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2024-03-29T14:25:00Z
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https://librepathology.org/w/index.php?title=Juxtaglomerular_cell_tumour&diff=52865
Juxtaglomerular cell tumour
2024-03-26T15:15:45Z
<p>Michael: more</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = <br />
| Width =<br />
| Caption = <br />
| Synonyms = juxtaglomerular tumour, reninoma<br />
| Micro =<br />
| Subtypes =<br />
| LMDDx = [[metanephric adenoma]], [[papillary renal cell carcinoma]], [[epithelioid angiomyolipoma]], [[chromophobe renal cell carcinoma]] <br />
| Stains =<br />
| IHC =<br />
| EM =<br />
| Molecular =<br />
| IF =<br />
| Gross =<br />
| Grossing =<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx = [[hypertension]]<br />
| Syndromes =<br />
| Clinicalhx = usu. young adult or adolescent<br />
| Signs = [[hypertension]]<br />
| Symptoms =<br />
| Prevalence = very rare<br />
| Bloodwork = increased plasma renin<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = benign (???)<br />
| Other =<br />
| ClinDDx = other causes of [[hypertension]]<br />
| Tx = surgery<br />
}}<br />
'''Juxtaglomerular cell tumour''', abbreviated '''JCT''', is a rare [[kidney tumour]] associated with [[hypertension]] and typically seen in adolescents or young adults.<br />
<br />
It is also known as '''juxtaglomerular tumour''' and '''reninoma'''.<ref name=pmid18192852>{{Cite journal | last1 = Wong | first1 = L. | last2 = Hsu | first2 = TH. | last3 = Perlroth | first3 = MG. | last4 = Hofmann | first4 = LV. | last5 = Haynes | first5 = CM. | last6 = Katznelson | first6 = L. | title = Reninoma: case report and literature review. | journal = J Hypertens | volume = 26 | issue = 2 | pages = 368-73 | month = Feb | year = 2008 | doi = 10.1097/HJH.0b013e3282f283f3 | PMID = 18192852 }}</ref><br />
<br />
==General==<br />
*Rare.<br />
*Etiology: increased renin.<br />
*Typically adolescents or young adults.<ref name=pmid21871063/><br />
<br />
Clinical:<ref name=pmid18192852>{{Cite journal | last1 = Wong | first1 = L. | last2 = Hsu | first2 = TH. | last3 = Perlroth | first3 = MG. | last4 = Hofmann | first4 = LV. | last5 = Haynes | first5 = CM. | last6 = Katznelson | first6 = L. | title = Reninoma: case report and literature review. | journal = J Hypertens | volume = 26 | issue = 2 | pages = 368-73 | month = Feb | year = 2008 | doi = 10.1097/HJH.0b013e3282f283f3 | PMID = 18192852 }}</ref><br />
*[[Hypertension]].<br />
*Increased aldosterone.<br />
**Causes hypokalemia. <br />
*Increased plasma renin.<br />
<br />
==Microscopic==<br />
Features:<ref name=pmid21191395/><br />
*Polygonal cells.<br />
*Abundant granular, eosinophilic cytoplasm.<ref name=pmid436071/><br />
*Perinuclear halo.<br />
<br />
DDx:<ref name=pmid21871063/><br />
*[[Metanephric adenoma]].<br />
*[[Papillary RCC]].<br />
*[[Epithelioid angiomyolipoma]].<br />
*[[Chromophobe renal cell carcinoma]], eosinophilic variant.{{fact}}<br />
<br />
Note - some similarities with:<br />
*[[Solitary fibrous tumour]].<br />
*[[Glomus tumour]].<br />
<br />
===Images===<br />
*[http://www.nature.com/ki/journal/v79/n2/fig_tab/ki2010445f1.html#figure-title Reninoma (nature.com)].<ref name=pmid21191395/><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3173291/figure/F1/ JCT (nih.gov)].<ref name=pmid21871063/><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3173291/figure/F2/ JCT (nih.gov)].<ref name=pmid21871063>{{Cite journal | last1 = Kuroda | first1 = N. | last2 = Gotoda | first2 = H. | last3 = Ohe | first3 = C. | last4 = Mikami | first4 = S. | last5 = Inoue | first5 = K. | last6 = Nagashima | first6 = Y. | last7 = Petersson | first7 = F. | last8 = Alvarado-Cabrero | first8 = I. | last9 = Pan | first9 = CC. | title = Review of juxtaglomerular cell tumor with focus on pathobiological aspect. | journal = Diagn Pathol | volume = 6 | issue = | pages = 80 | month = | year = 2011 | doi = 10.1186/1746-1596-6-80 | PMID = 21871063 }}</ref><br />
<br />
==Stains==<br />
Cytoplasmic granules.<ref name=pmid436071>{{Cite journal | last1 = Hanna | first1 = W. | last2 = Tepperman | first2 = B. | last3 = Logan | first3 = AG. | last4 = Robinette | first4 = MA. | last5 = Colapinto | first5 = R. | last6 = Phillips | first6 = MJ. | title = Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension. | journal = Can Med Assoc J | volume = 120 | issue = 8 | pages = 957-9 | month = Apr | year = 1979 | doi = | PMID = 436071 | PMC = 1819229 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1819229/?tool=pubmed }}</ref><br />
*PAS +ve.<br />
*PASD +ve.<br />
<br />
==IHC==<br />
Features:<br />
*CD34 +ve.<ref name=pmid21871063/><br />
*Vimentin +ve.<br />
*Actin +ve.<br />
*HMB-45 -ve.<ref name=pmid21191395/><br />
<br />
Others:<ref name=pmid21871063/><br />
*CD31 -ve. <br />
*Desmin -ve.<br />
*S-100 -ve.<br />
*Chromogranin -ve.<br />
*Synaptophysin -ve.<br />
*NSE -ve.<br />
*CAM5.2 +ve.<ref name=pmid21871063/> ‡<br />
*CK7 +ve.<ref name=pmid21871063/> ‡<br />
<br />
Note:<br />
*‡ Negative keratin staining reported by Chao ''et al.''.<ref name=pmid21191395>{{Cite journal | last1 = Chao | first1 = CT. | last2 = Chang | first2 = FC. | last3 = Wu | first3 = VC. | last4 = Chen | first4 = JC. | title = Reninoma. | journal = Kidney Int | volume = 79 | issue = 2 | pages = 260 | month = Jan | year = 2011 | doi = 10.1038/ki.2010.445 | PMID = 21191395 }}</ref><br />
<br />
==EM==<br />
Features:<br />
*Vesicles - contain renin.<ref name=upmc29>URL: [http://path.upmc.edu/cases/case29/micro.html http://path.upmc.edu/cases/case29/micro.html]. Accessed on: 18 December 2011.</ref><br />
<br />
==Molecular==<br />
*NOTCH1 rearrangement.<ref>PMID: 37749094</ref><br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumours with eosinophilic cytoplasm]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Juxtaglomerular_cell_tumour&diff=52864
Juxtaglomerular cell tumour
2024-03-26T15:10:29Z
<p>Michael: /* EM */</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = <br />
| Width =<br />
| Caption = <br />
| Synonyms = juxtaglomerular tumour, reninoma<br />
| Micro =<br />
| Subtypes =<br />
| LMDDx = [[metanephric adenoma]], [[papillary renal cell carcinoma]], [[epithelioid angiomyolipoma]], [[chromophobe renal cell carcinoma]] <br />
| Stains =<br />
| IHC =<br />
| EM =<br />
| Molecular =<br />
| IF =<br />
| Gross =<br />
| Grossing =<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx = [[hypertension]]<br />
| Syndromes =<br />
| Clinicalhx = usu. young adult or adolescent<br />
| Signs = [[hypertension]]<br />
| Symptoms =<br />
| Prevalence = very rare<br />
| Bloodwork = increased plasma renin<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = benign (???)<br />
| Other =<br />
| ClinDDx = other causes of [[hypertension]]<br />
| Tx = surgery<br />
}}<br />
'''Juxtaglomerular cell tumour''', abbreviated '''JCT''', is a rare [[kidney tumour]] associated with [[hypertension]] and typically seen in adolescents or young adults.<br />
<br />
It is also known as '''juxtaglomerular tumour''' and '''reninoma'''.<ref name=pmid18192852>{{Cite journal | last1 = Wong | first1 = L. | last2 = Hsu | first2 = TH. | last3 = Perlroth | first3 = MG. | last4 = Hofmann | first4 = LV. | last5 = Haynes | first5 = CM. | last6 = Katznelson | first6 = L. | title = Reninoma: case report and literature review. | journal = J Hypertens | volume = 26 | issue = 2 | pages = 368-73 | month = Feb | year = 2008 | doi = 10.1097/HJH.0b013e3282f283f3 | PMID = 18192852 }}</ref><br />
<br />
==General==<br />
*Rare.<br />
*Etiology: increased renin.<br />
*Typically adolescents or young adults.<ref name=pmid21871063/><br />
<br />
Clinical:<ref name=pmid18192852>{{Cite journal | last1 = Wong | first1 = L. | last2 = Hsu | first2 = TH. | last3 = Perlroth | first3 = MG. | last4 = Hofmann | first4 = LV. | last5 = Haynes | first5 = CM. | last6 = Katznelson | first6 = L. | title = Reninoma: case report and literature review. | journal = J Hypertens | volume = 26 | issue = 2 | pages = 368-73 | month = Feb | year = 2008 | doi = 10.1097/HJH.0b013e3282f283f3 | PMID = 18192852 }}</ref><br />
*[[Hypertension]].<br />
*Increased aldosterone.<br />
**Causes hypokalemia. <br />
*Increased plasma renin.<br />
<br />
==Microscopic==<br />
Features:<ref name=pmid21191395/><br />
*Polygonal cells.<br />
*Abundant granular, eosinophilic cytoplasm.<ref name=pmid436071/><br />
*Perinuclear halo.<br />
<br />
DDx:<ref name=pmid21871063/><br />
*[[Metanephric adenoma]].<br />
*[[Papillary RCC]].<br />
*[[Epithelioid angiomyolipoma]].<br />
*[[Chromophobe renal cell carcinoma]], eosinophilic variant.{{fact}}<br />
<br />
===Images===<br />
*[http://www.nature.com/ki/journal/v79/n2/fig_tab/ki2010445f1.html#figure-title Reninoma (nature.com)].<ref name=pmid21191395/><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3173291/figure/F1/ JCT (nih.gov)].<ref name=pmid21871063/><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3173291/figure/F2/ JCT (nih.gov)].<ref name=pmid21871063>{{Cite journal | last1 = Kuroda | first1 = N. | last2 = Gotoda | first2 = H. | last3 = Ohe | first3 = C. | last4 = Mikami | first4 = S. | last5 = Inoue | first5 = K. | last6 = Nagashima | first6 = Y. | last7 = Petersson | first7 = F. | last8 = Alvarado-Cabrero | first8 = I. | last9 = Pan | first9 = CC. | title = Review of juxtaglomerular cell tumor with focus on pathobiological aspect. | journal = Diagn Pathol | volume = 6 | issue = | pages = 80 | month = | year = 2011 | doi = 10.1186/1746-1596-6-80 | PMID = 21871063 }}</ref><br />
<br />
==Stains==<br />
Cytoplasmic granules.<ref name=pmid436071>{{Cite journal | last1 = Hanna | first1 = W. | last2 = Tepperman | first2 = B. | last3 = Logan | first3 = AG. | last4 = Robinette | first4 = MA. | last5 = Colapinto | first5 = R. | last6 = Phillips | first6 = MJ. | title = Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension. | journal = Can Med Assoc J | volume = 120 | issue = 8 | pages = 957-9 | month = Apr | year = 1979 | doi = | PMID = 436071 | PMC = 1819229 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1819229/?tool=pubmed }}</ref><br />
*PAS +ve.<br />
*PASD +ve.<br />
<br />
==IHC==<br />
Features:<br />
*CD34 +ve.<ref name=pmid21871063/><br />
*Vimentin +ve.<br />
*Actin +ve.<br />
*HMB-45 -ve.<ref name=pmid21191395/><br />
<br />
Others:<ref name=pmid21871063/><br />
*CD31 -ve. <br />
*Desmin -ve.<br />
*S-100 -ve.<br />
*Chromogranin -ve.<br />
*Synaptophysin -ve.<br />
*NSE -ve.<br />
*CAM5.2 +ve.<ref name=pmid21871063/> ‡<br />
*CK7 +ve.<ref name=pmid21871063/> ‡<br />
<br />
Note:<br />
*‡ Negative keratin staining reported by Chao ''et al.''.<ref name=pmid21191395>{{Cite journal | last1 = Chao | first1 = CT. | last2 = Chang | first2 = FC. | last3 = Wu | first3 = VC. | last4 = Chen | first4 = JC. | title = Reninoma. | journal = Kidney Int | volume = 79 | issue = 2 | pages = 260 | month = Jan | year = 2011 | doi = 10.1038/ki.2010.445 | PMID = 21191395 }}</ref><br />
<br />
==EM==<br />
Features:<br />
*Vesicles - contain renin.<ref name=upmc29>URL: [http://path.upmc.edu/cases/case29/micro.html http://path.upmc.edu/cases/case29/micro.html]. Accessed on: 18 December 2011.</ref><br />
<br />
==Molecular==<br />
*NOTCH1 rearrangement.<ref>PMID: 37749094</ref><br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumours with eosinophilic cytoplasm]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Glycoprotein_non-metastatic_B&diff=52863
Glycoprotein non-metastatic B
2024-03-26T13:33:06Z
<p>Michael: </p>
<hr />
<div>'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an [[immunostain]].<br />
<br />
It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
==Positive==<br />
*[[Angiomyolipoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[Low-grade oncocytic tumour]] (LOT).<br />
*[[PEComa]].<br />
*[[Renal cell carcinoma with fibromyomatous stroma]].<br />
<br />
Others:<br />
*[[Leiomyosarcoma]].{{fact}}<br />
<br />
==Negative==<br />
*[[Clear cell renal cell carcinoma]].<br />
*[[Papillary renal cell carcinoma]].<br />
*[[Chromophobe renal cell carcinoma]].<br />
*[[Clear cell papillary renal cell tumour]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Immunohistochemistry]]</div>
Michael
https://librepathology.org/w/index.php?title=ELOC-mutated_renal_cell_carcinoma&diff=52862
ELOC-mutated renal cell carcinoma
2024-03-26T13:31:14Z
<p>Michael: /* IHC */</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Renal cell carcinoma with clear cells and fibromuscular bands -- intermed mag.jpg <br />
| Width =<br />
| Caption = RCC with clear cells and fibromuscular band - morphology compatible with TCEB1 mutation. [[H&E stain]]. (WC)<br />
| Synonyms = TCEB1-mutated renal cell carcinoma (old name)<br />
| Micro = clear cells with abundant cytoplasm and basal nuclei (may have luminal nuclei), usually [[ISUP nucleolar grade]] 2, fibromuscular bands<br />
| Subtypes =<br />
| LMDDx = [[clear cell renal cell carcinoma]], [[clear cell papillary renal cell tumour]] (clear cell papillary RCC), [[renal cell carcinoma with fibromyomatous stroma]]<br />
| Stains =<br />
| IHC = CK7 +ve, CD10 +ve, [[CK34betaE12]] -ve<br />
| EM =<br />
| Molecular = TCEB1 mutation<br />
| IF =<br />
| Gross =<br />
| Grossing =<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx =<br />
| Syndromes = possibly [[tuberous sclerosis]] - see ''[[tuberous sclerosis-associated renal cell carcinoma]]''<br />
| Clinicalhx =<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = very rare - evolving entity<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = good - based on limited data<br />
| Other =<br />
| ClinDDx =<br />
| Tx =<br />
}}<br />
'''ELOC-mutated renal cell carcinoma''' is a type of [[renal cell carcinoma]] that is morphologically similar to [[clear cell renal cell carcinoma]] and [[clear cell papillary renal cell tumour]] (previously ''clear cell papillary renal cell carcinoma'').<ref name=pmid25676555>{{Cite journal | last1 = Hakimi | first1 = AA. | last2 = Tickoo | first2 = SK. | last3 = Jacobsen | first3 = A. | last4 = Sarungbam | first4 = J. | last5 = Sfakianos | first5 = JP. | last6 = Sato | first6 = Y. | last7 = Morikawa | first7 = T. | last8 = Kume | first8 = H. | last9 = Fukayama | first9 = M. | title = TCEB1-mutated renal cell carcinoma: a distinct genomic and morphological subtype. | journal = Mod Pathol | volume = 28 | issue = 6 | pages = 845-53 | month = Jun | year = 2015 | doi = 10.1038/modpathol.2015.6 | PMID = 25676555 }}</ref> It was formally recognized in the WHO 2022 classification of renal neoplasia.{{fact}}<br />
<br />
It was previously known as '''TCEB1-mutated renal cell carcinoma''' and '''renal cell carcinoma with TCEB1 mutation'''. It should '''not''' be confused with [[TFEB renal cell carcinoma]], also known as ''renal tumour with t(6;11) translocation''.<br />
<br />
It is unrelated to the previously described [[renal angiomyoadenomatous tumour]]<ref name=pmid25676555/><ref name=pmid19471960>{{Cite journal | last1 = Michal | first1 = M. | last2 = Hes | first2 = O. | last3 = Kuroda | first3 = N. | last4 = Kazakov | first4 = DV. | last5 = Hora | first5 = M. | title = Difference between RAT and clear cell papillary renal cell carcinoma/clear renal cell carcinoma. | journal = Virchows Arch | volume = 454 | issue = 6 | pages = 719 | month = Jun | year = 2009 | doi = 10.1007/s00428-009-0788-9 | PMID = 19471960 }}</ref> that was lumped with [[clear cell papillary renal cell carcinoma]] in the [[Vancouver classification|Vancouver modification of the WHO classification]].<br />
<br />
==General==<br />
*Entity part of WHO Blue Book (5th Edition).<br />
*TCEB1 (transcription elongation factor B, polypeptide 1) is part of a complex that binds with the product of the [[VHL]] gene;<ref>URL: [http://www.omim.org/entry/600788 http://www.omim.org/entry/600788]. Accessed on: 12 June 2015.</ref> thus, it acts as a [[tumour suppressor]].<br />
*Good prognosis - based on limited data.<ref name=pmid25676555/><br />
<br />
==Gross==<br />
*Cystic changes.<br />
<br />
==Microscopic==<br />
Features:<br />
*Clear cells with abundant cytoplasm and basal nuclei.<br />
**Luminal nuclei (like in ''clear cell tubulopapillary RCC'') may be seen.<br />
*Usually [[ISUP nucleolar grade]] 2.<br />
*Fibromuscular bands - '''key feature'''.<br />
<br />
DDx:<br />
*[[Clear cell renal cell carcinoma]].<br />
*[[Clear cell papillary renal cell tumour]] (previously ''clear cell papillary renal cell carcinoma'') - have luminal nuclei.<br />
*[[Tuberous sclerosis-associated renal cell carcinoma]].<br />
*[[Renal cell carcinoma with fibromyomatous stroma]] has TSC/mTOR pathway mutation.<br />
<br />
===Images===<br />
====Morphology compatible====<br />
<gallery><br />
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- very low mag.jpg | RCC CCFB - very low mag.<br />
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- low mag.jpg | RCC CCFB - low mag.<br />
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- intermed mag.jpg | RCC CCFB - intermed. mag.<br />
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- high mag.jpg | RCC CCFB - high mag.<br />
Image: Renal cell carcinoma with clear cells and fibromuscular bands - alt -- high mag.jpg | RCC CCFB - high mag.<br />
Image: Renal cell carcinoma with clear cells and fibromuscular bands -- very high mag.jpg | RCC CCFB - very high mag.<br />
</gallery><br />
<br />
==IHC==<br />
Features:<ref name=pmid25676555/><br />
*CK7 +ve.<br />
*CD10 +ve.<br />
**Typically negative in [[clear cell papillary renal cell tumour]].<br />
*CK34betaE12 -ve.<br />
**Usually positive in [[clear cell papillary renal cell tumour]].<br />
<br />
Others:<br />
*[[GPNMB]] -ve.<br />
**TSC1/TSC2/MTOR mutated RCC is GPNMB +ve.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
===Comparison between some renal tumours with clear cells===<br />
{| class="wikitable sortable" <br />
!Tumour <br />
!CK7<br />
!CD10<br />
!CK34betaE12 (K903)<br />
![[GPNMB]]<br />
|-<br />
|Clear cell renal cell carcinoma <br />
| -ve (may be focal)<br />
| +ve<br />
| -ve<br />
| usu. -ve <br />
|-<br />
|Clear cell papillary renal cell tumour <br />
| +ve (diffuse)<br />
| -ve<br />
| +ve<br />
| -ve<ref name=pmid37661807/><br />
|-<br />
|ELOC-mutated renal cell carcinoma <br />
| +ve<br />
| +ve<br />
| -ve<br />
| -ve<br />
|-<br />
|[[Renal cell carcinoma with fibromyomatous stroma|Renal cell carcinoma with fibromyomatous stroma and TSC/mTOR-mutation]]<br />
| +ve<br />
| +ve<br />
| -ve<br />
| +ve<br />
|-<br />
|}<br />
<br />
==Sign out==<br />
===Molecular lacking===<br />
<pre><br />
Right Kidney, Radical Nephrectomy: <br />
- RENAL CELL CARCINOMA with clear cells, cysts and leiomyomatous stroma, see comment.<br />
-- Margin clear.<br />
-- Please see synoptic report for details.<br />
<br />
Comment:<br />
The tumour stains as follows:<br />
POSITIVE: CD10 (moderate), EMA (patchy), CAM5.2, CK7 (focal), PAX8 (diffuse).<br />
NEGATIVE: CK34betaE12, CK20.<br />
<br />
The differential diagnosis includes: (1) clear cell renal cell carcinoma, (2) RCC with a mTOR pathway mutation, and (3) ELOC-mutated RCC.<br />
<br />
</pre><br />
<br />
==See also==<br />
*[[Vancouver classification]].<br />
*[[Renal angiomyoadenomatous tumour]] (renal cell carcinoma with angioleiomyoma-like stroma).<br />
*[[Renal cell carcinoma with fibromyomatous stroma]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Genitourinary pathology]]<br />
[[Category:Kidney tumours]]</div>
Michael
https://librepathology.org/w/index.php?title=Renal_cell_carcinoma_with_fibromyomatous_stroma&diff=52861
Renal cell carcinoma with fibromyomatous stroma
2024-03-26T13:30:38Z
<p>Michael: </p>
<hr />
<div>'''Renal cell carcinoma with fibromyomatous stroma''', abbreviated '''RCC FMS''', is an evolving subtype of [[renal cell carcinoma]].<ref>{{cite journal |authors=Trpkov K, Williamson SR, Gill AJ, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Hes O |title=Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia |journal=Mod Pathol |volume=34 |issue=6 |pages=1167–1184 |date=June 2021 |pmid=33526874 |doi=10.1038/s41379-021-00737-6 |url=}}</ref><br />
<br />
''Renal cell carcinoma with leiomyomatous stroma'' redirects here.<br />
<br />
==General==<br />
*Rare tumour: 12 in >500 renal cell carcinomas with clear cells.<ref name=pmid36592877/><br />
<br />
==Microscopic==<br />
Features:<ref name=pmid36592877>{{cite journal |authors=Tjota MY, Sharma A, Wanjari P, Fitzpatrick C, Segal J, Antic T |title=TSC/MTOR mutated renal cell carcinoma with leiomyomatous stroma is a distinct entity: a comprehensive study of 12 cases |journal=Hum Pathol |volume=134 |issue= |pages=124–133 |date=April 2023 |pmid=36592877 |doi=10.1016/j.humpath.2022.12.015 |url=}}</ref><br />
*Fibromyomatous stroma (smooth muscle stroma) - '''key feature'''.<br />
*Varied architecture: nested, solid, tubular, tubulopapillary. <br />
*WHO/ISUP grade 3 or 4.<br />
*Nuclei ''not'' polarized in the cell.<br />
<br />
DDx:<br />
*[[Clear cell renal cell carcinoma]].<br />
*[[ELOC-mutated renal cell carcinoma]].<br />
*[[Clear cell papillary renal cell tumour]].<br />
<br />
==IHC==<br />
Features:<ref name=pmid36592877/><br />
*CD10 +ve.<br />
*CK7 +ve (most).<br />
*CA9 +ve.<br />
<br />
===Comparison between some renal tumours with clear cells===<br />
{| class="wikitable sortable" <br />
!Tumour <br />
!CK7<br />
!CD10<br />
!CK34betaE12 (K903)<br />
![[GPNMB]]<br />
|-<br />
|Clear cell renal cell carcinoma <br />
| -ve (may be focal)<br />
| +ve<br />
| -ve<br />
| usu. -ve <br />
|-<br />
|Clear cell papillary renal cell tumour <br />
| +ve (diffuse)<br />
| -ve<br />
| +ve<br />
| -ve<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
|-<br />
|ELOC-mutated renal cell carcinoma <br />
| +ve<br />
| +ve<br />
| -ve<br />
| -ve<br />
|-<br />
|Renal cell carcinoma with fibromyomatous stroma<br />
| +ve<br />
| +ve<br />
| -ve<br />
| +ve<br />
|-<br />
|}<br />
==Molecular==<br />
*[[Renal neoplasms with TSC/mTOR pathway mutations|TSC/mTOR pathway mutation]].<ref name=pmid36592877/><br />
<br />
==See also==<br />
*[[Renal cell carcinoma]].<br />
*[[Clear cells in the kidney]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Genitourinary pathology]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=GPNMB&diff=52860
GPNMB
2024-03-26T13:29:56Z
<p>Michael: Redirected page to Glycoprotein non-metastatic B</p>
<hr />
<div>#redirect [[Glycoprotein non-metastatic B]]<br />
<br />
[[Category:Abbreviation]]</div>
Michael
https://librepathology.org/w/index.php?title=Glycoprotein_non-metastatic_B&diff=52859
Glycoprotein non-metastatic B
2024-03-26T13:29:27Z
<p>Michael: </p>
<hr />
<div>'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an [[immunostain]].<br />
<br />
It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
==Positive==<br />
*[[Angiomyolipoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[Low-grade oncocytic tumour]] (LOT).<br />
*[[PEComa]].<br />
*[[Renal cell carcinoma with fibromyomatous stroma]].<br />
<br />
==Negative==<br />
*[[Clear cell renal cell carcinoma]].<br />
*[[Papillary renal cell carcinoma]].<br />
*[[Chromophobe renal cell carcinoma]].<br />
*[[Clear cell papillary renal cell tumour]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Immunohistochemistry]]</div>
Michael
https://librepathology.org/w/index.php?title=Glycoprotein_non-metastatic_B&diff=52858
Glycoprotein non-metastatic B
2024-03-26T13:29:20Z
<p>Michael: </p>
<hr />
