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SMARCB1-deficient renal medullary carcinoma (view source)
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# | '''Renal medullary carcinoma''', abbreviated '''RMC''', is a rare [[malignant]] [[kidney tumour]] associated with sickle cell trait and a poor prognosis. | ||
==General== | |||
*Rare. | |||
*Usually young adults. | |||
*Associated with sickle cell trait (heterozygotes for the sickle cell allele).<ref name=pmid7528470>{{cite journal |author=Davis CJ, Mostofi FK, Sesterhenn IA |title=Renal medullary carcinoma. The seventh sickle cell nephropathy |journal=Am. J. Surg. Pathol. |volume=19 |issue=1 |pages=1–11 |year=1995 |month=January |pmid=7528470 |doi= |url=}}</ref> | |||
*Aggressive/poor prognosis.<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> | |||
Aside: | |||
*Kidney disease associated with sickle cell disorders:<ref name=pmid7528470/> | |||
*#Papillary necrosis. | |||
*#[[Nephrotic syndrome]]. | |||
*#Renal [[infarction]]. | |||
*#[[Pyelonephritis]]. | |||
==Gross== | |||
Features:<ref name=pmid17643096>{{cite journal |author=Watanabe IC, Billis A, Guimarães MS, ''et al.'' |title=Renal medullary carcinoma: report of seven cases from Brazil |journal=Mod. Pathol. |volume=20 |issue=9 |pages=914–20 |year=2007 |month=September |pmid=17643096 |doi=10.1038/modpathol.3800934 |url=}}</ref> | |||
*Well circumscribed. | |||
*Renal medulla. | |||
==Microscopic== | |||
Features:<ref name=pmid7528470/><ref name=pmid17643096>{{cite journal |author=Watanabe IC, Billis A, Guimarães MS, ''et al.'' |title=Renal medullary carcinoma: report of seven cases from Brazil |journal=Mod. Pathol. |volume=20 |issue=9 |pages=914–20 |year=2007 |month=September |pmid=17643096 |doi=10.1038/modpathol.3800934 |url=}}</ref> | |||
*Variable architecture: | |||
**Reticular - classic. | |||
**[[Adenoid cystic carcinoma]]-like appearance: | |||
***Cystic spaces. | |||
**Yolk sac-like. | |||
**Tubular. | |||
*Desmoplastic stroma - prominent. | |||
*Inflammation: | |||
**Lymphocytes. | |||
**Neutrophils - margination in vessels. | |||
DDx: | |||
*[[Yolk sac tumour]]. | |||
*[[Collecting duct carcinoma]]. | |||
===Image=== | |||
*[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)]. | |||
==IHC== | |||
*SMARCB1 (INI1) -ve.<ref name=Ref_WMSP286>{{Ref WMSP|286}}</ref> | |||
==See also== | |||
*[[Kidney tumours]]. | |||
*[[Collecting duct carcinoma]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Kidney tumours]] | |||