Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
Li Ka Shing Knowledge Institute, St. Michael's Hospital, Toronto, Ontario, Canada.
Virchows Arch. 2024 Sep;485(3):391-405. doi: 10.1007/s00428-024-03865-x. Epub 2024 Jul 12.
Historically, papillary renal cell carcinoma (PRCC) was divided into two types, type 1 and type 2, based solely on morphology. However, it is apparent that PRCC is far more complex and represents a histological, clinical, and molecular spectrum. There has been a significant evolution in our understanding of PRCC, highlighted by the recognition of new and molecularly defined entities that were previously included in PRCC type 2. This contemporary review addresses the evolving concepts regarding the PRCC, including why it is no longer needed to subtype PRCC, the current molecular landscape, prognostic parameters, and PRCC variants, including biphasic PRCC, papillary renal neoplasm with reverse polarity, and Warthin-like PRCC. Pathologists should also be aware of the potential mimickers of both low-grade and high-grade PRCCs as well as some new and emerging entities that may show papillary growth that should be excluded in the diagnostic workup. The evolving knowledge of PRCC biomarkers, morphologic patterns, and PRCC variants could also have important implications for clinical management. Lastly, the heterogeneity within the PRCC spectrum needs to be further studied, aiming to better stratify PRCC for appropriate clinical management and systemic therapy.
从历史上看，肾乳头状细胞癌（PRCC）仅基于形态学就被分为 1 型和 2 型。然而，PRCC 显然要复杂得多，代表了一种组织学、临床和分子谱。我们对 PRCC 的认识发生了重大演变，突出了新的和分子定义的实体的认识，这些实体以前包含在 PRCC 2 型中。这篇当代综述讨论了 PRCC 的不断发展的概念，包括为什么不再需要对 PRCC 进行亚型分类，当前的分子景观，预后参数以及 PRCC 变体，包括双相 PRCC、极性反转的乳头状肾肿瘤和沃辛样 PRCC。病理学家还应该意识到低级别和高级别 PRCC 的潜在类似物以及一些新出现的实体，这些实体可能表现出乳头状生长，在诊断工作中需要排除这些实体。PRCC 生物标志物、形态模式和 PRCC 变体的不断发展的知识也可能对临床管理产生重要影响。最后，需要进一步研究 PRCC 谱内的异质性，旨在为适当的临床管理和系统治疗更好地分层 PRCC。
