Difference between revisions of "Pediatric kidney tumours"

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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===
The most common ones are:
*Common abdominal [[pediatric pathology|pediatric]] tumour.
#[[Wilms 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>
#[[Clear cell sarcoma of the kidney]].
**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===
Other renal tumours (not covered in this article):
*Lobulated tan mass.
*Postneuroblastoma RCC.
*[[Neuroblastoma]].
*[[PNET]].
*[[Synovial sarcoma]].
*[[Lymphoma]].


Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)].
The [[Kidney_tumours#Renal_translocation_carcinomas|renal translocation carcinomas]] are covered in:
*[[Renal tumour with Xp11.2 translocation]].
*[[Renal tumour with t(6;11) translocation]].


===Microscopic===
=Specific tumours=
Features - classically three components (blastema, immature stroma, tubules):<ref name=Ref_PCPBoD8_254-5>{{Ref PCPBoD8|254-5}}</ref>
==Wilms tumour==
#Malignant [[Small round blue cell tumours|small round blue cells]] ("blastema"):
*[[AKA]] nephroblastoma, AKA Wilms' tumour.
#*Size = ~ 2x RBC diameter.
{{Main|Wilms tumour}}
#*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==
==Metanephric stromal tumour==
Line 133: Line 87:
====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===
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*No nucleoli.
*No nucleoli.


==Renal rhabdoid tumour==
Images:
*[[AKA]] ''rhabdoid tumour of the kidney'', abbreviated ''RTK''.
*[http://path.upmc.edu/cases/case201.html Clear cell sarcoma of the kidney - several images (upmc.edu)].


===General===
===IHC===
*Similar to ''[[extrarenal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
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>
*Arises from renal medulla.
*Vimentin +ve.
*May be associated with a CNS tumour.
*Desmin -ve.  
*Smooth muscle actin -ve.
*NSE -ve.
*S-100 -ve.


===Microscopic===
===Molecular===
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref><ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
*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>
*Variable architecture.
*Round cells.
*Abundant cytoplasm with eosinophilic inclusions.
*Eccentric vesicular nucleus.
*Prominent [[nucleolus]] -- '''key feature'''.


===IHC===
==Renal rhabdoid tumour==
*INI1 -ve.
*[[AKA]] ''rhabdoid tumour of the kidney'', abbreviated ''RTK''.
{{Main|Renal rhabdoid tumour}}


==Renal cell carcinoma==
==Renal cell carcinoma==
===General===
===General===
*Similar to in adults.
*Similar to in adults.
*Clear cell RCC not common - a tumour with the clear cell histomorphology is more often a ''renal tumour with a Xp11.2 translocation''.
*[[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====
====Papillary RCC====
Line 193: Line 150:
:See: ''[[Renal_cell_carcinoma|(adult) renal cell carcinoma]]''.
:See: ''[[Renal_cell_carcinoma|(adult) renal cell carcinoma]]''.


==See also==
=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]]

Latest revision as of 06:05, 11 September 2018

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.

Overview

These are diagnoses pediatric pathologists make.

The most common ones are:

  1. Wilms tumour.
  2. Clear cell sarcoma of the kidney.

Other renal tumours (not covered in this article):

The renal translocation carcinomas are covered in:

Specific tumours

Wilms tumour

  • AKA nephroblastoma, AKA Wilms' tumour.
Main article: Wilms tumour

Metanephric stromal tumour

  • Abbreviated MST.

General

Gross

Features:[1]

  • Usu. renal medulla.
  • Solid or cystic.

Microscopic

Features:[1]

  • "Collarettes" - tumour cells surround blood vessels or renal tubules.
  • Spindle cells/stellate cells.
  • Variable cellular density - imparts a nodular appearance at low power.
  • Indistinct cell borders/cytoplasm.
  • Induces epithelioid morphology in smooth muscle cells.

Notes:

  • +/-Heterologous elements.

DDx:

Images:

Metanephric adenofibroma

General

  • Adults and children.

Microsopic

Features:[1]

Mesoblastic nephroma

General

  • Almost exclusively in infants.

Subclassified:

  1. Classic.
  2. Cellular.
  3. Mixed.

Gross

  • Renal sinus infiltration - common.

Microscopic

Classic

Features:[3]

  • Spindle cells in fascicles.
  • Infiltrative border.

Cellular

Features:[3]

Mixed

  • Like the name implies - both classic pattern and cellular pattern areas are present.[3]

Molecular

Cellular mesoblastic nephroma:[4]

Clear cell sarcoma of the kidney

General

  • Common pediatric renal tumour - second only to Wilms tumour.
  • Aggressive.

Gross

  • Renal medulla.
  • +/-Cystic.

Image:

Microscopic

Features:[3]

  • Polygonal cells/Stellate cells.
  • Indistinct cell borders/cytoplasm; clear background.
  • No nucleoli.

Images:

IHC

Features:[5]

  • 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.[6]

Renal rhabdoid tumour

  • AKA rhabdoid tumour of the kidney, abbreviated RTK.
Main article: Renal rhabdoid tumour

Renal cell carcinoma

General

Papillary RCC

See: (adult) renal cell carcinoma.
  • Most common form of RCC in children.

Clear cell RCC

See: (adult) renal cell carcinoma.

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 283. ISBN 978-0781765275.
  2. ↑ 2.0 2.1 Palese, MA.; Ferrer, F.; Perlman, E.; Gearhart, JP. (Sep 2001). "Metanephric stromal tumor: a rare benign pediatric renal mass.". Urology 58 (3): 462. PMID 11549506.
  3. ↑ 3.0 3.1 3.2 3.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 284. ISBN 978-0781765275.
  4. ↑ Rudzinski, ER.; Lockwood, CM.; Stohr, BA.; Vargas, SO.; Sheridan, R.; Black, JO.; Rajaram, V.; Laetsch, TW. et al. (Jul 2018). "Pan-Trk Immunohistochemistry Identifies NTRK Rearrangements in Pediatric Mesenchymal Tumors.". Am J Surg Pathol 42 (7): 927-935. doi:10.1097/PAS.0000000000001062. PMID 29683818.
  5. ↑ Viswanathan, S.; Dave, BK.; Desai, SB. (Apr 2007). "Clear cell sarcoma of the kidney--a study of seven cases over a period of three years.". Indian J Pathol Microbiol 50 (2): 270-3. PMID 17883043.
  6. ↑ O'Meara, E.; Stack, D.; Lee, CH.; Garvin, AJ.; Morris, T.; Argani, P.; Han, JS.; Karlsson, J. et al. (May 2012). "Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney.". J Pathol 227 (1): 72-80. doi:10.1002/path.3985. PMID 22294382.
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