<div>'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an [[immunostain]].<br />
<br />
It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
==Positive==<br />
*[[Angiomyolipoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[Low-grade oncocytic tumour]] (LOT).<br />
*[[PEComa]].<br />
*[[Renal cell carcinoma with fibromyomatous stroma]].<br />
<br />
==Negative<br />
*[[Clear cell renal cell carcinoma]].<br />
*[[Papillary renal cell carcinoma]].<br />
*[[Chromophobe renal cell carcinoma]].<br />
*[[Clear cell papillary renal cell tumour]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Immunohistochemistry]]</div>
Michael
https://librepathology.org/w/index.php?title=Glycoprotein_non-metastatic_B&diff=52857
Glycoprotein non-metastatic B
2024-03-26T13:28:19Z
<p>Michael: </p>
<hr />
<div>'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an [[immunostain]].<br />
<br />
It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
<br />
==Positive==<br />
*[[Angiomyolipoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[Low-grade oncocytic tumour]] (LOT).<br />
*[[PEComa]].<br />
*[[Renal cell carcinoma with fibromyomatous stroma]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Immunohistochemistry]]</div>
Michael
https://librepathology.org/w/index.php?title=Glycoprotein_non-metastatic_B&diff=52856
Glycoprotein non-metastatic B
2024-03-26T13:28:04Z
<p>Michael: </p>
<hr />
<div>'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an [[immunostain]].<br />
<br />
It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
<br />
==Positive==<br />
*[[Angiomyolipoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[Low-grade oncocytic tumour]] (LOT).<br />
*[[PEComa]].<br />
*[[RCC with fibromyomatous stroma]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Immunohistochemistry]]</div>
Michael
https://librepathology.org/w/index.php?title=Glycoprotein_non-metastatic_B&diff=52855
Glycoprotein non-metastatic B
2024-03-26T13:27:52Z
<p>Michael: carete</p>
<hr />
<div>'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an [[immunostain]].<br />
<br />
It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
<br />
==Positive==<br />
*[[Angiomyolipoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[Low-grade oncocytic tumour]] (LOT).<br />
*[[PEComa]].<br />
*[[RCC with FMS]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Immunohistochemistry]]</div>
Michael
https://librepathology.org/w/index.php?title=Glycoprotein_non-metastatic_B&diff=52854
Glycoprotein non-metastatic B
2024-03-26T13:26:11Z
<p>Michael: Created page with "'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an immunostain. It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37..."</p>
<hr />
<div>'''Glycoprotein non-metastatic B''', abbreviated '''GPNMB''', is an [[immunostain]].<br />
<br />
It is typically positive in TSC1/TSC2/MTOR mutated renal cell carcinomas.<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref><br />
<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Immunohistochemistry]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB-rearranged_renal_cell_carcinoma&diff=52853
TFEB-rearranged renal cell carcinoma
2024-03-26T13:19:36Z
<p>Michael: </p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = TFEB-like renal cell carcinoma -- high mag.jpg<br />
| Width =<br />
| Caption = Renal cell carcinoma with morphology and IHC profile suggestive of TFEB RCC. [[H&E stain]].<br />
| Synonyms =<br />
| Micro = solid architecture with occasional [[rosette]]-like structures; two cell populations (1) large epithelioid cells with clear to eosinophilic cytoplasm, (2) small lymphocyte-like cells<br />
| Subtypes =<br />
| LMDDx = [[renal cell carcinoma, unclassified]], [[angiomyolipoma]] (epithelioid), other [[renal tumours with eosinophilic cytoplasm]]<br />
| Stains =<br />
| IHC = TFEB +ve (nuclear staining), HMB-45 +ve, TFE3 -ve<br />
| EM =<br />
| Molecular = TFEB rearrangement, t(6;11)(p21;q12) and others<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = typically children or young adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = very rare ~ 2 dozen reported cases<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = aggressive (?)<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
| Tx = [[nephrectomy]]<br />
}}<br />
'''TFEB-rearranged renal cell carcinoma''', also '''TFEB RCC''', is a rare [[renal cell carcinoma]] recognized by the WHO classification.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><ref name=pmid24877033/> <br />
<br />
It was previously known as ''renal tumour with t(6;11) translocation'', also ''t(6;11) renal cell carcinoma''. It was first described in children.<br />
<br />
It was added to WHO classification of renal neoplasia in the [[Vancouver classification|2012 Vancouver modification]].<ref name=pmid24025519>{{Cite journal | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref><br />
<br />
This entity should '''not''' be confused with [[ELOC-mutated renal cell carcinoma]] (previously known as ''TCEB1-mutated renal cell carcinoma'').<br />
<br />
==General==<br />
*Very rare - approximately 2 dozen reported cases as of 2014.<ref name=pmid24877033/><ref name=pmid22892601>{{Cite journal | last1 = Argani | first1 = P. | last2 = Yonescu | first2 = R. | last3 = Morsberger | first3 = L. | last4 = Morris | first4 = K. | last5 = Netto | first5 = GJ. | last6 = Smith | first6 = N. | last7 = Gonzalez | first7 = N. | last8 = Illei | first8 = PB. | last9 = Ladanyi | first9 = M. | title = Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum. | journal = Am J Surg Pathol | volume = 36 | issue = 10 | pages = 1516-26 | month = Oct | year = 2012 | doi = 10.1097/PAS.0b013e3182613d8f | PMID = 22892601 }}</ref><br />
*[[Lymph node metastases]] are common.<br />
*Traditionally thought of as a pediatric tumour... but cases in adults are reported.<ref name=pmid24877033>{{Cite journal | last1 = Hora | first1 = M. | last2 = Urge | first2 = T. | last3 = Trávníček | first3 = I. | last4 = Ferda | first4 = J. | last5 = Chudáček | first5 = Z. | last6 = Vaněček | first6 = T. | last7 = Michal | first7 = M. | last8 = Petersson | first8 = F. | last9 = Kuroda | first9 = N. | title = MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)]. | journal = Springerplus | volume = 3 | issue = | pages = 245 | month = | year = 2014 | doi = 10.1186/2193-1801-3-245 | PMID = 24877033 }}</ref><ref name=pmid21884304>{{Cite journal | last1 = Ishihara | first1 = A. | last2 = Yamashita | first2 = Y. | last3 = Takamori | first3 = H. | last4 = Kuroda | first4 = N. | title = Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case. | journal = Pathol Int | volume = 61 | issue = 9 | pages = 539-45 | month = Sep | year = 2011 | doi = 10.1111/j.1440-1827.2011.02711.x | PMID = 21884304 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=pmid18998233>{{Cite journal | last1 = Hora | first1 = M. | last2 = Hes | first2 = O. | last3 = Urge | first3 = T. | last4 = Eret | first4 = V. | last5 = Klecka | first5 = J. | last6 = Michal | first6 = M. | title = A distinctive translocation carcinoma of the kidney ["rosette-like forming," t(6;11), HMB45-positive renal tumor]. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 553-7 | month = | year = 2009 | doi = 10.1007/s11255-008-9495-8 | PMID = 18998233 }}</ref><br />
*Solid with occasional [[rosette]]-like structures.<br />
*Two cell populations:<br />
*#Large epithelioid cells with clear to eosinophilic cytoplasm.<br />
*#Small lymphocyte-like cells.<br />
<br />
DDx:<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Angiomyolipoma]] (epithelioid).<br />
*[[Clear cell renal cell carcinoma]] with eosinophilic cytoplasm.<br />
*Other [[renal tumours with eosinophilic cytoplasm]].<br />
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].<br />
<br />
===Images===<br />
====www====<br />
*[http://atlasgeneticsoncology.org/Tumors/Images/t0611p21q12RCCFig.jpg TFEB RCC (atlasgeneticsoncology.org)].<ref>URL: [http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html]. Accessed on: December 29, 2014.</ref><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC156324/figure/F1/ Renal tumour with t(6,11) (nih.gov)].<ref name=pmid12719541>{{Cite journal | last1 = Davis | first1 = IJ. | last2 = Hsi | first2 = BL. | last3 = Arroyo | first3 = JD. | last4 = Vargas | first4 = SO. | last5 = Yeh | first5 = YA. | last6 = Motyckova | first6 = G. | last7 = Valencia | first7 = P. | last8 = Perez-Atayde | first8 = AR. | last9 = Argani | first9 = P. | title = Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation. | journal = Proc Natl Acad Sci U S A | volume = 100 | issue = 10 | pages = 6051-6 | month = May | year = 2003 | doi = 10.1073/pnas.0931430100 | PMID = 12719541 }}</ref><br />
====IHC and morphology compatible====<br />
<gallery><br />
Image: TFEB-like renal cell carcinoma -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma -- very high mag.jpg | TFEB-like RCC - very high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - alt -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma - rosette-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - gland-like -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma - gland-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
</gallery><br />
<br />
==IHC==<br />
*TFEB +ve (nuclear staining).<br />
*TFE3 -ve.<br />
*Melan A +ve (13 +ve of 13 cases<ref name=pmid24618616>{{Cite journal | last1 = Smith | first1 = NE. | last2 = Illei | first2 = PB. | last3 = Allaf | first3 = M. | last4 = Gonzalez | first4 = N. | last5 = Morris | first5 = K. | last6 = Hicks | first6 = J. | last7 = Demarzo | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Amin | first9 = MB. | title = t(6;11) renal cell carcinoma (RCC): expanded immunohistochemical profile emphasizing novel RCC markers and report of 10 new genetically confirmed cases. | journal = Am J Surg Pathol | volume = 38 | issue = 5 | pages = 604-14 | month = May | year = 2014 | doi = 10.1097/PAS.0000000000000203 | PMID = 24618616 }}</ref>).<br />
*HMB-45 +ve (12 +ve of 13 cases<ref name=pmid24618616/>). <br />
*PAX8 +ve/-ve (14 +ve of 23<ref name=pmid24618616/>).<br />
*CAM5.2 +ve (8 +ve of 13<ref name=pmid24618616/>).<br />
*RCC +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*CD10 +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*Cathepsin K +ve.{{fact}}<br />
*PD-L1 +ve.<ref name=pmid33208882/><br />
<br />
Note:<br />
*Melan A and HMB-45 are considered a good screen for this entity and recommended for renal tumour with eosinophilic cytoplasm.<br />
<br />
==Molecular==<br />
*t(6;11)(p21;q12) MALAT1<ref>{{OMIM|607924}}</ref>/TFEB.<ref name=Ref_WMSP281>{{Ref WMSP|281}}</ref><br />
<br />
<br />
Others translocations involving TFEB have been described:<ref name=pmid33208882>{{cite journal |authors=Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G |title=TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1 |journal=Mod Pathol |volume=34 |issue=4 |pages=842–850 |date=April 2021 |pmid=33208882 |doi=10.1038/s41379-020-00713-6 |url=}}</ref><br />
*ACTB-TFEB.<br />
*NEAT1-TFEB.<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumour with Xp11.2 translocation]].<br />
*[[Chromosomal translocations]].<br />
*[[Pediatric pathology]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB-rearranged_renal_cell_carcinoma&diff=52852
TFEB-rearranged renal cell carcinoma
2024-03-26T13:18:54Z
<p>Michael: </p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = TFEB-like renal cell carcinoma -- high mag.jpg<br />
| Width =<br />
| Caption = Renal cell carcinoma with morphology and IHC profile suggestive of TFEB RCC. [[H&E stain]].<br />
| Synonyms =<br />
| Micro = solid architecture with occasional [[rosette]]-like structures; two cell populations (1) large epithelioid cells with clear to eosinophilic cytoplasm, (2) small lymphocyte-like cells<br />
| Subtypes =<br />
| LMDDx = [[renal cell carcinoma, unclassified]], [[angiomyolipoma]] (epithelioid), other [[renal tumours with eosinophilic cytoplasm]]<br />
| Stains =<br />
| IHC = TFEB +ve (nuclear staining), HMB-45 +ve, TFE3 -ve<br />
| EM =<br />
| Molecular = TFEB rearrangement, t(6;11)(p21;q12) and others<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = typically children or young adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = very rare ~ 2 dozen reported cases<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = aggressive (?)<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
| Tx = [[nephrectomy]]<br />
}}<br />
'''TFEB-rearranged renal cell carcinoma''', also '''TFEB RCC''', is a rare [[renal cell carcinoma]] recognized by the WHO classification.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><ref name=pmid24877033/> <br />
<br />
It was previously known as ''renal tumour with t(6;11) translocation'', also ''t(6;11) renal cell carcinoma''. It was first described in children.<br />
<br />
It was added to WHO classification of renal neoplasia in the [[Vancouver classification|2012 Vancouver modification]].<ref name=pmid24025519>{{Cite journal | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref><br />
<br />
This entity should '''not''' be confused with [[TCEB1-mutated renal cell carcinoma]]..<br />
<br />
==General==<br />
*Very rare - approximately 2 dozen reported cases as of 2014.<ref name=pmid24877033/><ref name=pmid22892601>{{Cite journal | last1 = Argani | first1 = P. | last2 = Yonescu | first2 = R. | last3 = Morsberger | first3 = L. | last4 = Morris | first4 = K. | last5 = Netto | first5 = GJ. | last6 = Smith | first6 = N. | last7 = Gonzalez | first7 = N. | last8 = Illei | first8 = PB. | last9 = Ladanyi | first9 = M. | title = Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum. | journal = Am J Surg Pathol | volume = 36 | issue = 10 | pages = 1516-26 | month = Oct | year = 2012 | doi = 10.1097/PAS.0b013e3182613d8f | PMID = 22892601 }}</ref><br />
*[[Lymph node metastases]] are common.<br />
*Traditionally thought of as a pediatric tumour... but cases in adults are reported.<ref name=pmid24877033>{{Cite journal | last1 = Hora | first1 = M. | last2 = Urge | first2 = T. | last3 = Trávníček | first3 = I. | last4 = Ferda | first4 = J. | last5 = Chudáček | first5 = Z. | last6 = Vaněček | first6 = T. | last7 = Michal | first7 = M. | last8 = Petersson | first8 = F. | last9 = Kuroda | first9 = N. | title = MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)]. | journal = Springerplus | volume = 3 | issue = | pages = 245 | month = | year = 2014 | doi = 10.1186/2193-1801-3-245 | PMID = 24877033 }}</ref><ref name=pmid21884304>{{Cite journal | last1 = Ishihara | first1 = A. | last2 = Yamashita | first2 = Y. | last3 = Takamori | first3 = H. | last4 = Kuroda | first4 = N. | title = Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case. | journal = Pathol Int | volume = 61 | issue = 9 | pages = 539-45 | month = Sep | year = 2011 | doi = 10.1111/j.1440-1827.2011.02711.x | PMID = 21884304 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=pmid18998233>{{Cite journal | last1 = Hora | first1 = M. | last2 = Hes | first2 = O. | last3 = Urge | first3 = T. | last4 = Eret | first4 = V. | last5 = Klecka | first5 = J. | last6 = Michal | first6 = M. | title = A distinctive translocation carcinoma of the kidney ["rosette-like forming," t(6;11), HMB45-positive renal tumor]. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 553-7 | month = | year = 2009 | doi = 10.1007/s11255-008-9495-8 | PMID = 18998233 }}</ref><br />
*Solid with occasional [[rosette]]-like structures.<br />
*Two cell populations:<br />
*#Large epithelioid cells with clear to eosinophilic cytoplasm.<br />
*#Small lymphocyte-like cells.<br />
<br />
DDx:<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Angiomyolipoma]] (epithelioid).<br />
*[[Clear cell renal cell carcinoma]] with eosinophilic cytoplasm.<br />
*Other [[renal tumours with eosinophilic cytoplasm]].<br />
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].<br />
<br />
===Images===<br />
====www====<br />
*[http://atlasgeneticsoncology.org/Tumors/Images/t0611p21q12RCCFig.jpg TFEB RCC (atlasgeneticsoncology.org)].<ref>URL: [http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html]. Accessed on: December 29, 2014.</ref><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC156324/figure/F1/ Renal tumour with t(6,11) (nih.gov)].<ref name=pmid12719541>{{Cite journal | last1 = Davis | first1 = IJ. | last2 = Hsi | first2 = BL. | last3 = Arroyo | first3 = JD. | last4 = Vargas | first4 = SO. | last5 = Yeh | first5 = YA. | last6 = Motyckova | first6 = G. | last7 = Valencia | first7 = P. | last8 = Perez-Atayde | first8 = AR. | last9 = Argani | first9 = P. | title = Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation. | journal = Proc Natl Acad Sci U S A | volume = 100 | issue = 10 | pages = 6051-6 | month = May | year = 2003 | doi = 10.1073/pnas.0931430100 | PMID = 12719541 }}</ref><br />
====IHC and morphology compatible====<br />
<gallery><br />
Image: TFEB-like renal cell carcinoma -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma -- very high mag.jpg | TFEB-like RCC - very high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - alt -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma - rosette-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - gland-like -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma - gland-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
</gallery><br />
<br />
==IHC==<br />
*TFEB +ve (nuclear staining).<br />
*TFE3 -ve.<br />
*Melan A +ve (13 +ve of 13 cases<ref name=pmid24618616>{{Cite journal | last1 = Smith | first1 = NE. | last2 = Illei | first2 = PB. | last3 = Allaf | first3 = M. | last4 = Gonzalez | first4 = N. | last5 = Morris | first5 = K. | last6 = Hicks | first6 = J. | last7 = Demarzo | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Amin | first9 = MB. | title = t(6;11) renal cell carcinoma (RCC): expanded immunohistochemical profile emphasizing novel RCC markers and report of 10 new genetically confirmed cases. | journal = Am J Surg Pathol | volume = 38 | issue = 5 | pages = 604-14 | month = May | year = 2014 | doi = 10.1097/PAS.0000000000000203 | PMID = 24618616 }}</ref>).<br />
*HMB-45 +ve (12 +ve of 13 cases<ref name=pmid24618616/>). <br />
*PAX8 +ve/-ve (14 +ve of 23<ref name=pmid24618616/>).<br />
*CAM5.2 +ve (8 +ve of 13<ref name=pmid24618616/>).<br />
*RCC +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*CD10 +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*Cathepsin K +ve.{{fact}}<br />
*PD-L1 +ve.<ref name=pmid33208882/><br />
<br />
Note:<br />
*Melan A and HMB-45 are considered a good screen for this entity and recommended for renal tumour with eosinophilic cytoplasm.<br />
<br />
==Molecular==<br />
*t(6;11)(p21;q12) MALAT1<ref>{{OMIM|607924}}</ref>/TFEB.<ref name=Ref_WMSP281>{{Ref WMSP|281}}</ref><br />
<br />
<br />
Others translocations involving TFEB have been described:<ref name=pmid33208882>{{cite journal |authors=Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G |title=TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1 |journal=Mod Pathol |volume=34 |issue=4 |pages=842–850 |date=April 2021 |pmid=33208882 |doi=10.1038/s41379-020-00713-6 |url=}}</ref><br />
*ACTB-TFEB.<br />
*NEAT1-TFEB.<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumour with Xp11.2 translocation]].<br />
*[[Chromosomal translocations]].<br />
*[[Pediatric pathology]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB-rearranged_RCC&diff=52851
TFEB-rearranged RCC
2024-03-26T13:18:22Z
<p>Michael: redirect</p>
<hr />
<div>#redirect [[TFEB-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=TFE3-rearranged_renal_cell_carcinoma&diff=52850
TFE3-rearranged renal cell carcinoma
2024-03-26T13:17:29Z
<p>Michael: </p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg <br />
| Width =<br />
| Caption = Xp11.2 translocation carcinoma. [[H&E stain]]. <br />
| Synonyms = Xp11 translocation renal cell carcinoma, renal tumour with Xp11 translocation, [[MiT family translocation renal cell carcinoma]] (obsolete term)<br />
| Micro = large cells with clear or eosinophilic cytoplasm, calcification (classic histomorphologic feature), +/-papillae, +/-nests, +/-[[psammoma bodies]] (common), +/-[[hyaline bodies]] (common)<br />
| Subtypes =<br />
| LMDDx = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]], [[epithelioid angiomyolipoma]], [[clear cell papillary renal cell carcinoma]], [[unclassified renal cell carcinoma]], [[melanotic Xp11 translocation renal cancer]] (evolving entity)<br />
| Stains =<br />
| IHC = TFE3 +ve (nucleus), CD10 +ve, vimentin +ve, CK7 -ve (usu.)<br />
| EM =<br />
| Molecular = [[translocation]] involving TFE3, e.g. t(X;1)(p11.2;q21)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see [[kidney tumours]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = children or younger adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = rare<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = poor<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
}}<br />
'''TFE3-rearranged renal cell carcinoma''' is a rare type of [[renal cell carcinoma]] recognized by the WHO classification as a distinct entity.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><br />
<br />
It was previously known as '''renal tumour with Xp11.2 translocation''', also '''Xp11.2 translocation carcinoma''' and '''Xp11 translocation renal cell carcinoma'''. It was previously lumped with other entities in the category [[MiT family translocation renal cell carcinoma]]. <br />
<br />
It should '''not''' be confused with ''[[TFEB-rearranged RCC]]'', another [[translocation carcinoma]] found in the [[kidney]].<br />
<br />
==General==<br />
*Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.<br />
*TFE3 is the gene involved in the translocation seen in [[alveolar soft part sarcoma]] (ASPS).<br />
*Poor prognosis - majority Stage III or Stage IV.<ref name=pmid25002378>{{Cite journal | last1 = Su | first1 = HH. | last2 = Sung | first2 = MT. | last3 = Chiang | first3 = PH. | last4 = Cheng | first4 = YT. | last5 = Chen | first5 = YT. | title = The preliminary experiences of translocation renal cell carcinoma and literature review. | journal = Kaohsiung J Med Sci | volume = 30 | issue = 8 | pages = 402-8 | month = Aug | year = 2014 | doi = 10.1016/j.kjms.2014.03.003 | PMID = 25002378 }}</ref><br />
*~1/3 of childhood RCC.<ref name=pmid17667536>{{Cite journal | last1 = Argani | first1 = P. | last2 = Olgac | first2 = S. | last3 = Tickoo | first3 = SK. | last4 = Goldfischer | first4 = M. | last5 = Moch | first5 = H. | last6 = Chan | first6 = DY. | last7 = Eble | first7 = JN. | last8 = Bonsib | first8 = SM. | last9 = Jimeno | first9 = M. | title = Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. | journal = Am J Surg Pathol | volume = 31 | issue = 8 | pages = 1149-60 | month = Aug | year = 2007 | doi = 10.1097/PAS.0b013e318031ffff | PMID = 17667536 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
*Large cells.<br />
*Clear or eosinophilic cytoplasm.<br />
*Papillae or nests.<br />
*[[Psammoma bodies]] - common.<ref name=pmid17102051>{{Cite journal | last1 = Prasad | first1 = SR. | last2 = Humphrey | first2 = PA. | last3 = Catena | first3 = JR. | last4 = Narra | first4 = VR. | last5 = Srigley | first5 = JR. | last6 = Cortez | first6 = AD. | last7 = Dalrymple | first7 = NC. | last8 = Chintapalli | first8 = KN. | title = Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. | journal = Radiographics | volume = 26 | issue = 6 | pages = 1795-806; discussion 1806-10 | month = | year = | doi = 10.1148/rg.266065010 | PMID = 17102051 }}</ref><br />
