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

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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]].
| Micro      = apical nuclei, clear cells, pseudopapillae, cystic and/or tubular architectures
| 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
| Subtypes  =
| Subtypes  =
| LMDDx      = [[papillary renal cell carcinoma]], [[clear cell 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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| IF        =
| IF        =
| Gross      =
| Gross      =
| Grossing  =
| Grossing  = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
| Staging    = [[kidney cancer staging]]
| Site      = [[kidney]] - see [[renal tumours]]
| Site      = [[kidney]] - see [[renal tumours]]
| Assdx      =
| Assdx      =
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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>  
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==
*New entity - not in the 2004 WHO classification of renal tumours.<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>
*Relatively new entity.
**Not in the (original) 2004 [[WHO]] classification of renal tumours.<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>
***Added in ''[[Vancouver classification|The 2012 Vancouver modification of the WHO classification]]''.<ref name=pmid24025519>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref>
*Shown to be distinct on a molecular basis.<ref>{{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>
*Shown to be distinct on a molecular basis.<ref>{{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>
**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''.
**Most common (in order): [[clear cell RCC]], [[papillary RCC]], [[chromophobe RCC]], [[clear cell papillary RCC]].
**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.


==Microscopic==
==Microscopic==
Features:<ref name=pmid21602815/>
Features:<ref name=pmid21602815/>
*Features of both:
*Some features of both:
*#Clear cell RCC.
*#Clear cell RCC.
*#*Clear cells.
*#*Clear cells.
*#Papillary RCC.
*#Papillary RCC.
*#*Pseudopapillae - often do not have true papillae.
*#*Pseudopapillae - often do not have true papillae.
*Apical nuclei - '''key feature'''.<ref name=bing>{{Cite journal  | last1 = Bing | first1 = Z. | last2 = Tomaszewski | first2 = JE. | title = Case Report: Clear Cell Papillary Renal Cell Carcinoma in the  Bilateral Native Kidneys after 2 Years of Renal Transplantation:  Report of a Case and Review of  the Literature. | journal = Case Reports in Transplantation | volume = 2011 | issue = | pages =  | month =  | year = 2011 | doi = 10.1155/2011/387645 | PMID =  | url = http://www.hindawi.com/crim/transplantation/2011/387645/cta/ }}</ref>
*Apical nuclei - should be predominant ‡ - '''key feature'''.<ref name=bing>{{Cite journal  | last1 = Bing | first1 = Z. | last2 = Tomaszewski | first2 = JE. | title = Case Report: Clear Cell Papillary Renal Cell Carcinoma in the  Bilateral Native Kidneys after 2 Years of Renal Transplantation:  Report of a Case and Review of  the Literature. | journal = Case Reports in Transplantation | volume = 2011 | issue = | pages =  | month =  | year = 2011 | doi = 10.1155/2011/387645 | PMID =  | url = http://www.hindawi.com/crim/transplantation/2011/387645/cta/ }}</ref>
**In most glandular structures the nuclei are usu. basally located, i.e. in the cytoplasm adjacent to the basement membrane.
**In most glandular structures the nuclei are usu. basally located, i.e. in the cytoplasm adjacent to the basement membrane.
*Tubular and/or cystic architecture.
*Tubular and/or cystic architecture.
Notes: ‡
*Apical nuclei may be seen focally in [[clear cell renal cell carcinoma]].
*Nuclei should be low grade ([[ISUP nucleolar grade]] 1 or 2).


DDx:
DDx:
*[[Clear cell renal cell carcinoma]] - main DDx.
*[[Papillary renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Clear cell renal cell carcinoma]]
*[[Xp11.2 translocation carcinoma]].
*[[Xp11.2 translocation carcinoma]].
Note:
*[[Renal angiomyoadenomatous tumour]] has been proposed as separate entity.<ref name=pmid23434146>{{Cite journal  | last1 = Petersson | first1 = F. | last2 = Grossmann | first2 = P. | last3 = Hora | first3 = M. | last4 = Sperga | first4 = M. | last5 = Montiel | first5 = DP. | last6 = Martinek | first6 = P. | last7 = Gutierrez | first7 = ME. | last8 = Bulimbasic | first8 = S. | last9 = Michal | first9 = M. | title = Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma. | journal = Hum Pathol | volume = 44 | issue = 7 | pages = 1412-20 | month = Jul | year = 2013 | doi = 10.1016/j.humpath.2012.11.019 | PMID = 23434146 }}</ref> ''The Vancouver modification of the WHO classification'' does '''not''' consider it a separate entity; it lumps it with ''clear cell papillary renal cell carcinoma''.


