Srigley John R, Delahunt Brett
Department of Pathology and Molecular Medicine, McMaster University, c/o The Credit Valley Hospital, 2200 Eglinton Avenue West, Mississauga, ON L5M2N1, Canada.
Mod Pathol. 2009 Jun;22 Suppl 2:S2-S23. doi: 10.1038/modpathol.2009.70.
Major consensus conferences held over a decade ago laid the foundations for the current (2004) WHO classification of renal carcinoma. Clear cell, papillary and chromophobe carcinomas account for 85-90% carcinomas seen in routine practice. The remaining 10-15% of carcinomas consist of rare sporadic and hereditary tumors, some of which had been long recognized, but many of which only emerged as distinct entities in the decade leading up to the WHO publication. Collecting-duct carcinoma is a rare, often lethal form of carcinoma. Medullary carcinoma associated with sickle cell trait, has emerged as a distinctive tumor showing some overlapping features with upper tract urothelial carcinoma. Mucinous tubular and spindle-cell carcinoma and tubulocystic carcinoma were earlier considered as patterns of low-grade collecting-duct carcinoma, but are now recognized as separate tumor entities. Carcinomas associated with somatic translocations of TFE3 and TFEB comprise a significant proportion of pediatric renal carcinomas. Oncocytoid renal carcinomas in neuroblastoma survivors was recognized as a unique tumor category in the WHO classification. Renal carcinoma associated with end-stage renal disease is now recognized as having distinct morphological patterns and behavior. In addition there is a group of rare recently described carcinomas, including clear cell papillary carcinoma, oncocytic papillary renal cell carcinoma, follicular renal carcinoma and leiomyomatous renal cell carcinoma. It behooves the surgical pathologist to not only be capable of diagnosing the common forms of renal cancer, but also to be aware of the rare types of renal carcinoma, many of which have emerged in recent years.
十多年前召开的主要共识会议为当前（2004年）世界卫生组织（WHO）的肾癌分类奠定了基础。透明细胞癌、乳头状癌和嫌色细胞癌占常规实践中所见肾癌的85 - 90%。其余10 - 15%的肾癌由罕见的散发性和遗传性肿瘤组成，其中一些早已为人所知，但许多直到WHO发布前十年才作为独特的实体出现。集合管癌是一种罕见的、通常致命的癌。与镰状细胞性状相关的髓样癌已成为一种独特的肿瘤，显示出与上尿路尿路上皮癌有一些重叠特征。黏液性小管状和梭形细胞癌以及微囊型癌早期被认为是低级别集合管癌的形态，但现在被认为是独立的肿瘤实体。与TFE3和TFEB体细胞易位相关的癌在儿童肾癌中占很大比例。神经母细胞瘤幸存者中的嗜酸细胞样肾癌在WHO分类中被认为是一种独特的肿瘤类型。与终末期肾病相关的肾癌现在被认为具有独特的形态模式和行为。此外，还有一组最近描述的罕见癌，包括透明细胞乳头状癌、嗜酸细胞乳头状肾细胞癌、滤泡性肾癌和平滑肌瘤样肾细胞癌。外科病理学家不仅应该能够诊断常见类型的肾癌，还应该了解近年来出现的许多罕见类型的肾癌。
