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Difference between revisions of "Wilms tumour"
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# | '''Wilms tumour''', also '''nephroblastoma''' and '''Wilms' tumour''', is the most common [[Pediatric_kidney_tumours|pediatric kidney tumour]]. | ||
==General== | |||
*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== | |||
*Lobulated tan mass. | |||
Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)]. | |||
==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]], biphasic - especially 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=== | |||
<gallery> | |||
Image:Wilms_tumour_-_low_mag.jpg | Wilms tumour - low mag. (WC/Nephron) | |||
Image:Wilms tumour - intermed mag.jpg | Wilms tumour - intermed. mag. (WC/Nephron) | |||
Image:Wilms tumour - high mag.jpg | Wilms tumour - high mag. (WC/Nephron) | |||
Image:Wilms_tumour_-_very_high_mag.jpg | Wilms tumour - very high mag. (WC/Nephron) | |||
</gallery> | |||
www: | |||
*[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)]. | |||
===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. | |||
==See also== | |||
*[[Pediatric kidney tumours]]. | |||
*[[Kidney tumours]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Pediatric kidney tumours]] | |||