Difference between revisions of "Gastrointestinal stromal tumour"

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*[[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>
*[[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:
===Images===
*www:
*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/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://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>
**[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>
*[[WC]]:
 
**[http://commons.wikimedia.org/wiki/File:Gastric_GIST_%282%29.jpg GIST - low mag. (WC)].
<gallery>
**[http://commons.wikimedia.org/wiki/File:Gastric_GIST_%281%29.jpg GIST - high mag. (WC)].
Image:Gastric_GIST_%282%29.jpg | GIST - low mag. (WC/KGH)
**[http://commons.wikimedia.org/wiki/File:Gastrointestinal_stromal_tumour_-_low_mag.jpg Intestinal spindle cell GIST - low mag. (WC/Nephron)].
Image:Gastric_GIST_%281%29.jpg | GIST - high mag. (WC/KGH)  
**[http://commons.wikimedia.org/wiki/File:Gastrointestinal_stromal_tumour_-_intermed_mag.jpg Intestinal spindle cell GIST - intermed. mag. (WC/Nephron)].
Image:Gastrointestinal_stromal_tumour_-_low_mag.jpg | Intestinal spindle cell GIST - low mag. (WC/Nephron)
**[http://commons.wikimedia.org/wiki/File:Gastrointestinal_stromal_tumour_-_high_mag.jpg Intestinal spindle cell GIST - high mag. (WC/Nephron)].
Image:Gastrointestinal_stromal_tumour_-_intermed_mag.jpg | Intestinal spindle cell GIST - intermed. mag. (WC/Nephron)
**[http://commons.wikimedia.org/wiki/File:Gastrointestinal_stromal_tumour_-_superf_-_intermed_mag.jpg Epithelioid GIST - intermed. mag. (WC/Nephron)].
Image:Gastrointestinal_stromal_tumour_-_high_mag.jpg | Intestinal spindle cell GIST - high mag. (WC/Nephron)
Image:Gastrointestinal_stromal_tumour_-_superf_-_intermed_mag.jpg | Epithelioid GIST - intermed. mag. (WC/Nephron)
</gallery>


==IHC==
==IHC==

Revision as of 18:38, 18 May 2013

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]

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 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.

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]
  • Classically splits the layers of the muscularis propria - as this is where the interstitial cells of Cajal are located.[6]
  • +/-Skenoid fibres - extracellular collagen bundles[7] ~ 2-5 x 60 micrometers - uncommon finding.

DDx

Images

  • GIST - low mag. (WC/KGH)

  • GIST - high mag. (WC/KGH)

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

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

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

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

IHC

  • CD34 +ve in 70%.[1]
  • CD117 +ve in 95%.[1]
  • Desmin +ve in 5%.[1]
  • DOG1 +ve.[10]
    • More sensitive than CD117.

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.[11]

Sign out

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.[12]

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. ↑ 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.
  7. ↑ 7.0 7.1 7.2 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.
  8. ↑ 8.0 8.1 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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.
  12. ↑ 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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