Abstract:
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.
摘要:
历史上,乳头状肾细胞癌(PRCC)分为两种类型,1型和2型,仅基于形态。然而,很明显,PRCC要复杂得多,代表组织学,临床,和分子光谱。我们对PRCC的理解有了很大的进步,通过识别以前包含在PRCC2型中的新的和分子定义的实体来突出显示。这篇当代评论讨论了关于PRCC的不断发展的概念,包括为什么不再需要它来亚型PRCC,当前的分子景观,预后参数,和PRCC变体,包括双相PRCC,具相反极性的乳头状肾肿瘤,和类似Warthin的PRCC。病理学家还应该意识到低级别和高级别PRCC的潜在模拟者,以及一些新的和新兴的实体,这些实体可能显示乳头状生长,应在诊断检查中排除。PRCC生物标志物的不断发展的知识,形态学模式,和PRCC变体也可能对临床管理具有重要意义。最后,PRCC光谱内的异质性需要进一步研究,旨在更好地对PRCC进行分层,以进行适当的临床管理和系统治疗。