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

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

Latest revision as of 14:09, 16 August 2024

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

Evolving entity.
Thought to be distinct from eosinophilic papillary renal cell carcinoma.^[2]^[3]
Early data suggests an indolent behaviour.^[1]

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:

Eosinophilic papillary renal cell carcinoma - considered distinct from this entity.
Renal tumours with eosinophilic cytoplasm.

Images

PRNRP - very low mag.
PRNRP - low mag.
PRNRP - low mag.
PRNRP - intermed. mag.
PRNRP - high mag.
PRNRP - very high mag.

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

KRAS mutations.^[3]

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/

References

↑ ^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.
↑ 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.
↑ ^3.0 ^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.

[pmid31135486-1] 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.

[pmid38456605-2] 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.

[pmid37797754-3] 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.

[1]

[2]

[3]

@@ Line 1: / Line 1: @@
-'''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>
-==Micro==
+==Microscopic==
 Features:<ref name=pmid31135486/>
 *Branching papillae with:
@@ Line 16: / Line 49: @@
 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/>
-*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).
 *CD117 -ve.
@@ Line 28: / Line 73: @@
 *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.
+. Pathol Int. 2024 Apr;74(4):222-226. doi: 10.1111/pin.13417. https://pubmed.ncbi.nlm.nih.gov/38456605/
+. Hum Pathol. 2023 Dec:142:1-6. doi: 10.1016/j.humpath.2023.09.011. https://pubmed.ncbi.nlm.nih.gov/37797754/
+. 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==
@@ Line 36: / Line 126: @@
 ==References==
-{{Reflist|1}}
+{{Reflist|2}}
 [[Category:Diagnosis]]
+[[Category:Kidney tumours]]

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

Latest revision as of 14:09, 16 August 2024

Contents