Difference between revisions of "Wilms tumour"

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#redirect [[Pediatric_kidney_tumours#Wilms_tumour]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Wilms_tumour_-_very_high_mag.jpg
| Width      =
| Caption    = Wilms tumour. [[H&E stain]].
| Synonyms  = nephroblastoma
| Micro      =
| Subtypes  =
| LMDDx      = [[metanephric adenoma]], nephrogenic nests, [[small round cell tumours]], Immature teratoma
| Stains    =
| IHC        = WT-1 +ve, CD56 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[kidney]] - see ''[[pediatric kidney tumours]]''
| Assdx      =
| Syndromes  = WAGR syndrome, [[Beckwith-Wiedemann syndrome]], [[Denys-Drash syndrome]]
| Clinicalhx =
| Signs      = +/-abdominal mass
| Symptoms  =
| Prevalence = most common pediatric kidney tumour
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Wilms tumour''', also '''nephroblastoma''' and '''Wilms' tumour''', is the most common [[Pediatric_kidney_tumours|pediatric kidney tumour]].<ref name=pmid10935424>{{cite journal |author=Coppes MJ, Wolff JE, Ritchey ML |title=Wilms tumour: diagnosis and treatment |journal=Paediatr Drugs |volume=1 |issue=4 |pages=251–62 |year=1999 |pmid=10935424 |doi= |url=}}</ref><ref name=pmid15738594>{{cite journal |author=Stefanowicz J, Sierota D, Balcerska A, Stoba C |title=[Wilms' tumour of unfavorable histology--results of treatment with the SIOP 93-01 protocol at the Gdańsk centre. Preliminary report] |language=Polish |journal=Med Wieku Rozwoj |volume=8 |issue=2 Pt 1 |pages=197–200 |year=2004 |pmid=15738594 |doi= |url=}}</ref>
 
==General==
*Common abdominal [[pediatric pathology|pediatric]] tumour.
**Affects approximately 1 in 8000 children.
**There is no sex predilection and the mean patient age at diagnosis ranges among 37 to 43 months.
*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==
Features <ref name="pmid10193955">{{Cite journal  | last1 = Coppes | first1 = MJ. | last2 = Arnold | first2 = M. | last3 = Beckwith | first3 = JB. | last4 = Ritchey | first4 = ML. | last5 = D'Angio | first5 = GJ. | last6 = Green | first6 = DM. | last7 = Breslow | first7 = NE. | title = Factors affecting the risk of contralateral Wilms tumor development: a report from the National Wilms Tumor Study Group. | journal = Cancer | volume = 85 | issue = 7 | pages = 1616-25 | month = Apr | year = 1999 | doi =  | PMID = 10193955 }}</ref>
*Most nephroblastomas are unifocal.
**Usually solitary, rounded, multilobular masses sharply demarcated from adjacent parenchyma.
**The cut surface is most commonly pale grey or tan.
**Cyst most be prominent in some cases.
*Multifocal masses in a single kidney and bilateral primary lesions are less frequent.
 
===Images===
<gallery>
Image:Wilms_tumor.jpg | Wilms tumour. (WC/AFIP)
</gallery>
www:
*[http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)].
*[http://www.webpathology.com/image.asp?case=73&n=1 Wilms tumour (WebPathology)].
 
==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"):
#*The blastemal component is the least differentiated cellular element.
#*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.
#Epithelial components ("tubules"):
#*Primitive rossete-like tubules, well-formed maturing and mature tubules, glomerular structures and  variably papillary architecture.
#**Usually clustered.
#**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.
*Immature teratoma.
 
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)
File:Wilms Tumor (Nephroblastoma) (4882456062).jpg | Wilms tumor - low mag. (WC/Ed Uthman)
</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 (nuclear).
*CD56 +ve.<ref name=pmid11688464>{{Cite journal  | last1 = Muir | first1 = TE. | last2 = Cheville | first2 = JC. | last3 = Lager | first3 = DJ. | title = Metanephric adenoma, nephrogenic rests, and Wilms' tumor: a histologic and immunophenotypic comparison. | journal = Am J Surg Pathol | volume = 25 | issue = 10 | pages = 1290-6 | month = Oct | year = 2001 | doi =  | PMID = 11688464 }}</ref>
**-ve in [[metanephric adenoma]].
 
==Molecular==
*Cytogenetics<ref>{{Cite journal  | last1 = Md Zin | first1 = R. | last2 = Murch | first2 = A. | last3 = Charles | first3 = A. | title = Pathology, genetics and cytogenetics of Wilms' tumour. | journal = Pathology | volume = 43 | issue = 4 | pages = 302-12 | month = Jun | year = 2011 | doi = 10.1097/PAT.0b013e3283463575 | PMID = 21516053 }}</ref>
**Partial gains of 1q.
**Partial losses of 1p, 1q, 4q, 11q, 16q, 22q.
**Complete loss of chromosome 16, 11, 12, 22.
**Trisomy of chromosome 8, 12, 13, 18.
 
