T.; Hamakawa, T.; Hirota, S.; Naka, T.; Miyazaki, Y.; Makino, T. et al. (2016). "Detecting Secondary C-KIT Mutations in the Peripheral Blood of Patients with Imatinib-Resistant Gastrointestinal Stromal Tumor.". Oncology 90 (2): 112-7. doi:10.1159/000442948. PMID 26779618.
  15. ↑ Coccolini, F.; Catena, F.; Ansaloni, L.; Pinna, AD. (Feb 2012). "Gastrointestinal stromal tumor and mitosis, pay attention.". World J Gastroenterol 18 (6): 587-8. doi:10.3748/wjg.v18.i6.587. PMID 22363128.
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