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

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Papillary renal neoplasm with reverse polarity (view source)

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'''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>
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Papillary renal neoplasm with reverse polarity - alt -- high mag.jpg
| Width      =
| Caption    = Papillary renal neoplasm with reverse polarity. [[H&E stain]].
| Synonyms  =
| Micro      = papillary structures, eosinophilic cytoplasm, reverse polarity of nuclei (nuclei at luminal aspect of cell), low nuclear grade
| Subtypes  =
| LMDDx      = eosinophilic [[papillary renal cell carcinoma]], other [[renal tumours with eosinophilic cytoplasm]]
| Stains    =
| IHC        = GATA3 +ve, PAX-8 +ve, CK7 +ve, CD10 +ve, CD117 -ve
| EM        =
| Molecular  = [[KRAS]] mutations
| IF        =
| Gross      =
| Grossing  = [[partial nephrectomy]], [[radical nephrectomy]]
| Staging    =
| Site      = [[kidney]] - see ''[[kidney tumours]]''
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = indolent - based on limited data
| Other      =
| ClinDDx    = other [[kidney tumours]]
| Tx        =
}}
'''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==
*Evolving entity.
*Thought to be distinct from ''eosinophilic [[papillary renal cell carcinoma]]''.<ref name=pmid38456605>{{cite journal |authors=Castillo VF, Trpkov K, Van der Kwast T, Rotondo F, Hamdani M, Saleeb R |title=Papillary renal neoplasm with reverse polarity is biologically and clinically distinct from eosinophilic papillary renal cell carcinoma |journal=Pathol Int |volume=74 |issue=4 |pages=222–226 |date=April 2024 |pmid=38456605 |doi=10.1111/pin.13417 |url=}}</ref><ref name=pmid37797754>{{cite journal |authors=Kim B, Lee S, Moon KC |title=Papillary renal neoplasm with reverse polarity: a clinicopathologic study of 43 cases with a focus on the expression of KRAS signaling pathway downstream effectors |journal=Hum Pathol |volume=142 |issue= |pages=1–6 |date=December 2023 |pmid=37797754 |doi=10.1016/j.humpath.2023.09.011 |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>
 
==Microscopic==
Features:<ref name=pmid31135486/>
*Branching papillae with:
**Thin fibrovascular cores.
**Cuboidal to columnar lining cells with granular eosinophilic cytoplasm.
***May have occasional cytoplasmic clearing.
**Smooth luminal borders.
**Reverse polarized nuclei (luminal nuclei; nuclei closer to lumen than basement membrane).
**Nucleoli absent or small.
 
DDx:
*Eosinophilic [[papillary renal cell carcinoma]] - considered distinct from this entity.
*[[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==
*[[KRAS]] mutations.<ref name=pmid37797754/>
==Sign out==
<pre>
A. Right Kidney, Tumour, Partial Nephrectomy:
- Papillary renal neoplasm with reverse polarity, clear of margin, see comment.
Comment:
The tumour has a papillary architecture, eosinophilic cytoplasm, low nuclear grade, and reversed nuclear polarity.
It stains as follows:
POSITIVE: PAX8 (moderate, diffuse), CK7 (strong, diffuse), AE1/AE3 (strong, diffuse), GATA3 (moderate, diffuse), AMACR (moderate, diffuse).
NEGATIVE: vimentin (stroma), CD117, CD10.
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/
2. Hum Pathol. 2023 Dec:142:1-6. doi: 10.1016/j.humpath.2023.09.011. https://pubmed.ncbi.nlm.nih.gov/37797754/
3. Am J Surg Pathol. 2019 Aug;43(8):1099-1111. doi: 10.1097/PAS.0000000000001288. https://pubmed.ncbi.nlm.nih.gov/31135486/
</pre>


==See also==
==See also==
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==References==
==References==
{{Reflist|1}}
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Kidney tumours]]
Michael
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