Difference between revisions of "Kidney tumours"

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=Pediatric=
=Pediatric=
==Wilms tumour==
{{Pediatric kidney tumours}}
*[[AKA]] nephroblastoma, AKA Wilms' tumour.


===General===
The most common is [[nephroblastoma]] (Wilms tumour).
*Common abdominal [[pediatric pathology|pediatric]] tumour.
*May be associated with a syndrome:<ref>URL: [http://emedicine.medscape.com/article/989398-overview http://emedicine.medscape.com/article/989398-overview]. Accessed on: 9 March 2011.</ref>
**WAGR syndrome (Wilms tumour, Aniridia (absence of iris), GU abnormalities, Retardation).<ref>{{OMIM|194072}}</ref>
**[[Beckwith-Wiedemann syndrome]].<ref>{{OMIM|130650}}</ref>
**Denys-Drash syndrome.<ref>{{OMIM|194080}}</ref>


===Gross===
Others include:
*Lobulated tan mass.
*[[Metanephric stromal tumour]].
 
*[[Metanephric adenofibroma]]
Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)].
*[[Metanephric adenoma]].
 
*[[Clear cell sarcoma of the kidney]].
===Microscopic===
Features - classically three components (blastema, immature stroma, tubules):<ref name=Ref_PCPBoD8_254-5>{{Ref PCPBoD8|254-5}}</ref>
#Malignant [[Small round blue cell tumours|small round blue cells]] ("blastema"):
#*Size = ~ 2x RBC diameter.
#*Nuclear pleomorphism (variation of size, shape and staining).
#**Irregular nuclear membrane - '''important'''.
#*Scant/difficult to discern cytoplasm - basophilic (light blue).
#*Mitoses - common.
#Stroma ("immature stroma"):
#*Spindle cells:
#**Elliptical nuclear membrane.
#**Abundant loose cytoplasm.
#Tubular structures ("tubules"):
#*Usually clustered.
#*Vaguely resemble a glomerulus.
#*Usu. have a central (clear/white) space surrounded by a rim of intensely eosinophilic cytoplasm.
#*Nuclei of tubular structures often elongated and palisaded.
 
Other findings:
*Commonly seen in association with ''nephrogenic rests''.
**Cluster of cells small (blue) cells; lack nuclear atypia seen in Wilms tumour.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970416-8 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970416-8]. Accessed on: 28 March 2011.</ref>
*+/-Heterologous elements (skeletal muscle, smooth muscle adipose tissue, cartilage).<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
**Heterologous = doesn't normally belong there.<ref>URL: [http://www.biology-online.org/dictionary/Heterologous http://www.biology-online.org/dictionary/Heterologous]. Accessed on: 1 October 2011.</ref>
 
DDx:
*Metanephric adenoma.
*Nephrogenic nests.
*Other small round cell tumours.
*[[Synovial sarcoma]] - esp. in adults.
 
Notes:
*Palisade = fence made of stakes driven into the ground.<ref>URL: [http://www.thefreedictionary.com/palisaded http://www.thefreedictionary.com/palisaded]. Accessed on: 2 February 2011.</ref>
*Approximately 30-40% Wilms tumour cases have nephrogenic rests.<ref name=pmid8047084>{{cite journal |author=Coppes MJ, Haber DA, Grundy PE |title=Genetic events in the development of Wilms' tumor |journal=N. Engl. J. Med. |volume=331 |issue=9 |pages=586–90 |year=1994 |month=September |pmid=8047084 |doi=10.1056/NEJM199409013310906 |url=}}</ref>
*The three phases are also called ''blastemal, epithelial and stromal''.<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
 
Images:
*[http://www.biologydisease.com/images/kidney/nephrogenic-rests/nephrogenic-rest.jpg.php Nephrogenic rests (biologydisease.com)].
*[http://www.webpathology.com/image.asp?n=1&Case=73 Wilms tumour (webpathology.com)].
*[http://commons.wikimedia.org/wiki/File:Wilms_tumour_-_low_mag.jpg Wilms tumour - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Wilms_tumour_-_very_high_mag.jpg Wilms tumour - very high mag. (WC)].
 
====Anaplasia====
Subclassified as:<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
#Focal anaplasia.
#Diffuse anaplasia.
 
Criteria (all of the following):<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
#Atypical mitoses.
#Nuclear hyperchromasia.
#Nuclear size variation (of the tumour cells) > 3x.
 
===IHC===
*WT-1 +ve.
 
==Metanephric stromal tumour==
*Abbreviated ''MST''.
===General===
*Infants and children.
*Diagnosed as [[mesoblastic nephroma]] in the past.<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
*Benign and rare.<ref name=pmid11549506>{{Cite journal  | last1 = Palese | first1 = MA. | last2 = Ferrer | first2 = F. | last3 = Perlman | first3 = E. | last4 = Gearhart | first4 = JP. | title = Metanephric stromal tumor: a rare benign pediatric renal mass. | journal = Urology | volume = 58 | issue = 3 | pages = 462 | month = Sep | year = 2001 | doi =  | PMID = 11549506 }}</ref>
 
===Gross===
Features:<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
*Usu. renal medulla.
*Solid or cystic.
 
===Microscopic===
Features:<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
*"Collarettes" - tumour cells surround blood vessels ''or'' renal tubules.
*Spindle cells/stellate cells.
*Variable cellular density - imparts a nodular appearance at low power.
*Scant cytoplasm.
*Induces epithelioid morphology in smooth muscle cells.
 
Notes:
*+/-Heterologous elements.
 
DDx:
*Clear cell sarcoma of the kidney.<ref name=pmid11549506/>
*[[Mesoblastic nephroma]].
 
==Metanephric adenofibroma==
===General===
*Adults and children.
 
===Microsopic===
Features:<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
*Has features of:
*#Stromal component.
*#*Similar to [[metanephric stromal tumour]].
*#Epithelial nodules.
*#*Similar to [[metanephric adenoma]].


=See also=
=See also=
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