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Pediatric kidney tumours (view source)

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This article cover '''pediatric kidney tumours'''.  Adult kidney tumour are covered in ''[[kidney tumours]]'' article.
This article cover '''pediatric [[kidney]] tumours'''.  Adult kidney tumour are covered in ''[[kidney tumours]]'' article. An introduction to pediatric pathology is in the ''[[pediatric pathology]]'' article.


==Wilms tumour==
=Overview=
*[[AKA]] nephroblastoma, AKA Wilms' tumour.
These are diagnoses pediatric pathologists make.
 
===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===
The most common ones are:
*Lobulated tan mass.
#[[Wilms tumour]].
#[[Clear cell sarcoma of the kidney]].


Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)].
Other renal tumours (not covered in this article):
*Postneuroblastoma RCC.
*[[Neuroblastoma]].
*[[PNET]].
*[[Synovial sarcoma]].
*[[Lymphoma]].


===Microscopic===
The [[Kidney_tumours#Renal_translocation_carcinomas|renal translocation carcinomas]] are covered in:
Features - classically three components (blastema, immature stroma, tubules):<ref name=Ref_PCPBoD8_254-5>{{Ref PCPBoD8|254-5}}</ref>
*[[Renal tumour with Xp11.2 translocation]].
#Malignant [[Small round blue cell tumours|small round blue cells]] ("blastema"):
*[[Renal tumour with t(6;11) translocation]].
#*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:
=Specific tumours=
*Commonly seen in association with ''nephrogenic rests''.
==Wilms tumour==
**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>
*[[AKA]] nephroblastoma, AKA Wilms' tumour.
*+/-Heterologous elements (skeletal muscle, smooth muscle adipose tissue, cartilage).<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
{{Main|Wilms tumour}}
**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==
==Metanephric stromal tumour==
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====Cellular====
====Cellular====
Features:<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
Features:<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
*Plump cells with vesicular nuclei.
*Plump cells with [[vesicular nuclei]].
*Well-defined border.
*Well-defined border.
*Mitotically active.
*Mitotically active.
Line 141: Line 95:


===Molecular===
===Molecular===
Cellular mesoblastic nephroma:
Cellular mesoblastic nephroma:<ref name=pmid29683818>{{Cite journal  | last1 = Rudzinski | first1 = ER. | last2 = Lockwood | first2 = CM. | last3 = Stohr | first3 = BA. | last4 = Vargas | first4 = SO. | last5 = Sheridan | first5 = R. | last6 = Black | first6 = JO. | last7 = Rajaram | first7 = V. | last8 = Laetsch | first8 = TW. | last9 = Davis | first9 = JL. | title = Pan-Trk Immunohistochemistry Identifies NTRK Rearrangements in Pediatric Mesenchymal Tumors. | journal = Am J Surg Pathol | volume = 42 | issue = 7 | pages = 927-935 | month = Jul | year = 2018 | doi = 10.1097/PAS.0000000000001062 | PMID = 29683818 }}</ref>
*t(12:15)(p13;q25) ETV6/NTRK3.
*t(12:15)(p13;q25) ETV6/[[NTRK3]].
**Same [[translocation]] if found in ''[[infantile fibrosarcoma]]''.
**Same [[translocation]] if found in ''[[infantile fibrosarcoma]]''.


Line 155: Line 109:
*Renal medulla.
*Renal medulla.
*+/-Cystic.
*+/-Cystic.
Image:
*[http://www.flickr.com/photos/35441329@N05/4171714950/in/photostream/ CCS of the kidney (flickr.com)].
*[http://www.flickr.com/photos/35441329@N05/4171714940/in/photostream/ CCS of the kidney (flickr.com)].


===Microscopic===
===Microscopic===
Features:<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
Features:<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
*Polygonal cells/Stellate cells.
*Polygonal cells/Stellate cells.
*Clear cytoplasm.
*Indistinct cell borders/cytoplasm; clear background.
*No nucleoli.
*No nucleoli.


==See also==
Images:
*[http://path.upmc.edu/cases/case201.html Clear cell sarcoma of the kidney - several images (upmc.edu)].
 
===IHC===
Features:<ref name=pmid17883043>{{Cite journal  | last1 = Viswanathan | first1 = S. | last2 = Dave | first2 = BK. | last3 = Desai | first3 = SB. | title = Clear cell sarcoma of the kidney--a study of seven cases over a period of three years. | journal = Indian J Pathol Microbiol | volume = 50 | issue = 2 | pages = 270-3 | month = Apr | year = 2007 | doi =  | PMID = 17883043 }}</ref>
*Vimentin +ve.
*Desmin -ve.
*Smooth muscle actin -ve.
*NSE -ve.
*S-100 -ve.
 
===Molecular===
*t(10;17)(q22;p13) - reported in 6 of 50 cases.<ref name=pmid22294382>{{Cite journal  | last1 = O'Meara | first1 = E. | last2 = Stack | first2 = D. | last3 = Lee | first3 = CH. | last4 = Garvin | first4 = AJ. | last5 = Morris | first5 = T. | last6 = Argani | first6 = P. | last7 = Han | first7 = JS. | last8 = Karlsson | first8 = J. | last9 = Gisselson | first9 = D. | title = Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney. | journal = J Pathol | volume = 227 | issue = 1 | pages = 72-80 | month = May | year = 2012 | doi = 10.1002/path.3985 | PMID = 22294382 }}</ref>
 
==Renal rhabdoid tumour==
*[[AKA]] ''rhabdoid tumour of the kidney'', abbreviated ''RTK''.
{{Main|Renal rhabdoid tumour}}
 
==Renal cell carcinoma==
===General===
*Similar to in adults.
*[[Clear cell renal cell carcinoma|Clear cell RCC]] not common - a tumour with the clear cell histomorphology is more often a ''[[renal tumour with Xp11.2 translocation]]''.
 
====Papillary RCC====
:See: ''[[Renal_cell_carcinoma|(adult) renal cell carcinoma]]''.
*Most common form of RCC in children.
 
====Clear cell RCC====
:See: ''[[Renal_cell_carcinoma|(adult) renal cell carcinoma]]''.
 
=See also=
*[[Genitourinary pathology]]
*[[Genitourinary pathology]]
*[[Kidney tumours]].
*[[Kidney tumours]].
*[[Pediatric pathology]].
*[[Pediatric pathology]].


==References==
=References=
{{Reflist|2}}
{{Reflist|2}}


[[Category:Genitourinary pathology]]
[[Category:Genitourinary pathology]]
Michael
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