**Calcification is considered the '''classic histomorphologic feature'''. <br />
*[[Hyaline bodies]] - common.<br />
<br />
Notes:<br />
*Looks clear cell RCC ''or'' papillary RCC ''or'' a hybrid between the two.<br />
*May resemble [[alveolar soft part sarcoma]].<br />
<br />
DDx:<br />
*[[Clear cell RCC]].<br />
*[[Papillary RCC]].<br />
*[[Epithelioid angiomyolipoma]].<br />
*[[Clear cell papillary renal cell carcinoma]].<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Adrenal gland]] - cortex.<br />
*[[Melanotic Xp11 translocation renal cancer]] - an evolving entity that looks like Xp11 translocation carcinoma, keratin negative.<ref name=pmid19065101>{{Cite journal | last1 = Argani | first1 = P. | last2 = Aulmann | first2 = S. | last3 = Karanjawala | first3 = Z. | last4 = Fraser | first4 = RB. | last5 = Ladanyi | first5 = M. | last6 = Rodriguez | first6 = MM. | title = Melanotic Xp11 translocation renal cancers: a distinctive neoplasm with overlapping features of PEComa, carcinoma, and melanoma. | journal = Am J Surg Pathol | volume = 33 | issue = 4 | pages = 609-19 | month = Apr | year = 2009 | doi = 10.1097/PAS.0b013e31818fbdff | PMID = 19065101 }}</ref><br />
<br />
===Images===<br />
====Case 1====<br />
<gallery><br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_intermed_mag.jpg | Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg | Xp11.2 translocation RCC - high mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_very_high_mag.jpg | Xp11.2 translocation RCC - very high mag. (WC/Nephron)<br />
</gallery><br />
====Case 2====<br />
<gallery><br />
Image: Translocation carcinoma - kidney -- low mag.jpg | TCK - low mag.<br />
Image: Translocation carcinoma - kidney -- intermed mag.jpg | TCK - intermed. mag.<br />
Image: Translocation carcinoma - kidney -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney - alt -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney -- very high mag.jpg | TCK - very high mag.<br />
</gallery><br />
====www====<br />
*[http://www.flickr.com/photos/40764007@N08/7403237732/in/photostream/ Translocation carcinoma (flickr.com)].<br />
<br />
==IHC==<br />
Key stains - as per [[ISUP]]:<ref name=pmid25025364 >{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref><br />
*TFE3 +ve (nucleus).<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref> ‡<br />
*TFEB +ve.<br />
*Cathepsin K +ve.<ref name=pmid21602817>{{cite journal |author=Martignoni G, Gobbo S, Camparo P, ''et al.'' |title=Differential expression of cathepsin K in neoplasms harboring TFE3 gene fusions |journal=Mod. Pathol. |volume=24 |issue=10 |pages=1313–9 |year=2011 |month=October |pmid=21602817 |doi=10.1038/modpathol.2011.93 |url=}}</ref><br />
<br />
Others:<br />
*CD10 +ve.<br />
*Vimentin +ve.<br />
*CK7 -ve.<br />
**Positive in ~20% of cases.<ref name=pmid21804394>{{Cite journal | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref><br />
*HMB-45 -ve.<br />
**Positive in ''epithelioid [[angiomyolipoma]]''.<br />
*Melan A -ve/+ve.<ref name=pmid22207547>{{Cite journal | last1 = Kuroda | first1 = N. | last2 = Mikami | first2 = S. | last3 = Pan | first3 = CC. | last4 = Cohen | first4 = RJ. | last5 = Hes | first5 = O. | last6 = Michal | first6 = M. | last7 = Nagashima | first7 = Y. | last8 = Tanaka | first8 = Y. | last9 = Inoue | first9 = K. | title = Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect. | journal = Histol Histopathol | volume = 27 | issue = 2 | pages = 133-40 | month = Feb | year = 2012 | doi = | PMID = 22207547 }}</ref><br />
**Should exclude adrenal if +ve.<br />
*EMA usually -ve.<ref name=pmid20679884>{{Cite journal | last1 = Argani | first1 = P. | last2 = Hicks | first2 = J. | last3 = De Marzo | first3 = AM. | last4 = Albadine | first4 = R. | last5 = Illei | first5 = PB. | last6 = Ladanyi | first6 = M. | last7 = Reuter | first7 = VE. | last8 = Netto | first8 = GJ. | title = Xp11 translocation renal cell carcinoma (RCC): extended immunohistochemical profile emphasizing novel RCC markers. | journal = Am J Surg Pathol | volume = 34 | issue = 9 | pages = 1295-303 | month = Sep | year = 2010 | doi = 10.1097/PAS.0b013e3181e8ce5b | PMID = 20679884 }}</ref><br />
*CD117 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*CK (pooled) -ve.<ref name=pmid20679884/> (???)<br />
*WT1 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*PAX8 +ve (16 +ve/21<ref name=pmid20679884/>).<br />
<br />
Notes:<br />
*Poor man's panel - if one doesn't have the more informative stains: <br />
**CK (pooled), EMA, Melan A, HMB-45, CK7, AMACR, CD10, vimentin, RCC.<br />
*‡ The IHC (in comparsion to FISH) has both false negatives and false positives; in one study by Qu ''et al.'' the false positive rate was 7% (2/30) and false negative rate was 4% (2/46).<ref>{{Cite journal | last1 = Qu | first1 = Y. | last2 = Gu | first2 = C. | last3 = Wang | first3 = H. | last4 = Chang | first4 = K. | last5 = Yang | first5 = X. | last6 = Zhou | first6 = X. | last7 = Dai | first7 = B. | last8 = Zhu | first8 = Y. | last9 = Shi | first9 = G. | title = Diagnosis of adults Xp11.2 translocation renal cell carcinoma by immunohistochemistry and FISH assays: clinicopathological data from ethnic Chinese population. | journal = Sci Rep | volume = 6 | issue = | pages = 21677 | month = | year = 2016 | doi = 10.1038/srep21677 | PMID = 26880493 }}</ref><br />
<br />
==Molecular==<br />
*[[Translocation]] involving TFE3.<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
**A very low list of partners have been described.<br />
<br />
The most common are:<ref name=pmid24309327 >{{Cite journal | last1 = Ellis | first1 = CL. | last2 = Eble | first2 = JN. | last3 = Subhawong | first3 = AP. | last4 = Martignoni | first4 = G. | last5 = Zhong | first5 = M. | last6 = Ladanyi | first6 = M. | last7 = Epstein | first7 = JI. | last8 = Netto | first8 = GJ. | last9 = Argani | first9 = P. | title = Clinical heterogeneity of Xp11 translocation renal cell carcinoma: impact of fusion subtype, age, and stage. | journal = Mod Pathol | volume = | issue = | pages = | month = Dec | year = 2013 | doi = 10.1038/modpathol.2013.208 | PMID = 24309327 }}</ref><br />
*t(x;1)(p11.2;q21) PRCC-TFE3.<br />
*t(x;17)(p11.2;q25) ASPSCR1-TFE3.<br />
**Also seen in [[alveolar soft part sarcoma]].<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Chromosomal translocation]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Diagnosis]]<br />
[[Category:Kidney tumours]]</div>
Michael
https://librepathology.org/w/index.php?title=Xp11.2_translocation_carcinoma&diff=52849
Xp11.2 translocation carcinoma
2024-03-26T13:13:31Z
<p>Michael: Changed redirect target from Renal tumour with Xp11.2 translocation to TFE3-rearranged renal cell carcinoma</p>
<hr />
<div>#redirect [[TFE3-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=Xp11_translocation_renal_cell_carcinoma&diff=52848
Xp11 translocation renal cell carcinoma
2024-03-26T13:13:13Z
<p>Michael: Changed redirect target from Renal tumour with Xp11.2 translocation to TFE3-rearranged renal cell carcinoma</p>
<hr />
<div>#redirect [[TFE3-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=TFE3-rearranged_renal_cell_carcinoma&diff=52847
TFE3-rearranged renal cell carcinoma
2024-03-26T13:12:03Z
<p>Michael: </p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg <br />
| Width =<br />
| Caption = Xp11.2 translocation carcinoma. [[H&E stain]]. <br />
| Synonyms = Xp11 translocation renal cell carcinoma, renal tumour with Xp11 translocation, [[MiT family translocation renal cell carcinoma]] (obsolete term)<br />
| Micro = large cells with clear or eosinophilic cytoplasm, calcification (classic histomorphologic feature), +/-papillae, +/-nests, +/-[[psammoma bodies]] (common), +/-[[hyaline bodies]] (common)<br />
| Subtypes =<br />
| LMDDx = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]], [[epithelioid angiomyolipoma]], [[clear cell papillary renal cell carcinoma]], [[unclassified renal cell carcinoma]], [[melanotic Xp11 translocation renal cancer]] (evolving entity)<br />
| Stains =<br />
| IHC = TFE3 +ve (nucleus), CD10 +ve, vimentin +ve, CK7 -ve (usu.)<br />
| EM =<br />
| Molecular = [[translocation]] involving TFE3, e.g. t(X;1)(p11.2;q21)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see [[kidney tumours]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = children or younger adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = rare<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = poor<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
}}<br />
'''TFE3-rearranged renal cell carcinoma''' is a rare type of [[renal cell carcinoma]] recognized by the WHO classification as a distinct entity.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><br />
<br />
It was previously known as '''renal tumour with Xp11.2 translocation''', also '''Xp11.2 translocation carcinoma''' and '''Xp11 translocation renal cell carcinoma'''. It was previously lumped with other entities in the category [[MiT family translocation renal cell carcinoma]]. <br />
<br />
It should '''not''' be confused with ''TFEB RCC'', another [[translocation carcinoma]] found in the [[kidney]].<br />
<br />
==General==<br />
*Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.<br />
*TFE3 is the gene involved in the translocation seen in [[alveolar soft part sarcoma]] (ASPS).<br />
*Poor prognosis - majority Stage III or Stage IV.<ref name=pmid25002378>{{Cite journal | last1 = Su | first1 = HH. | last2 = Sung | first2 = MT. | last3 = Chiang | first3 = PH. | last4 = Cheng | first4 = YT. | last5 = Chen | first5 = YT. | title = The preliminary experiences of translocation renal cell carcinoma and literature review. | journal = Kaohsiung J Med Sci | volume = 30 | issue = 8 | pages = 402-8 | month = Aug | year = 2014 | doi = 10.1016/j.kjms.2014.03.003 | PMID = 25002378 }}</ref><br />
*~1/3 of childhood RCC.<ref name=pmid17667536>{{Cite journal | last1 = Argani | first1 = P. | last2 = Olgac | first2 = S. | last3 = Tickoo | first3 = SK. | last4 = Goldfischer | first4 = M. | last5 = Moch | first5 = H. | last6 = Chan | first6 = DY. | last7 = Eble | first7 = JN. | last8 = Bonsib | first8 = SM. | last9 = Jimeno | first9 = M. | title = Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. | journal = Am J Surg Pathol | volume = 31 | issue = 8 | pages = 1149-60 | month = Aug | year = 2007 | doi = 10.1097/PAS.0b013e318031ffff | PMID = 17667536 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
*Large cells.<br />
*Clear or eosinophilic cytoplasm.<br />
*Papillae or nests.<br />
*[[Psammoma bodies]] - common.<ref name=pmid17102051>{{Cite journal | last1 = Prasad | first1 = SR. | last2 = Humphrey | first2 = PA. | last3 = Catena | first3 = JR. | last4 = Narra | first4 = VR. | last5 = Srigley | first5 = JR. | last6 = Cortez | first6 = AD. | last7 = Dalrymple | first7 = NC. | last8 = Chintapalli | first8 = KN. | title = Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. | journal = Radiographics | volume = 26 | issue = 6 | pages = 1795-806; discussion 1806-10 | month = | year = | doi = 10.1148/rg.266065010 | PMID = 17102051 }}</ref><br />
**Calcification is considered the '''classic histomorphologic feature'''. <br />
*[[Hyaline bodies]] - common.<br />
<br />
Notes:<br />
*Looks clear cell RCC ''or'' papillary RCC ''or'' a hybrid between the two.<br />
*May resemble [[alveolar soft part sarcoma]].<br />
<br />
DDx:<br />
*[[Clear cell RCC]].<br />
*[[Papillary RCC]].<br />
*[[Epithelioid angiomyolipoma]].<br />
*[[Clear cell papillary renal cell carcinoma]].<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Adrenal gland]] - cortex.<br />
*[[Melanotic Xp11 translocation renal cancer]] - an evolving entity that looks like Xp11 translocation carcinoma, keratin negative.<ref name=pmid19065101>{{Cite journal | last1 = Argani | first1 = P. | last2 = Aulmann | first2 = S. | last3 = Karanjawala | first3 = Z. | last4 = Fraser | first4 = RB. | last5 = Ladanyi | first5 = M. | last6 = Rodriguez | first6 = MM. | title = Melanotic Xp11 translocation renal cancers: a distinctive neoplasm with overlapping features of PEComa, carcinoma, and melanoma. | journal = Am J Surg Pathol | volume = 33 | issue = 4 | pages = 609-19 | month = Apr | year = 2009 | doi = 10.1097/PAS.0b013e31818fbdff | PMID = 19065101 }}</ref><br />
<br />
===Images===<br />
====Case 1====<br />
<gallery><br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_intermed_mag.jpg | Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg | Xp11.2 translocation RCC - high mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_very_high_mag.jpg | Xp11.2 translocation RCC - very high mag. (WC/Nephron)<br />
</gallery><br />
====Case 2====<br />
<gallery><br />
Image: Translocation carcinoma - kidney -- low mag.jpg | TCK - low mag.<br />
Image: Translocation carcinoma - kidney -- intermed mag.jpg | TCK - intermed. mag.<br />
Image: Translocation carcinoma - kidney -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney - alt -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney -- very high mag.jpg | TCK - very high mag.<br />
</gallery><br />
====www====<br />
*[http://www.flickr.com/photos/40764007@N08/7403237732/in/photostream/ Translocation carcinoma (flickr.com)].<br />
<br />
==IHC==<br />
Key stains - as per [[ISUP]]:<ref name=pmid25025364 >{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref><br />
*TFE3 +ve (nucleus).<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref> ‡<br />
*TFEB +ve.<br />
*Cathepsin K +ve.<ref name=pmid21602817>{{cite journal |author=Martignoni G, Gobbo S, Camparo P, ''et al.'' |title=Differential expression of cathepsin K in neoplasms harboring TFE3 gene fusions |journal=Mod. Pathol. |volume=24 |issue=10 |pages=1313–9 |year=2011 |month=October |pmid=21602817 |doi=10.1038/modpathol.2011.93 |url=}}</ref><br />
<br />
Others:<br />
*CD10 +ve.<br />
*Vimentin +ve.<br />
*CK7 -ve.<br />
**Positive in ~20% of cases.<ref name=pmid21804394>{{Cite journal | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref><br />
*HMB-45 -ve.<br />
**Positive in ''epithelioid [[angiomyolipoma]]''.<br />
*Melan A -ve/+ve.<ref name=pmid22207547>{{Cite journal | last1 = Kuroda | first1 = N. | last2 = Mikami | first2 = S. | last3 = Pan | first3 = CC. | last4 = Cohen | first4 = RJ. | last5 = Hes | first5 = O. | last6 = Michal | first6 = M. | last7 = Nagashima | first7 = Y. | last8 = Tanaka | first8 = Y. | last9 = Inoue | first9 = K. | title = Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect. | journal = Histol Histopathol | volume = 27 | issue = 2 | pages = 133-40 | month = Feb | year = 2012 | doi = | PMID = 22207547 }}</ref><br />
**Should exclude adrenal if +ve.<br />
*EMA usually -ve.<ref name=pmid20679884>{{Cite journal | last1 = Argani | first1 = P. | last2 = Hicks | first2 = J. | last3 = De Marzo | first3 = AM. | last4 = Albadine | first4 = R. | last5 = Illei | first5 = PB. | last6 = Ladanyi | first6 = M. | last7 = Reuter | first7 = VE. | last8 = Netto | first8 = GJ. | title = Xp11 translocation renal cell carcinoma (RCC): extended immunohistochemical profile emphasizing novel RCC markers. | journal = Am J Surg Pathol | volume = 34 | issue = 9 | pages = 1295-303 | month = Sep | year = 2010 | doi = 10.1097/PAS.0b013e3181e8ce5b | PMID = 20679884 }}</ref><br />
*CD117 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*CK (pooled) -ve.<ref name=pmid20679884/> (???)<br />
*WT1 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*PAX8 +ve (16 +ve/21<ref name=pmid20679884/>).<br />
<br />
Notes:<br />
*Poor man's panel - if one doesn't have the more informative stains: <br />
**CK (pooled), EMA, Melan A, HMB-45, CK7, AMACR, CD10, vimentin, RCC.<br />
*‡ The IHC (in comparsion to FISH) has both false negatives and false positives; in one study by Qu ''et al.'' the false positive rate was 7% (2/30) and false negative rate was 4% (2/46).<ref>{{Cite journal | last1 = Qu | first1 = Y. | last2 = Gu | first2 = C. | last3 = Wang | first3 = H. | last4 = Chang | first4 = K. | last5 = Yang | first5 = X. | last6 = Zhou | first6 = X. | last7 = Dai | first7 = B. | last8 = Zhu | first8 = Y. | last9 = Shi | first9 = G. | title = Diagnosis of adults Xp11.2 translocation renal cell carcinoma by immunohistochemistry and FISH assays: clinicopathological data from ethnic Chinese population. | journal = Sci Rep | volume = 6 | issue = | pages = 21677 | month = | year = 2016 | doi = 10.1038/srep21677 | PMID = 26880493 }}</ref><br />
<br />
==Molecular==<br />
*[[Translocation]] involving TFE3.<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
**A very low list of partners have been described.<br />
<br />
The most common are:<ref name=pmid24309327 >{{Cite journal | last1 = Ellis | first1 = CL. | last2 = Eble | first2 = JN. | last3 = Subhawong | first3 = AP. | last4 = Martignoni | first4 = G. | last5 = Zhong | first5 = M. | last6 = Ladanyi | first6 = M. | last7 = Epstein | first7 = JI. | last8 = Netto | first8 = GJ. | last9 = Argani | first9 = P. | title = Clinical heterogeneity of Xp11 translocation renal cell carcinoma: impact of fusion subtype, age, and stage. | journal = Mod Pathol | volume = | issue = | pages = | month = Dec | year = 2013 | doi = 10.1038/modpathol.2013.208 | PMID = 24309327 }}</ref><br />
*t(x;1)(p11.2;q21) PRCC-TFE3.<br />
*t(x;17)(p11.2;q25) ASPSCR1-TFE3.<br />
**Also seen in [[alveolar soft part sarcoma]].<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Chromosomal translocation]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Diagnosis]]<br />
[[Category:Kidney tumours]]</div>
Michael
https://librepathology.org/w/index.php?title=MiT_family_translocation_renal_cell_carcinoma&diff=52846
MiT family translocation renal cell carcinoma
2024-03-26T13:11:09Z
<p>Michael: </p>
<hr />
<div>'''MiT family translocation renal cell carcinoma''' is an obsolete name that grouped [[renal cell carcinoma]]s with [[chromosomal translocations]].<br />
<br />
It included what are now distinct entities in the WHO classification:<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
<br />
==See also==<br />
*[[Translocation carcinoma]].<br />
*[[MITF]].<br />
<br />
{{Disambiguation}}<br />
<br />
[[Category:Stuff]]</div>
Michael
https://librepathology.org/w/index.php?title=MiT_family_translocation_renal_cell_carcinoma&diff=52845
MiT family translocation renal cell carcinoma
2024-03-26T13:10:43Z
<p>Michael: </p>
<hr />
<div>'''MiT family translocation renal cell carcinoma''' is an obsolete name that grouped [[renal cell carcinoma]]s with [[chromosomal translocations]].<br />
<br />
It included:<br />
*[[TFEB-rearranged renal cell carcinoma]].<br />
*[[TFE3-rearranged renal cell carcinoma]].<br />
<br />
==See also==<br />
*[[Translocation carcinoma]].<br />
*[[MITF]].<br />
<br />
{{Disambiguation}}<br />
<br />
[[Category:Stuff]]</div>
Michael
https://librepathology.org/w/index.php?title=TFE3-rearranged_renal_cell_carcinoma&diff=52844
TFE3-rearranged renal cell carcinoma
2024-03-26T13:08:39Z
<p>Michael: rename</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg <br />
| Width =<br />
| Caption = Xp11.2 translocation carcinoma. [[H&E stain]]. <br />
| Synonyms = Xp11 translocation renal cell carcinoma, renal tumour with Xp11 translocation<br />
| Micro = large cells with clear or eosinophilic cytoplasm, calcification (classic histomorphologic feature), +/-papillae, +/-nests, +/-[[psammoma bodies]] (common), +/-[[hyaline bodies]] (common)<br />
| Subtypes =<br />
| LMDDx = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]], [[epithelioid angiomyolipoma]], [[clear cell papillary renal cell carcinoma]], [[unclassified renal cell carcinoma]], [[melanotic Xp11 translocation renal cancer]] (evolving entity)<br />
| Stains =<br />
| IHC = TFE3 +ve (nucleus), CD10 +ve, vimentin +ve, CK7 -ve (usu.)<br />
| EM =<br />
| Molecular = [[translocation]] involving TFE3, e.g. t(X;1)(p11.2;q21)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see [[kidney tumours]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = children or younger adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = rare<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = poor<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
}}<br />
'''TFE3-rearranged renal cell carcinoma''' is a rare type of [[renal cell carcinoma]] recognized by the WHO classification as a distinct entity.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><br />
<br />
It was previously known as '''renal tumour with Xp11.2 translocation''', also '''Xp11.2 translocation carcinoma''' and '''Xp11 translocation renal cell carcinoma'''. It was previously lumped with other entities in the category [[MiT family translocation renal cell carcinoma]]. <br />
<br />
It should '''not''' be confused with ''TFEB RCC'', another [[translocation carcinoma]] found in the [[kidney]].<br />
<br />
==General==<br />
*Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.<br />
*TFE3 is the gene involved in the translocation seen in [[alveolar soft part sarcoma]] (ASPS).<br />
*Poor prognosis - majority Stage III or Stage IV.<ref name=pmid25002378>{{Cite journal | last1 = Su | first1 = HH. | last2 = Sung | first2 = MT. | last3 = Chiang | first3 = PH. | last4 = Cheng | first4 = YT. | last5 = Chen | first5 = YT. | title = The preliminary experiences of translocation renal cell carcinoma and literature review. | journal = Kaohsiung J Med Sci | volume = 30 | issue = 8 | pages = 402-8 | month = Aug | year = 2014 | doi = 10.1016/j.kjms.2014.03.003 | PMID = 25002378 }}</ref><br />
*~1/3 of childhood RCC.<ref name=pmid17667536>{{Cite journal | last1 = Argani | first1 = P. | last2 = Olgac | first2 = S. | last3 = Tickoo | first3 = SK. | last4 = Goldfischer | first4 = M. | last5 = Moch | first5 = H. | last6 = Chan | first6 = DY. | last7 = Eble | first7 = JN. | last8 = Bonsib | first8 = SM. | last9 = Jimeno | first9 = M. | title = Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. | journal = Am J Surg Pathol | volume = 31 | issue = 8 | pages = 1149-60 | month = Aug | year = 2007 | doi = 10.1097/PAS.0b013e318031ffff | PMID = 17667536 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
*Large cells.<br />
*Clear or eosinophilic cytoplasm.<br />
*Papillae or nests.<br />
*[[Psammoma bodies]] - common.<ref name=pmid17102051>{{Cite journal | last1 = Prasad | first1 = SR. | last2 = Humphrey | first2 = PA. | last3 = Catena | first3 = JR. | last4 = Narra | first4 = VR. | last5 = Srigley | first5 = JR. | last6 = Cortez | first6 = AD. | last7 = Dalrymple | first7 = NC. | last8 = Chintapalli | first8 = KN. | title = Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. | journal = Radiographics | volume = 26 | issue = 6 | pages = 1795-806; discussion 1806-10 | month = | year = | doi = 10.1148/rg.266065010 | PMID = 17102051 }}</ref><br />
**Calcification is considered the '''classic histomorphologic feature'''. <br />
*[[Hyaline bodies]] - common.<br />
<br />
Notes:<br />
*Looks clear cell RCC ''or'' papillary RCC ''or'' a hybrid between the two.<br />
*May resemble [[alveolar soft part sarcoma]].<br />
<br />
DDx:<br />
*[[Clear cell RCC]].<br />
*[[Papillary RCC]].<br />
*[[Epithelioid angiomyolipoma]].<br />
*[[Clear cell papillary renal cell carcinoma]].<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Adrenal gland]] - cortex.<br />
