Difference between revisions of "Pediatric kidney tumours"
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This article cover '''pediatric kidney tumours'''. Adult kidney tumour are covered in ''[[kidney tumours]]'' article. | This article cover '''pediatric [[kidney]] tumours'''. Adult kidney tumour are covered in ''[[kidney tumours]]'' article. An introduction to pediatric pathology is in the ''[[pediatric pathology]]'' article. | ||
= | =Overview= | ||
These are diagnoses pediatric pathologists make. | |||
The most common ones are: | |||
#[[Wilms tumour]]. | |||
#[[Clear cell sarcoma of the kidney]]. | |||
Other renal tumours (not covered in this article): | |||
* | *Postneuroblastoma RCC. | ||
*[[Neuroblastoma]]. | |||
*[[PNET]]. | |||
*[[Synovial sarcoma]]. | |||
*[[Lymphoma]]. | |||
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]]. | |||
= | =Specific tumours= | ||
==Wilms tumour== | |||
*[[AKA]] nephroblastoma, AKA Wilms' tumour. | |||
{{Main|Wilms tumour}} | |||
*[[ | |||
==Metanephric stromal tumour== | ==Metanephric stromal tumour== | ||
Line 87: | Line 41: | ||
*Spindle cells/stellate cells. | *Spindle cells/stellate cells. | ||
*Variable cellular density - imparts a nodular appearance at low power. | *Variable cellular density - imparts a nodular appearance at low power. | ||
*Indistinct cytoplasm. | *Indistinct cell borders/cytoplasm. | ||
*Induces epithelioid morphology in smooth muscle cells. | *Induces epithelioid morphology in smooth muscle cells. | ||
Line 96: | Line 50: | ||
*Clear cell sarcoma of the kidney.<ref name=pmid11549506/> | *Clear cell sarcoma of the kidney.<ref name=pmid11549506/> | ||
*[[Mesoblastic nephroma]]. | *[[Mesoblastic nephroma]]. | ||
Images: | |||
*[http://www.flickr.com/photos/ckrishnan/3974742809/in/photostream/ MST - low mag. (flickr.com)]. | |||
*[http://www.flickr.com/photos/ckrishnan/3974742603/in/photostream MST - high mag. (flickr.com)]. | |||
==Metanephric adenofibroma== | ==Metanephric adenofibroma== | ||
Line 129: | Line 87: | ||
====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. | ||
Line 137: | Line 95: | ||
===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]]''. | ||
==See also | ==Clear cell sarcoma of the kidney== | ||
*Not to be confused with ''[[clear cell sarcoma]]''. | |||
===General=== | |||
*Common pediatric renal tumour - second only to [[Wilms tumour]]. | |||
*Aggressive. | |||
===Gross=== | |||
*Renal medulla. | |||
*+/-Cystic. | |||
Image: | |||
*[http://www.flickr.com/photos/35441329@N05/4171714950/in/photostream/ CCS of the kidney (flickr.com)]. | |||
*[http://www.flickr.com/photos/35441329@N05/4171714940/in/photostream/ CCS of the kidney (flickr.com)]. | |||
===Microscopic=== | |||
Features:<ref name=Ref_WMSP284>{{Ref WMSP|284}}</ref> | |||
*Polygonal cells/Stellate cells. | |||
*Indistinct cell borders/cytoplasm; clear background. | |||
*No nucleoli. | |||
Images: | |||
*[http://path.upmc.edu/cases/case201.html Clear cell sarcoma of the kidney - several images (upmc.edu)]. | |||
===IHC=== | |||
Features:<ref name=pmid17883043>{{Cite journal | last1 = Viswanathan | first1 = S. | last2 = Dave | first2 = BK. | last3 = Desai | first3 = SB. | title = Clear cell sarcoma of the kidney--a study of seven cases over a period of three years. | journal = Indian J Pathol Microbiol | volume = 50 | issue = 2 | pages = 270-3 | month = Apr | year = 2007 | doi = | PMID = 17883043 }}</ref> | |||
*Vimentin +ve. | |||
*Desmin -ve. | |||
*Smooth muscle actin -ve. | |||
*NSE -ve. | |||
*S-100 -ve. | |||
===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> | |||
==Renal rhabdoid tumour== | |||
*[[AKA]] ''rhabdoid tumour of the kidney'', abbreviated ''RTK''. | |||
{{Main|Renal rhabdoid tumour}} | |||
==Renal cell carcinoma== | |||
===General=== | |||
*Similar to in adults. | |||
*[[Clear cell renal cell carcinoma|Clear cell RCC]] not common - a tumour with the clear cell histomorphology is more often a ''[[renal tumour with Xp11.2 translocation]]''. | |||
====Papillary RCC==== | |||
:See: ''[[Renal_cell_carcinoma|(adult) renal cell carcinoma]]''. | |||
*Most common form of RCC in children. | |||
====Clear cell RCC==== | |||
:See: ''[[Renal_cell_carcinoma|(adult) renal cell carcinoma]]''. | |||
=See also= | |||
*[[Genitourinary pathology]] | *[[Genitourinary pathology]] | ||
*[[Kidney tumours]]. | *[[Kidney tumours]]. | ||
*[[Pediatric pathology]]. | *[[Pediatric pathology]]. | ||
=References= | |||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Genitourinary pathology]] | [[Category:Genitourinary pathology]] |
Latest revision as of 06:05, 11 September 2018
This article cover pediatric kidney tumours. Adult kidney tumour are covered in kidney tumours article. An introduction to pediatric pathology is in the pediatric pathology article.
