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| Other renal tumours (not covered in this article): | | Other renal tumours (not covered in this article): |
| *Postneuroblastoma RCC. | | *Postneuroblastoma RCC. |
| *Neuroblastoma. | | *[[Neuroblastoma]]. |
| *[[PNET]]. | | *[[PNET]]. |
| *[[Synovial sarcoma]]. | | *[[Synovial sarcoma]]. |
| *[[Lymphoma]]. | | *[[Lymphoma]]. |
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| The ''[[Kidney_tumours#Renal_translocation_carcinomas|translocation carcinomas]]'' are covered in the ''[[kidney tumours]]'' article | | 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]]. |
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| =Specific tumours= | | =Specific tumours= |
| ==Wilms tumour== | | ==Wilms tumour== |
| *[[AKA]] nephroblastoma, AKA Wilms' tumour. | | *[[AKA]] nephroblastoma, AKA Wilms' tumour. |
| | | {{Main|Wilms tumour}} |
| ===General===
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| *Common abdominal [[pediatric pathology|pediatric]] tumour.
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| *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>
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| **WAGR syndrome (Wilms tumour, Aniridia (absence of iris), GU abnormalities, Retardation).<ref>{{OMIM|194072}}</ref>
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| **[[Beckwith-Wiedemann syndrome]].<ref>{{OMIM|130650}}</ref>
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| **[[Denys-Drash syndrome]].<ref>{{OMIM|194080}}</ref>
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| ===Gross===
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| *Lobulated tan mass.
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| Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)].
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| ===Microscopic===
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| Features - classically three components (blastema, immature stroma, tubules):<ref name=Ref_PCPBoD8_254-5>{{Ref PCPBoD8|254-5}}</ref>
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| #Malignant [[Small round blue cell tumours|small round blue cells]] ("blastema"):
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| #*Size = ~ 2x RBC diameter.
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| #*Nuclear pleomorphism (variation of size, shape and staining).
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| #**Irregular nuclear membrane - '''important'''.
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| #*Scant/difficult to discern cytoplasm - basophilic (light blue).
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| #*Mitoses - common.
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| #Stroma ("immature stroma"):
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| #*Spindle cells:
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| #**Elliptical nuclear membrane.
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| #**Abundant loose cytoplasm.
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| #Tubular structures ("tubules"):
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| #*Usually clustered.
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| #*Vaguely resemble a glomerulus.
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| #*Usu. have a central (clear/white) space surrounded by a rim of intensely eosinophilic cytoplasm.
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| #*Nuclei of tubular structures often elongated and palisaded.
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| Other findings:
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| *Commonly seen in association with ''nephrogenic rests''.
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| **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>
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| *+/-Heterologous elements (skeletal muscle, smooth muscle adipose tissue, cartilage).<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
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| **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>
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| DDx:
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| *[[Metanephric adenoma]].
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| *Nephrogenic nests.
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| *Other [[small round cell tumours]].
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| *[[Synovial sarcoma]], biphasic - especially in adults.
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| Notes:
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| *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>
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| *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>
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| *The three phases are also called ''blastemal, epithelial and stromal''.<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
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| ====Images====
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| <gallery>
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| Image:Wilms_tumour_-_low_mag.jpg | Wilms tumour - low mag. (WC/Nephron)
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| Image:Wilms tumour - intermed mag.jpg | Wilms tumour - intermed. mag. (WC/Nephron)
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| Image:Wilms tumour - high mag.jpg | Wilms tumour - high mag. (WC/Nephron)
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| Image:Wilms_tumour_-_very_high_mag.jpg | Wilms tumour - very high mag. (WC/Nephron)
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| </gallery>
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| www:
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| *[http://www.biologydisease.com/images/kidney/nephrogenic-rests/nephrogenic-rest.jpg.php Nephrogenic rests (biologydisease.com)].
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| *[http://www.webpathology.com/image.asp?n=1&Case=73 Wilms tumour (webpathology.com)].
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| ====Anaplasia====
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| Subclassified as:<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
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| #Focal anaplasia.
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| #Diffuse anaplasia.
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| Criteria (all of the following):<ref name=Ref_WMSP282>{{Ref WMSP|282}}</ref>
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| #Atypical mitoses.
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| #Nuclear hyperchromasia.
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| #Nuclear size variation (of the tumour cells) > 3x.
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| ===IHC===
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| *WT-1 +ve.
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| ==Metanephric stromal tumour== | | ==Metanephric stromal tumour== |
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| ====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. |
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| ===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]]''. |
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| *NSE -ve. | | *NSE -ve. |
| *S-100 -ve. | | *S-100 -ve. |
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| | ===Molecular=== |
| | *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> |
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| ==Renal rhabdoid tumour== | | ==Renal rhabdoid tumour== |
| *[[AKA]] ''rhabdoid tumour of the kidney'', abbreviated ''RTK''. | | *[[AKA]] ''rhabdoid tumour of the kidney'', abbreviated ''RTK''. |
| | | {{Main|Renal rhabdoid tumour}} |
| ===General===
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| *Similar to ''[[extrarenal malignant rhabdoid tumour]]''.<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
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| *Arises from renal medulla.
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| *May be associated with a CNS tumour.
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref><ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref>
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| *Variable architecture.
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| *Round cells.
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| *Abundant cytoplasm with eosinophilic inclusions.
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| *Eccentric vesicular nucleus.
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| *Prominent [[nucleolus]] -- '''key feature'''.
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| Images:
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| *[http://www.flickr.com/photos/ckrishnan/3954115280/in/photostream RTK - low mag. (flickr.com)].
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| *[http://www.flickr.com/photos/ckrishnan/3953336593/in/photostream RTK - high mag. (flickr.com)].
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| ===IHC===
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| *INI1 -ve.
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| ==Renal cell carcinoma== | | ==Renal cell carcinoma== |