Cystic nephroma pathophysiology: Difference between revisions

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{{CMG}}; {{AE}} {{SC}}
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==Overview==
==Overview==
On gross pathology, large, unilateral, multiloculated, and well-encapsulated [[cystic]] [[tumor]] is the characteristic finding of cystic nephroma. On microscopic histopathological analysis, [[cyst]]s lined by a simple [[epithelium]] with a hobnail morphology and [[ovary|ovarian]]-like [[stroma]] are characteristic findings of cystic nephroma.


===Pathologic diagnosis===
==Gross Pathology==
[[Image:Cystic nephroma high mag.jpg|thumb|right|250px|High magnification micrograph of a '''cystic nephroma''' showing the characteristic [[simple epithelium]] with hobnail morphology, and the [[ovary|ovarian]]-like [[stroma of ovary|stroma]]. [[H&E stain]].]]
Characteristic findings of cystic nephroma on gross pathology include:
The characteristics of cystic nephromas are:
* Large, unilateral, and multiloculated cystic [[tumor]]
*[[Cyst]]s lined by a simple epithelium with a ''hobnail morphology'', i.e. the nuclei of the cyst lining epithelium bulges into the lumen of the cysts,
* Well-encapsulated
*[[w:ovary|Ovarian]]-like [[stroma (animal tissue)|stroma]] that has a:
* [[Nodular]] surface
**[[w:spindle cell|Spindle cell]] morphology, and has a
**Basophilic [[w:cytoplasm|cytoplasm]].


Cystic nephromas have an immunostaining pattern like ovarian stroma; they are positive for:
==Microscopic Pathology==
*[[w:Estrogen receptor|Estrogen receptor (ER)]],
The characteristics of cystic nephromas include:
*[[w:Progesterone receptor|Progesterone receptor (PR)]] and
*[[Cyst]]s lined by a simple epithelium with a hobnail morphology(i.e. the nuclei of the cyst lining epithelium bulges into the [[lumen]] of the [[cyst]]s)
*[[w:CD10|CD10]].
*[[ovary|Ovarian]]-like [[stroma]]
:*[[Spindle cell]] morphology
:*[[Basophilic]] [[cytoplasm]]
 
<gallery>
Image:
Cystic nephroma high mag.jpg|Cystic nephroma<ref>Cystic nephroma. Wikipedia 2015. https://en.wikipedia.org/wiki/Cystic_nephroma#/media/File:Cystic_nephroma_high_mag.jpg Accessed on October, 30 2015 </ref>
</gallery>
 
==Genetics==
* [[DICER1]] mutations are involved in the pathogenesis of cystic nephroma.<ref name="pmid24481001">{{cite journal |vauthors=Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, A Schultz K, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Hill DA |title=DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma |journal=[[Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc]] |volume=27 |issue=9 |pages=1267–80 |year=2014 |pmid=24481001 |pmc=4117822 |doi=10.1038/modpathol.2013.242 |url=http://dx.doi.org/10.1038/modpathol.2013.242}}</ref>
 
* [[DICER1]] is a human [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: DICER1 Dicer1, Dcr-1 homolog (Drosophila)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=23405| accessdate = }}</ref> This gene encodes a protein possessing an RNA helicase motif containing a DEXH box in its amino terminus and an RNA motif in the carboxy terminus. The encoded protein functions as a ribonuclease and is required by the RNA interference and small temporal RNA (stRNA) pathways to produce the active small RNA component that represses gene expression. Two transcript variants encoding the same protein have been identified for this gene.<ref name="entrez">{{cite web | title = Entrez Gene: DICER1 Dicer1, Dcr-1 homolog (Drosophila)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=23405| accessdate = }}</ref>


==References==
==References==

Latest revision as of 15:01, 1 December 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]

Overview

On gross pathology, large, unilateral, multiloculated, and well-encapsulated cystic tumor is the characteristic finding of cystic nephroma. On microscopic histopathological analysis, cysts lined by a simple epithelium with a hobnail morphology and ovarian-like stroma are characteristic findings of cystic nephroma.

Gross Pathology

Characteristic findings of cystic nephroma on gross pathology include:

  • Large, unilateral, and multiloculated cystic tumor
  • Well-encapsulated
  • Nodular surface

Microscopic Pathology

The characteristics of cystic nephromas include:

  • Cysts lined by a simple epithelium with a hobnail morphology(i.e. the nuclei of the cyst lining epithelium bulges into the lumen of the cysts
  • Ovarian-like stroma

Genetics

  • DICER1 mutations are involved in the pathogenesis of cystic nephroma.[2]
  • DICER1 is a human gene.[3] This gene encodes a protein possessing an RNA helicase motif containing a DEXH box in its amino terminus and an RNA motif in the carboxy terminus. The encoded protein functions as a ribonuclease and is required by the RNA interference and small temporal RNA (stRNA) pathways to produce the active small RNA component that represses gene expression. Two transcript variants encoding the same protein have been identified for this gene.[3]

References

  1. Cystic nephroma. Wikipedia 2015. https://en.wikipedia.org/wiki/Cystic_nephroma#/media/File:Cystic_nephroma_high_mag.jpg Accessed on October, 30 2015
  2. Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, A Schultz K, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Hill DA (2014). "DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma". Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc. 27 (9): 1267–80. doi:10.1038/modpathol.2013.242. PMC 4117822. PMID 24481001.
  3. 3.0 3.1 "Entrez Gene: DICER1 Dicer1, Dcr-1 homolog (Drosophila)".

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