Difference between revisions of "Cystic nephroma"

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(Replaced content with "'''Cystic nephroma''' refers to two morphologically identical tumours with different molecular changes: *Cystic nephroma, adult type - do '''not''' have DICER1 muation...")
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{{ Infobox diagnosis
'''Cystic nephroma''' refers to two morphologically identical tumours with different molecular changes:
| Name      = {{PAGENAME}}
*[[Cystic nephroma, adult type]] - do '''not''' have DICER1 muations.
| Image      = Cystic nephroma - alt -- high mag.jpg
*[[Cystic nephroma, juvenile type]] - DICER1 mutations present.
| Width      =
| Caption    = Micrograph of cystic nephroma showing the characteristic stroma and hobnailed epithelium. [[H&E stain]].
| Synonyms  =
| Micro      = cysts lined by simple epithelium with hobnailing, stroma has an ovarian look (blue, spindle cells)
| Subtypes  =
| LMDDx      = [[mixed epithelial stromal tumour]], [[localized cystic disease of the kidney]]
| Stains    =
| IHC        = ER +ve, PR +ve, CD10 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      = bubble wrap-like appearance
| Grossing  =
| Site      = [[kidney]] - see ''[[kidney tumours]]''
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    = other cystic kidney tumours
| Tx        =
}}
'''Cystic nephroma''' is a rare, benign [[kidney tumours|kidney tumour]].


Turbiner ''et al.''<ref name=pmid17414095>{{Cite journal  | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref>  have suggested that ''cystic nephroma'' and ''[[mixed epithelial and stromal tumour]] (MEST)'' are one tumour; the lumping term is ''[[renal epithelial and stromal tumour]]'' (abbreviated ''REST'').
{{disambiguation}}
 
[[Category:Genitourinary pathology]]
==General==
*Rare.<ref name=pmid20427881>{{Cite journal  | last1 = Mohanty | first1 = D. | last2 = Jain | first2 = BK. | last3 = Agrawal | first3 = V. | last4 = Gupta | first4 = A. | title = Cystic nephroma: a diagnostic dilemma. | journal = Saudi J Kidney Dis Transpl | volume = 21 | issue = 3 | pages = 518-20 | month = May | year = 2010 | doi =  | PMID = 20427881 }}</ref>
*Good prognosis/benign.<ref name=pmid20427881/><ref name=pmid19172100/>
*Considered to be on a spectrum with ''cystic partially differentiated [[nephroblastoma]]'' and ''[[Wilms tumour]].<ref name=pmid19172100>{{Cite journal  | last1 = van den Hoek | first1 = J. | last2 = de Krijger | first2 = R. | last3 = van de Ven | first3 = K. | last4 = Lequin | first4 = M. | last5 = van den Heuvel-Eibrink | first5 = MM. | title = Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas. | journal = Urol Int | volume = 82 | issue = 1 | pages = 65-70 | month =  | year = 2009 | doi = 10.1159/000176028 | PMID = 19172100 }}</ref>
 
==Gross==
*Bubble wrap-like appearance.
 
===Image===
<gallery>
Image:Bubble_Wrap.jpg| Bubble wrap. (WC)
</gallery>
www:
*[http://www.sjkdt.org/viewimage.asp?img=SaudiJKidneyDisTranspl_2010_21_3_518_62699_u2.jpg Cystic nephroma (sjkdt.org)].<ref name=pmid20427881/>
==Microscopic==
Features:
*Cysts lined by simple epithelium with hobnailing - '''key feature'''.
*Stroma has an ovarian look:
**''Blue'' (basophilic).
**''Spindle cells''.
 
Notes:
*Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts.
 
DDx:
*[[Mixed epithelial stromal tumour]].
*[[Localized cystic disease of the kidney]].
 
===Images===
====Case 1====
<gallery>
Image:Cystic_nephroma_low_mag.jpg |Cystic nephroma - low mag. (WC/Nephron)
Image:Cystic_nephroma_intermed_mag.jpg |Cystic nephroma - intermed. mag. (WC/Nephron)
</gallery>
====Case 2====
<gallery>
Image: Cystic nephroma -- low mag.jpg | CN - low mag. (WC/Nephron)
Image: Cystic nephroma -- intermed mag.jpg | CN - intermed. mag. (WC/Nephron)
Image: Cystic nephroma -- high mag.jpg | CN - high mag. (WC/Nephron)
Image: Cystic nephroma - alt -- high mag.jpg | CN - high mag. (WC/Nephron)
Image: Cystic nephroma -- very high mag.jpg | CN - very high mag. (WC/Nephron)
 
Image: Cystic nephroma - a -- low mag.jpg | CN - low mag. (WC/Nephron)
Image: Cystic nephroma - b -- low mag.jpg | CN - low mag. (WC/Nephron)
Image: Cystic nephroma - b -- intermed mag.jpg | CN - intermed. mag. (WC/Nephron)
Image: Cystic nephroma - b -- high mag.jpg | CN - high mag. (WC/Nephron)
</gallery>
 
==IHC==
Features:
*ER +ve.
*PR +ve.
*CD10 +ve.
 
==See also==
*[[Kidney tumours]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Kidney tumours]]

Revision as of 18:22, 13 March 2016

Cystic nephroma refers to two morphologically identical tumours with different molecular changes: