Difference between revisions of "Wilms tumour"

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#redirect [[Pediatric_kidney_tumours#Wilms_tumour]]
'''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]]

Revision as of 03:19, 3 May 2014

Wilms tumour, also nephroblastoma and Wilms' tumour, is the most common pediatric kidney tumour.

General

Gross

  • Lobulated tan mass.

Image: Wilms tumour (med.utah.edu).

Microscopic

Features - classically three components (blastema, immature stroma, tubules):[5]

  1. Malignant 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.
  2. Stroma ("immature stroma"):
    • Spindle cells:
      • Elliptical nuclear membrane.
      • Abundant loose cytoplasm.
  3. 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.[6]
  • +/-Heterologous elements (skeletal muscle, smooth muscle adipose tissue, cartilage).[7]
    • Heterologous = doesn't normally belong there.[8]

DDx:

Notes:

  • Palisade = fence made of stakes driven into the ground.[9]
  • Approximately 30-40% Wilms tumour cases have nephrogenic rests.[10]
  • The three phases are also called blastemal, epithelial and stromal.[7]

Images

  • Wilms tumour - low mag. (WC/Nephron)

  • Wilms tumour - intermed. mag. (WC/Nephron)

  • Wilms tumour - high mag. (WC/Nephron)

  • Wilms tumour - very high mag. (WC/Nephron)

www:

Anaplasia

Subclassified as:[7]

  1. Focal anaplasia.
  2. Diffuse anaplasia.

Criteria (all of the following):[7]

  1. Atypical mitoses.
  2. Nuclear hyperchromasia.
  3. Nuclear size variation (of the tumour cells) > 3x.

IHC

  • WT-1 +ve.

See also

References

  1. ↑ URL: http://emedicine.medscape.com/article/989398-overview. Accessed on: 9 March 2011.
  2. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 194072
  3. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 130650
  4. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 194080
  5. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 254-5. ISBN 978-1416054542.
  6. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970416-8. Accessed on: 28 March 2011.
  7. ↑ 7.0 7.1 7.2 7.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 282. ISBN 978-0781765275.
  8. ↑ URL: http://www.biology-online.org/dictionary/Heterologous. Accessed on: 1 October 2011.
  9. ↑ URL: http://www.thefreedictionary.com/palisaded. Accessed on: 2 February 2011.
  10. ↑ Coppes MJ, Haber DA, Grundy PE (September 1994). "Genetic events in the development of Wilms' tumor". N. Engl. J. Med. 331 (9): 586–90. doi:10.1056/NEJM199409013310906. PMID 8047084.
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