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Difference between revisions of "SMARCB1-deficient renal medullary carcinoma"
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SMARCB1-deficient renal medullary carcinoma (view source)
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = | | Image = Renal medullary carcinoma - 2 -- high mag.jpg | ||
| Width = | | Width = | ||
| Caption = | | Caption = Renal medullary carcinoma (right of image), reactive urothelium and sickled [[red blood cell]]s. [[H&E stain]]. (WC/Nephron) | ||
| Synonyms = | | Synonyms = | ||
| Micro = | | Micro = | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[collecting duct carcinoma]], [[yolk sac tumour]] | | LMDDx = [[collecting duct carcinoma]], [[yolk sac tumour]], [[hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma]], [[ALK translocation renal cell carcinoma]] | ||
| Stains = | | Stains = | ||
| IHC = INI1 -ve, vimentin +ve, EMA +ve | | IHC = INI1 -ve, vimentin +ve, EMA +ve | ||
| Line 14: | Line 14: | ||
| IF = | | IF = | ||
| Gross = well-circumscribed mass in renal medulla | | Gross = well-circumscribed mass in renal medulla | ||
| Grossing = | | Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]] | ||
| Staging = [[kidney cancer staging]] | |||
| Site = [[kidney]], medulla - see ''[[kidney tumours]]'' | | Site = [[kidney]], medulla - see ''[[kidney tumours]]'' | ||
| Assdx = [[sickle cell disease]] or sickle cell trait | | Assdx = [[sickle cell disease]] or sickle cell trait | ||
| Line 48: | Line 49: | ||
==Gross== | ==Gross== | ||
Features:<ref name=pmid17643096>{{ | Features:<ref name=pmid17643096>{{Cite journal | last1 = Watanabe | first1 = IC. | last2 = Billis | first2 = A. | last3 = Guimarães | first3 = MS. | last4 = Alvarenga | first4 = M. | last5 = de Matos | first5 = AC. | last6 = Cardinalli | first6 = IA. | last7 = Filippi | first7 = RZ. | last8 = de Castro | first8 = MG. | last9 = Suzigan | first9 = S. | title = Renal medullary carcinoma: report of seven cases from Brazil. | journal = Mod Pathol | volume = 20 | issue = 9 | pages = 914-20 | month = Sep | year = 2007 | doi = 10.1038/modpathol.3800934 | PMID = 17643096 |url = http://www.nature.com/modpathol/journal/v20/n9/full/3800934a.html }}</ref> | ||
*Well circumscribed. | *Well circumscribed. | ||
*Renal medulla. | *Renal medulla. | ||
==Microscopic== | ==Microscopic== | ||
Features:<ref name=pmid7528470/><ref name=pmid17643096>{{ | Features:<ref name=pmid7528470/><ref name=pmid17643096>{{Cite journal | last1 = Watanabe | first1 = IC. | last2 = Billis | first2 = A. | last3 = Guimarães | first3 = MS. | last4 = Alvarenga | first4 = M. | last5 = de Matos | first5 = AC. | last6 = Cardinalli | first6 = IA. | last7 = Filippi | first7 = RZ. | last8 = de Castro | first8 = MG. | last9 = Suzigan | first9 = S. | title = Renal medullary carcinoma: report of seven cases from Brazil. | journal = Mod Pathol | volume = 20 | issue = 9 | pages = 914-20 | month = Sep | year = 2007 | doi = 10.1038/modpathol.3800934 | PMID = 17643096 |url = http://www.nature.com/modpathol/journal/v20/n9/full/3800934a.html }}</ref> | ||
*Variable architecture: | *Variable architecture: | ||
**Reticular - classic. | **Reticular - classic. | ||
| Line 60: | Line 61: | ||
**Yolk sac-like. | **Yolk sac-like. | ||
**Tubular. | **Tubular. | ||
*Desmoplastic stroma - prominent. | *[[Desmoplastic stroma]] - prominent. | ||
*Inflammation: | *Inflammation: | ||
**Lymphocytes. | **Lymphocytes. | ||
| Line 68: | Line 69: | ||
*[[Yolk sac tumour]]. | *[[Yolk sac tumour]]. | ||
*[[Collecting duct carcinoma]]. | *[[Collecting duct carcinoma]]. | ||
*[[Hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma]]. | |||
*[[ALK translocation renal cell carcinoma]]. | |||
===Images=== | ===Images=== | ||
====Case==== | |||
<gallery> | |||
Image: Renal medullary carcinoma -- low mag.jpg | RMC - low mag. | |||
Image: Renal medullary carcinoma -- intermed mag.jpg | RMC - intermed. mag. | |||
Image: Renal medullary carcinoma -- high mag.jpg | RMC - high mag. | |||
Image: Renal medullary carcinoma - alt1 -- high mag.jpg | RMC - high mag. | |||
Image: Renal medullary carcinoma - alt2 -- high mag.jpg | RMC - high mag. | |||
Image: Renal medullary carcinoma -- very high mag.jpg | RMC - very high mag. | |||
Image: Renal medullary carcinoma - 2 -- intermed mag.jpg | RMC - intermed. mag. | |||
Image: Renal medullary carcinoma - 2 -- high mag.jpg | RMC - high mag. | |||
Image: Renal medullary carcinoma - 2 -- very high mag.jpg | RMC - very high mag. | |||
Image: Urothelium with reactive changes and RMC -- intermed mag.jpg | URC and RMC - intermed. mag. | |||
Image: Urothelium with reactive changes and RMC -- high mag.jpg | URC and RMC - high mag. | |||
Image: Urothelium with reactive changes -- very high mag.jpg | Urothelium with RC - very high mag. | |||
</gallery> | |||
====www==== | |||
*[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)]. | *[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)]. | ||
*[http://www.nature.com/modpathol/journal/v22/n2s/fig_tab/modpathol200970f5.html#figure-title RMC - low mag. (nature.com)].<ref name=pmid19494850/> | *[http://www.nature.com/modpathol/journal/v22/n2s/fig_tab/modpathol200970f5.html#figure-title RMC - low mag. (nature.com)].<ref name=pmid19494850/> | ||
| Line 77: | Line 98: | ||
==IHC== | ==IHC== | ||
Features:<ref name=pmid18327209>{{cite journal |author=Cheng JX, Tretiakova M, Gong C, Mandal S, Krausz T, Taxy JB |title=Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior |journal=Mod. Pathol. |volume=21 |issue=6 |pages=647–52 |year=2008 |month=June |pmid=18327209 |doi=10.1038/modpathol.2008.44 |url=}}</ref> | Features:<ref name=pmid18327209>{{cite journal |author=Cheng JX, Tretiakova M, Gong C, Mandal S, Krausz T, Taxy JB |title=Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior |journal=Mod. Pathol. |volume=21 |issue=6 |pages=647–52 |year=2008 |month=June |pmid=18327209 |doi=10.1038/modpathol.2008.44 |url=}}</ref> | ||
*SMARCB1 (INI1) -ve.<ref name=Ref_WMSP286>{{Ref WMSP|286}}</ref> | *[[SMARCB1]] (INI1) -ve.<ref name=Ref_WMSP286>{{Ref WMSP|286}}</ref> | ||
*Vimentin +ve. | *Vimentin +ve. | ||
*EMA +ve. | *EMA +ve. | ||
*CAM5.2 +ve. | *CAM5.2 +ve. | ||
*AE1/AE3 +ve. | *AE1/AE3 +ve. | ||
*Cyclin D1 +ve.<ref name=pmid22686875/> | |||
==See also== | ==See also== | ||