*[[Melanotic Xp11 translocation renal cancer]] - an evolving entity that looks like Xp11 translocation carcinoma, keratin negative.<ref name=pmid19065101>{{Cite journal | last1 = Argani | first1 = P. | last2 = Aulmann | first2 = S. | last3 = Karanjawala | first3 = Z. | last4 = Fraser | first4 = RB. | last5 = Ladanyi | first5 = M. | last6 = Rodriguez | first6 = MM. | title = Melanotic Xp11 translocation renal cancers: a distinctive neoplasm with overlapping features of PEComa, carcinoma, and melanoma. | journal = Am J Surg Pathol | volume = 33 | issue = 4 | pages = 609-19 | month = Apr | year = 2009 | doi = 10.1097/PAS.0b013e31818fbdff | PMID = 19065101 }}</ref><br />
<br />
===Images===<br />
====Case 1====<br />
<gallery><br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_intermed_mag.jpg | Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg | Xp11.2 translocation RCC - high mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_very_high_mag.jpg | Xp11.2 translocation RCC - very high mag. (WC/Nephron)<br />
</gallery><br />
====Case 2====<br />
<gallery><br />
Image: Translocation carcinoma - kidney -- low mag.jpg | TCK - low mag.<br />
Image: Translocation carcinoma - kidney -- intermed mag.jpg | TCK - intermed. mag.<br />
Image: Translocation carcinoma - kidney -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney - alt -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney -- very high mag.jpg | TCK - very high mag.<br />
</gallery><br />
====www====<br />
*[http://www.flickr.com/photos/40764007@N08/7403237732/in/photostream/ Translocation carcinoma (flickr.com)].<br />
<br />
==IHC==<br />
Key stains - as per [[ISUP]]:<ref name=pmid25025364 >{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref><br />
*TFE3 +ve (nucleus).<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref> ‡<br />
*TFEB +ve.<br />
*Cathepsin K +ve.<ref name=pmid21602817>{{cite journal |author=Martignoni G, Gobbo S, Camparo P, ''et al.'' |title=Differential expression of cathepsin K in neoplasms harboring TFE3 gene fusions |journal=Mod. Pathol. |volume=24 |issue=10 |pages=1313–9 |year=2011 |month=October |pmid=21602817 |doi=10.1038/modpathol.2011.93 |url=}}</ref><br />
<br />
Others:<br />
*CD10 +ve.<br />
*Vimentin +ve.<br />
*CK7 -ve.<br />
**Positive in ~20% of cases.<ref name=pmid21804394>{{Cite journal | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref><br />
*HMB-45 -ve.<br />
**Positive in ''epithelioid [[angiomyolipoma]]''.<br />
*Melan A -ve/+ve.<ref name=pmid22207547>{{Cite journal | last1 = Kuroda | first1 = N. | last2 = Mikami | first2 = S. | last3 = Pan | first3 = CC. | last4 = Cohen | first4 = RJ. | last5 = Hes | first5 = O. | last6 = Michal | first6 = M. | last7 = Nagashima | first7 = Y. | last8 = Tanaka | first8 = Y. | last9 = Inoue | first9 = K. | title = Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect. | journal = Histol Histopathol | volume = 27 | issue = 2 | pages = 133-40 | month = Feb | year = 2012 | doi = | PMID = 22207547 }}</ref><br />
**Should exclude adrenal if +ve.<br />
*EMA usually -ve.<ref name=pmid20679884>{{Cite journal | last1 = Argani | first1 = P. | last2 = Hicks | first2 = J. | last3 = De Marzo | first3 = AM. | last4 = Albadine | first4 = R. | last5 = Illei | first5 = PB. | last6 = Ladanyi | first6 = M. | last7 = Reuter | first7 = VE. | last8 = Netto | first8 = GJ. | title = Xp11 translocation renal cell carcinoma (RCC): extended immunohistochemical profile emphasizing novel RCC markers. | journal = Am J Surg Pathol | volume = 34 | issue = 9 | pages = 1295-303 | month = Sep | year = 2010 | doi = 10.1097/PAS.0b013e3181e8ce5b | PMID = 20679884 }}</ref><br />
*CD117 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*CK (pooled) -ve.<ref name=pmid20679884/> (???)<br />
*WT1 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*PAX8 +ve (16 +ve/21<ref name=pmid20679884/>).<br />
<br />
Notes:<br />
*Poor man's panel - if one doesn't have the more informative stains: <br />
**CK (pooled), EMA, Melan A, HMB-45, CK7, AMACR, CD10, vimentin, RCC.<br />
*‡ The IHC (in comparsion to FISH) has both false negatives and false positives; in one study by Qu ''et al.'' the false positive rate was 7% (2/30) and false negative rate was 4% (2/46).<ref>{{Cite journal | last1 = Qu | first1 = Y. | last2 = Gu | first2 = C. | last3 = Wang | first3 = H. | last4 = Chang | first4 = K. | last5 = Yang | first5 = X. | last6 = Zhou | first6 = X. | last7 = Dai | first7 = B. | last8 = Zhu | first8 = Y. | last9 = Shi | first9 = G. | title = Diagnosis of adults Xp11.2 translocation renal cell carcinoma by immunohistochemistry and FISH assays: clinicopathological data from ethnic Chinese population. | journal = Sci Rep | volume = 6 | issue = | pages = 21677 | month = | year = 2016 | doi = 10.1038/srep21677 | PMID = 26880493 }}</ref><br />
<br />
==Molecular==<br />
*[[Translocation]] involving TFE3.<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
**A very low list of partners have been described.<br />
<br />
The most common are:<ref name=pmid24309327 >{{Cite journal | last1 = Ellis | first1 = CL. | last2 = Eble | first2 = JN. | last3 = Subhawong | first3 = AP. | last4 = Martignoni | first4 = G. | last5 = Zhong | first5 = M. | last6 = Ladanyi | first6 = M. | last7 = Epstein | first7 = JI. | last8 = Netto | first8 = GJ. | last9 = Argani | first9 = P. | title = Clinical heterogeneity of Xp11 translocation renal cell carcinoma: impact of fusion subtype, age, and stage. | journal = Mod Pathol | volume = | issue = | pages = | month = Dec | year = 2013 | doi = 10.1038/modpathol.2013.208 | PMID = 24309327 }}</ref><br />
*t(x;1)(p11.2;q21) PRCC-TFE3.<br />
*t(x;17)(p11.2;q25) ASPSCR1-TFE3.<br />
**Also seen in [[alveolar soft part sarcoma]].<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Chromosomal translocation]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Diagnosis]]<br />
[[Category:Kidney tumours]]</div>
Michael
https://librepathology.org/w/index.php?title=Talk:Renal_tumour_with_Xp11.2_translocation&diff=52843
Talk:Renal tumour with Xp11.2 translocation
2024-03-26T13:04:37Z
<p>Michael: Michael moved page Talk:Renal tumour with Xp11.2 translocation to Talk:TFE3-rearranged renal cell carcinoma: WHO renamed</p>
<hr />
<div>#REDIRECT [[Talk:TFE3-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=Talk:TFE3-rearranged_renal_cell_carcinoma&diff=52842
Talk:TFE3-rearranged renal cell carcinoma
2024-03-26T13:04:37Z
<p>Michael: Michael moved page Talk:Renal tumour with Xp11.2 translocation to Talk:TFE3-rearranged renal cell carcinoma: WHO renamed</p>
<hr />
<div>== IHC result, not clinically validated FISH ==<br />
<br />
<pre><br />
An outside TFE3 immunostain is POSITIVE in the renal cell carcinoma. <br />
<br />
This suggests the tumour is an Xp11 TRANSLOCATION (RENAL CELL) CARCINOMA.<br />
<br />
Note:<br />
TFE3 breakapart FISH testing was done at XXXX. Details are in report YYYY. <br />
This testing is not validated for clinical use; however, the findings are <br />
in keeping the TFE3 immunostaining.<br />
</pre><br />
<br />
== Negative TFE3 IHC==<br />
<br />
<pre>A TFE3 immunostain was done and is NEGATIVE in the renal cell carcinoma. The control tissue <br />
stains appropriately.<br />
<br />
Overall, the findings are in keeping with CLEAR CELL RENAL CELL CARCINOMA.<br />
</pre></div>
Michael
https://librepathology.org/w/index.php?title=Renal_tumour_with_Xp11.2_translocation&diff=52841
Renal tumour with Xp11.2 translocation
2024-03-26T13:04:37Z
<p>Michael: Michael moved page Renal tumour with Xp11.2 translocation to TFE3-rearranged renal cell carcinoma: WHO renamed</p>
<hr />
<div>#REDIRECT [[TFE3-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=TFE3-rearranged_renal_cell_carcinoma&diff=52840
TFE3-rearranged renal cell carcinoma
2024-03-26T13:04:37Z
<p>Michael: Michael moved page Renal tumour with Xp11.2 translocation to TFE3-rearranged renal cell carcinoma: WHO renamed</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg <br />
| Width =<br />
| Caption = Xp11.2 translocation carcinoma. [[H&E stain]]. <br />
| Synonyms = Xp11 translocation renal cell carcinoma, renal tumour with Xp11 translocation<br />
| Micro = large cells with clear or eosinophilic cytoplasm, calcification (classic histomorphologic feature), +/-papillae, +/-nests, +/-[[psammoma bodies]] (common), +/-[[hyaline bodies]] (common)<br />
| Subtypes =<br />
| LMDDx = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]], [[epithelioid angiomyolipoma]], [[clear cell papillary renal cell carcinoma]], [[unclassified renal cell carcinoma]], [[melanotic Xp11 translocation renal cancer]] (evolving entity)<br />
| Stains =<br />
| IHC = TFE3 +ve (nucleus), CD10 +ve, vimentin +ve, CK7 -ve (usu.)<br />
| EM =<br />
| Molecular = [[translocation]] involving TFE3, e.g. t(X;1)(p11.2;q21)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see [[kidney tumours]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = children or younger adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = rare<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = poor<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
}}<br />
'''Renal tumour with Xp11.2 translocation''', also '''Xp11.2 translocation carcinoma''' and '''Xp11 translocation renal cell carcinoma''', is an uncommon [[malignant]] [[kidney tumour]] in the [[MiT family translocation renal cell carcinoma]]. <br />
<br />
It should '''not''' be confused with ''[[renal tumour with t(6;11) translocation]]'' (also known as ''TFEB RCC''), another [[translocation carcinoma]] found in the [[kidney]] that is also in the ''MiT family translocation renal cell carcinoma''. <br />
<br />
==General==<br />
*Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.<br />
*TFE3 is the gene involved in the translocation seen in [[alveolar soft part sarcoma]] (ASPS).<br />
*Poor prognosis - majority Stage III or Stage IV.<ref name=pmid25002378>{{Cite journal | last1 = Su | first1 = HH. | last2 = Sung | first2 = MT. | last3 = Chiang | first3 = PH. | last4 = Cheng | first4 = YT. | last5 = Chen | first5 = YT. | title = The preliminary experiences of translocation renal cell carcinoma and literature review. | journal = Kaohsiung J Med Sci | volume = 30 | issue = 8 | pages = 402-8 | month = Aug | year = 2014 | doi = 10.1016/j.kjms.2014.03.003 | PMID = 25002378 }}</ref><br />
*~1/3 of childhood RCC.<ref name=pmid17667536>{{Cite journal | last1 = Argani | first1 = P. | last2 = Olgac | first2 = S. | last3 = Tickoo | first3 = SK. | last4 = Goldfischer | first4 = M. | last5 = Moch | first5 = H. | last6 = Chan | first6 = DY. | last7 = Eble | first7 = JN. | last8 = Bonsib | first8 = SM. | last9 = Jimeno | first9 = M. | title = Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. | journal = Am J Surg Pathol | volume = 31 | issue = 8 | pages = 1149-60 | month = Aug | year = 2007 | doi = 10.1097/PAS.0b013e318031ffff | PMID = 17667536 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
*Large cells.<br />
*Clear or eosinophilic cytoplasm.<br />
*Papillae or nests.<br />
*[[Psammoma bodies]] - common.<ref name=pmid17102051>{{Cite journal | last1 = Prasad | first1 = SR. | last2 = Humphrey | first2 = PA. | last3 = Catena | first3 = JR. | last4 = Narra | first4 = VR. | last5 = Srigley | first5 = JR. | last6 = Cortez | first6 = AD. | last7 = Dalrymple | first7 = NC. | last8 = Chintapalli | first8 = KN. | title = Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. | journal = Radiographics | volume = 26 | issue = 6 | pages = 1795-806; discussion 1806-10 | month = | year = | doi = 10.1148/rg.266065010 | PMID = 17102051 }}</ref><br />
**Calcification is considered the '''classic histomorphologic feature'''. <br />
*[[Hyaline bodies]] - common.<br />
<br />
Notes:<br />
*Looks clear cell RCC ''or'' papillary RCC ''or'' a hybrid between the two.<br />
*May resemble [[alveolar soft part sarcoma]].<br />
<br />
DDx:<br />
*[[Clear cell RCC]].<br />
*[[Papillary RCC]].<br />
*[[Epithelioid angiomyolipoma]].<br />
*[[Clear cell papillary renal cell carcinoma]].<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Adrenal gland]] - cortex.<br />
*[[Melanotic Xp11 translocation renal cancer]] - an evolving entity that looks like Xp11 translocation carcinoma, keratin negative.<ref name=pmid19065101>{{Cite journal | last1 = Argani | first1 = P. | last2 = Aulmann | first2 = S. | last3 = Karanjawala | first3 = Z. | last4 = Fraser | first4 = RB. | last5 = Ladanyi | first5 = M. | last6 = Rodriguez | first6 = MM. | title = Melanotic Xp11 translocation renal cancers: a distinctive neoplasm with overlapping features of PEComa, carcinoma, and melanoma. | journal = Am J Surg Pathol | volume = 33 | issue = 4 | pages = 609-19 | month = Apr | year = 2009 | doi = 10.1097/PAS.0b013e31818fbdff | PMID = 19065101 }}</ref><br />
<br />
===Images===<br />
====Case 1====<br />
<gallery><br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_intermed_mag.jpg | Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg | Xp11.2 translocation RCC - high mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_very_high_mag.jpg | Xp11.2 translocation RCC - very high mag. (WC/Nephron)<br />
</gallery><br />
====Case 2====<br />
<gallery><br />
Image: Translocation carcinoma - kidney -- low mag.jpg | TCK - low mag.<br />
Image: Translocation carcinoma - kidney -- intermed mag.jpg | TCK - intermed. mag.<br />
Image: Translocation carcinoma - kidney -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney - alt -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney -- very high mag.jpg | TCK - very high mag.<br />
</gallery><br />
====www====<br />
*[http://www.flickr.com/photos/40764007@N08/7403237732/in/photostream/ Translocation carcinoma (flickr.com)].<br />
<br />
==IHC==<br />
Key stains - as per [[ISUP]]:<ref name=pmid25025364 >{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref><br />
*TFE3 +ve (nucleus).<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref> ‡<br />
*TFEB +ve.<br />
*Cathepsin K +ve.<ref name=pmid21602817>{{cite journal |author=Martignoni G, Gobbo S, Camparo P, ''et al.'' |title=Differential expression of cathepsin K in neoplasms harboring TFE3 gene fusions |journal=Mod. Pathol. |volume=24 |issue=10 |pages=1313–9 |year=2011 |month=October |pmid=21602817 |doi=10.1038/modpathol.2011.93 |url=}}</ref><br />
<br />
Others:<br />
*CD10 +ve.<br />
*Vimentin +ve.<br />
*CK7 -ve.<br />
**Positive in ~20% of cases.<ref name=pmid21804394>{{Cite journal | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref><br />
*HMB-45 -ve.<br />
**Positive in ''epithelioid [[angiomyolipoma]]''.<br />
*Melan A -ve/+ve.<ref name=pmid22207547>{{Cite journal | last1 = Kuroda | first1 = N. | last2 = Mikami | first2 = S. | last3 = Pan | first3 = CC. | last4 = Cohen | first4 = RJ. | last5 = Hes | first5 = O. | last6 = Michal | first6 = M. | last7 = Nagashima | first7 = Y. | last8 = Tanaka | first8 = Y. | last9 = Inoue | first9 = K. | title = Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect. | journal = Histol Histopathol | volume = 27 | issue = 2 | pages = 133-40 | month = Feb | year = 2012 | doi = | PMID = 22207547 }}</ref><br />
**Should exclude adrenal if +ve.<br />
*EMA usually -ve.<ref name=pmid20679884>{{Cite journal | last1 = Argani | first1 = P. | last2 = Hicks | first2 = J. | last3 = De Marzo | first3 = AM. | last4 = Albadine | first4 = R. | last5 = Illei | first5 = PB. | last6 = Ladanyi | first6 = M. | last7 = Reuter | first7 = VE. | last8 = Netto | first8 = GJ. | title = Xp11 translocation renal cell carcinoma (RCC): extended immunohistochemical profile emphasizing novel RCC markers. | journal = Am J Surg Pathol | volume = 34 | issue = 9 | pages = 1295-303 | month = Sep | year = 2010 | doi = 10.1097/PAS.0b013e3181e8ce5b | PMID = 20679884 }}</ref><br />
*CD117 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*CK (pooled) -ve.<ref name=pmid20679884/> (???)<br />
*WT1 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*PAX8 +ve (16 +ve/21<ref name=pmid20679884/>).<br />
<br />
Notes:<br />
*Poor man's panel - if one doesn't have the more informative stains: <br />
**CK (pooled), EMA, Melan A, HMB-45, CK7, AMACR, CD10, vimentin, RCC.<br />
*‡ The IHC (in comparsion to FISH) has both false negatives and false positives; in one study by Qu ''et al.'' the false positive rate was 7% (2/30) and false negative rate was 4% (2/46).<ref>{{Cite journal | last1 = Qu | first1 = Y. | last2 = Gu | first2 = C. | last3 = Wang | first3 = H. | last4 = Chang | first4 = K. | last5 = Yang | first5 = X. | last6 = Zhou | first6 = X. | last7 = Dai | first7 = B. | last8 = Zhu | first8 = Y. | last9 = Shi | first9 = G. | title = Diagnosis of adults Xp11.2 translocation renal cell carcinoma by immunohistochemistry and FISH assays: clinicopathological data from ethnic Chinese population. | journal = Sci Rep | volume = 6 | issue = | pages = 21677 | month = | year = 2016 | doi = 10.1038/srep21677 | PMID = 26880493 }}</ref><br />
<br />
==Molecular==<br />
*[[Translocation]] involving TFE3.<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
**A very low list of partners have been described.<br />
<br />
The most common are:<ref name=pmid24309327 >{{Cite journal | last1 = Ellis | first1 = CL. | last2 = Eble | first2 = JN. | last3 = Subhawong | first3 = AP. | last4 = Martignoni | first4 = G. | last5 = Zhong | first5 = M. | last6 = Ladanyi | first6 = M. | last7 = Epstein | first7 = JI. | last8 = Netto | first8 = GJ. | last9 = Argani | first9 = P. | title = Clinical heterogeneity of Xp11 translocation renal cell carcinoma: impact of fusion subtype, age, and stage. | journal = Mod Pathol | volume = | issue = | pages = | month = Dec | year = 2013 | doi = 10.1038/modpathol.2013.208 | PMID = 24309327 }}</ref><br />
*t(x;1)(p11.2;q21) PRCC-TFE3.<br />
*t(x;17)(p11.2;q25) ASPSCR1-TFE3.<br />
**Also seen in [[alveolar soft part sarcoma]].<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Chromosomal translocation]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Diagnosis]]<br />
[[Category:Kidney tumours]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB_renal_cell_carcinoma&diff=52839
TFEB renal cell carcinoma
2024-03-26T13:02:22Z
<p>Michael: Changed redirect target from Renal tumour with t(6;11) translocation to TFEB-rearranged renal cell carcinoma</p>
<hr />
<div>#redirect [[TFEB-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB_RCC&diff=52838
TFEB RCC
2024-03-26T13:01:46Z
<p>Michael: Changed redirect target from Renal tumour with t(6;11) translocation to TFEB-rearranged renal cell carcinoma</p>
<hr />
<div>#redirect [[TFEB-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=T(6;11)_renal_cell_carcinoma&diff=52837
T(6;11) renal cell carcinoma
2024-03-26T13:01:21Z
<p>Michael: Changed redirect target from Renal tumour with t(6;11) translocation to TFEB-rearranged renal cell carcinoma</p>
<hr />
<div>#redirect [[TFEB-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB-rearranged_renal_cell_carcinoma&diff=52836
TFEB-rearranged renal cell carcinoma
2024-03-26T13:00:18Z
<p>Michael: </p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = TFEB-like renal cell carcinoma -- high mag.jpg<br />
| Width =<br />
| Caption = Renal cell carcinoma with morphology and IHC profile suggestive of TFEB RCC. [[H&E stain]].<br />
| Synonyms =<br />
| Micro = solid architecture with occasional [[rosette]]-like structures; two cell populations (1) large epithelioid cells with clear to eosinophilic cytoplasm, (2) small lymphocyte-like cells<br />
| Subtypes =<br />
| LMDDx = [[renal cell carcinoma, unclassified]], [[angiomyolipoma]] (epithelioid), other [[renal tumours with eosinophilic cytoplasm]]<br />
| Stains =<br />
| IHC = TFEB +ve (nuclear staining), HMB-45 +ve, TFE3 -ve<br />
| EM =<br />
| Molecular = TFEB rearrangement, t(6;11)(p21;q12) and others<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = typically children or young adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = very rare ~ 2 dozen reported cases<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = aggressive (?)<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
| Tx = [[nephrectomy]]<br />
}}<br />
'''TFEB-rearranged renal cell carcinoma''', also '''TFEB RCC''', is a rare [[renal cell carcinoma]] recognized by the WHO classification.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><ref name=pmid24877033/> <br />
<br />
It was previously known as '''renal tumour with t(6;11) translocation''', also '''t(6;11) renal cell carcinoma'''. It was first described in children.<br />
<br />
It was added to WHO classification of renal neoplasia in the [[Vancouver classification|2012 Vancouver modification]].<ref name=pmid24025519>{{Cite journal | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref><br />
<br />
This entity should '''not''' be confused with [[TCEB1-mutated renal cell carcinoma]]..<br />
<br />
==General==<br />
*Very rare - approximately 2 dozen reported cases as of 2014.<ref name=pmid24877033/><ref name=pmid22892601>{{Cite journal | last1 = Argani | first1 = P. | last2 = Yonescu | first2 = R. | last3 = Morsberger | first3 = L. | last4 = Morris | first4 = K. | last5 = Netto | first5 = GJ. | last6 = Smith | first6 = N. | last7 = Gonzalez | first7 = N. | last8 = Illei | first8 = PB. | last9 = Ladanyi | first9 = M. | title = Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum. | journal = Am J Surg Pathol | volume = 36 | issue = 10 | pages = 1516-26 | month = Oct | year = 2012 | doi = 10.1097/PAS.0b013e3182613d8f | PMID = 22892601 }}</ref><br />
*[[Lymph node metastases]] are common.<br />
*Traditionally thought of as a pediatric tumour... but cases in adults are reported.<ref name=pmid24877033>{{Cite journal | last1 = Hora | first1 = M. | last2 = Urge | first2 = T. | last3 = Trávníček | first3 = I. | last4 = Ferda | first4 = J. | last5 = Chudáček | first5 = Z. | last6 = Vaněček | first6 = T. | last7 = Michal | first7 = M. | last8 = Petersson | first8 = F. | last9 = Kuroda | first9 = N. | title = MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)]. | journal = Springerplus | volume = 3 | issue = | pages = 245 | month = | year = 2014 | doi = 10.1186/2193-1801-3-245 | PMID = 24877033 }}</ref><ref name=pmid21884304>{{Cite journal | last1 = Ishihara | first1 = A. | last2 = Yamashita | first2 = Y. | last3 = Takamori | first3 = H. | last4 = Kuroda | first4 = N. | title = Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case. | journal = Pathol Int | volume = 61 | issue = 9 | pages = 539-45 | month = Sep | year = 2011 | doi = 10.1111/j.1440-1827.2011.02711.x | PMID = 21884304 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=pmid18998233>{{Cite journal | last1 = Hora | first1 = M. | last2 = Hes | first2 = O. | last3 = Urge | first3 = T. | last4 = Eret | first4 = V. | last5 = Klecka | first5 = J. | last6 = Michal | first6 = M. | title = A distinctive translocation carcinoma of the kidney ["rosette-like forming," t(6;11), HMB45-positive renal tumor]. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 553-7 | month = | year = 2009 | doi = 10.1007/s11255-008-9495-8 | PMID = 18998233 }}</ref><br />
*Solid with occasional [[rosette]]-like structures.<br />
*Two cell populations:<br />
*#Large epithelioid cells with clear to eosinophilic cytoplasm.<br />
*#Small lymphocyte-like cells.<br />
<br />
DDx:<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Angiomyolipoma]] (epithelioid).<br />
*[[Clear cell renal cell carcinoma]] with eosinophilic cytoplasm.<br />
*Other [[renal tumours with eosinophilic cytoplasm]].<br />
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].<br />
<br />
===Images===<br />
====www====<br />
