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 | | 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 = [[ | | 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 | ||
| Line 13: | Line 14: | ||
| 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 = | ||
| Line 28: | Line 30: | ||
| ClinDDx = other [[renal tumours]] | | ClinDDx = other [[renal tumours]] | ||
}} | }} | ||
'''Clear cell papillary renal cell | '''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''' | 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== | ||
* | *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 - | *Good prognosis. | ||
* | *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. | ||
==Microscopic== | ==Microscopic== | ||
Features:<ref name=pmid21602815/> | Features:<ref name=pmid21602815/> | ||
* | *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]]. | ||
*[[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 | | 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 | | Image:Clear_cell_papillary_renal_cell_carcinoma_-_very_high_mag.jpg | CCPRCT - very high mag. (WC/Nephron) | ||
</gallery> | </gallery> | ||
www: | www: | ||
| Line 68: | Line 84: | ||
==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
- Relatively new entity.
- Shown to be distinct on a molecular basis.[3]
- No vHL gene mutations,[4] seen in clear cell renal cell carcinoma.
- Good prognosis.
- Previously classified as carcinoma.
- Relatively common - if one considers it a RCC: it is the fourth most common type.[7]
- Most common (in order): clear cell RCC, papillary RCC, chromophobe RCC, clear cell papillary RCC.
Microscopic
Features:[1]
- Some features of both:
- Clear cell RCC.
- Clear cells.
- Papillary RCC.
- Pseudopapillae - often do not have true papillae.
- Clear cell RCC.
- 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: ‡
- Apical nuclei may be seen focally in clear cell renal cell carcinoma.
- Nuclei should be low grade (ISUP nucleolar grade 1 or 2).
DDx:
- Clear cell renal cell carcinoma - main DDx.
- Papillary renal cell carcinoma.
- Xp11.2 translocation carcinoma.
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
CCPRCT - high mag. (WC/Nephron)
CCPRCT - very high mag. (WC/Nephron)
www:
IHC
Features:[1]
- CK7 +ve - esp. diffuse.[10]
- Usually -ve (or patchy) in clear cell RCC.
- CD10 -ve (0 +ve/9 cases in one series[1]).
- Usually +ve in clear cell RCC.
- Positive in Xp11.2 translocation carcinoma.[11]
- TFE3 -ve.
- By definition +ve in Xp11.2 translocation carcinoma.
- AMACR -ve.[12]
- Usu. +ve in Papillary RCC.
- Smooth muscle actin +ve (focal).[13]
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
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Rohan, SM.; Xiao, Y.; Liang, Y.; Dudas, ME.; Al-Ahmadie, HA.; Fine, SW.; Gopalan, A.; Reuter, VE. et al. (Sep 2011). "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.". Mod Pathol 24 (9): 1207-20. doi:10.1038/modpathol.2011.80. PMID 21602815.
- ↑ Srigley, JR.; Delahunt, B.; Eble, JN.; Egevad, L.; Epstein, JI.; Grignon, D.; Hes, O.; Moch, H. et al. (Oct 2013). "The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.". Am J Surg Pathol 37 (10): 1469-89. doi:10.1097/PAS.0b013e318299f2d1. PMID 24025519.
- ↑ Rohan, SM.; Xiao, Y.; Liang, Y.; Dudas, ME.; Al-Ahmadie, HA.; Fine, SW.; Gopalan, A.; Reuter, VE. et al. (Sep 2011). "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.". Mod Pathol 24 (9): 1207-20. doi:10.1038/modpathol.2011.80. PMID 21602815.
- ↑ Aydin, H.; Chen, L.; Cheng, L.; Vaziri, S.; He, H.; Ganapathi, R.; Delahunt, B.; Magi-Galluzzi, C. et al. (Nov 2010). "Clear cell tubulopapillary renal cell carcinoma: a study of 36 distinctive low-grade epithelial tumors of the kidney.". Am J Surg Pathol 34 (11): 1608-21. doi:10.1097/PAS.0b013e3181f2ee0b. PMID 20924276.
