Difference between revisions of "Clear cell papillary renal cell tumour"

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Clear cell papillary renal cell tumour (view source)

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| Image      = Clear_cell_papillary_renal_cell_carcinoma_-_very_high_mag.jpg
| Image      = Clear_cell_papillary_renal_cell_carcinoma_-_very_high_mag.jpg
| Width      =
| Width      =
| Caption    = Clear cell papillary renal cell carcinoma. [[H&E stain]].
| Caption    = Clear cell papillary renal cell tumour. [[H&E stain]].
| Synonyms  = clear cell tubulopapillary renal cell carcinoma
| Synonyms  = previously known as ''clear cell tubulopapillary renal cell carcinoma'' and ''clear cell papillary renal cell carcinoma''
| Micro      = low ([[ISUP nucleolar grade|ISUP nucleolar]]) grade apical nuclei, clear cells, pseudopapillae, cystic and/or tubular architectures
| Micro      = low ([[ISUP nucleolar grade|ISUP nucleolar]]) grade apical nuclei, clear cells, pseudopapillae, cystic and/or tubular architectures
| Subtypes  =
| Subtypes  =
| LMDDx      = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]],[[Xp11.2 translocation carcinoma]]
| LMDDx      = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]], [[Xp11.2 translocation carcinoma]]
| Stains    =
| Stains    =
| IHC        = CK7 +ve, AMACR -ve, CD10 -ve, TFE3 -ve
| IHC        = CK7 +ve, AMACR -ve, CD10 -ve, TFE3 -ve
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| ClinDDx    = other [[renal tumours]]
| ClinDDx    = other [[renal tumours]]
}}
}}
'''Clear cell papillary renal cell carcinoma''', abbreviated '''CCPRCC''', is recently described type of [[renal cell carcinoma]].
'''Clear cell papillary renal cell tumour''', abbreviated '''CCPRCT''', is a relatively common benign renal tumour.  


It is also known as '''clear cell tubulopapillary renal cell carcinoma''',<ref name=pmid21602815>{{Cite journal  | last1 = Rohan | first1 = SM. | last2 = Xiao | first2 = Y. | last3 = Liang | first3 = Y. | last4 = Dudas | first4 = ME. | last5 = Al-Ahmadie | first5 = HA. | last6 = Fine | first6 = SW. | last7 = Gopalan | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Rosenblum | first9 = MK. | title = Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel-Lindau gene and hypoxia-inducible factor pathway-related proteins. | journal = Mod Pathol | volume = 24 | issue = 9 | pages = 1207-20 | month = Sep | year = 2011 | doi = 10.1038/modpathol.2011.80 | PMID = 21602815 }}</ref> abbreviated '''CCTPRCC'''.
It was previously known as '''clear cell papillary renal cell carcinoma''', abbreviated '''CCPRCC'''
 
It is also previously known as '''clear cell tubulopapillary renal cell carcinoma''',<ref name=pmid21602815>{{Cite journal  | last1 = Rohan | first1 = SM. | last2 = Xiao | first2 = Y. | last3 = Liang | first3 = Y. | last4 = Dudas | first4 = ME. | last5 = Al-Ahmadie | first5 = HA. | last6 = Fine | first6 = SW. | last7 = Gopalan | first7 = A. | last8 = Reuter | first8 = VE. | last9 = Rosenblum | first9 = MK. | title = Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel-Lindau gene and hypoxia-inducible factor pathway-related proteins. | journal = Mod Pathol | volume = 24 | issue = 9 | pages = 1207-20 | month = Sep | year = 2011 | doi = 10.1038/modpathol.2011.80 | PMID = 21602815 }}</ref> abbreviated '''CCTPRCC'''.


