|
|
Line 1: |
Line 1: |
| {{ 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]] | |