*[http://atlasgeneticsoncology.org/Tumors/Images/t0611p21q12RCCFig.jpg TFEB RCC (atlasgeneticsoncology.org)].<ref>URL: [http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html]. Accessed on: December 29, 2014.</ref><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC156324/figure/F1/ Renal tumour with t(6,11) (nih.gov)].<ref name=pmid12719541>{{Cite journal | last1 = Davis | first1 = IJ. | last2 = Hsi | first2 = BL. | last3 = Arroyo | first3 = JD. | last4 = Vargas | first4 = SO. | last5 = Yeh | first5 = YA. | last6 = Motyckova | first6 = G. | last7 = Valencia | first7 = P. | last8 = Perez-Atayde | first8 = AR. | last9 = Argani | first9 = P. | title = Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation. | journal = Proc Natl Acad Sci U S A | volume = 100 | issue = 10 | pages = 6051-6 | month = May | year = 2003 | doi = 10.1073/pnas.0931430100 | PMID = 12719541 }}</ref><br />
====IHC and morphology compatible====<br />
<gallery><br />
Image: TFEB-like renal cell carcinoma -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma -- very high mag.jpg | TFEB-like RCC - very high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - alt -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma - rosette-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - gland-like -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma - gland-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
</gallery><br />
<br />
==IHC==<br />
*TFEB +ve (nuclear staining).<br />
*TFE3 -ve.<br />
*Melan A +ve (13 +ve of 13 cases<ref name=pmid24618616>{{Cite journal | last1 = Smith | first1 = NE. | last2 = Illei | first2 = PB. | last3 = Allaf | first3 = M. | last4 = Gonzalez | first4 = N. | last5 = Morris | first5 = K. | last6 = Hicks | first6 = J. | last7 = Demarzo | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Amin | first9 = MB. | title = t(6;11) renal cell carcinoma (RCC): expanded immunohistochemical profile emphasizing novel RCC markers and report of 10 new genetically confirmed cases. | journal = Am J Surg Pathol | volume = 38 | issue = 5 | pages = 604-14 | month = May | year = 2014 | doi = 10.1097/PAS.0000000000000203 | PMID = 24618616 }}</ref>).<br />
*HMB-45 +ve (12 +ve of 13 cases<ref name=pmid24618616/>). <br />
*PAX8 +ve/-ve (14 +ve of 23<ref name=pmid24618616/>).<br />
*CAM5.2 +ve (8 +ve of 13<ref name=pmid24618616/>).<br />
*RCC +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*CD10 +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*Cathepsin K +ve.{{fact}}<br />
*PD-L1 +ve.<ref name=pmid33208882/><br />
<br />
Note:<br />
*Melan A and HMB-45 are considered a good screen for this entity and recommended for renal tumour with eosinophilic cytoplasm.<br />
<br />
==Molecular==<br />
*t(6;11)(p21;q12) MALAT1<ref>{{OMIM|607924}}</ref>/TFEB.<ref name=Ref_WMSP281>{{Ref WMSP|281}}</ref><br />
<br />
<br />
Others translocations involving TFEB have been described:<ref name=pmid33208882>{{cite journal |authors=Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G |title=TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1 |journal=Mod Pathol |volume=34 |issue=4 |pages=842–850 |date=April 2021 |pmid=33208882 |doi=10.1038/s41379-020-00713-6 |url=}}</ref><br />
*ACTB-TFEB.<br />
*NEAT1-TFEB.<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumour with Xp11.2 translocation]].<br />
*[[Chromosomal translocations]].<br />
*[[Pediatric pathology]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB-rearranged_renal_cell_carcinoma&diff=52835
TFEB-rearranged renal cell carcinoma
2024-03-26T12:59:30Z
<p>Michael: </p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = TFEB-like renal cell carcinoma -- high mag.jpg<br />
| Width =<br />
| Caption = Renal cell carcinoma with morphology and IHC profile suggestive of TFEB RCC. [[H&E stain]].<br />
| Synonyms =<br />
| Micro = solid architecture with occasional [[rosette]]-like structures; two cell populations (1) large epithelioid cells with clear to eosinophilic cytoplasm, (2) small lymphocyte-like cells<br />
| Subtypes =<br />
| LMDDx = [[renal cell carcinoma, unclassified]], [[angiomyolipoma]] (epithelioid), other [[renal tumours with eosinophilic cytoplasm]]<br />
| Stains =<br />
| IHC = TFEB +ve (nuclear staining), HMB-45 +ve, TFE3 -ve<br />
| EM =<br />
| Molecular = t(6;11)(p21;q12)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = typically children or young adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = very rare ~ 2 dozen reported cases<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = aggressive (?)<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
| Tx = [[nephrectomy]]<br />
}}<br />
'''TFEB-rearranged renal cell carcinoma''', also '''TFEB RCC''', is a rare [[renal cell carcinoma]] recognized by the WHO classification.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><ref name=pmid24877033/> <br />
<br />
It was previously known as '''renal tumour with t(6;11) translocation''', also '''t(6;11) renal cell carcinoma'''. It was first described in children.<br />
<br />
It was added to WHO classification of renal neoplasia in the [[Vancouver classification|2012 Vancouver modification]].<ref name=pmid24025519>{{Cite journal | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref><br />
<br />
This entity should '''not''' be confused with [[TCEB1-mutated renal cell carcinoma]]..<br />
<br />
==General==<br />
*Very rare - approximately 2 dozen reported cases as of 2014.<ref name=pmid24877033/><ref name=pmid22892601>{{Cite journal | last1 = Argani | first1 = P. | last2 = Yonescu | first2 = R. | last3 = Morsberger | first3 = L. | last4 = Morris | first4 = K. | last5 = Netto | first5 = GJ. | last6 = Smith | first6 = N. | last7 = Gonzalez | first7 = N. | last8 = Illei | first8 = PB. | last9 = Ladanyi | first9 = M. | title = Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum. | journal = Am J Surg Pathol | volume = 36 | issue = 10 | pages = 1516-26 | month = Oct | year = 2012 | doi = 10.1097/PAS.0b013e3182613d8f | PMID = 22892601 }}</ref><br />
*[[Lymph node metastases]] are common.<br />
*Traditionally thought of as a pediatric tumour... but cases in adults are reported.<ref name=pmid24877033>{{Cite journal | last1 = Hora | first1 = M. | last2 = Urge | first2 = T. | last3 = Trávníček | first3 = I. | last4 = Ferda | first4 = J. | last5 = Chudáček | first5 = Z. | last6 = Vaněček | first6 = T. | last7 = Michal | first7 = M. | last8 = Petersson | first8 = F. | last9 = Kuroda | first9 = N. | title = MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)]. | journal = Springerplus | volume = 3 | issue = | pages = 245 | month = | year = 2014 | doi = 10.1186/2193-1801-3-245 | PMID = 24877033 }}</ref><ref name=pmid21884304>{{Cite journal | last1 = Ishihara | first1 = A. | last2 = Yamashita | first2 = Y. | last3 = Takamori | first3 = H. | last4 = Kuroda | first4 = N. | title = Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case. | journal = Pathol Int | volume = 61 | issue = 9 | pages = 539-45 | month = Sep | year = 2011 | doi = 10.1111/j.1440-1827.2011.02711.x | PMID = 21884304 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=pmid18998233>{{Cite journal | last1 = Hora | first1 = M. | last2 = Hes | first2 = O. | last3 = Urge | first3 = T. | last4 = Eret | first4 = V. | last5 = Klecka | first5 = J. | last6 = Michal | first6 = M. | title = A distinctive translocation carcinoma of the kidney ["rosette-like forming," t(6;11), HMB45-positive renal tumor]. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 553-7 | month = | year = 2009 | doi = 10.1007/s11255-008-9495-8 | PMID = 18998233 }}</ref><br />
*Solid with occasional [[rosette]]-like structures.<br />
*Two cell populations:<br />
*#Large epithelioid cells with clear to eosinophilic cytoplasm.<br />
*#Small lymphocyte-like cells.<br />
<br />
DDx:<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Angiomyolipoma]] (epithelioid).<br />
*[[Clear cell renal cell carcinoma]] with eosinophilic cytoplasm.<br />
*Other [[renal tumours with eosinophilic cytoplasm]].<br />
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].<br />
<br />
===Images===<br />
====www====<br />
*[http://atlasgeneticsoncology.org/Tumors/Images/t0611p21q12RCCFig.jpg TFEB RCC (atlasgeneticsoncology.org)].<ref>URL: [http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html]. Accessed on: December 29, 2014.</ref><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC156324/figure/F1/ Renal tumour with t(6,11) (nih.gov)].<ref name=pmid12719541>{{Cite journal | last1 = Davis | first1 = IJ. | last2 = Hsi | first2 = BL. | last3 = Arroyo | first3 = JD. | last4 = Vargas | first4 = SO. | last5 = Yeh | first5 = YA. | last6 = Motyckova | first6 = G. | last7 = Valencia | first7 = P. | last8 = Perez-Atayde | first8 = AR. | last9 = Argani | first9 = P. | title = Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation. | journal = Proc Natl Acad Sci U S A | volume = 100 | issue = 10 | pages = 6051-6 | month = May | year = 2003 | doi = 10.1073/pnas.0931430100 | PMID = 12719541 }}</ref><br />
====IHC and morphology compatible====<br />
<gallery><br />
Image: TFEB-like renal cell carcinoma -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma -- very high mag.jpg | TFEB-like RCC - very high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - alt -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma - rosette-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - gland-like -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma - gland-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
</gallery><br />
<br />
==IHC==<br />
*TFEB +ve (nuclear staining).<br />
*TFE3 -ve.<br />
*Melan A +ve (13 +ve of 13 cases<ref name=pmid24618616>{{Cite journal | last1 = Smith | first1 = NE. | last2 = Illei | first2 = PB. | last3 = Allaf | first3 = M. | last4 = Gonzalez | first4 = N. | last5 = Morris | first5 = K. | last6 = Hicks | first6 = J. | last7 = Demarzo | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Amin | first9 = MB. | title = t(6;11) renal cell carcinoma (RCC): expanded immunohistochemical profile emphasizing novel RCC markers and report of 10 new genetically confirmed cases. | journal = Am J Surg Pathol | volume = 38 | issue = 5 | pages = 604-14 | month = May | year = 2014 | doi = 10.1097/PAS.0000000000000203 | PMID = 24618616 }}</ref>).<br />
*HMB-45 +ve (12 +ve of 13 cases<ref name=pmid24618616/>). <br />
*PAX8 +ve/-ve (14 +ve of 23<ref name=pmid24618616/>).<br />
*CAM5.2 +ve (8 +ve of 13<ref name=pmid24618616/>).<br />
*RCC +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*CD10 +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*Cathepsin K +ve.{{fact}}<br />
*PD-L1 +ve.<ref name=pmid33208882/><br />
<br />
Note:<br />
*Melan A and HMB-45 are considered a good screen for this entity and recommended for renal tumour with eosinophilic cytoplasm.<br />
<br />
==Molecular==<br />
*t(6;11)(p21;q12) MALAT1<ref>{{OMIM|607924}}</ref>/TFEB.<ref name=Ref_WMSP281>{{Ref WMSP|281}}</ref><br />
<br />
<br />
Others translocations involving TFEB have been described:<ref name=pmid33208882>{{cite journal |authors=Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G |title=TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1 |journal=Mod Pathol |volume=34 |issue=4 |pages=842–850 |date=April 2021 |pmid=33208882 |doi=10.1038/s41379-020-00713-6 |url=}}</ref><br />
*ACTB-TFEB.<br />
*NEAT1-TFEB.<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumour with Xp11.2 translocation]].<br />
*[[Chromosomal translocations]].<br />
*[[Pediatric pathology]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB-rearranged_renal_cell_carcinoma&diff=52834
TFEB-rearranged renal cell carcinoma
2024-03-26T12:58:12Z
<p>Michael: rename</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = TFEB-like renal cell carcinoma -- high mag.jpg<br />
| Width =<br />
| Caption = Renal cell carcinoma with morphology and IHC profile suggestive of TFEB RCC. [[H&E stain]].<br />
| Synonyms =<br />
| Micro = solid architecture with occasional [[rosette]]-like structures; two cell populations (1) large epithelioid cells with clear to eosinophilic cytoplasm, (2) small lymphocyte-like cells<br />
| Subtypes =<br />
| LMDDx = [[renal cell carcinoma, unclassified]], [[angiomyolipoma]] (epithelioid), other [[renal tumours with eosinophilic cytoplasm]]<br />
| Stains =<br />
| IHC = TFEB +ve (nuclear staining), HMB-45 +ve, TFE3 -ve<br />
| EM =<br />
| Molecular = t(6;11)(p21;q12)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = typically children or young adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = very rare ~ 2 dozen reported cases<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = aggressive (?)<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
| Tx = [[nephrectomy]]<br />
}}<br />
'''TFEB-rearranged renal cell carcinoma''', also '''TFEB RCC''', is a rare [[renal cell carcinoma]] recognized by the WHO classification.<ref name=pmid36645398>{{cite journal |authors=Alaghehbandan R, Siadat F, Trpkov K |title=What's new in the WHO 2022 classification of kidney tumours? |journal=Pathologica |volume=115 |issue=1 |pages=8–22 |date=February 2022 |pmid=36645398 |pmc=10342217 |doi=10.32074/1591-951X-818 |url=}}</ref><ref name=pmid24877033/> <br />
<br />
It was previously known as '''renal tumour with t(6;11) translocation''', also '''t(6;11) renal cell carcinoma'''. It was first described in children.<br />
<br />
It was added to WHO classification of renal neoplasia in the [[Vancouver classification|2012 Vancouver modification]].<ref name=pmid24025519>{{Cite journal | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref><br />
<br />
This entity should '''not''' be confused with [[TCEB1-mutated renal cell carcinoma]], a subset of [[clear cell carcinoma]] that is ''not'' an entity in the WHO classification of renal neoplasia.<br />
<br />
==General==<br />
*Very rare - approximately 2 dozen reported cases as of 2014.<ref name=pmid24877033/><ref name=pmid22892601>{{Cite journal | last1 = Argani | first1 = P. | last2 = Yonescu | first2 = R. | last3 = Morsberger | first3 = L. | last4 = Morris | first4 = K. | last5 = Netto | first5 = GJ. | last6 = Smith | first6 = N. | last7 = Gonzalez | first7 = N. | last8 = Illei | first8 = PB. | last9 = Ladanyi | first9 = M. | title = Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum. | journal = Am J Surg Pathol | volume = 36 | issue = 10 | pages = 1516-26 | month = Oct | year = 2012 | doi = 10.1097/PAS.0b013e3182613d8f | PMID = 22892601 }}</ref><br />
*[[Lymph node metastases]] are common.<br />
*Traditionally thought of as a pediatric tumour... but cases in adults are reported.<ref name=pmid24877033>{{Cite journal | last1 = Hora | first1 = M. | last2 = Urge | first2 = T. | last3 = Trávníček | first3 = I. | last4 = Ferda | first4 = J. | last5 = Chudáček | first5 = Z. | last6 = Vaněček | first6 = T. | last7 = Michal | first7 = M. | last8 = Petersson | first8 = F. | last9 = Kuroda | first9 = N. | title = MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)]. | journal = Springerplus | volume = 3 | issue = | pages = 245 | month = | year = 2014 | doi = 10.1186/2193-1801-3-245 | PMID = 24877033 }}</ref><ref name=pmid21884304>{{Cite journal | last1 = Ishihara | first1 = A. | last2 = Yamashita | first2 = Y. | last3 = Takamori | first3 = H. | last4 = Kuroda | first4 = N. | title = Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case. | journal = Pathol Int | volume = 61 | issue = 9 | pages = 539-45 | month = Sep | year = 2011 | doi = 10.1111/j.1440-1827.2011.02711.x | PMID = 21884304 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=pmid18998233>{{Cite journal | last1 = Hora | first1 = M. | last2 = Hes | first2 = O. | last3 = Urge | first3 = T. | last4 = Eret | first4 = V. | last5 = Klecka | first5 = J. | last6 = Michal | first6 = M. | title = A distinctive translocation carcinoma of the kidney ["rosette-like forming," t(6;11), HMB45-positive renal tumor]. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 553-7 | month = | year = 2009 | doi = 10.1007/s11255-008-9495-8 | PMID = 18998233 }}</ref><br />
*Solid with occasional [[rosette]]-like structures.<br />
*Two cell populations:<br />
*#Large epithelioid cells with clear to eosinophilic cytoplasm.<br />
*#Small lymphocyte-like cells.<br />
<br />
DDx:<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Angiomyolipoma]] (epithelioid).<br />
*[[Clear cell renal cell carcinoma]] with eosinophilic cytoplasm.<br />
*Other [[renal tumours with eosinophilic cytoplasm]].<br />
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].<br />
<br />
===Images===<br />
====www====<br />
*[http://atlasgeneticsoncology.org/Tumors/Images/t0611p21q12RCCFig.jpg TFEB RCC (atlasgeneticsoncology.org)].<ref>URL: [http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html]. Accessed on: December 29, 2014.</ref><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC156324/figure/F1/ Renal tumour with t(6,11) (nih.gov)].<ref name=pmid12719541>{{Cite journal | last1 = Davis | first1 = IJ. | last2 = Hsi | first2 = BL. | last3 = Arroyo | first3 = JD. | last4 = Vargas | first4 = SO. | last5 = Yeh | first5 = YA. | last6 = Motyckova | first6 = G. | last7 = Valencia | first7 = P. | last8 = Perez-Atayde | first8 = AR. | last9 = Argani | first9 = P. | title = Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation. | journal = Proc Natl Acad Sci U S A | volume = 100 | issue = 10 | pages = 6051-6 | month = May | year = 2003 | doi = 10.1073/pnas.0931430100 | PMID = 12719541 }}</ref><br />
====IHC and morphology compatible====<br />
<gallery><br />
Image: TFEB-like renal cell carcinoma -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma -- very high mag.jpg | TFEB-like RCC - very high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - alt -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma - rosette-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - gland-like -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma - gland-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
</gallery><br />
<br />
==IHC==<br />
*TFEB +ve (nuclear staining).<br />
*TFE3 -ve.<br />
*Melan A +ve (13 +ve of 13 cases<ref name=pmid24618616>{{Cite journal | last1 = Smith | first1 = NE. | last2 = Illei | first2 = PB. | last3 = Allaf | first3 = M. | last4 = Gonzalez | first4 = N. | last5 = Morris | first5 = K. | last6 = Hicks | first6 = J. | last7 = Demarzo | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Amin | first9 = MB. | title = t(6;11) renal cell carcinoma (RCC): expanded immunohistochemical profile emphasizing novel RCC markers and report of 10 new genetically confirmed cases. | journal = Am J Surg Pathol | volume = 38 | issue = 5 | pages = 604-14 | month = May | year = 2014 | doi = 10.1097/PAS.0000000000000203 | PMID = 24618616 }}</ref>).<br />
*HMB-45 +ve (12 +ve of 13 cases<ref name=pmid24618616/>). <br />
*PAX8 +ve/-ve (14 +ve of 23<ref name=pmid24618616/>).<br />
*CAM5.2 +ve (8 +ve of 13<ref name=pmid24618616/>).<br />
*RCC +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*CD10 +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*Cathepsin K +ve.{{fact}}<br />
*PD-L1 +ve.<ref name=pmid33208882/><br />
<br />
Note:<br />
*Melan A and HMB-45 are considered a good screen for this entity and recommended for renal tumour with eosinophilic cytoplasm.<br />
<br />
==Molecular==<br />
*t(6;11)(p21;q12) MALAT1<ref>{{OMIM|607924}}</ref>/TFEB.<ref name=Ref_WMSP281>{{Ref WMSP|281}}</ref><br />
<br />
<br />
Others translocations involving TFEB have been described:<ref name=pmid33208882>{{cite journal |authors=Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G |title=TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1 |journal=Mod Pathol |volume=34 |issue=4 |pages=842–850 |date=April 2021 |pmid=33208882 |doi=10.1038/s41379-020-00713-6 |url=}}</ref><br />
*ACTB-TFEB.<br />
*NEAT1-TFEB.<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumour with Xp11.2 translocation]].<br />
*[[Chromosomal translocations]].<br />
*[[Pediatric pathology]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Renal_tumour_with_t(6;11)_translocation&diff=52833
Renal tumour with t(6;11) translocation
2024-03-26T12:54:59Z
<p>Michael: Michael moved page Renal tumour with t(6;11) translocation to TFEB-rearranged renal cell carcinoma: renamed by WHO... not only t(6;11)</p>
<hr />
<div>#REDIRECT [[TFEB-rearranged renal cell carcinoma]]</div>
Michael
https://librepathology.org/w/index.php?title=TFEB-rearranged_renal_cell_carcinoma&diff=52832
TFEB-rearranged renal cell carcinoma
2024-03-26T12:54:59Z
<p>Michael: Michael moved page Renal tumour with t(6;11) translocation to TFEB-rearranged renal cell carcinoma: renamed by WHO... not only t(6;11)</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = TFEB-like renal cell carcinoma -- high mag.jpg<br />
| Width =<br />
| Caption = Renal cell carcinoma with morphology and IHC profile suggestive of TFEB RCC. [[H&E stain]].<br />
| Synonyms =<br />
| Micro = solid architecture with occasional [[rosette]]-like structures; two cell populations (1) large epithelioid cells with clear to eosinophilic cytoplasm, (2) small lymphocyte-like cells<br />
| Subtypes =<br />
| LMDDx = [[renal cell carcinoma, unclassified]], [[angiomyolipoma]] (epithelioid), other [[renal tumours with eosinophilic cytoplasm]]<br />
| Stains =<br />
| IHC = TFEB +ve (nuclear staining), HMB-45 +ve, TFE3 -ve<br />
| EM =<br />
| Molecular = t(6;11)(p21;q12)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see ''[[kidney tumours]]''<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = typically children or young adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = very rare ~ 2 dozen reported cases<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = aggressive (?)<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
| Tx = [[nephrectomy]]<br />
}}<br />
'''Renal tumour with t(6;11) translocation''', also '''t(6;11) renal cell carcinoma''', is a rare [[kidney tumour]] seen primarily in children.<br />
<br />
It also known as '''TFEB RCC'''.<ref name=pmid24877033/> <br />
<br />
It was added to WHO classification of renal neoplasia in the [[Vancouver classification|2012 Vancouver modification]].<ref name=pmid24025519>{{Cite journal | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref><br />
<br />
This entity should '''not''' be confused with [[TCEB1-mutated renal cell carcinoma]], a subset of [[clear cell carcinoma]] that is ''not'' an entity in the WHO classification of renal neoplasia.<br />
<br />
==General==<br />
*Very rare - approximately 2 dozen reported cases as of 2014.<ref name=pmid24877033/><ref name=pmid22892601>{{Cite journal | last1 = Argani | first1 = P. | last2 = Yonescu | first2 = R. | last3 = Morsberger | first3 = L. | last4 = Morris | first4 = K. | last5 = Netto | first5 = GJ. | last6 = Smith | first6 = N. | last7 = Gonzalez | first7 = N. | last8 = Illei | first8 = PB. | last9 = Ladanyi | first9 = M. | title = Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum. | journal = Am J Surg Pathol | volume = 36 | issue = 10 | pages = 1516-26 | month = Oct | year = 2012 | doi = 10.1097/PAS.0b013e3182613d8f | PMID = 22892601 }}</ref><br />
*[[Lymph node metastases]] are common.<br />