- ↑ Diolombi, ML.; Cheng, L.; Argani, P.; Epstein, JI. (Dec 2015). "Do Clear Cell Papillary Renal Cell Carcinomas Have Malignant Potential?". Am J Surg Pathol 39 (12): 1621-34. doi:10.1097/PAS.0000000000000513. PMID 26426379.
- ↑ Williamson SR (January 2021). "Clear cell papillary renal cell carcinoma: an update after 15 years". Pathology 53 (1): 109–119. doi:10.1016/j.pathol.2020.10.002. PMID 33223139.
- ↑ Zhou, H.; Zheng, S.; Truong, LD.; Ro, JY.; Ayala, AG.; Shen, SS. (Oct 2013). "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.". Hum Pathol. doi:10.1016/j.humpath.2013.08.004. PMID 24182559.
- ↑ Bing, Z.; Tomaszewski, JE. (2011). "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.". Case Reports in Transplantation 2011. doi:10.1155/2011/387645. http://www.hindawi.com/crim/transplantation/2011/387645/cta/.
- ↑ Petersson, F.; Grossmann, P.; Hora, M.; Sperga, M.; Montiel, DP.; Martinek, P.; Gutierrez, ME.; Bulimbasic, S. et al. (Jul 2013). "Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma.". Hum Pathol 44 (7): 1412-20. doi:10.1016/j.humpath.2012.11.019. PMID 23434146.
- ↑ Williamson, SR.; Gupta, NS.; Eble, JN.; Rogers, CG.; Michalowski, S.; Zhang, S.; Wang, M.; Grignon, DJ. et al. (Nov 2015). "Clear Cell Renal Cell Carcinoma With Borderline Features of Clear Cell Papillary Renal Cell Carcinoma: Combined Morphologic, Immunohistochemical, and Cytogenetic Analysis.". Am J Surg Pathol 39 (11): 1502-10. doi:10.1097/PAS.0000000000000514. PMID 26457355.
- ↑ He, H.; Zhou, GX.; Zhou, M.; Chen, L. (Sep 2011). "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.". Int J Gynecol Pathol 30 (5): 425-30. doi:10.1097/PGP.0b013e318214dd4f. PMID 21804394.
- ↑ Pramick, M.; Ziober, A.; Bing, Z. (Oct 2013). "Useful immunohistochemical panel for differentiating clear cell papillary renal cell carcinoma from its mimics.". Ann Diagn Pathol 17 (5): 437-40. doi:10.1016/j.anndiagpath.2013.05.004. PMID 23791764.
- ↑ Williamson, SR.; Eble, JN.; Cheng, L.; Grignon, DJ. (May 2013). "Clear cell papillary renal cell carcinoma: differential diagnosis and extended immunohistochemical profile.". Mod Pathol 26 (5): 697-708. doi:10.1038/modpathol.2012.204. PMID 23238627.
- ↑ Kuroda, N.; Ohe, C.; Kawakami, F.; Mikami, S.; Furuya, M.; Matsuura, K.; Moriyama, M.; Nagashima, Y. et al. (2014). "Clear cell papillary renal cell carcinoma: a review.". Int J Clin Exp Pathol 7 (11): 7312-8. PMID 25550767.
- ↑ Hakimi, AA.; Tickoo, SK.; Jacobsen, A.; Sarungbam, J.; Sfakianos, JP.; Sato, Y.; Morikawa, T.; Kume, H. et al. (Jun 2015). "TCEB1-mutated renal cell carcinoma: a distinct genomic and morphological subtype.". Mod Pathol 28 (6): 845-53. doi:10.1038/modpathol.2015.6. PMID 25676555.
- ↑ Mantilla JG, Antic T, Tretiakova M (August 2017). "GATA3 as a valuable marker to distinguish clear cell papillary renal cell carcinomas from morphologic mimics". Hum Pathol 66: 152–158. doi:10.1016/j.humpath.2017.06.016. PMID 28705707.