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Difference between revisions of "Wilms tumour"
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{{ 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> | '''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== | ==General== | ||
*Common abdominal [[pediatric pathology|pediatric]] tumour. | *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> | *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> | **WAGR syndrome (Wilms tumour, Aniridia (absence of iris), GU abnormalities, Retardation).<ref>{{OMIM|194072}}</ref> | ||
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==Gross== | ==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. | |||
Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)]. | ===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== | ==Microscopic== | ||
Features - classically three components (blastema, immature stroma, tubules):<ref name=Ref_PCPBoD8_254-5>{{Ref PCPBoD8|254-5}}</ref> | 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"): | #Malignant [[Small round blue cell tumours|small round blue cells]] ("blastema"): | ||
#*The blastemal component is the least differentiated cellular element. | |||
#*Size = ~ 2x RBC diameter. | #*Size = ~ 2x RBC diameter. | ||
#*Nuclear pleomorphism (variation of size, shape and staining). | #*Nuclear pleomorphism (variation of size, shape and staining). | ||
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#**Elliptical nuclear membrane. | #**Elliptical nuclear membrane. | ||
#**Abundant loose cytoplasm. | #**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. | #**Usu. have a central (clear/white) space surrounded by a rim of intensely eosinophilic cytoplasm. | ||
#*Nuclei of tubular structures often elongated and palisaded. | #**Nuclei of tubular structures often elongated and palisaded. | ||
Other findings: | Other findings: | ||
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*Other [[small round cell tumours]]. | *Other [[small round cell tumours]]. | ||
*[[Synovial sarcoma]], biphasic - especially in adults. | *[[Synovial sarcoma]], biphasic - especially in adults. | ||
*Immature teratoma. | |||
Notes: | Notes: | ||
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Image:Wilms tumour - high mag.jpg | Wilms tumour - high 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) | 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> | </gallery> | ||
www: | www: | ||
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==IHC== | ==IHC== | ||
*WT-1 +ve. | *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== | ==See also== | ||
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[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Pediatric kidney tumours]] | [[Category:Pediatric kidney tumours]] | ||
[[Category:Small round blue cell tumours]] | |||