Difference between revisions of "SMARCB1-deficient renal medullary carcinoma"

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#redirect [[Kidney_tumours#Renal_medullary_carcinoma]]
'''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]]

Revision as of 04:12, 3 June 2014

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).[1]
  • Aggressive/poor prognosis.[2]

Aside:

Gross

Features:[2]

  • Well circumscribed.
  • Renal medulla.

Microscopic

Features:[1][2]

  • Variable architecture:
  • Desmoplastic stroma - prominent.
  • Inflammation:
    • Lymphocytes.
    • Neutrophils - margination in vessels.

DDx:

Image

IHC

  • SMARCB1 (INI1) -ve.[3]

See also

References

  1. ↑ 1.0 1.1 1.2 Davis CJ, Mostofi FK, Sesterhenn IA (January 1995). "Renal medullary carcinoma. The seventh sickle cell nephropathy". Am. J. Surg. Pathol. 19 (1): 1–11. PMID 7528470.
  2. ↑ 2.0 2.1 2.2 Watanabe, IC.; Billis, A.; Guimarães, MS.; Alvarenga, M.; de Matos, AC.; Cardinalli, IA.; Filippi, RZ.; de Castro, MG. et al. (Sep 2007). "Renal medullary carcinoma: report of seven cases from Brazil.". Mod Pathol 20 (9): 914-20. doi:10.1038/modpathol.3800934. PMID 17643096. http://www.nature.com/modpathol/journal/v20/n9/full/3800934a.html. Cite error: Invalid <ref> tag; name "pmid17643096" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid17643096" defined multiple times with different content
  3. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 286. ISBN 978-0781765275.
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