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> | ||
| Line 9: | Line 42: | ||
==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). | ||
| Line 25: | Line 70: | ||
#**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: | ||
| Line 42: | Line 87: | ||
*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: | ||
| Line 54: | Line 100: | ||
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: | ||
| Line 70: | Line 117: | ||
==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== | ||
| Line 81: | Line 137: | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Pediatric kidney tumours]] | [[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]
- WAGR syndrome (Wilms tumour, Aniridia (absence of iris), GU abnormalities, Retardation).[4]
- Beckwith-Wiedemann syndrome.[5]
- Denys-Drash syndrome.[6]
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]
- 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.
- Stroma ("immature stroma"):
- Spindle cells:
- Elliptical nuclear membrane.
- Abundant loose cytoplasm.
- Spindle cells:
- 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.
- Primitive rossete-like tubules, well-formed maturing and mature tubules, glomerular structures and variably papillary architecture.
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:
- 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.[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)
_(4882456062).jpg)
www:
Anaplasia
Subclassified as:[10]
- Focal anaplasia.
- Diffuse anaplasia.
Criteria (all of the following):[10]
- Atypical mitoses.
- Nuclear hyperchromasia.
- Nuclear size variation (of the tumour cells) > 3x.
IHC
- WT-1 +ve (nuclear).
- CD56 +ve.[14]
- -ve in metanephric adenoma.
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
- ↑ Coppes MJ, Wolff JE, Ritchey ML (1999). "Wilms tumour: diagnosis and treatment". Paediatr Drugs 1 (4): 251–62. PMID 10935424.
- ↑ 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.
- ↑ URL: http://emedicine.medscape.com/article/989398-overview. Accessed on: 9 March 2011.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 194072
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 130650
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 194080
- ↑ 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.
- ↑ 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.
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970416-8. Accessed on: 28 March 2011.
- ↑ 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.
- ↑ URL: http://www.biology-online.org/dictionary/Heterologous. Accessed on: 1 October 2011.
- ↑ URL: http://www.thefreedictionary.com/palisaded. Accessed on: 2 February 2011.
- ↑ 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.
- ↑ 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.
- ↑ 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.