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

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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==
Features:<ref name=pmid31135486/>
*GATA3 +ve (43 of 43 cases<ref name=pmid37797754/>) - '''key stain'''.
**Negative in other papillary RCCs.<ref name=pmid37797754/>
*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).
*CD117 -ve.
*AE1/AE3 +ve.
*EMA +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==
*[[Renal tumours with eosinophilic cytoplasm]].


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


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

Latest revision as of 14:09, 16 August 2024

Papillary renal neoplasm with reverse polarity
Diagnosis in short

Papillary renal neoplasm with reverse polarity. H&E stain.

LM papillary structures, eosinophilic cytoplasm, reverse polarity of nuclei (nuclei at luminal aspect of cell), low nuclear grade
LM DDx eosinophilic papillary renal cell carcinoma, other renal tumours with eosinophilic cytoplasm
IHC GATA3 +ve, PAX-8 +ve, CK7 +ve, CD10 +ve, CD117 -ve
Molecular KRAS mutations
Grossing notes partial nephrectomy, radical nephrectomy
Site kidney - see kidney tumours

Prevalence rare
Prognosis indolent - based on limited data
Clin. DDx other kidney tumours

Papillary renal neoplasm with reverse polarity is a rare renal tumour and an evolving entity.[1] It has a distinctive morphology that matches the name.

General

Microscopic

Features:[1]

  • 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:

Images

IHC

Features:[1]

  • GATA3 +ve (43 of 43 cases[3]) - key stain.
    • Negative in other papillary RCCs.[3]
  • CK7 +ve (88% +ve, 37 of 42 cases[3]).
  • CD10 +ve/-ve (50% +ve, 21 of 42 cases[3]).
  • Vimentin -ve (all cases).
  • CD117 -ve.
  • AE1/AE3 +ve.
  • EMA +ve.
  • L1CAM +ve.
  • PAX8 +ve.
  • AMACR -ve (4 of 43 cases[3]).
  • RCC -ve (1 of 43 cases[3]).

Differentiating between papillary RCC and PRNRP

Adapted from Kim et al.:[3]

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

Sign out

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/

See also

References

  1. 1.0 1.1 1.2 1.3 Al-Obaidy KI, Eble JN, Cheng L, Williamson SR, Sakr WA, Gupta N, Idrees MT, Grignon DJ (August 2019). "Papillary Renal Neoplasm With Reverse Polarity: A Morphologic, Immunohistochemical, and Molecular Study". Am J Surg Pathol 43 (8): 1099–1111. doi:10.1097/PAS.0000000000001288. PMID 31135486.
  2. Castillo VF, Trpkov K, Van der Kwast T, Rotondo F, Hamdani M, Saleeb R (April 2024). "Papillary renal neoplasm with reverse polarity is biologically and clinically distinct from eosinophilic papillary renal cell carcinoma". Pathol Int 74 (4): 222–226. doi:10.1111/pin.13417. PMID 38456605.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Kim B, Lee S, Moon KC (December 2023). "Papillary renal neoplasm with reverse polarity: a clinicopathologic study of 43 cases with a focus on the expression of KRAS signaling pathway downstream effectors". Hum Pathol 142: 1–6. doi:10.1016/j.humpath.2023.09.011. PMID 37797754.