*Traditionally thought of as a pediatric tumour... but cases in adults are reported.<ref name=pmid24877033>{{Cite journal | last1 = Hora | first1 = M. | last2 = Urge | first2 = T. | last3 = Trávníček | first3 = I. | last4 = Ferda | first4 = J. | last5 = Chudáček | first5 = Z. | last6 = Vaněček | first6 = T. | last7 = Michal | first7 = M. | last8 = Petersson | first8 = F. | last9 = Kuroda | first9 = N. | title = MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)]. | journal = Springerplus | volume = 3 | issue = | pages = 245 | month = | year = 2014 | doi = 10.1186/2193-1801-3-245 | PMID = 24877033 }}</ref><ref name=pmid21884304>{{Cite journal | last1 = Ishihara | first1 = A. | last2 = Yamashita | first2 = Y. | last3 = Takamori | first3 = H. | last4 = Kuroda | first4 = N. | title = Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case. | journal = Pathol Int | volume = 61 | issue = 9 | pages = 539-45 | month = Sep | year = 2011 | doi = 10.1111/j.1440-1827.2011.02711.x | PMID = 21884304 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=pmid18998233>{{Cite journal | last1 = Hora | first1 = M. | last2 = Hes | first2 = O. | last3 = Urge | first3 = T. | last4 = Eret | first4 = V. | last5 = Klecka | first5 = J. | last6 = Michal | first6 = M. | title = A distinctive translocation carcinoma of the kidney ["rosette-like forming," t(6;11), HMB45-positive renal tumor]. | journal = Int Urol Nephrol | volume = 41 | issue = 3 | pages = 553-7 | month = | year = 2009 | doi = 10.1007/s11255-008-9495-8 | PMID = 18998233 }}</ref><br />
*Solid with occasional [[rosette]]-like structures.<br />
*Two cell populations:<br />
*#Large epithelioid cells with clear to eosinophilic cytoplasm.<br />
*#Small lymphocyte-like cells.<br />
<br />
DDx:<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Angiomyolipoma]] (epithelioid).<br />
*[[Clear cell renal cell carcinoma]] with eosinophilic cytoplasm.<br />
*Other [[renal tumours with eosinophilic cytoplasm]].<br />
*[[Biphasic hyalinizing psammomatous renal cell carcinoma]].<br />
<br />
===Images===<br />
====www====<br />
*[http://atlasgeneticsoncology.org/Tumors/Images/t0611p21q12RCCFig.jpg TFEB RCC (atlasgeneticsoncology.org)].<ref>URL: [http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html]. Accessed on: December 29, 2014.</ref><br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC156324/figure/F1/ Renal tumour with t(6,11) (nih.gov)].<ref name=pmid12719541>{{Cite journal | last1 = Davis | first1 = IJ. | last2 = Hsi | first2 = BL. | last3 = Arroyo | first3 = JD. | last4 = Vargas | first4 = SO. | last5 = Yeh | first5 = YA. | last6 = Motyckova | first6 = G. | last7 = Valencia | first7 = P. | last8 = Perez-Atayde | first8 = AR. | last9 = Argani | first9 = P. | title = Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation. | journal = Proc Natl Acad Sci U S A | volume = 100 | issue = 10 | pages = 6051-6 | month = May | year = 2003 | doi = 10.1073/pnas.0931430100 | PMID = 12719541 }}</ref><br />
====IHC and morphology compatible====<br />
<gallery><br />
Image: TFEB-like renal cell carcinoma -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma -- very high mag.jpg | TFEB-like RCC - very high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - alt -- high mag.jpg | TFEB-like RCC - high mag.<br />
Image: TFEB-like renal cell carcinoma - rosette-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
<br />
Image: TFEB-like renal cell carcinoma - gland-like -- intermed mag.jpg | TFEB-like RCC - intermed. mag.<br />
Image: TFEB-like renal cell carcinoma - gland-like -- high mag.jpg | TFEB-like RCC - high mag.<br />
</gallery><br />
<br />
==IHC==<br />
*TFEB +ve (nuclear staining).<br />
*TFE3 -ve.<br />
*Melan A +ve (13 +ve of 13 cases<ref name=pmid24618616>{{Cite journal | last1 = Smith | first1 = NE. | last2 = Illei | first2 = PB. | last3 = Allaf | first3 = M. | last4 = Gonzalez | first4 = N. | last5 = Morris | first5 = K. | last6 = Hicks | first6 = J. | last7 = Demarzo | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Amin | first9 = MB. | title = t(6;11) renal cell carcinoma (RCC): expanded immunohistochemical profile emphasizing novel RCC markers and report of 10 new genetically confirmed cases. | journal = Am J Surg Pathol | volume = 38 | issue = 5 | pages = 604-14 | month = May | year = 2014 | doi = 10.1097/PAS.0000000000000203 | PMID = 24618616 }}</ref>).<br />
*HMB-45 +ve (12 +ve of 13 cases<ref name=pmid24618616/>). <br />
*PAX8 +ve/-ve (14 +ve of 23<ref name=pmid24618616/>).<br />
*CAM5.2 +ve (8 +ve of 13<ref name=pmid24618616/>).<br />
*RCC +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*CD10 +ve (10 +ve of 14<ref name=pmid24618616/>).<br />
*Cathepsin K +ve.{{fact}}<br />
*PD-L1 +ve.<ref name=pmid33208882/><br />
<br />
Note:<br />
*Melan A and HMB-45 are considered a good screen for this entity and recommended for renal tumour with eosinophilic cytoplasm.<br />
<br />
==Molecular==<br />
*t(6;11)(p21;q12) MALAT1<ref>{{OMIM|607924}}</ref>/TFEB.<ref name=Ref_WMSP281>{{Ref WMSP|281}}</ref><br />
<br />
<br />
Others translocations involving TFEB have been described:<ref name=pmid33208882>{{cite journal |authors=Caliò A, Harada S, Brunelli M, Pedron S, Segala D, Portillo SC, Magi-Galluzzi C, Netto GJ, Mackinnon AC, Martignoni G |title=TFEB rearranged renal cell carcinoma. A clinicopathologic and molecular study of 13 cases. Tumors harboring MALAT1-TFEB, ACTB-TFEB, and the novel NEAT1-TFEB translocations constantly express PDL1 |journal=Mod Pathol |volume=34 |issue=4 |pages=842–850 |date=April 2021 |pmid=33208882 |doi=10.1038/s41379-020-00713-6 |url=}}</ref><br />
*ACTB-TFEB.<br />
*NEAT1-TFEB.<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Renal tumour with Xp11.2 translocation]].<br />
*[[Chromosomal translocations]].<br />
*[[Pediatric pathology]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Kidney tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=TFE3-rearranged_renal_cell_carcinoma&diff=52831
TFE3-rearranged renal cell carcinoma
2024-03-26T12:50:09Z
<p>Michael: /* Molecular */</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg <br />
| Width =<br />
| Caption = Xp11.2 translocation carcinoma. [[H&E stain]]. <br />
| Synonyms = Xp11 translocation renal cell carcinoma, renal tumour with Xp11 translocation<br />
| Micro = large cells with clear or eosinophilic cytoplasm, calcification (classic histomorphologic feature), +/-papillae, +/-nests, +/-[[psammoma bodies]] (common), +/-[[hyaline bodies]] (common)<br />
| Subtypes =<br />
| LMDDx = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]], [[epithelioid angiomyolipoma]], [[clear cell papillary renal cell carcinoma]], [[unclassified renal cell carcinoma]], [[melanotic Xp11 translocation renal cancer]] (evolving entity)<br />
| Stains =<br />
| IHC = TFE3 +ve (nucleus), CD10 +ve, vimentin +ve, CK7 -ve (usu.)<br />
| EM =<br />
| Molecular = [[translocation]] involving TFE3, e.g. t(X;1)(p11.2;q21)<br />
| IF =<br />
| Gross =<br />
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]<br />
| Staging = [[kidney cancer staging]]<br />
| Site = [[kidney]] - see [[kidney tumours]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = children or younger adults<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = rare<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = poor<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
}}<br />
'''Renal tumour with Xp11.2 translocation''', also '''Xp11.2 translocation carcinoma''' and '''Xp11 translocation renal cell carcinoma''', is an uncommon [[malignant]] [[kidney tumour]] in the [[MiT family translocation renal cell carcinoma]]. <br />
<br />
It should '''not''' be confused with ''[[renal tumour with t(6;11) translocation]]'' (also known as ''TFEB RCC''), another [[translocation carcinoma]] found in the [[kidney]] that is also in the ''MiT family translocation renal cell carcinoma''. <br />
<br />
==General==<br />
*Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.<br />
*TFE3 is the gene involved in the translocation seen in [[alveolar soft part sarcoma]] (ASPS).<br />
*Poor prognosis - majority Stage III or Stage IV.<ref name=pmid25002378>{{Cite journal | last1 = Su | first1 = HH. | last2 = Sung | first2 = MT. | last3 = Chiang | first3 = PH. | last4 = Cheng | first4 = YT. | last5 = Chen | first5 = YT. | title = The preliminary experiences of translocation renal cell carcinoma and literature review. | journal = Kaohsiung J Med Sci | volume = 30 | issue = 8 | pages = 402-8 | month = Aug | year = 2014 | doi = 10.1016/j.kjms.2014.03.003 | PMID = 25002378 }}</ref><br />
*~1/3 of childhood RCC.<ref name=pmid17667536>{{Cite journal | last1 = Argani | first1 = P. | last2 = Olgac | first2 = S. | last3 = Tickoo | first3 = SK. | last4 = Goldfischer | first4 = M. | last5 = Moch | first5 = H. | last6 = Chan | first6 = DY. | last7 = Eble | first7 = JN. | last8 = Bonsib | first8 = SM. | last9 = Jimeno | first9 = M. | title = Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. | journal = Am J Surg Pathol | volume = 31 | issue = 8 | pages = 1149-60 | month = Aug | year = 2007 | doi = 10.1097/PAS.0b013e318031ffff | PMID = 17667536 }}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
*Large cells.<br />
*Clear or eosinophilic cytoplasm.<br />
*Papillae or nests.<br />
*[[Psammoma bodies]] - common.<ref name=pmid17102051>{{Cite journal | last1 = Prasad | first1 = SR. | last2 = Humphrey | first2 = PA. | last3 = Catena | first3 = JR. | last4 = Narra | first4 = VR. | last5 = Srigley | first5 = JR. | last6 = Cortez | first6 = AD. | last7 = Dalrymple | first7 = NC. | last8 = Chintapalli | first8 = KN. | title = Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. | journal = Radiographics | volume = 26 | issue = 6 | pages = 1795-806; discussion 1806-10 | month = | year = | doi = 10.1148/rg.266065010 | PMID = 17102051 }}</ref><br />
**Calcification is considered the '''classic histomorphologic feature'''. <br />
*[[Hyaline bodies]] - common.<br />
<br />
Notes:<br />
*Looks clear cell RCC ''or'' papillary RCC ''or'' a hybrid between the two.<br />
*May resemble [[alveolar soft part sarcoma]].<br />
<br />
DDx:<br />
*[[Clear cell RCC]].<br />
*[[Papillary RCC]].<br />
*[[Epithelioid angiomyolipoma]].<br />
*[[Clear cell papillary renal cell carcinoma]].<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Adrenal gland]] - cortex.<br />
*[[Melanotic Xp11 translocation renal cancer]] - an evolving entity that looks like Xp11 translocation carcinoma, keratin negative.<ref name=pmid19065101>{{Cite journal | last1 = Argani | first1 = P. | last2 = Aulmann | first2 = S. | last3 = Karanjawala | first3 = Z. | last4 = Fraser | first4 = RB. | last5 = Ladanyi | first5 = M. | last6 = Rodriguez | first6 = MM. | title = Melanotic Xp11 translocation renal cancers: a distinctive neoplasm with overlapping features of PEComa, carcinoma, and melanoma. | journal = Am J Surg Pathol | volume = 33 | issue = 4 | pages = 609-19 | month = Apr | year = 2009 | doi = 10.1097/PAS.0b013e31818fbdff | PMID = 19065101 }}</ref><br />
<br />
===Images===<br />
====Case 1====<br />
<gallery><br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_intermed_mag.jpg | Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_high_mag.jpg | Xp11.2 translocation RCC - high mag. (WC/Nephron)<br />
Image:Xp11.2_translocation_renal_cell_carcinoma_-_very_high_mag.jpg | Xp11.2 translocation RCC - very high mag. (WC/Nephron)<br />
</gallery><br />
====Case 2====<br />
<gallery><br />
Image: Translocation carcinoma - kidney -- low mag.jpg | TCK - low mag.<br />
Image: Translocation carcinoma - kidney -- intermed mag.jpg | TCK - intermed. mag.<br />
Image: Translocation carcinoma - kidney -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney - alt -- high mag.jpg | TCK - high mag.<br />
Image: Translocation carcinoma - kidney -- very high mag.jpg | TCK - very high mag.<br />
</gallery><br />
====www====<br />
*[http://www.flickr.com/photos/40764007@N08/7403237732/in/photostream/ Translocation carcinoma (flickr.com)].<br />
<br />
==IHC==<br />
Key stains - as per [[ISUP]]:<ref name=pmid25025364 >{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref><br />
*TFE3 +ve (nucleus).<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref> ‡<br />
*TFEB +ve.<br />
*Cathepsin K +ve.<ref name=pmid21602817>{{cite journal |author=Martignoni G, Gobbo S, Camparo P, ''et al.'' |title=Differential expression of cathepsin K in neoplasms harboring TFE3 gene fusions |journal=Mod. Pathol. |volume=24 |issue=10 |pages=1313–9 |year=2011 |month=October |pmid=21602817 |doi=10.1038/modpathol.2011.93 |url=}}</ref><br />
<br />
Others:<br />
*CD10 +ve.<br />
*Vimentin +ve.<br />
*CK7 -ve.<br />
**Positive in ~20% of cases.<ref name=pmid21804394>{{Cite journal | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref><br />
*HMB-45 -ve.<br />
**Positive in ''epithelioid [[angiomyolipoma]]''.<br />
*Melan A -ve/+ve.<ref name=pmid22207547>{{Cite journal | last1 = Kuroda | first1 = N. | last2 = Mikami | first2 = S. | last3 = Pan | first3 = CC. | last4 = Cohen | first4 = RJ. | last5 = Hes | first5 = O. | last6 = Michal | first6 = M. | last7 = Nagashima | first7 = Y. | last8 = Tanaka | first8 = Y. | last9 = Inoue | first9 = K. | title = Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect. | journal = Histol Histopathol | volume = 27 | issue = 2 | pages = 133-40 | month = Feb | year = 2012 | doi = | PMID = 22207547 }}</ref><br />
**Should exclude adrenal if +ve.<br />
*EMA usually -ve.<ref name=pmid20679884>{{Cite journal | last1 = Argani | first1 = P. | last2 = Hicks | first2 = J. | last3 = De Marzo | first3 = AM. | last4 = Albadine | first4 = R. | last5 = Illei | first5 = PB. | last6 = Ladanyi | first6 = M. | last7 = Reuter | first7 = VE. | last8 = Netto | first8 = GJ. | title = Xp11 translocation renal cell carcinoma (RCC): extended immunohistochemical profile emphasizing novel RCC markers. | journal = Am J Surg Pathol | volume = 34 | issue = 9 | pages = 1295-303 | month = Sep | year = 2010 | doi = 10.1097/PAS.0b013e3181e8ce5b | PMID = 20679884 }}</ref><br />
*CD117 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*CK (pooled) -ve.<ref name=pmid20679884/> (???)<br />
*WT1 -ve (0 +ve/21 cases<ref name=pmid20679884/>).<br />
*PAX8 +ve (16 +ve/21<ref name=pmid20679884/>).<br />
<br />
Notes:<br />
*Poor man's panel - if one doesn't have the more informative stains: <br />
**CK (pooled), EMA, Melan A, HMB-45, CK7, AMACR, CD10, vimentin, RCC.<br />
*‡ The IHC (in comparsion to FISH) has both false negatives and false positives; in one study by Qu ''et al.'' the false positive rate was 7% (2/30) and false negative rate was 4% (2/46).<ref>{{Cite journal | last1 = Qu | first1 = Y. | last2 = Gu | first2 = C. | last3 = Wang | first3 = H. | last4 = Chang | first4 = K. | last5 = Yang | first5 = X. | last6 = Zhou | first6 = X. | last7 = Dai | first7 = B. | last8 = Zhu | first8 = Y. | last9 = Shi | first9 = G. | title = Diagnosis of adults Xp11.2 translocation renal cell carcinoma by immunohistochemistry and FISH assays: clinicopathological data from ethnic Chinese population. | journal = Sci Rep | volume = 6 | issue = | pages = 21677 | month = | year = 2016 | doi = 10.1038/srep21677 | PMID = 26880493 }}</ref><br />
<br />
==Molecular==<br />
*[[Translocation]] involving TFE3.<ref name=Ref_WMSP285>{{Ref WMSP|285}}</ref><br />
**A very low list of partners have been described.<br />
<br />
The most common are:<ref name=pmid24309327 >{{Cite journal | last1 = Ellis | first1 = CL. | last2 = Eble | first2 = JN. | last3 = Subhawong | first3 = AP. | last4 = Martignoni | first4 = G. | last5 = Zhong | first5 = M. | last6 = Ladanyi | first6 = M. | last7 = Epstein | first7 = JI. | last8 = Netto | first8 = GJ. | last9 = Argani | first9 = P. | title = Clinical heterogeneity of Xp11 translocation renal cell carcinoma: impact of fusion subtype, age, and stage. | journal = Mod Pathol | volume = | issue = | pages = | month = Dec | year = 2013 | doi = 10.1038/modpathol.2013.208 | PMID = 24309327 }}</ref><br />
*t(x;1)(p11.2;q21) PRCC-TFE3.<br />
*t(x;17)(p11.2;q25) ASPSCR1-TFE3.<br />
**Also seen in [[alveolar soft part sarcoma]].<br />
<br />
==See also==<br />
*[[Kidney tumours]].<br />
*[[Chromosomal translocation]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Diagnosis]]<br />
[[Category:Kidney tumours]]</div>
Michael
https://librepathology.org/w/index.php?title=Perinephric_myxoid_pseudotumour_of_fat&diff=52830
Perinephric myxoid pseudotumour of fat
2024-03-26T12:46:56Z
<p>Michael: </p>
<hr />
<div>'''Perinephric myxoid pseudotumour of fat''', abbreviated '''PMPF''', is a rare lesion found around the [[kidney]].<ref>{{cite journal |authors=Dashti NK, Fritchie KJ, Folpe AL |title=Perinephric myxoid pseudotumor of fat: a distinctive pseudoneoplasm most often associated with non-neoplastic renal disease |journal=Hum Pathol |volume=87 |issue= |pages=37–43 |date=May 2019 |pmid=30826321 |doi=10.1016/j.humpath.2019.02.005 |url=}}</ref><br />
<br />
==General==<br />
*Often elderly male.<br />
*Non-tumour kidney - typically [[medical kidney disease]].<br />
*Good outcome.<br />
*Typically an incidental finding.<br />
<br />
==Microscopic==<br />
Features:<br />
*Myxoid background.<br />
*Mature fat without significant atypia.<br />
*Perivascular hyalinization.<br />
*Fibrosis.<br />
<br />
DDx:<br />
*[[Angiomyolipoma]]<br />
*[[IgG4-related disease]].<br />
*[[Myelolipoma]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=PMPF&diff=52829
PMPF
2024-03-26T12:46:31Z
<p>Michael: </p>
<hr />
<div>#redirect [[Perinephric myxoid pseudotumour of fat]]<br />
<br />
[[Category:Abbreviation]]</div>
Michael
https://librepathology.org/w/index.php?title=PMPF&diff=52828
PMPF
2024-03-26T12:46:25Z
<p>Michael: Redirected page to Perinephric myxoid pseudotumour of fat</p>
<hr />
<div>#redirect [[Perinephric myxoid pseudotumour of fat]]<br />
<br />
[[Category:Abbreviations]]</div>
Michael
https://librepathology.org/w/index.php?title=Perinephric_myxoid_pseudotumour_of_fat&diff=52827
Perinephric myxoid pseudotumour of fat
2024-03-26T12:45:41Z
<p>Michael: </p>
<hr />
<div>'''Perinephric myxoid pseudotumour of fat''', abbreviated '''PMPF''', is a rare lesion found around the [[kidney]].<ref>{{cite journal |authors=Dashti NK, Fritchie KJ, Folpe AL |title=Perinephric myxoid pseudotumor of fat: a distinctive pseudoneoplasm most often associated with non-neoplastic renal disease |journal=Hum Pathol |volume=87 |issue= |pages=37–43 |date=May 2019 |pmid=30826321 |doi=10.1016/j.humpath.2019.02.005 |url=}}</ref><br />
<br />
==General==<br />
*Often elderly male.<br />
*Non-tumour kidney - typically [[medical kidney disease]].<br />
*Good outcome.<br />
<br />
==Microscopic==<br />
Features:<br />
*Myxoid background.<br />
*Mature fat without significant atypia.<br />
*Perivascular hyalinization.<br />
*Fibrosis.<br />
<br />
DDx:<br />
*[[Angiomyolipoma]]<br />
*[[IgG4-related disease]].<br />
*[[Myelolipoma]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Perinephric_myxoid_pseudotumour_of_fat&diff=52826
Perinephric myxoid pseudotumour of fat
2024-03-26T12:44:58Z
<p>Michael: Created page with "'''Perinephric myxoid pseudotumour of fat''', abbreviated '''PMPF''', is a rare lesion found around the kidney.<ref>{{cite journal |authors=Dashti NK, Fritchie KJ, Folpe A..."</p>
<hr />
<div>'''Perinephric myxoid pseudotumour of fat''', abbreviated '''PMPF''', is a rare lesion found around the [[kidney]].<ref>{{cite journal |authors=Dashti NK, Fritchie KJ, Folpe AL |title=Perinephric myxoid pseudotumor of fat: a distinctive pseudoneoplasm most often associated with non-neoplastic renal disease |journal=Hum Pathol |volume=87 |issue= |pages=37–43 |date=May 2019 |pmid=30826321 |doi=10.1016/j.humpath.2019.02.005 |url=}}</ref><br />
<br />
==General==<br />
*Often elderly male.<br />
*Non-tumour kidney - typically [[medical kidney disease]].<br />
*Good outcome.<br />
<br />
==Microscopic==<br />
Features:<br />
*Myxoid background.<br />
*Mature fat without significant atypia.<br />
*Perivascular hyalinization.<br />
*Fibrosis.<br />
<br />
DDx:<br />
*[[Angiomyolipoma]]<br />
*[[IgG4-disease]].<br />
*[[Myelolipoma]].<br />
<br />
==References==<br />
{{Reflist|1}}<br />
<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Low-grade_oncocytic_renal_tumour&diff=52825
Low-grade oncocytic renal tumour
2024-03-26T12:37:07Z
<p>Michael: +L1CAM</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Low grade oncocytic renal tumour -- very high mag.jpg<br />
| Width =<br />
| Caption = Low-grade renal oncocytic tumour. [[H&E stain]].<br />
| Micro = abundant eosinophilic cytoplasm, nuclei uniform and round without significant pleomorphism, solid or tubular architecture<br />
| Subtypes =<br />
| LMDDx = [[renal oncocytoma]], [[chromophobe renal cell carcinoma]] eosinophilic variant, [[clear cell renal cell carcinoma]] eosinophilic variant, [[papillary renal cell carcinoma]] oncocytic variant, [[tubulocystic carcinoma of the kidney]], other [[renal tumours with eosinophilic cytoplasm]], benign [[liver]]<br />
| Stains = <br />
| IHC = CK7 +ve, CD117 -ve, GATA3 +ve, [[PAX8]] +ve<br />
| EM = <br />
| Molecular = TSC/mTOR pathway mutation<br />
| IF =<br />
| Gross = <br />
| Grossing = [[partial nephrectomy grossing]], [[total nephrectomy for tumour grossing]]<br />
| Site = [[kidney]] - see [[kidney tumours]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx = often incidentally discovered mass<br />
| Signs =<br />
| Symptoms = <br />
| Prevalence =<br />
| Bloodwork =<br />
| Rads = <br />
| Endoscopy =<br />
| Prognosis = likely benign<br />
| Other =<br />
| ClinDDx = other [[kidney tumours]]<br />
}}<br />
'''Low-grade oncocytic renal tumour''', abbreviated '''LOT''', is an unusual [[renal tumour]] that does not neatly fit into any WHO renal neoplasia category. It is also known as '''renal oncocytic tumour that is CD117 negative and CK7 positive'''. <br />
<br />
''Low-grade oncocytic tumour'' directs to here.<br />
<br />
Whether it is a distinct entity is not clear as of 2019.<ref name=pmid30895640/> It can be considered an emerging entity that may be in the WHO classification of renal neoplasia in the future.<ref name=pmid31936678>{{cite journal |authors=Siadat F, Trpkov K |title=ESC, ALK, HOT and LOT: Three Letter Acronyms of Emerging Renal Entities Knocking on the Door of the WHO Classification |journal=Cancers (Basel) |volume=12 |issue=1 |pages= |date=January 2020 |pmid=31936678 |pmc=7017067 |doi=10.3390/cancers12010168 |url=}}</ref><br />
<br />
==General==<br />
*Very rare.<br />
*Benign behaviour based on a small series.<ref name=pmid30895640>{{cite journal |authors=Trpkov K, Williamson SR, Gao Y, Martinek P, Cheng L, Sangoi AR, Yilmaz A, Wang C, San Miguel Fraile P, Perez Montiel DM, Bulimbasić S, Rogala J, Hes O |title=Low-grade oncocytic tumour of kidney (CD117-negative, cytokeratin 7-positive): a distinct entity? |journal=Histopathology |volume=75 |issue=2 |pages=174–184 |date=August 2019 |pmid=30895640 |doi=10.1111/his.13865 |url=}}</ref><br />
<br />
==Microscopic==<br />
Features:<ref name=Trpkov>Trpkov K, Williamson S, Martinek P, Cheng L, San Miguel Fraile P, Yilmaz A, Hes O, Oncocytic Renal Tumors with CD117 Negative, Cytokeratin 7 Positive Immunoprofile are Different from Eosinophilic Chromophobe Renal Cell Carcinoma (ChrRCC) and Oncocytoma. United States and Canadian Academy of Pathology Annual Meeting 2018.</ref><br />
*Oncocytic cytoplasm.<br />
*Round nuclei.<br />
*Tubular or solid architecture.<br />
*Edematous areas with loosly arranged cells.<br />
*Low [[ISUP nucleolar grade]] - grade 1 or 2.<br />
<br />
DDx:<br />
*[[Renal oncocytoma]].<br />
*[[Chromophobe renal cell carcinoma]].<br />
*[[Renal hybrid oncocytic/chromophobe tumour]].<br />
*[[Renal cell carcinoma, unclassified]].<br />
*[[Renal tumours with eosinophilic cytoplasm]].<br />
<br />
===Images===<br />
<gallery><br />
