Difference between revisions of "Papillary renal neoplasm with reverse polarity"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      =  
| Image      = Papillary renal neoplasm with reverse polarity - alt -- high mag.jpg
| Width      =
| Width      =
| Caption    =  
| Caption    = Papillary renal neoplasm with reverse polarity. [[H&E stain]].
| Synonyms  =
| Synonyms  =
| Micro      = papillary structures, eosinophilic cytoplasm, reverse polarity of nuclei (nuclei at luminal aspect of cell), low nuclear grade
| Micro      = papillary structures, eosinophilic cytoplasm, reverse polarity of nuclei (nuclei at luminal aspect of cell), low nuclear grade
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| Tx        =
| Tx        =
}}
}}
'''Papillary renal neoplasm with reverse polarity''' is a rare [[renal tumour]] and evolving entity.<ref name=pmid31135486>{{cite journal |authors=Al-Obaidy KI, Eble JN, Cheng L, Williamson SR, Sakr WA, Gupta N, Idrees MT, Grignon DJ |title=Papillary Renal Neoplasm With Reverse Polarity: A Morphologic, Immunohistochemical, and Molecular Study |journal=Am J Surg Pathol |volume=43 |issue=8 |pages=1099–1111 |date=August 2019 |pmid=31135486 |doi=10.1097/PAS.0000000000001288 |url=}}</ref>
'''Papillary renal neoplasm with reverse polarity''' is a rare [[renal tumour]] and an evolving entity.<ref name=pmid31135486>{{cite journal |authors=Al-Obaidy KI, Eble JN, Cheng L, Williamson SR, Sakr WA, Gupta N, Idrees MT, Grignon DJ |title=Papillary Renal Neoplasm With Reverse Polarity: A Morphologic, Immunohistochemical, and Molecular Study |journal=Am J Surg Pathol |volume=43 |issue=8 |pages=1099–1111 |date=August 2019 |pmid=31135486 |doi=10.1097/PAS.0000000000001288 |url=}}</ref> It has a distinctive morphology that matches the name.


==General==
==General==
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*Early data suggests an indolent behaviour.<ref name=pmid31135486>{{cite journal |authors=Al-Obaidy KI, Eble JN, Cheng L, Williamson SR, Sakr WA, Gupta N, Idrees MT, Grignon DJ |title=Papillary Renal Neoplasm With Reverse Polarity: A Morphologic, Immunohistochemical, and Molecular Study |journal=Am J Surg Pathol |volume=43 |issue=8 |pages=1099–1111 |date=August 2019 |pmid=31135486 |doi=10.1097/PAS.0000000000001288 |url=}}</ref>
*Early data suggests an indolent behaviour.<ref name=pmid31135486>{{cite journal |authors=Al-Obaidy KI, Eble JN, Cheng L, Williamson SR, Sakr WA, Gupta N, Idrees MT, Grignon DJ |title=Papillary Renal Neoplasm With Reverse Polarity: A Morphologic, Immunohistochemical, and Molecular Study |journal=Am J Surg Pathol |volume=43 |issue=8 |pages=1099–1111 |date=August 2019 |pmid=31135486 |doi=10.1097/PAS.0000000000001288 |url=}}</ref>


==Micro==
==Microscopic==
Features:<ref name=pmid31135486/>
Features:<ref name=pmid31135486/>
*Branching papillae with:
*Branching papillae with:
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DDx:
DDx:
*Eosinophilic [[papillary renal cell carcinoma]] - considered distinct from this entity.
*[[Renal tumours with eosinophilic cytoplasm]].
*[[Renal tumours with eosinophilic cytoplasm]].
===Images===
<gallery>
Image: Papillary renal neoplasm with reverse polarity -- very low mag.jpg | PRNRP - very low mag.
Image: Papillary renal neoplasm with reverse polarity -- low mag.jpg | PRNRP - low mag.
Image: Papillary renal neoplasm with reverse polarity - alt -- low mag.jpg | PRNRP - low mag.
Image: Papillary renal neoplasm with reverse polarity -- intermed mag.jpg | PRNRP - intermed. mag.
Image: Papillary renal neoplasm with reverse polarity -- high mag.jpg | PRNRP - high mag.
Image: Papillary renal neoplasm with reverse polarity -- very high mag.jpg | PRNRP - very high mag.
</gallery>


==IHC==
==IHC==
Features:<ref name=pmid31135486/>
Features:<ref name=pmid31135486/>
*CK7 +ve.
*GATA3 +ve (43 of 43 cases<ref name=pmid37797754/>) - '''key stain'''.
*CD10 +ve (all cases).
**Negative in other papillary RCCs.<ref name=pmid37797754/>
*GATA3 +ve (all cases).
*CK7 +ve (88% +ve, 37 of 42 cases<ref name=pmid37797754/>).
*CD10 +ve/-ve (50% +ve, 21 of 42 cases<ref name=pmid37797754/>).
*Vimentin -ve (all cases).
*Vimentin -ve (all cases).
*CD117 -ve.
*CD117 -ve.
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*EMA +ve.
*EMA +ve.
*L1CAM +ve.
*L1CAM +ve.
*PAX8 +ve.
*AMACR -ve (4 of 43 cases<ref name=pmid37797754/>).
*RCC -ve (1 of 43 cases<ref name=pmid37797754/>).
===Differentiating between papillary RCC and PRNRP===
Adapted from Kim ''et al.'':<ref name=pmid37797754/>
{| class="wikitable sortable"
!Diagnosis
!GATA3
!AMACR
!RCC
!Vimentin
|-
|Papillary RCC
| 0% +ve
| 85-90% +ve
| 60-75% +ve
| 60-70% +ve
|-
|PRNRP
| 100% +ve
| ~10% +ve
| ~2% +ve
| ~2% +ve
|-
|}


==Molecular==
==Molecular==
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Comment:
Comment:
The tumour has a papillary architecture, eosinophilic cytoplasm and reversed nuclear polarity.
The tumour has a papillary architecture, eosinophilic cytoplasm, low nuclear grade, and reversed nuclear polarity.


It stains as follows:
It stains as follows:
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NEGATIVE: vimentin (stroma), CD117, CD10.
NEGATIVE: vimentin (stroma), CD117, CD10.


PRNRP is thought to be distinct from papillary RCC.[1][2]  Limited data suggests PRNRP has an indolent behaviour.[3] Follow-up is recommended.  
The findings are in keeping with papillary renal neoplasm with reverse polarity (PRNRP). PRNRP is thought to be distinct from papillary RCC.[1][2]  Limited data suggests PRNRP has an indolent behaviour.[3] Follow-up is recommended.  


1. Pathol Int. 2024 Apr;74(4):222-226. doi: 10.1111/pin.13417. https://pubmed.ncbi.nlm.nih.gov/38456605/
1. Pathol Int. 2024 Apr;74(4):222-226. doi: 10.1111/pin.13417. https://pubmed.ncbi.nlm.nih.gov/38456605/
48,849

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