==General==
==General==
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**No [[vHL]] gene mutations,<ref name=pmid20924276>{{Cite journal  | last1 = Aydin | first1 = H. | last2 = Chen | first2 = L. | last3 = Cheng | first3 = L. | last4 = Vaziri | first4 = S. | last5 = He | first5 = H. | last6 = Ganapathi | first6 = R. | last7 = Delahunt | first7 = B. | last8 = Magi-Galluzzi | first8 = C. | last9 = Zhou | first9 = M. | title = Clear cell tubulopapillary renal cell carcinoma: a study of 36 distinctive low-grade epithelial tumors of the kidney. | journal = Am J Surg Pathol | volume = 34 | issue = 11 | pages = 1608-21 | month = Nov | year = 2010 | doi = 10.1097/PAS.0b013e3181f2ee0b | PMID = 20924276 }}
**No [[vHL]] gene mutations,<ref name=pmid20924276>{{Cite journal  | last1 = Aydin | first1 = H. | last2 = Chen | first2 = L. | last3 = Cheng | first3 = L. | last4 = Vaziri | first4 = S. | last5 = He | first5 = H. | last6 = Ganapathi | first6 = R. | last7 = Delahunt | first7 = B. | last8 = Magi-Galluzzi | first8 = C. | last9 = Zhou | first9 = M. | title = Clear cell tubulopapillary renal cell carcinoma: a study of 36 distinctive low-grade epithelial tumors of the kidney. | journal = Am J Surg Pathol | volume = 34 | issue = 11 | pages = 1608-21 | month = Nov | year = 2010 | doi = 10.1097/PAS.0b013e3181f2ee0b | PMID = 20924276 }}
</ref> seen in [[clear cell renal cell carcinoma]].
</ref> seen in [[clear cell renal cell carcinoma]].
*Good prognosis - based on a small series.<ref name=pmid20924276/>
*Good prognosis.
*Fourth most common type of RCC is a recent series.<ref name=pmid24182559>{{Cite journal  | last1 = Zhou | first1 = H. | last2 = Zheng | first2 = S. | last3 = Truong | first3 = LD. | last4 = Ro | first4 = JY. | last5 = Ayala | first5 = AG. | last6 = Shen | first6 = SS. | title = Clear cell papillary renal cell carcinoma is the fourth most common histologic type of renal cell carcinoma in 290 consecutive nephrectomies for renal cell carcinoma. | journal = Hum Pathol | volume =  | issue =  | pages =  | month = Oct | year = 2013 | doi = 10.1016/j.humpath.2013.08.004 | PMID = 24182559 }}</ref>
*Previously classified as ''carcinoma''.
**On the basis of 268 cases: it has was suggested that ''clear cell papillary renal cell carcinoma'' be renamed ''clear cell papillary neoplasm of low malignant potential''.<ref name=pmid26426379>{{Cite journal  | last1 = Diolombi | first1 = ML. | last2 = Cheng | first2 = L. | last3 = Argani | first3 = P. | last4 = Epstein | first4 = JI. | title = Do Clear Cell Papillary Renal Cell Carcinomas Have Malignant Potential? | journal = Am J Surg Pathol | volume = 39 | issue = 12 | pages = 1621-34 | month = Dec | year = 2015 | doi = 10.1097/PAS.0000000000000513 | PMID = 26426379 }}</ref>
**The name ''clear cell papillary (cyst)adenoma'' was also proposed.<ref name=pmid33223139>{{cite journal |authors=Williamson SR |title=Clear cell papillary renal cell carcinoma: an update after 15 years |journal=Pathology |volume=53 |issue=1 |pages=109–119 |date=January 2021 |pmid=33223139 |doi=10.1016/j.pathol.2020.10.002 |url=}}</ref>
*Relatively common - if one considers it a RCC: it is the fourth most common type.<ref name=pmid24182559>{{Cite journal  | last1 = Zhou | first1 = H. | last2 = Zheng | first2 = S. | last3 = Truong | first3 = LD. | last4 = Ro | first4 = JY. | last5 = Ayala | first5 = AG. | last6 = Shen | first6 = SS. | title = Clear cell papillary renal cell carcinoma is the fourth most common histologic type of renal cell carcinoma in 290 consecutive nephrectomies for renal cell carcinoma. | journal = Hum Pathol | volume =  | issue =  | pages =  | month = Oct | year = 2013 | doi = 10.1016/j.humpath.2013.08.004 | PMID = 24182559 }}</ref>
**Most common (in order): [[clear cell RCC]], [[papillary RCC]], [[chromophobe RCC]], clear cell papillary RCC.
**Most common (in order): [[clear cell RCC]], [[papillary RCC]], [[chromophobe RCC]], clear cell papillary RCC.