Image: Low grade oncocytic renal tumour -- low mag.jpg | LGORT - low mag. (WC)<br />
Image: Low grade oncocytic renal tumour -- intermed mag.jpg | LGORT - intermed. mag. (WC)<br />
Image: Low grade oncocytic renal tumour - alt -- intermed mag.jpg | LGORT - intermed. mag. (WC)<br />
Image: Low grade oncocytic renal tumour -- high mag.jpg | LGORT - high mag. (WC)<br />
Image: Low grade oncocytic renal tumour - alt - high mag.jpg | LGORT - high mag. (WC)<br />
Image: Low grade oncocytic renal tumour -- very high mag.jpg | LGORT - high mag. (WC)<br />
</gallery><br />
<br />
==IHC==<br />
Features:<ref>{{cite journal |authors=Ricci C, Ambrosi F, Franceschini T, Giunchi F, Grillini A, Franchini E, Grillini M, Schiavina R, Massari F, Mollica V, Tateo V, Bianchi FM, Bianchi L, Droghetti M, Maloberti T, Tallini G, Colecchia M, Acosta AM, Lobo J, Trpkov K, Fiorentino M, de Biase D |title=Evaluation of an institutional series of low-grade oncocytic tumor (LOT) of the kidney and review of the mutational landscape of LOT |journal=Virchows Arch |volume= |issue= |pages= |date=October 2023 |pmid=37845471 |doi=10.1007/s00428-023-03673-9 |url=}}</ref><br />
*CK7 +ve.<br />
*[[GATA3]] +ve.<br />
**GATA3 very rarely positive in RCCs (1/71 clear cell RCC, 0/53 papillary RCC, 2/33 chromophobe RCC).<ref name=pmid2431520>{{cite journal |authors=Gonzalez-Roibon N, Faraj SF, Munari E, Bezerra SM, Albadine R, Sharma R, Argani P, Allaf ME, Netto GJ |title=Comprehensive profile of GATA binding protein 3 immunohistochemical expression in primary and metastatic renal neoplasms |journal=Hum Pathol |volume=45 |issue=2 |pages=244–8 |date=February 2014 |pmid=24315206 |doi=10.1016/j.humpath.2013.08.020 |url=}}</ref><br />
**GATA3 occasionally positive in [[renal oncocytoma]] (9/47 cases).<ref name=pmid2431520/><br />
*PAX8 +ve.<br />
*CD117 -ve.<br />
<br />
Others:<br />
*CD10 -ve/+ve.<br />
*AMACR -ve/+ve.<br />
*CA9 -ve.<br />
*CK20 -ve.<br />
*CD10 -ve.<br />
*L1CAM +ve.<ref name=pmid38460672>{{cite journal |authors=Alghamdi M, Chen JF, Jungbluth A, Koutzaki S, Palmer MB, Al-Ahmadie HA, Fine SW, Gopalan A, Sarungbam J, Sirintrapun SJ, Tickoo SK, Reuter VE, Chen YB |title=L1CAM Expression and Molecular Alterations Distinguish Low Grade Oncocytic Tumor (LOT) from Eosinophilic Chromophobe Renal Cell Carcinoma |journal=Mod Pathol |volume= |issue= |pages=100467 |date=March 2024 |pmid=38460672 |doi=10.1016/j.modpat.2024.100467 |url=}}</ref><br />
<br />
==Molecular==<br />
*[[Renal neoplasms with TSC/mTOR pathway mutations|TSC/mTOR pathway mutation]].<ref name=pmid35203531>{{cite journal |authors=Pivovarcikova K, Alaghehbandan R, Vanecek T, Ohashi R, Pitra T, Hes O |title=TSC/mTOR Pathway Mutation Associated Eosinophilic/Oncocytic Renal Neoplasms: A Heterogeneous Group of Tumors with Distinct Morphology, Immunohistochemical Profile, and Similar Genetic Background |journal=Biomedicines |volume=10 |issue=2 |pages= |date=January 2022 |pmid=35203531 |pmc=8869370 |doi=10.3390/biomedicines10020322 |url=}}</ref><br />
<br />
==Sign out==<br />
<pre><br />
Right Kidney Mass, Biopsy:<br />
- Low-grade oncocytic tumour (see comment).<br />
<br />
Comment:<br />
The tumour stains as follows:<br />
POSITIVE: CK7 (strong, diffuse), PAX8 (strong, diffuse), AE1/AE3, GATA-3 (moderate, diffuse).<br />
NEGATIVE: CD117, CD10, CK20, vimentin, CA9.<br />
<br />
It is in keeping with "low-grade oncocytic tumour" (LOT), an evolving entity. Based on limited data, LOT appears to have an indolent behavior. <br />
<br />
This tumour does not fit the diagnosis of renal oncocytoma or eosinophilic chromophobe renal cell carcinoma. <br />
</pre><br />
<br />
==See also==<br />
*[[Renal oncocytoma]].<br />
*[[Renal tumours with eosinophilic cytoplasm]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Diagnosis]]<br />
[[Category:Genitourinary pathology]]</div>
Michael
https://librepathology.org/w/index.php?title=Inflammatory_myofibroblastic_tumour&diff=52824
Inflammatory myofibroblastic tumour
2024-03-26T00:28:04Z
<p>Michael: /* IHC */</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Inflammatory_myofibroblastic_tumour_-_very_high_mag.jpg <br />
| Width =<br />
| Caption = Inflammatory myofibroblastic tumour. [[H&E stain]].<br />
| Synonyms =<br />
| Micro = inflammation ([[plasma cells]] - predominant, lymphocytes, eosinophils), [[spindle cells]] without atypia +/-fascicular architecture, +/-mitoses (none atypical), +/-[[necrosis]], +/-hemorrhage, +/-calcification<br />
| Subtypes =<br />
| LMDDx = [[calcifying fibrous pseudotumour]], [[inflammatory fibroid tumour]], [[nodular fasciitis]], [[gastrointestinal stromal tumour]], [[epithelioid inflammatory myofibroblastic sarcoma]]<br />
| Stains =<br />
| IHC =<br />
| EM =<br />
| Molecular =<br />
| IF =<br />
| Gross =<br />
| Grossing =<br />
| Site = [[soft tissue lesions|soft tissue]] - see ''[[fibroblastic/myofibroblastic tumours]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx =<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = uncommon<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy =<br />
| Prognosis = benign<br />
| Other =<br />
| ClinDDx = other soft tissue lesions<br />
| Tx =<br />
}}<br />
'''Inflammatory myofibroblastic tumour''', abbreviated '''IMT''', is an uncommon [[soft tissue lesion]].<br />
<br />
It is also known as '''inflammatory pseudotumour''', and '''inflammatory fibrosarcoma'''<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref> and '''plasma cell granuloma'''.<ref>URL: [http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung]. Accessed on: 27 November 2011.</ref><ref name=pmid21772725>{{Cite journal | last1 = Manohar | first1 = B. | last2 = Bhuvaneshwari | first2 = S. | title = Plasma cell granuloma of gingiva. | journal = J Indian Soc Periodontol | volume = 15 | issue = 1 | pages = 64-6 | month = Jan | year = 2011 | doi = 10.4103/0972-124X.82275 | PMID = 21772725 }}</ref><br />
<br />
==General==<br />
*Mostly benign.<br />
*Children & young adults.<br />
<br />
May be treated with:<br />
*[[ALK inhibitors]].<ref name=pmid30790150>{{Cite journal | last1 = Honda | first1 = K. | last2 = Kadowaki | first2 = S. | last3 = Kato | first3 = K. | last4 = Hanai | first4 = N. | last5 = Hasegawa | first5 = Y. | last6 = Yatabe | first6 = Y. | last7 = Muro | first7 = K. | title = Durable response to the ALK inhibitor alectinib in inflammatory myofibroblastic tumor of the head and neck with a novel SQSTM1-ALK fusion: a case report. | journal = Invest New Drugs | volume = | issue = | pages = | month = Feb | year = 2019 | doi = 10.1007/s10637-019-00742-2 | PMID = 30790150 }}</ref> <br />
*Drugs that act on ROS1 rearrangements, e.g. [[crizotinib]].<ref name=pmid30642440>{{Cite journal | last1 = Mai | first1 = S. | last2 = Xiong | first2 = G. | last3 = Diao | first3 = D. | last4 = Wang | first4 = W. | last5 = Zhou | first5 = Y. | last6 = Cai | first6 = R. | title = Case report: Crizotinib is effective in a patient with ROS1-rearranged pulmonary inflammatory myofibroblastic tumor. | journal = Lung Cancer | volume = 128 | issue = | pages = 101-104 | month = Feb | year = 2019 | doi = 10.1016/j.lungcan.2018.12.016 | PMID = 30642440 }}</ref><br />
<br />
==Gross==<br />
*Classically located in mesentery of ileocolic region or [[small bowel]].<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref><br />
*May be seen in the [[urinary bladder]].<ref name=pmid15577680/><br />
<br />
==Microscopic==<br />
Features:<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref><br />
*Inflammation:<br />
**[[Plasma cells]] - predominant - '''key feature'''.<ref name=pmid21297584>{{Cite journal | last1 = Saab | first1 = ST. | last2 = Hornick | first2 = JL. | last3 = Fletcher | first3 = CD. | last4 = Olson | first4 = SJ. | last5 = Coffin | first5 = CM. | title = IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link? | journal = Mod Pathol | volume = 24 | issue = 4 | pages = 606-12 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.226 | PMID = 21297584 }}</ref><br />
**Lymphocytes.<br />
**[[Eosinophil]]s.<br />
*Spindle cells without atypia.<br />
*+/-Fascicular architecture.<br />
*Mitoses -- though none atypical.<br />
*+/-Necrosis.<br />
*+/-Hemorrhage.<br />
*Calcifications.<br />
<br />
DDx:<br />
*[[Calcifying fibrous pseudotumour]] (has [[psammoma body|psammomatous calcifications]]).<br />
*[[Inflammatory fibroid tumour]].<br />
*[[Nodular fasciitis]].<br />
*[[Gastrointestinal stromal tumour]].<ref name=pmid24938355>{{Cite journal | last1 = Kataoka | first1 = TR. | last2 = Yamashita | first2 = N. | last3 = Furuhata | first3 = A. | last4 = Hirata | first4 = M. | last5 = Ishida | first5 = T. | last6 = Nakamura | first6 = I. | last7 = Hirota | first7 = S. | last8 = Haga | first8 = H. | last9 = Katsuyama | first9 = E. | title = An inflammatory myofibroblastic tumor exhibiting immunoreactivity to KIT: a case report focusing on a diagnostic pitfall. | journal = World J Surg Oncol | volume = 12 | issue = | pages = 186 | month = | year = 2014 | doi = 10.1186/1477-7819-12-186 | PMID = 24938355 }}</ref><br />
*[[IgG4-related systemic disease]].<ref name=pmid21297584/><br />
*[[Epithelioid inflammatory myofibroblastic sarcoma]].<br />
<br />
Notes:<br />
*Some consider this a wastebasket diagnosis... for benign appearing spindle cell lesions.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2]. Accessed on: 10 May 2011.</ref><br />
<br />
===Images===<br />
<gallery><br />
Image:Inflammatory_myofibroblastic_tumour_-_high_mag.jpg | IMT - high mag. (WC)<br />
Image:Inflammatory_myofibroblastic_tumour_-_very_high_mag.jpg | IMT - very high mag. (WC)<br />
</gallery><br />
<br />
==IHC==<br />
Features - dependent on site:<br />
*SMA +ve.<ref name=pmid20350216>{{Cite journal | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}</ref><br />
*Vimentin +ve.<br />
*ALK-1 +ve.<ref name=pmid15577680>{{Cite journal | last1 = Tsuzuki | first1 = T. | last2 = Magi-Galluzzi | first2 = C. | last3 = Epstein | first3 = JI. | title = ALK-1 expression in inflammatory myofibroblastic tumor of the urinary bladder. | journal = Am J Surg Pathol | volume = 28 | issue = 12 | pages = 1609-14 | month = Dec | year = 2004 | doi = | PMID = 15577680 }}</ref><br />
*CD117 -ve.<br />
**Case report of CD117 +ve.<ref name=pmid24938355/><br />
*CD30 -ve.<br />
<br />
Variable staining with:<br />
*CD34, AE1/AE3, calretinin.<ref name=pmid20216379>{{Cite journal | last1 = Miyamoto | first1 = H. | last2 = Montgomery | first2 = EA. | last3 = Epstein | first3 = JI. | title = Paratesticular fibrous pseudotumor: a morphologic and immunohistochemical study of 13 cases. | journal = Am J Surg Pathol | volume = 34 | issue = 4 | pages = 569-74 | month = Apr | year = 2010 | doi = 10.1097/PAS.0b013e3181d438cb | PMID = 20216379 }}</ref><ref name=pmid20350216>{{Cite journal | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}</ref><br />
<br />
Negative:<ref name=pmid20350216/><br />
*S100, CD117, CD68.<br />
<br />
==Molecular==<br />
*ALK rearrangements.<ref name=pmid21297584/><br />
*ROS1 rearrangements.<ref name=pmid30642440>{{Cite journal | last1 = Mai | first1 = S. | last2 = Xiong | first2 = G. | last3 = Diao | first3 = D. | last4 = Wang | first4 = W. | last5 = Zhou | first5 = Y. | last6 = Cai | first6 = R. | title = Case report: Crizotinib is effective in a patient with ROS1-rearranged pulmonary inflammatory myofibroblastic tumor. | journal = Lung Cancer | volume = 128 | issue = | pages = 101-104 | month = Feb | year = 2019 | doi = 10.1016/j.lungcan.2018.12.016 | PMID = 30642440 }}</ref><br />
<br />
==See also==<br />
*[[Soft tissue lesions]].<br />
*[[Fibroblastic/myofibroblastic tumours]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Soft tissue lesions]]<br />
[[Category:Fibroblastic/myofibroblastic tumours]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Syphilis&diff=52823
Syphilis
2024-03-26T00:15:21Z
<p>Michael: </p>
<hr />
<div>'''Syphilis''' is the infectious disease caused by ''Treponema pallidum''.<br />
<br />
==General==<br />
*Classically affects the [[testis]] before the epididymis.<ref name=Ref_WMSP364>{{Ref WMSP|364}}</ref><br />
**[[Tuberculosis]]<ref name=pmid19468469>{{Cite journal | last1 = Das | first1 = P. | last2 = Ahuja | first2 = A. | last3 = Gupta | first3 = SD. | title = Incidence, etiopathogenesis and pathological aspects of genitourinary tuberculosis in India: A journey revisited. | journal = Indian J Urol | volume = 24 | issue = 3 | pages = 356-61 | month = Jul | year = 2008 | doi = 10.4103/0970-1591.42618 | PMID = 19468469 }}</ref> and gonorrhea usually affect the epididymis before the testis.<br />
*Considered one of the [[TORCH infections]].<br />
<br />
==Gross==<br />
Aorta:<br />
*Wrinkled intimal surface - "tree bark" appearance.<ref>{{Ref AoGP|80}}</ref><br />
<br />
==Microscopic==<br />
Features:<br />
*[[Plasma cells]] - '''key feature'''.<ref name=pmid18948765>{{Cite journal | last1 = Brinster | first1 = NK. | title = Dermatopathology for the surgical pathologist: a pattern-based approach to the diagnosis of inflammatory skin disorders (part II). | journal = Adv Anat Pathol | volume = 15 | issue = 6 | pages = 350-69 | month = Nov | year = 2008 | doi = 10.1097/PAP.0b013e31818b1ac6 | PMID = 18948765 }}</ref><br />
*Spirochetes - '''diagnostic'''.<br />
*+/-[[Granulomas]]<ref name=Ref_PCPBoD8_47>{{Ref PCPBoD8|47}}</ref> - ''gumma'' - in the tertiary phase.<ref>URL: [http://pediatrics.aappublications.org/cgi/content/full/104/1/e4 http://pediatrics.aappublications.org/cgi/content/full/104/1/e4]. Accessed on: 17 November 2010.</ref><br />
<br />
DDx:<br />
*[[Plasma cell neoplasm]].<br />
*Reactive changes.<br />
*[[Zoon balanitis]].<br />
*Other spirochetes, e.g. ''Borrelia burgdorferi''.<br />
<br />
==[[Stains]]==<br />
*[[Dieterle stain]].<br />
<br />
===Image===<br />
<gallery><br />
Image:Treponema_pallidum_-_very_high_mag_-_extreme_crop.jpg | T. pallidum - very high mag. cropped. [[Dieterle stain]]. (WC)<br />
Image:Treponema_pallidum_-_very_high_mag.jpg | T. pallidum - very high mag. [[Dieterle stain]]. (WC)<br />
</gallery><br />
<br />
==See also==<br />
*[[Vascular disease]].<br />
*[[Microorganisms]].<br />
*[[Intestinal spirochetosis]].<br />
*[[Inflammatory skin disorders]].<br />
*[[Granulomatous orchitis]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Weird stuff]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Autoimmune_metaplastic_atrophic_gastritis&diff=52822
Autoimmune metaplastic atrophic gastritis
2024-03-26T00:08:30Z
<p>Michael: /* Microscopic */</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Metaplastic atrophic gastritis - body -- intermed mag.jpg<br />
| Width = Metaplastic atrophic gastritis - body of stomach. [[H&E stains]].<br />
| Caption = Atrophic gastritis (body) without appreciable parietal cells. [[H&E stain]].<br />
| Micro = corpus predominant inflammation - usu. moderate or severe, loss of parietal cells, increased G cells in the antrum<br />
| Subtypes =<br />
| LMDDx = [[chronic gastritis]], [[intestinal metaplasia of the stomach]], [[gastric neuroendocrine tumour]]<br />
| Stains =<br />
| IHC =<br />
| EM =<br />
| Molecular =<br />
| IF =<br />
| Gross =<br />
| Grossing =<br />
| Site = [[stomach]]<br />
| Assdx =<br />
| Syndromes =<br />
| Clinicalhx =<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = uncommon<br />
| Bloodwork = antibodies to parietal cells & intrinsic factor, [[macrocytic anemia]], increased gastrin level<br />
| Rads =<br />
| Endoscopy = erythema - corpus only<br />
| Prognosis =<br />
| Other =<br />
| ClinDDx = diffuse chronic gastritis<br />
}}<br />
'''Autoimmune metaplastic atrophic gastritis''', also '''autoimmune gastritis'''<ref name=pmid16382988>{{Cite journal | last1 = Chlumská | first1 = A. | last2 = Boudová | first2 = L. | last3 = Benes | first3 = Z. | last4 = Zámecník | first4 = M. | title = Autoimmune gastritis. A clinicopathologic study of 25 cases. | journal = Cesk Patol | volume = 41 | issue = 4 | pages = 137-42 | month = Oct | year = 2005 | doi = | PMID = 16382988 }}</ref> (abbreviated '''AIG'''), is a rare pathology of the [[stomach]]. It is closely associated with '''pernicious anemia'''.<br />
<br />
==General==<br />
*Pathology: loss of parietal cells, gastric atrophy.<br />
*Lab: classically considered to have [[macrocytic anemia]]; however, normocytic and microcystic more common.<ref name=pmid16239424>{{Cite journal | last1 = Hershko | first1 = C. | last2 = Ronson | first2 = A. | last3 = Souroujon | first3 = M. | last4 = Maschler | first4 = I. | last5 = Heyd | first5 = J. | last6 = Patz | first6 = J. | title = Variable hematologic presentation of autoimmune gastritis: age-related progression from iron deficiency to cobalamin depletion. | journal = Blood | volume = 107 | issue = 4 | pages = 1673-9 | month = Feb | year = 2006 | doi = 10.1182/blood-2005-09-3534 | PMID = 16239424 }}</ref><br />
*Etiology: autoimmune.<br />
<br />
Diagnosis based on serology for antibodies to:<ref name=pmid12643357>{{Cite journal | last1 = Oh | first1 = R. | last2 = Brown | first2 = DL. | title = Vitamin B12 deficiency. | journal = Am Fam Physician | volume = 67 | issue = 5 | pages = 979-86 | month = Mar | year = 2003 | doi = | PMID = 12643357 }}</ref><br />
*Parietal cells.<br />
*Intrinsic factor. <br />
<br />
Others:<br />
*Gastrin level (increased).<ref name=pmid21947876>{{Cite journal | last1 = Annibale | first1 = B. | last2 = Lahner | first2 = E. | last3 = Fave | first3 = GD. | title = Diagnosis and management of pernicious anemia. | journal = Curr Gastroenterol Rep | volume = 13 | issue = 6 | pages = 518-24 | month = Dec | year = 2011 | doi = 10.1007/s11894-011-0225-5 | PMID = 21947876 }}</ref><br />
**Normal < 100 pg/mL.<ref>URL: [http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/8512 http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/8512]. Accessed on: 14 August 2012.</ref><br />
<br />
Note:<br />
*Parietal cells produce ''intrinsic factor'' (important for vitamin B12 absorption) and ''hydrogen chloride'', i.e. stomach acid.<br />
<br />
==Gross==<br />
*Erythema - corpus involved, antrum spared.<br />
<br />
==Microscopic==<br />
Features:<br />
*Corpus predominant inflammation - usu. moderate or severe - '''key feature'''.<br />
*Loss of parietal cells.<br />
*Increased G cells in the antrum.<br />
**Produce gastrin to stimulate the (missing) parietal cells.<br />
<br />
Notes:<br />
*Compare with other types of ''[[gastric atrophy]]''.<br />
<br />
DDx:<br />
*[[Gastric neuroendocrine tumour]].<br />
*[[Intestinal metaplasia of the stomach]] with chronic inflammation.<br />
*[[Chronic gastritis]].<br />
<br />
===Images===<br />
<gallery><br />
Image: Metaplastic atrophic gastritis - body -- very low mag.jpg | MAG (body) - very low mag.<br />
Image: Metaplastic atrophic gastritis - body -- low mag.jpg | MAG (body) - low mag.<br />
Image: Metaplastic atrophic gastritis - body - alt -- low mag.jpg | MAG (body) - low mag.<br />
Image: Metaplastic atrophic gastritis - body -- intermed mag.jpg | MAG (body) - intermed. mag.<br />
Image: Metaplastic atrophic gastritis - body -- high mag.jpg | MAG (body) - high mag.<br />
Image: Metaplastic atrophic gastritis - body - alt -- high mag.jpg | MAG (body) - high mag.<br />
Image: Metaplastic atrophic gastritis - body -- very high mag.jpg | MAG (body) - very high mag.<br />
<br />
Image: Metaplastic atrophic gastritis - body 2 -- very low mag.jpg | MAG (body) - very low mag. <br />
Image: Metaplastic atrophic gastritis - body 2 -- low mag.jpg | MAG (body) - low mag.<br />
Image: Metaplastic atrophic gastritis - body 2 - alt -- low mag.jpg | MAG (body) - low mag.<br />
Image: Metaplastic atrophic gastritis - body 2 -- intermed mag.jpg | MAG (body) - intermed. mag.<br />
Image: Metaplastic atrophic gastritis - body 2 -- high mag.jpg | MAG (body) - high mag.<br />
Image: Metaplastic atrophic gastritis - body 2 - alt -- high mag.jpg | MAG (body) - high mag.<br />
<br />
<br />
<br />
<br />
Image: Metaplastic atrophic gastritis - antrum -- low mag.jpg | MAG - antrum - low mag.<br />
Image: Metaplastic atrophic gastritis - antrum -- intermed mag.jpg | MAG - antrum - low mag.<br />
Image: Metaplastic atrophic gastritis - antrum - alt -- intermed mag.jpg | MAG - antrum - low mag.<br />
<br />
<br />
</gallery><br />
<br />
==IHC==<br />
Features:<ref name=pmid20975338>{{Cite journal | last1 = Park | first1 = JY. | last2 = Cornish | first2 = TC. | last3 = Lam-Himlin | first3 = D. | last4 = Shi | first4 = C. | last5 = Montgomery | first5 = E. | title = Gastric lesions in patients with autoimmune metaplastic atrophic gastritis (AMAG) in a tertiary care setting. | journal = Am J Surg Pathol | volume = 34 | issue = 11 | pages = 1591-8 | month = Nov | year = 2010 | doi = 10.1097/PAS.0b013e3181f623af | PMID = 20975338 }}</ref><br />
*Chromogranin A +ve (demonstrates ''nodular enterochromaffin-like cell hyperplasia'').<br />
*Gastrin -ve (body of stomach).<br />
**+ve in antrum.<br />
<br />
===Images===<br />
====Body====<br />
<gallery><br />
Image: Metaplastic atrophic gastritis - body - chromogranin A -- very low mag.jpg | MAG (body) - chromogranin A - very low mag. <br />
Image: Metaplastic atrophic gastritis - body - chromogranin A -- low mag.jpg | MAG (body) - chromogranin A - low mag.<br />
Image: Metaplastic atrophic gastritis - body - chromogranin A -- intermed mag.jpg | MAG (body) - chromogranin A - intermed. mag.<br />
Image: Metaplastic atrophic gastritis - body - chromogranin A -- high mag.jpg | MAG (body) - chromogranin A - high mag.<br />
<br />
Image: Metaplastic atrophic gastritis - body - gastrin -- low mag.jpg | MAG (body) - gastrin - low mag. <br />
Image: Metaplastic atrophic gastritis - body - alt - gastrin -- low mag.jpg | MAG (body) - gastrin - low mag.<br />
</gallery><br />
====Antrum====<br />
<gallery><br />
Image: Metaplastic atrophic gastritis - antrum - gastrin -- low mag.jpg | MAG (antrum) - gastrin - low mag.<br />
Image: Metaplastic atrophic gastritis - antrum - gastrin -- intermed mag.jpg | MAG (antrum) - gastrin - intermed. mag.<br />
</gallery><br />
<br />
====www====<br />
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2575912/figure/f5/ Autoimmune gastritis - chromogranin A (nih.gov)].<ref name=pmid18719002>{{Cite journal | last1 = Pritchard | first1 = DM. | last2 = Berry | first2 = D. | last3 = Przemeck | first3 = SM. | last4 = Campbell | first4 = F. | last5 = Edwards | first5 = SW. | last6 = Varro | first6 = A. | title = Gastrin increases mcl-1 expression in type I gastric carcinoid tumors and a gastric epithelial cell line that expresses the CCK-2 receptor. | journal = Am J Physiol Gastrointest Liver Physiol | volume = 295 | issue = 4 | pages = G798-805 | month = Oct | year = 2008 | doi = 10.1152/ajpgi.00015.2008 | PMID = 18719002 }}</ref><br />
**Findings may be seen in hypergastrinemia and nodular enterochromaffin cell-like (ECL) hyperplasia.<br />
<br />
==Sign out==<br />
<pre><br />
Stomach, Body, Biopsy:<br />
- Gastric mucosa with INTESTINAL METAPLASIA, <br />
moderate chronic inactive inflammation and atrophic features, SEE COMMENT.<br />
- NEGATIVE for apparent parietal cells, SEE COMMENT.<br />
- NEGATIVE for Helicobacter-like organisms.<br />
- NEGATIVE for dysplasia and NEGATIVE for malignancy.<br />
<br />
Comment:<br />
Immunostains show rows of Chromogranin A positive cells and a lack of gastrin staining. <br />
<br />
These findings suggest an autoimmune (metaplastic atrophic) gastritis; correlation with blood work and clinical findings is recommended.<br />
