Clear cell papillary renal cell carcinoma: an update after 15 years.

Pathology
Sean R Williamson

Abstract

Fifteen years since the first recognition of clear cell papillary renal cell carcinoma, this distinct renal tumour type is now well accepted as a distinct entity in major classification schemes. It occurs both with and without end-stage renal disease and may be multifocal or bilateral in both scenarios. Recognisable morphological features include clear cells lining branching glands and variable papillary formations with nuclear alignment. Most tumours are small (pT1a) and nucleolar grade 1-2. Immunohistochemistry consistently shows positivity for carbonic anhydrase IX and cytokeratin 7, and often high molecular weight cytokeratin or GATA3, the latter suggesting distal nephron phenotype. Labeling for AMACR and CD10 is consistently negative or minimal. Despite a resemblance to clear cell renal cell carcinoma, molecular alterations of VHL and chromosome 3p are typically lacking, with debatable rare exceptions. Potential mimics include clear cell renal cell carcinoma (with branching architecture or nuclear alignment), papillary renal cell carcinoma with clear cytoplasm, or rarely MITF family translocation renal cell carcinoma. Clinical behaviour is highly favourable with rare, debatable reports of aggressive behaviour. Combined wit...Continue Reading

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Citations

Dec 1, 2020·Pathology·Lars EgevadJohn N Eble
Jun 22, 2021·Frontiers in Cell and Developmental Biology·Zhengjin HeHai Jiang
Oct 7, 2021·Virchows Archiv : an International Journal of Pathology·Sofia Canete-PortilloCristina Magi-Galluzzi

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