Clear cell papillary renal cell tumour: Difference between revisions

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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 (reverse polarized), 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, GATA-3 +ve, MA903 +ve, CD10 -ve, AMACR -ve, TFE3 -ve
| EM        =
| EM        =
| Molecular  =
| Molecular  =
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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 was 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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</ref> seen in [[clear cell renal cell carcinoma]].
</ref> seen in [[clear cell renal cell carcinoma]].
*Good prognosis.
*Good prognosis.
**On the basis of 268 cases: it has been 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>
*Previously classified as ''carcinoma''.
**The name ''clear cell papillary (cyst)adenoma'' has also been proposed.<ref name=pmid33223139>{{cite journal |vauthors=Williamson SR |title=Clear cell papillary renal cell carcinoma: an update after 15 years |journal=Pathology |volume= |issue= |pages= |date=November 2020 |pmid=33223139 |doi=10.1016/j.pathol.2020.10.002 |url=}}</ref>
**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>
*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>
**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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*[[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>
*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''.
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==Sign out==
==Sign out==
<pre>
<pre>
Left Kidney Mass, Core Biopsy:
A. Left Kidney Mass, Radical Nephrectomy:
    - CLEAR CELL TUBULOPAPILARY RENAL CELL CARCINOMA (clear cell
    - CLEAR CELL PAPILLARY RENAL CELL TUMOUR, nucleolar grade 1 of 4, see comment.
      papillary renal cell carcinoma), preliminary ISUP nucleolar  
    -- Margins clear.
      grade 1 of 4, see comment.
    -- Please see synoptic report.


Comment:
Comment:
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The tumour stains as follows:  
The tumour stains as follows:  
POSITIVE: PAX8 (diffuse), CK7 (strong, diffuse), CK34betaE12 (strong, diffuse), vimentin (diffuse, strong), EMA (moderate, diffuse).
POSITIVE: PAX8 (strong, diffuse), CK7 (strong, diffuse), MA903 (strong, diffuse), CAIX (moderate, patchy cup-like), AE1/AE3 (moderate, diffuse), GATA3 (moderate, diffuse).
NEGATIVE: CD10.
NEGATIVE: CD10, AMACR.


The findings are in keeping with clear cell tubulopapillary renal cell carcinoma.  
The findings are in keeping with clear cell papillary renal cell tumour. These tumours were previously called "clear cell papillary renal cell carcinoma".[1]


These tumours have a highly favourable clinical behaviour.[1,2] The terms "clear cell papillary neoplasm of low malignant potential"[1] and "clear cell papillary (cyst)adenoma"[2] have been proposed as names that better reflect their biology.
These tumours have a highly favourable clinical behaviour.[2,3]  


1. Am J Surg Pathol. 2015 Dec;39(12):1621-34. DOI: 10.1097/PAS.0000000000000513
1. Pathologica. 2022 Feb;115(1):8-22. doi: 10.32074/1591-951X-818. https://pmc.ncbi.nlm.nih.gov/articles/PMC10342217/
2. Pathology. 2020 Nov 19;S0031-3025(20)30957-0. DOI: 10.1016/j.pathol.2020.10.002
2. Am J Surg Pathol. 2015 Dec;39(12):1621-34. DOI: 10.1097/PAS.0000000000000513
3. Pathology. 2020 Nov 19;S0031-3025(20)30957-0. DOI: 10.1016/j.pathol.2020.10.002
</pre>
</pre>
===Biopsy===
Diagnosing the this entity on biopsy should be done with caution as:
*Overlaps are described between ''clear cell renal cell carcinoma'' and ''clear cell papillary renal cell tumour''.<ref name=pmid26752401>{{cite journal |vauthors=Dhakal HP, McKenney JK, Khor LY, Reynolds JP, Magi-Galluzzi C, Przybycin CG |title=Renal Neoplasms With Overlapping Features of Clear Cell Renal Cell Carcinoma and Clear Cell Papillary Renal Cell Carcinoma: A Clinicopathologic Study of 37 Cases From a Single Institution |journal=Am J Surg Pathol |volume=40 |issue=2 |pages=141–54 |date=February 2016 |pmid=26752401 |doi=10.1097/PAS.0000000000000583 |url=}}</ref>
*''Clear cell papillary renal cell tumour'' may also be seen in conjunction with (other) renal cell carcinoma(s).<ref name=pmid26857533>{{cite journal |authors=Shao T, Yousef P, Shipilova I, Saleeb R, Lee JY, Krizova A |title=Clear cell papillary renal cell carcinoma as part of histologically discordant multifocal renal cell carcinoma: A case report and review of literature |journal=Pathol Res Pract |volume=212 |issue=3 |pages=229–33 |date=March 2016 |pmid=26857533 |doi=10.1016/j.prp.2015.12.007 |url=}}</ref>


==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==
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