Papillary renal neoplasm with reverse polarity

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Papillary renal neoplasm with reverse polarity is a rare renal tumour and evolving entity.[1]

General

Micro

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:

IHC

Features:[1]

  • CK7 +ve.
  • CD10 +ve (all cases).
  • GATA3 +ve (all cases).
  • Vimentin -ve (all cases).
  • CD117 -ve.
  • AE1/AE3 +ve.
  • EMA +ve.
  • L1CAM +ve.

Molecular

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A. Right Kidney, Tumour, Partial Nephrectomy:
	- Papillary renal neoplasm with reverse polarity, see comment.

Comment:
The tumour has a papillary architecture, eosinophilic cytoplasm 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.

These tumour are thought to be distinct from papillary RCC.[1][2]  Limited data suggests they have an indolent behaviour.[3]  

1. Pathol Int. 2024 Apr;74(4):222-226. doi: 10.1111/pin.13417. Epub 2024 Mar 8.
2. Hum Pathol. 2023 Dec:142:1-6. doi: 10.1016/j.humpath.2023.09.011. Epub 2023 Oct 4.
3. Am J Surg Pathol. 2019 Aug;43(8):1099-1111. doi: 10.1097/PAS.0000000000001288.

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 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.
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