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===Images===
===Images===
<gallery>
<gallery>
Image:Clear_cell_papillary_renal_cell_carcinoma_-_high_mag.jpg | CCPRCC - high mag. (WC/Nephron)
Image:Clear_cell_papillary_renal_cell_carcinoma_-_high_mag.jpg | CCPRCT - high mag. (WC/Nephron)
Image:Clear_cell_papillary_renal_cell_carcinoma_-_very_high_mag.jpg | CCPRCC - very high mag. (WC/Nephron)
Image:Clear_cell_papillary_renal_cell_carcinoma_-_very_high_mag.jpg | CCPRCT - very high mag. (WC/Nephron)
</gallery>
</gallery>
www:
www:
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*HIF-1alpha +ve.  
*HIF-1alpha +ve.  
*GLUT-1 +ve.
*GLUT-1 +ve.
*CA9 +ve (cup-shaped pattern).<ref name=pmid25550767>{{Cite journal  | last1 = Kuroda | first1 = N. | last2 = Ohe | first2 = C. | last3 = Kawakami | first3 = F. | last4 = Mikami | first4 = S. | last5 = Furuya | first5 = M. | last6 = Matsuura | first6 = K. | last7 = Moriyama | first7 = M. | last8 = Nagashima | first8 = Y. | last9 = Zhou | first9 = M. | title = Clear cell papillary renal cell carcinoma: a review. | journal = Int J Clin Exp Pathol | volume = 7 | issue = 11 | pages = 7312-8 | month =  | year = 2014 | doi =  | PMID = 25550767 }}</ref>
*[[CA9]] +ve (cup-shaped pattern).<ref name=pmid25550767>{{Cite journal  | last1 = Kuroda | first1 = N. | last2 = Ohe | first2 = C. | last3 = Kawakami | first3 = F. | last4 = Mikami | first4 = S. | last5 = Furuya | first5 = M. | last6 = Matsuura | first6 = K. | last7 = Moriyama | first7 = M. | last8 = Nagashima | first8 = Y. | last9 = Zhou | first9 = M. | title = Clear cell papillary renal cell carcinoma: a review. | journal = Int J Clin Exp Pathol | volume = 7 | issue = 11 | pages = 7312-8 | month =  | year = 2014 | doi =  | PMID = 25550767 }}</ref>
*CK34betaE12 +ve.<ref name=pmid25676555>{{Cite journal  | last1 = Hakimi | first1 = AA. | last2 = Tickoo | first2 = SK. | last3 = Jacobsen | first3 = A. | last4 = Sarungbam | first4 = J. | last5 = Sfakianos | first5 = JP. | last6 = Sato | first6 = Y. | last7 = Morikawa | first7 = T. | last8 = Kume | first8 = H. | last9 = Fukayama | first9 = M. | title = TCEB1-mutated renal cell carcinoma: a distinct genomic and morphological subtype. | journal = Mod Pathol | volume = 28 | issue = 6 | pages = 845-53 | month = Jun | year = 2015 | doi = 10.1038/modpathol.2015.6 | PMID = 25676555 }}</ref>
*[[GATA3]] +ve.<ref name=pmid28705707>{{cite journal |authors=Mantilla JG, Antic T, Tretiakova M |title=GATA3 as a valuable marker to distinguish clear cell papillary renal cell carcinomas from morphologic mimics |journal=Hum Pathol |volume=66 |issue= |pages=152–158 |date=August 2017 |pmid=28705707 |doi=10.1016/j.humpath.2017.06.016 |url=}}</ref>


How to remember: ''The two key stains are opposite of what the name implies''.
How to remember: ''The two key stains are opposite of what the name implies''.
*''Clear cell'' RCC: CK7 negative.  In this tumour it is the opposite - CK7 is positive.
*''Clear cell'' RCC: CK7 negative.  In this tumour it is the opposite - CK7 is positive.
*''Papillary'' RCC: AMACR positive.  In this tumour it is the opposite - AMACR is negative.
*''Papillary'' RCC: AMACR positive.  In this tumour it is the opposite - AMACR is negative.
==Sign out==
<pre>
Left Kidney Mass, Core Biopsy:
    - CLEAR CELL PAPILARY RENAL CELL TUMOUR, preliminary ISUP nucleolar grade 1 of 4, see comment.
Comment:
The tumour has a tubulocystic architecture and is composed of cuboidal cells with clear cytoplasm. The nuclei are reverse stratified.
The tumour stains as follows:
POSITIVE: PAX8 (diffuse), CK7 (strong, diffuse), CK34betaE12 (strong, diffuse), vimentin (diffuse, strong), EMA (moderate, diffuse).
NEGATIVE: CD10.
The findings are in keeping with clear cell tubulopapillary renal cell tumour. These tumours were previously called "clear cell papillary renal cell carcinoma".
These tumours have a highly favourable clinical behaviour.[1,2]
1. Am J Surg Pathol. 2015 Dec;39(12):1621-34. DOI: 10.1097/PAS.0000000000000513
2. Pathology. 2020 Nov 19;S0031-3025(20)30957-0. DOI: 10.1016/j.pathol.2020.10.002
</pre>


==See also==
==See also==
*[[Kidney tumours]].
*[[Kidney tumours]].
*[[Tubulocystic carcinoma of the kidney]].
*[[Tubulocystic carcinoma of the kidney]].
*[[TCEB1-mutated renal cell carcinoma]].
*[[ELOC-mutated renal cell carcinoma]].


==References==
==References==
Michael
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