===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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==IHC==
==IHC==
Features:<ref name=pmid21602815/>
Features:<ref name=pmid21602815/>
*CK7 +ve.
*CK7 +ve - esp. diffuse.<ref name=pmid26457355>{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Gupta | first2 = NS. | last3 = Eble | first3 = JN. | last4 = Rogers | first4 = CG. | last5 = Michalowski | first5 = S. | last6 = Zhang | first6 = S. | last7 = Wang | first7 = M. | last8 = Grignon | first8 = DJ. | last9 = Cheng | first9 = L. | title = Clear Cell Renal Cell Carcinoma With Borderline Features of Clear Cell Papillary Renal Cell Carcinoma: Combined Morphologic, Immunohistochemical, and Cytogenetic Analysis. | journal = Am J Surg Pathol | volume = 39 | issue = 11 | pages = 1502-10 | month = Nov | year = 2015 | doi = 10.1097/PAS.0000000000000514 | PMID = 26457355 }}</ref>
**Usually -ve in clear cell RCC.
**Usually -ve (or patchy) in clear cell RCC.
*CD10 -ve.
*CD10 -ve (0 +ve/9 cases in one series<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>).  
**Usually +ve in clear cell RCC.
**Usually +ve in clear cell RCC.
**Positive in Xp11.2 translocation carcinoma.<ref name=pmid21804394>{{Cite journal  | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref>
**Positive in Xp11.2 translocation carcinoma.<ref name=pmid21804394>{{Cite journal  | last1 = He | first1 = H. | last2 = Zhou | first2 = GX. | last3 = Zhou | first3 = M. | last4 = Chen | first4 = L. | title = The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray. | journal = Int J Gynecol Pathol | volume = 30 | issue = 5 | pages = 425-30 | month = Sep | year = 2011 | doi = 10.1097/PGP.0b013e318214dd4f | PMID = 21804394 }}</ref>
*TFE3 -ve.
*TFE3 -ve.
**By definition +ve in [[Xp11.2 translocation carcinoma]].
**By definition +ve in [[Xp11.2 translocation carcinoma]].
*AMACR -ve.
*[[AMACR]] -ve.<ref name=pmid23791764>{{Cite journal  | last1 = Pramick | first1 = M. | last2 = Ziober | first2 = A. | last3 = Bing | first3 = Z. | title = Useful immunohistochemical panel for differentiating clear cell papillary renal cell carcinoma from its mimics. | journal = Ann Diagn Pathol | volume = 17 | issue = 5 | pages = 437-40 | month = Oct | year = 2013 | doi = 10.1016/j.anndiagpath.2013.05.004 | PMID = 23791764 }}</ref>
**Usu. +ve in [[Papillary RCC]].
**Usu. +ve in [[Papillary RCC]].
*Smooth muscle actin +ve (focal).<ref name=pmid23238627>{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Eble | first2 = JN. | last3 = Cheng | first3 = L. | last4 = Grignon | first4 = DJ. | title = Clear cell papillary renal cell carcinoma: differential diagnosis and extended immunohistochemical profile. | journal = Mod Pathol | volume = 26 | issue = 5 | pages = 697-708 | month = May | year = 2013 | doi = 10.1038/modpathol.2012.204 | PMID = 23238627 }}</ref>
*Smooth muscle actin +ve (focal).<ref name=pmid23238627>{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Eble | first2 = JN. | last3 = Cheng | first3 = L. | last4 = Grignon | first4 = DJ. | title = Clear cell papillary renal cell carcinoma: differential diagnosis and extended immunohistochemical profile. | journal = Mod Pathol | volume = 26 | issue = 5 | pages = 697-708 | month = May | year = 2013 | doi = 10.1038/modpathol.2012.204 | PMID = 23238627 }}</ref>
Line 82: Line 98:
*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>
*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]].
*[[ELOC-mutated renal cell carcinoma]].


==References==
==References==

Latest revision as of 02:55, 20 March 2024

Clear cell papillary renal cell tumour
Diagnosis in short

Clear cell papillary renal cell tumour. H&E stain.

Synonyms previously known as clear cell tubulopapillary renal cell carcinoma and clear cell papillary renal cell carcinoma

LM low (ISUP nucleolar) grade apical nuclei, clear cells, pseudopapillae, cystic and/or tubular architectures
LM DDx clear cell renal cell carcinoma, papillary renal cell carcinoma, Xp11.2 translocation carcinoma
IHC CK7 +ve, AMACR -ve, CD10 -ve, TFE3 -ve
Grossing notes total nephrectomy for tumour grossing, partial nephrectomy grossing
Staging kidney cancer staging
Site kidney - see renal tumours

Prevalence uncommon
Prognosis good
Clin. DDx other renal tumours

Clear cell papillary renal cell tumour, abbreviated CCPRCT, is a relatively common benign renal tumour.

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,[1] abbreviated CCTPRCC.

General

Microscopic

Features:[1]

  • Some features of both:
    1. Clear cell RCC.
      • Clear cells.
    2. Papillary RCC.
      • Pseudopapillae - often do not have true papillae.
  • Apical nuclei - should be predominant ‡ - key feature.[8]
    • In most glandular structures the nuclei are usu. basally located, i.e. in the cytoplasm adjacent to the basement membrane.
  • Tubular and/or cystic architecture.

Notes: ‡

DDx:

Note:

  • Renal angiomyoadenomatous tumour has been proposed as separate entity.[9] The Vancouver modification of the WHO classification does not consider it a separate entity; it lumps it with clear cell papillary renal cell carcinoma.

Images

www:

IHC

Features:[1]

Others:[1]

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.
  • Papillary RCC: AMACR positive. In this tumour it is the opposite - AMACR is negative.

Sign out

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

See also

References

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