</pre><br />
===Block letters===<br />
<pre><br />
STOMACH, BIOPSY:<br />
- SEVERE CHRONIC ACTIVE GASTRITIS WITH EXTENSIVE INTESTINAL METAPLASIA.<br />
- NEGATIVE FOR HELICOBACTER-LIKE ORGANISMS.<br />
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.<br />
<br />
COMMENT:<br />
Parietal cells are not apparent on the H&E stained sections. Immunostains show <br />
rows of Chromogranin A positive cells and a lack of gastrin staining. <br />
<br />
These findings suggest an autoimmune gastritis; correlation with blood work <br />
is suggested.<br />
</pre><br />
<br />
==See also==<br />
*[[Stomach]].<br />
*[[Gastritis]].<br />
**[[Chronic gastritis]].<br />
*[[Anemia]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Stomach]]<br />
[[Category:Diagnosis]]</div>
Michael
https://librepathology.org/w/index.php?title=Gastritis&diff=52821
Gastritis
2024-03-26T00:06:30Z
<p>Michael: /* Etiology */</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Chronic gastritis -- high mag.jpg<br />
| Width =<br />
| Caption = Chronic gastritis. [[H&E stain]].<br />
| Micro = acute: [[PMN]]s in the lamina propria or intraepithelial; (usual) chronic: cluster of plasma cells (mild), several clusters (moderate), +/-mucosal erosions (severe); lymphocytic: intraepithelial lymphocytes (25 lymphocytes/100 epithelial cells)<br />
| Subtypes = [[acute gastritis]], [[chronic gastritis]], focal acute gastritis (pill gastritis), [[lymphocytic gastritis]]<br />
| LMDDx = [[Helicobacter gastritis]], [[collagenous gastritis]], [[Crohn's disease]], [[intestinal metaplasia of the stomach]], [[gastric columnar dysplasia]], [[gastric carcinoma]]<br />
| Stains = [[Diff-Quik stain|Diff-Quik]] -ve, [[Cresyl violet stain|cresyl violet]] -ve<br />
| IHC = Helicobacter -ve <br />
| EM =<br />
| Molecular =<br />
| IF =<br />
| Gross =<br />
| Grossing =<br />
| Site = [[stomach]]<br />
| Assdx = [[intestinal metaplasia of the stomach]] - esp. in chronic gastritis<br />
| Syndromes =<br />
| Clinicalhx = dyspepsia<br />
| Signs =<br />
| Symptoms = upper abdominal pain<br />
| Prevalence = very common<br />
| Bloodwork = H. pylori -ve or previously +ve<br />
| Rads =<br />
| Endoscopy = erythema<br />
| Prognosis = good<br />
| Other = neg. Helicobacter breath test<br />
| ClinDDx = [[reactive gastropathy]]<br />
}}<br />
'''Gastritis''' refers to an inflammatory process that affects the [[stomach]].<br />
<br />
There are several types of gastritis:<br />
*[[Chronic gastritis]].<br />
*[[Helicobacter pylori gastritis]].<br />
*[[Acute gastritis]].<br />
*"Chemical gastritis" - not really a gastritis; the preferred term is ''[[reactive gastropathy]]''.<br />
*[[Autoimmune gastritis]].<br />
*[[Lymphocytic gastritis]].<br />
*[[Collagenous gastritis]].<br />
<br />
This article gives an overview of causes of gastritis. It also deals with ''[[acute gastritis]]'' and the usual ''[[chronic gastritis]]'' without an apparent histologic cause. <br />
<br />
Other forms of gastritis are dealt with in their own articles.<br />
<br />
==General==<br />
*Very common.<br />
*A specific cause is uncommonly identified histologically.<br />
<br />
Clinical:<br />
*Dyspepsia - esp. upper abdominal pain.<br />
<br />
===Etiology===<br />
Gastritis causes:<ref name=Ref_PBoD812-3>{{Ref PBoD|812-3}}</ref><br />
*Infectious:<br />
**[[H. pylori]] infection.<br />
**[[Tuberculosis]].<br />
**Salmonellosis.<br />
**[[CMV]].<br />
**[[EBV]].<br />
**[[Syphilis]].<br />
*Endocrine-related:<br />
**[[Pernicious anemia]].<br />
**[[Diabetes mellitus]] - gastric atony.<br />
*Trauma, e.g. NG tube.<br />
*Vascular, ischemia.<br />
*Autoimmune:<br />
**[[Crohn's disease]].<br />
*Toxins:<br />
**[[Alcohol]]. <br />
**Medications, especially [[NSAID]]s.<br />
**Uremia.<br />
**[[Smoking]] (heavy).<br />
*Radiation.<br />
<br />
===Endoscopic appearance===<br />
*Erythematous.<br />
<br />
==Microscopic==<br />
*Inflammatory cells - see below.<br />
<br />
===Acute gastritis===<br />
*[[AKA]] ''active gastritis''.<br />
<br />
Features:<br />
*Neutrophils - especially when intraepithelial.<br />
<br />
====Focal active gastritis====<br />
DDx:<br />
#Drugs,<ref>{{Cite journal | last1 = Parfitt | first1 = JR. | last2 = Driman | first2 = DK. | title = Pathological effects of drugs on the gastrointestinal tract: a review. | journal = Hum Pathol | volume = 38 | issue = 4 | pages = 527-36 | month = Apr | year = 2007 | doi = 10.1016/j.humpath.2007.01.014 | PMID = 17367604 }}<br />
</ref> esp. [[NSAIDs]].<br />
#Infectious.<br />
#Inflammatory bowel disease.<br />
<br />
===Chronic gastritis===<br />
Features:<br />
*[[Plasma cells]] (in lamina propria).<br />
**Various criteria:<br />
**#Two plasma cells kissing, i.e. two plasma cells touching/overlapping.<br />
**#Three is a crowd, i.e. three plasma cells in close proximity.<br />
<br />
Note:<br />
*Approximately 20% of cases with an inflamed cardia will have [[intestinal metaplasia]].<ref name=pmid10566710>{{Cite journal | last1 = Voutilainen | first1 = M. | last2 = Färkkilä | first2 = M. | last3 = Mecklin | first3 = JP. | last4 = Juhola | first4 = M. | last5 = Sipponen | first5 = P. | title = Chronic inflammation at the gastroesophageal junction (carditis) appears to be a specific finding related to Helicobacter pylori infection and gastroesophageal reflux disease. The Central Finland Endoscopy Study Group. | journal = Am J Gastroenterol | volume = 94 | issue = 11 | pages = 3175-80 | month = Nov | year = 1999 | doi = 10.1111/j.1572-0241.1999.01513.x | PMID = 10566710 }}</ref><br />
<br />
====Images====<br />
<gallery><br />
Image: Chronic gastritis -- intermed mag.jpg | Moderate chronic gastritis - intermed. mag. (WC)<br />
Image: Chronic gastritis -- high mag.jpg | Moderate chronic gastritis - high mag. (WC)<br />
Image: Chronic gastritis -- very high mag.jpg | Moderate chronic gastritis - very high mag. (WC)<br />
</gallery><br />
<br />
===Sydney criteria for gastritis===<br />
A bunch of pathologists in Sydney came-up with criteria... and these were revised in Houston.<ref name=pmid8827022>{{cite journal |author=Dixon MF, Genta RM, Yardley JH, Correa P |title=Classification and grading of gastritis. The updated Sydney System. International Workshop on the Histopathology of Gastritis, Houston 1994 |journal=Am. J. Surg. Pathol. |volume=20 |issue=10 |pages=1161-81 |year=1996 |month=October |pmid=8827022 |doi= |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=20&issue=10&spage=1161}}</ref><br />
<br />
====Classification====<br />
Updated Sydney classification:<ref name=pmid8827022/><br />
{| class="wikitable"<br />
| '''Feature''' || '''Non-atrophic Helicobacter''' || '''Atrophic Helicobacter''' || '''Autoimmune'''<br />
|-<br />
| Inflammation pattern || antral or diffuse || antrum & corpus, mild inflammation || corpus only<br />
|-<br />
| Atrophy & metaplasia || nil || atrophy present, metaplasia at incisura || corpus only<br />
|-<br />
|}<br />
Notes: <br />
*''Corpus'' = gastric body.<br />
*''Incisura'' = angular incisure, incisura angularis (Latin) - notched transition point on lesser curvature of the stomach between pylorus and body.<ref>[http://en.wikipedia.org/wiki/Angular_incisure http://en.wikipedia.org/wiki/Angular_incisure]</ref><br />
<br />
====Severity====<br />
The Sydney group suggests grading severity with the following language:<ref name=pmid8827022/><br />
*Mild.<br />
*Moderate.<br />
*Marked.<br />
<br />
These terms are applied to the parameters described in a biopsy. The Sydney criteria lists ''H. pylori'', ''neutrophils'', ''mononuclear cells'', ''antrum (atrophy)'', ''corpus (atrophy)'' and ''intestinal metaplasia''. The paper that discusses this also give a visual analogue scale.<br />
<br />
Parameters & Severity (adapted from Dixon et al.<ref name=pmid8827022/>):<br />
{| class="wikitable sortable"<br />
! Feature <br />
! Mild <br />
! Moderate<br />
! Marked<br />
|-<br />
| H. pylori <br />
| few touching <br />
| many touching <br />
| piles<br />
|-<br />
| Neutrophils <br />
| few <br />
| bunches <br />
| crowded<br />
|-<br />
| Mononuclear cells <br />
| not touching <br />
| kissing <br />
| partying<br />
|-<br />
|}<br />
<br />
==Sign out==<br />
===Minimal chronic inactive===<br />
<pre><br />
STOMACH, BIOPSY: <br />
- BODY AND ANTRAL-TYPE GASTRIC MUCOSA WITH MINIMAL CHRONIC INACTIVE INFLAMMATION. <br />
- NEGATIVE FOR HELICOBACTER-LIKE ORGANISMS. <br />
- NEGATIVE FOR INTESTINAL METAPLASIA. <br />
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.<br />
</pre><br />
<br />
===Mild chronic inactive===<br />
<pre><br />
Stomach, Antrum, Biopsy:<br />
- Antral-type gastric mucosa with mild chronic inactive inflammation.<br />
- NEGATIVE for Helicobacter-like organisms.<br />
- NEGATIVE for intestinal metaplasia.<br />
- NEGATIVE for dysplasia and NEGATIVE for malignancy.<br />
</pre><br />
<br />
<pre><br />
Stomach, Antrum, Biopsy:<br />
- Antral-type gastric mucosa with mild focal chronic inactive inflammation, and mild focal gland tortousity without smooth muscle hyperplasia.<br />
- NEGATIVE for Helicobacter-like organisms.<br />
- NEGATIVE for intestinal metaplasia.<br />
- NEGATIVE for dysplasia and NEGATIVE for malignancy.<br />
</pre><br />
<br />
====Block letters====<br />
<pre><br />
STOMACH, BIOPSY: <br />
- BODY AND ANTRAL-TYPE GASTRIC MUCOSA WITH MILD CHRONIC INACTIVE INFLAMMATION. <br />
- NEGATIVE FOR HELICOBACTER-LIKE ORGANISMS. <br />
- NEGATIVE FOR INTESTINAL METAPLASIA. <br />
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.<br />
</pre><br />
<br />
====Moderate chronic inactive antrum with mild chronic inactive body====<br />
<pre><br />
STOMACH, BIOPSY:<br />
- ANTRAL-TYPE GASTRIC MUCOSA WITH MODERATE CHRONIC INACTIVE INFLAMMATION.<br />
- BODY-TYPE GASTRIC MUCOSA WITH MILD CHRONIC INACTIVE INFLAMMATION.<br />
- NEGATIVE FOR HELICOBACTER-LIKE ORGANISMS.<br />
- NEGATIVE FOR INTESTINAL METAPLASIA.<br />
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.<br />
</pre><br />
<br />
====Moderate chronic active====<br />
<pre><br />
STOMACH, BIOPSY: <br />
- Body and antral-type gastric mucosa with moderate chronic active inflammation.<br />
- NEGATIVE for Helicobacter-like organisms.<br />
- NEGATIVE for intestinal metaplasia.<br />
- NEGATIVE for dysplasia and NEGATIVE for malignancy. <br />
</pre><br />
<br />
=====Block letters=====<br />
<pre><br />
STOMACH, BIOPSY: <br />
- BODY AND ANTRAL-TYPE GASTRIC MUCOSA WITH MODERATE CHRONIC ACTIVE INFLAMMATION. <br />
- NEGATIVE FOR HELICOBACTER-LIKE ORGANISMS. <br />
- NEGATIVE FOR INTESTINAL METAPLASIA. <br />
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.<br />
</pre><br />
<br />
====Severe active====<br />
<pre><br />
Stomach, Biopsy: <br />
- Antral-type gastric mucosa with severe chronic active inflammation <br />
with evidence of ulceration (fibrin, necro-inflammatory debris).<br />
- NEGATIVE for Helicobacter-like organisms. <br />
- NEGATIVE for intestinal metaplasia. <br />
- NEGATIVE for dysplasia and NEGATIVE for malignancy. <br />
<br />
Comment:<br />
Follow-up is suggested.<br />
</pre><br />
<br />
====Sleeve gastrectomy====<br />
<pre><br />
Partial Stomach, Sleeve Gastrectomy:<br />
- Stomach wall with focal mild chronic inactive inflammation of the mucosa.<br />
- NEGATIVE for Helicobacter-like organisms.<br />
- NEGATIVE for intestinal metaplasia.<br />
- NEGATIVE for dysplasia and NEGATIVE for malignancy.<br />
</pre><br />
<br />
=====Alternate=====<br />
<pre><br />
Partial Stomach, Sleeve Gastrectomy:<br />
- Stomach wall with mild chronic inactive inflammation of the mucosa, otherwise <br />
within normal limits.<br />
</pre><br />
<br />
=====Block letters=====<br />
<pre><br />
STOMACH, GREATER CURVATURE, SLEEVE GASTRECTOMY:<br />
- STOMACH WALL WITH FOCAL MILD CHRONIC ACTIVE INFLAMMATION OF THE MUCOSA.<br />
- NEGATIVE FOR HELICOBACTER-LIKE ORGANISMS.<br />
- NEGATIVE FOR INTESTINAL METAPLASIA.<br />
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.<br />
</pre><br />
<br />
====Micro - inactive====<br />
The sections show gastric body type mucosa with small clusters of plasma cells. There are no intraepithelial neutrophils. Goblet cells are not identified. The epithelium matures normally to the surface. No Helicobacter organisms are seen.<br />
<br />
==See also==<br />
*[[Helicobacter pylori gastritis]].<br />
*[[Collagenous gastritis]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Stomach]]<br />
[[Category:Gastrointestinal pathology]]</div>
Michael
https://librepathology.org/w/index.php?title=Bright_red_blood_per_rectum&diff=52820
Bright red blood per rectum
2024-03-25T23:57:08Z
<p>Michael: </p>
<hr />
<div>'''Bright red blood per rectum''', abbreviated '''BRBPR''', is a nonspecific sign with a longer differential diagnosis.<br />
<br />
''Lower GI bleed'' redirects here.<br />
==Causes==<br />
Classic causes of lower GI bleed (mnemonic ''CHAND''):<br />
*[[Colitis]].<br />
**[[Infectious colitis]].<br />
**[[Ischemic colitis]].<br />
**[[Inflammatory bowel disease]].<br />
**[[Radiation colitis]].<br />
*[[Hemorrhoids]].<br />
*[[Angiodysplasia]].<br />
*Neoplasms - [[colorectal carcinoma]] and others.<br />
*[[Diverticular disease]].<br />
<br />
Other:<br />
*[[Solitary rectal ulcer]].<br />
<br />
==See also==<br />
*[[Gastrointestinal bleeding]].<br />
*[[Upper GI bleed]].<br />
*[[Clinical_history]].<br />
<br />
[[Category:Clinical]]</div>
Michael
https://librepathology.org/w/index.php?title=Bright_red_blood_per_rectum&diff=52819
Bright red blood per rectum
2024-03-25T23:54:31Z
<p>Michael: </p>
<hr />
<div>'''Bright red blood per rectum''', abbreviated '''BRBPR''', is a nonspecific sign with a longer differential diagnosis.<br />
<br />
''Lower GI bleed'' redirects here.<br />
==Causes==<br />
Classic causes of lower GI bleed (mnemonic ''CHAND''):<br />
*[[Colitis]].<br />
**[[Infectious colitis]].<br />
**[[Ischemic colitis]].<br />
**[[Inflammatory bowel disease]].<br />
**[[Radiation colitis]].<br />
*[[Hemorrhoids]].<br />
*[[Angiodysplasia]].<br />
*Neoplasms - [[colorectal carcinoma]] and others.<br />
*[[Diverticular disease]].<br />
<br />
Other:<br />
*[[Solitary rectal ulcer]].<br />
<br />
==See also==<br />
*[[Gastrointestinal bleeding]].<br />
*[[Upper GI bleed]].<br />
<br />
[[Category:Clinical]]</div>
Michael
https://librepathology.org/w/index.php?title=Gastrointestinal_bleeding&diff=52818
Gastrointestinal bleeding
2024-03-25T23:53:36Z
<p>Michael: Created page with "'''Gastrointestinal bleeding''', also '''gastrointestinal bleed''', is bleeding from the gastrointestinal tract. It is generally divided into: *Lower GI bleed. *Upper G..."</p>
<hr />
<div>'''Gastrointestinal bleeding''', also '''gastrointestinal bleed''', is bleeding from the gastrointestinal tract.<br />
<br />
It is generally divided into:<br />
*[[Lower GI bleed]].<br />
*[[Upper GI bleed]].<br />
<br />
<br />
[[Category:Clinical]]<br />
[[Category:Gastrointestinal pathology]]</div>
Michael
https://librepathology.org/w/index.php?title=Upper_GI_bleed&diff=52817
Upper GI bleed
2024-03-25T23:51:36Z
<p>Michael: redirect</p>
<hr />
<div>#redirect [[Clinical_history#Upper_GI_bleed]]</div>
Michael
https://librepathology.org/w/index.php?title=Signet_ring_cell_carcinoma&diff=52816
Signet ring cell carcinoma
2024-03-25T23:49:28Z
<p>Michael: /* Microscopic */</p>
<hr />
<div>{{ Infobox diagnosis<br />
| Name = {{PAGENAME}}<br />
| Image = Signet ring cell carcinoma - very high mag.jpg <br />
| Width =<br />
| Caption = Signet ring cell carcinoma. [[H&E stain]]<br />
| Micro = ovoid cells with abundant cytoplasm and a peripheral crescentic hyperchromatic nucleus <br />
| Subtypes =<br />
| LMDDx = [[serous fat atrophy]], benign histiocytes ([[mucocele]], [[gastric xanthoma|xanthoma]])<br />
| Stains = [[PAS stain]]<br />
| IHC = pankeratin +ve, CD68 -ve<br />
| EM =<br />
| Molecular =<br />
| IF =<br />
| Gross =<br />
| Grossing =<br />
| Site = [[stomach]], [[small intestine]], [[large intestine]], [[breast]], [[pancreas]], [[urinary bladder]], [[prostate gland]], [[lung]]<br />
| Assdx = [[Invasive lobular carcinoma]], [[mucinous carcinoma]]<br />
| Syndromes = [[familial diffuse gastric cancer]]<br />
| Clinicalhx =<br />
| Signs =<br />
| Symptoms =<br />
| Prevalence = uncommon<br />
| Bloodwork =<br />
| Rads =<br />
| Endoscopy = [[linitis plastica]] (classic finding in the stomach)<br />
| Prognosis = poor<br />
| Other =<br />
| ClinDDx =<br />
}}<br />
{{ Infobox external links<br />
| Name = {{PAGENAME}}<br />
| EHVSC = 9982<br />
| pathprotocols = <br />
| wikipedia = Signet ring cell carcinoma<br />
| pathoutlines =<br />
}}<br />
'''Signet ring cell carcinoma''', abbreviated '''SRCC''', is a type of malignant epithelial neoplasm that can arise from a number of places. It is commonly associated with the [[stomach]].<br />
<br />
==General==<br />
*Signet ring cell carcinoma are notoriously easy to miss in a small biopsy.<br />
**It has been said that there are two types of pathologists... those that have missed SRCCs ''and'' those that will miss SRCCs.<br />
*The name comes from the shape of cells. They look like signet rings that are lying flat on the ground and one is looking from above - see microscopic section.<br />
<br />
===Anatomical Site===<br />
It may arise from the:<ref>URL: [http://cancerhelp.cancerresearchuk.org/about-cancer/cancer-questions/what-is-a-signet-cell-cancer http://cancerhelp.cancerresearchuk.org/about-cancer/cancer-questions/what-is-a-signet-cell-cancer]. Accessed on: 7 March 2012.</ref><br />
*[[Esophagus]].<br />
**>50% signet ring cells - as per a study definition.<ref>URL: [https://clinicaltrials.gov/ct2/show/NCT01824966 https://clinicaltrials.gov/ct2/show/NCT01824966]. Accessed on: April 23, 2022.</ref><br />
*[[Stomach]].<br />
*[[Small intestine]]<br />
*[[Large intestine]].<br />
*[[Breast]]<br />
*[[Pancreas]].<br />
*[[Urinary bladder]].<br />
*[[Prostatic_signet_ring_cell_carcinoma|Prostate gland]]. <br />
*[[Lung]].<br />
<br />
==Microscopic==<br />
Features:<br />
*Signet ring cells resemble signet rings.<br />
**They contain a large amount of mucin, which pushes the nucleus to the cell periphery. <br />
**The pool of mucin in a signet ring cell mimics the appearance of the finger hole.<br />
**The nucleus mimics the appearance of the face of the ring in profile.<br />
*Signet ring cells are typically 2-3x the size of a lymphocyte.<br />
**Smaller than the typical adipocyte.<br />
*Often have a crescent-shaped ''or'' ovoid nucleus.<br />
**Capillaries sectioned on their lumen have endothelial cells - the nuclei of these are more spindled.<br />
<br />
Note:<br />
*SRCs are usually close to friend, i.e. they are adjacent to another SRC.<br />
**This helps differentiate SRCs from capillaries sectioned on their lumen.<br />
*The mucin is often clear on H&E... but maybe eosinophilic.<br />
<br />
DDx:<br />
*[[Serous fat atrophy]].<ref name=pmid6222282>{{Cite journal | last1 = Clarke | first1 = BE. | last2 = Brown | first2 = DJ. | last3 = Xipell | first3 = JM. | title = Gelatinous transformation of the bone marrow. | journal = Pathology | volume = 15 | issue = 1 | pages = 85-8 | month = Jan | year = 1983 | doi = | PMID = 6222282 }}</ref><br />
*[[Mucocele]] - muciphages may mimic signet ring cells.<ref name=pmid9591723>{{Cite journal | last1 = De Petris | first1 = G. | last2 = Lev | first2 = R. | last3 = Siew | first3 = S. | title = Peritumoral and nodal muciphages. | journal = Am J Surg Pathol | volume = 22 | issue = 5 | pages = 545-9 | month = May | year = 1998 | doi = | PMID = 9591723 }}</ref><br />
**Muciphages = cytoplasm lightly eosinophilic, multivaculated (classic) or finely reticulated.<br />
*[[Gastric xanthoma]].<br />
*[[Goblet cell adenocarcinoma]] (''crypt cell carcinoma'' or ''goblet cell carcinoid'').<ref name=pmid22933998>{{Cite journal | last1 = Pericleous | first1 = M. | last2 = Lumgair | first2 = H. | last3 = Baneke | first3 = A. | last4 = Morgan-Rowe | first4 = L. | last5 = E Caplin | first5 = M. | last6 = Luong | first6 = TV. | last7 = Thirlwell | first7 = C. | last8 = Gillmore | first8 = R. | last9 = Toumpanakis | first9 = C. | title = Appendiceal goblet cell carcinoid tumour: a case of unexpected lung metastasis. | journal = Case Rep Oncol | volume = 5 | issue = 2 | pages = 332-8 | month = May | year = 2012 | doi = 000339607 | PMID = 22933998 }}</ref><br />
*Ischemic mucosal changes - cells within the lumen.<ref name=pmid22082777>{{Cite journal | last1 = Dhingra | first1 = S. | last2 = Wang | first2 = H. | title = Nonneoplastic signet-ring cell change in gastrointestinal and biliary tracts: a pitfall for overdiagnosis. | journal = Ann Diagn Pathol | volume = 15 | issue = 6 | pages = 490-6 | month = Dec | year = 2011 | doi = 10.1016/j.anndiagpath.2011.07.006 | PMID = 22082777 }}</ref> <br />
<br />
===Images===<br />
<gallery><br />
Image:Medieval Signet Ring (FindID 226489).jpg|A medieval signet ring. On the left it is lying flat and looks similar to a so called signet ring cell. (WC/Staves)<br />
</gallery><br />
====www====<br />
*[http://www.engravingarts.com/sales/LVX2.jpg Signet rings (engravingarts.com)]. <br />
====Case - stomach====<br />
<gallery><br />
Image:Signet_ring_cell_carcinoma_-_low_mag.jpg | SRCC - low mag. - demonstrating that it can be subtle. (WC/Nephron)<br />
Image:Signet_ring_cell_carcinoma_-_high_mag.jpg | SRCC - high mag. (WC/Nephron)<br />
Image:Signet_ring_cell_carcinoma_-_very_high_mag.jpg | SRCC - very high mag. (WC/Nephron)<br />
</gallery><br />
====Case - bladder====<br />
<gallery><br />
Image: Signet ring cell carcinoma of the urinary bladder -- intermed mag.jpg | SRCC - bladder - intermed. mag.<br />
Image: Signet ring cell carcinoma of the urinary bladder - alt -- intermed mag.jpg | SRCC - bladder - intermed. mag.<br />
Image: Signet ring cell carcinoma of the urinary bladder -- high mag.jpg | SRCC - bladder - high mag.<br />
Image: Signet ring cell carcinoma of the urinary bladder -- very high mag.jpg | SRCC - bladder - very high mag.<br />
</gallery><br />
<br />
====Additional cases====<br />
<gallery><br />
Image:Signet_ring_cells_5.jpg | SRCC metastasis. (WC/Nephron)<br />
Image:Gastric_signet_ring_cell_carcinoma_histopatholgy_(1).jpg | SRCC. (WC/KGH)<br />
Image:Gastric_signet_ring_cell_carcinoma_histopatholgy_(2)_PAS_stain.jpg | SRCC - PAS stain. (WC/KGH)<br />
</gallery><br />
<br />
==Stains==<br />
*Alcian blue-PAS stain +ve (stomach and colorectum).<ref name=pmid23573317>{{Cite journal | last1 = Terada | first1 = T. | title = An immunohistochemical study of primary signet-ring cell carcinoma of the stomach and colorectum: I. Cytokeratin profile in 42 cases. | journal = Int J Clin Exp Pathol | volume = 6 | issue = 4 | pages = 703-10 | month = | year = 2013 | doi = | PMID = 23573317 }}</ref><br />
*PAS stain +ve.<br />
*[[Mucicarmine stain]] -ve (stomach).{{fact}}<br />
<br />
Note:<br />
*Mucin staining varies somewhat by the anatomical site.<ref name=pmid16740030>{{cite journal |authors=Nguyen MD, Plasil B, Wen P, Frankel WL |title=Mucin profiles in signet-ring cell carcinoma |journal=Arch Pathol Lab Med |volume=130 |issue=6 |pages=799–804 |date=June 2006 |pmid=16740030 |doi=10.5858/2006-130-799-MPISCC |url=}}</ref><br />
<br />
==IHC==<br />
*AE1/AE3 +ve.<br />
*CK7 +ve (usually).<br />
<br />
==See also==<br />
*[[Cancer]].<br />
*[[Starvation]].<br />
<br />
==References==<br />
{{Reflist|2}}<br />
<br />
[[Category:Gastrointestinal pathology]]<br />
[[Category:Diagnosis]]</div>
Michael