|
|
Line 593: |
Line 593: |
|
| |
|
| =Pediatric= | | =Pediatric= |
| ==Wilms tumour==
| | {{Pediatric kidney tumours}} |
| *[[AKA]] nephroblastoma, AKA Wilms' tumour.
| |
|
| |
|
| ===General===
| | The most common is [[nephroblastoma]] (Wilms tumour). |
| *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===
| | Others include: |
| *Lobulated tan mass. | | *[[Metanephric stromal tumour]]. |
| | | *[[Metanephric adenofibroma]] |
| Image: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Wilms tumour (med.utah.edu)].
| | *[[Metanephric adenoma]]. |
| | | *[[Clear cell sarcoma of the kidney]]. |
| ===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]] - esp. 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:
| |
| *[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)].
| |
| *[http://commons.wikimedia.org/wiki/File:Wilms_tumour_-_low_mag.jpg Wilms tumour - low mag. (WC)]. | |
| *[http://commons.wikimedia.org/wiki/File:Wilms_tumour_-_very_high_mag.jpg Wilms tumour - very high mag. (WC)].
| |
| | |
| ====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.
| |
| | |
| ==Metanephric stromal tumour==
| |
| *Abbreviated ''MST''.
| |
| ===General===
| |
| *Infants and children.
| |
| *Diagnosed as [[mesoblastic nephroma]] in the past.<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
| |
| *Benign and rare.<ref name=pmid11549506>{{Cite journal | last1 = Palese | first1 = MA. | last2 = Ferrer | first2 = F. | last3 = Perlman | first3 = E. | last4 = Gearhart | first4 = JP. | title = Metanephric stromal tumor: a rare benign pediatric renal mass. | journal = Urology | volume = 58 | issue = 3 | pages = 462 | month = Sep | year = 2001 | doi = | PMID = 11549506 }}</ref>
| |
| | |
| ===Gross===
| |
| Features:<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
| |
| *Usu. renal medulla.
| |
| *Solid or cystic.
| |
| | |
| ===Microscopic===
| |
| Features:<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
| |
| *"Collarettes" - tumour cells surround blood vessels ''or'' renal tubules.
| |
| *Spindle cells/stellate cells.
| |
| *Variable cellular density - imparts a nodular appearance at low power.
| |
| *Scant cytoplasm.
| |
| *Induces epithelioid morphology in smooth muscle cells.
| |
| | |
| Notes:
| |
| *+/-Heterologous elements.
| |
| | |
| DDx:
| |
| *Clear cell sarcoma of the kidney.<ref name=pmid11549506/>
| |
| *[[Mesoblastic nephroma]].
| |
| | |
| ==Metanephric adenofibroma==
| |
| ===General===
| |
| *Adults and children.
| |
| | |
| ===Microsopic===
| |
| Features:<ref name=Ref_WMSP283>{{Ref WMSP|283}}</ref>
| |
| *Has features of:
| |
| *#Stromal component.
| |
| *#*Similar to [[metanephric stromal tumour]].
| |
| *#Epithelial nodules.
| |
| *#*Similar to [[metanephric adenoma]].
| |
|
| |
|
| =See also= | | =See also= |