Papillary renal neoplasm with reverse polarity

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Papillary renal neoplasm with reverse polarity
Diagnosis in short
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
IHC GATA3 +ve, PAX-8 +ve, CK7 +ve, CD10 +ve, CD117 -ve
Site kidney - see kidney tumours

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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