==See also==
*[[Pediatric kidney tumours]].
*[[Kidney tumours]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Pediatric kidney tumours]]
[[Category:Small round blue cell tumours]]

Latest revision as of 05:48, 14 October 2018

Wilms tumour
Diagnosis in short

Wilms tumour. H&E stain.
Synonyms nephroblastoma
LM DDx metanephric adenoma, nephrogenic nests, small round cell tumours, Immature teratoma
IHC WT-1 +ve, CD56 +ve
Site kidney - see pediatric kidney tumours
Syndromes WAGR syndrome, Beckwith-Wiedemann syndrome, Denys-Drash syndrome
Signs +/-abdominal mass
Prevalence most common pediatric kidney tumour

Wilms tumour, also nephroblastoma and Wilms' tumour, is the most common pediatric kidney tumour.[1][2]

General

  • Common abdominal pediatric tumour.
    • Affects approximately 1 in 8000 children.
    • There is no sex predilection and the mean patient age at diagnosis ranges among 37 to 43 months.
  • May be associated with a syndrome:[3]

Gross

Features [7]

  • Most nephroblastomas are unifocal.
    • Usually solitary, rounded, multilobular masses sharply demarcated from adjacent parenchyma.
    • The cut surface is most commonly pale grey or tan.
    • Cyst most be prominent in some cases.
  • Multifocal masses in a single kidney and bilateral primary lesions are less frequent.

Images

  • Wilms tumour. (WC/AFIP)

www:

Microscopic

Features - classically three components (blastema, immature stroma, tubules):[8]

  1. Malignant small round blue cells ("blastema"):
    • The blastemal component is the least differentiated cellular element.
    • 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. Epithelial components ("tubules"):
    • Primitive rossete-like tubules, well-formed maturing and mature tubules, glomerular structures and variably papillary architecture.
      • Usually clustered.
      • 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.[9]
  • +/-Heterologous elements (skeletal muscle, smooth muscle adipose tissue, cartilage).[10]
    • Heterologous = doesn't normally belong there.[11]

DDx:

Notes:

  • Palisade = fence made of stakes driven into the ground.[12]
  • Approximately 30-40% Wilms tumour cases have nephrogenic rests.[13]
  • The three phases are also called blastemal, epithelial and stromal.[10]

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)

  • Wilms tumor - low mag. (WC/Ed Uthman)

www:

Anaplasia

Subclassified as:[10]

  1. Focal anaplasia.
  2. Diffuse anaplasia.

Criteria (all of the following):[10]

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

IHC

Molecular

  • Cytogenetics[15]
    • Partial gains of 1q.
    • Partial losses of 1p, 1q, 4q, 11q, 16q, 22q.
    • Complete loss of chromosome 16, 11, 12, 22.
    • Trisomy of chromosome 8, 12, 13, 18.

See also

References

  1. ↑ Coppes MJ, Wolff JE, Ritchey ML (1999). "Wilms tumour: diagnosis and treatment". Paediatr Drugs 1 (4): 251–62. PMID 10935424.
  2. ↑ Stefanowicz J, Sierota D, Balcerska A, Stoba C (2004). "[Wilms' tumour of unfavorable histology--results of treatment with the SIOP 93-01 protocol at the Gdańsk centre. Preliminary report]" (in Polish). Med Wieku Rozwoj 8 (2 Pt 1): 197–200. PMID 15738594.
  3. ↑ URL: http://emedicine.medscape.com/article/989398-overview. Accessed on: 9 March 2011.
  4. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 194072
  5. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 130650
  6. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 194080
  7. ↑ Coppes, MJ.; Arnold, M.; Beckwith, JB.; Ritchey, ML.; D'Angio, GJ.; Green, DM.; Breslow, NE. (Apr 1999). "Factors affecting the risk of contralateral Wilms tumor development: a report from the National Wilms Tumor Study Group.". Cancer 85 (7): 1616-25. PMID 10193955.
  8. ↑ 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.
  9. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970416-8. Accessed on: 28 March 2011.
  10. ↑ 10.0 10.1 10.2 10.3 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.
  11. ↑ URL: http://www.biology-online.org/dictionary/Heterologous. Accessed on: 1 October 2011.
  12. ↑ URL: http://www.thefreedictionary.com/palisaded. Accessed on: 2 February 2011.
  13. ↑ 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.
  14. ↑ Muir, TE.; Cheville, JC.; Lager, DJ. (Oct 2001). "Metanephric adenoma, nephrogenic rests, and Wilms' tumor: a histologic and immunophenotypic comparison.". Am J Surg Pathol 25 (10): 1290-6. PMID 11688464.
  15. ↑ Md Zin, R.; Murch, A.; Charles, A. (Jun 2011). "Pathology, genetics and cytogenetics of Wilms' tumour.". Pathology 43 (4): 302-12. doi:10.1097/PAT.0b013e3283463575. PMID 21516053.
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