Overview
These are diagnoses pediatric pathologists make.
The most common ones are:
Other renal tumours (not covered in this article):
- Postneuroblastoma RCC.
- Neuroblastoma.
- PNET.
- Synovial sarcoma.
- Lymphoma.
The renal translocation carcinomas are covered in:
Specific tumours
Wilms tumour
- AKA nephroblastoma, AKA Wilms' tumour.
Main article: Wilms tumour
Metanephric stromal tumour
- Abbreviated MST.
General
- Infants and children.
- Diagnosed as mesoblastic nephroma in the past.[1]
- Benign and rare.[2]
Gross
Features:[1]
- Usu. renal medulla.
- Solid or cystic.
Microscopic
Features:[1]
- "Collarettes" - tumour cells surround blood vessels or renal tubules.
- Spindle cells/stellate cells.
- Variable cellular density - imparts a nodular appearance at low power.
- Indistinct cell borders/cytoplasm.
- Induces epithelioid morphology in smooth muscle cells.
Notes:
- +/-Heterologous elements.
DDx:
- Clear cell sarcoma of the kidney.[2]
- Mesoblastic nephroma.
Images:
Metanephric adenofibroma
General
- Adults and children.
Microsopic
Features:[1]
- Has features of:
- Stromal component.
- Similar to metanephric stromal tumour.
- Epithelial nodules.
- Similar to metanephric adenoma.
- Stromal component.
Mesoblastic nephroma
General
- Almost exclusively in infants.
Subclassified:
- Classic.
- Cellular.
- Mixed.
Gross
- Renal sinus infiltration - common.
Microscopic
Classic
Features:[3]
- Spindle cells in fascicles.
- Infiltrative border.
Cellular
Features:[3]
- Plump cells with vesicular nuclei.
- Well-defined border.
- Mitotically active.
Mixed
- Like the name implies - both classic pattern and cellular pattern areas are present.[3]
Molecular
Cellular mesoblastic nephroma:[4]
- t(12:15)(p13;q25) ETV6/NTRK3.
- Same translocation if found in infantile fibrosarcoma.
Clear cell sarcoma of the kidney
- Not to be confused with clear cell sarcoma.
General
- Common pediatric renal tumour - second only to Wilms tumour.
- Aggressive.
Gross
- Renal medulla.
- +/-Cystic.
Image:
Microscopic
Features:[3]
- Polygonal cells/Stellate cells.
- Indistinct cell borders/cytoplasm; clear background.
- No nucleoli.
Images:
IHC
Features:[5]
- Vimentin +ve.
- Desmin -ve.
- Smooth muscle actin -ve.
- NSE -ve.
- S-100 -ve.
Molecular
- t(10;17)(q22;p13) - reported in 6 of 50 cases.[6]
Renal rhabdoid tumour
- AKA rhabdoid tumour of the kidney, abbreviated RTK.
Main article: Renal rhabdoid tumour
Renal cell carcinoma
General
- Similar to in adults.
- Clear cell RCC not common - a tumour with the clear cell histomorphology is more often a renal tumour with Xp11.2 translocation.
Papillary RCC
- Most common form of RCC in children.
Clear cell RCC
See also
References
- ↑ 1.0 1.1 1.2 1.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 283. ISBN 978-0781765275.
- ↑ 2.0 2.1 Palese, MA.; Ferrer, F.; Perlman, E.; Gearhart, JP. (Sep 2001). "Metanephric stromal tumor: a rare benign pediatric renal mass.". Urology 58 (3): 462. PMID 11549506.
- ↑ 3.0 3.1 3.2 3.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 284. ISBN 978-0781765275.
- ↑ Rudzinski, ER.; Lockwood, CM.; Stohr, BA.; Vargas, SO.; Sheridan, R.; Black, JO.; Rajaram, V.; Laetsch, TW. et al. (Jul 2018). "Pan-Trk Immunohistochemistry Identifies NTRK Rearrangements in Pediatric Mesenchymal Tumors.". Am J Surg Pathol 42 (7): 927-935. doi:10.1097/PAS.0000000000001062. PMID 29683818.
- ↑ Viswanathan, S.; Dave, BK.; Desai, SB. (Apr 2007). "Clear cell sarcoma of the kidney--a study of seven cases over a period of three years.". Indian J Pathol Microbiol 50 (2): 270-3. PMID 17883043.
- ↑ O'Meara, E.; Stack, D.; Lee, CH.; Garvin, AJ.; Morris, T.; Argani, P.; Han, JS.; Karlsson, J. et al. (May 2012). "Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney.". J Pathol 227 (1): 72-80. doi:10.1002/path.3985. PMID 22294382.