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2015年于瑞士苏黎世召开的WHO共识会议上确定通过后,2016年发布的第四版世界卫生组织(World Health Organization,WHO)泌尿生殖系统肿瘤分类(“蓝皮书”)(图1)进行了重要修订.本综述总结了新版分类与之前版本在肾、阴茎和睾丸肿瘤方面的主要差异.最新确定的肾上皮性肿瘤包括遗传性平滑肌瘤病和肾细胞癌综合征相关性肾细胞癌(hereditary leiomyomatosis and renal cell carcinoma syndrome-associated RCC, HLRCC-associated RCC)、琥珀酸脱氢酶缺陷型肾细胞癌(succinate dehydrogenase-deficient RCC, SDH-deficient RCC)、管状囊性肾细胞癌、获得性囊性疾病相关性肾细胞癌(acquired cystic disease-associated RCC)和透明细胞乳头状肾细胞癌(clear cell papillary RCC).蓝皮书推荐WHO/国际泌尿病理学会肾肿瘤分级系统供临床采用,并修改了肾乳头状腺瘤的定义.基于人乳头状瘤病毒的合并感染情况,WHO 对阴茎鳞状细胞癌重新分类,确定了相应的组织学亚型.对于侵袭性生殖细胞肿瘤的前驱病变,WHO 推荐使用睾丸原位生殖细胞瘤(germ cell neoplasia in situ,GCNIS)作为规范术语,并将睾丸生殖细胞肿瘤分为完全不同的两类:源自 GCNIS 的睾丸生殖细胞肿瘤和与 CNIS 无关的睾丸生殖细胞肿瘤.在2016年 WHO 分类中,精母细胞性精原细胞瘤(spermatocytic seminoma)被认定为精母细胞性肿瘤,并隶属于非GCNIS 相关性肿瘤.“,”The fourth edition of the World Health Organization(WHO)classification of urogenital tumours(WHO“blue book”), published in 2016, contains significant revisions. These revisions were performed after consideration by a large international group of pathologists with special expertise in this area. A subgroup of these persons met at the WHO Consensus Conference in Zurich, Switzerland, in 2015 to finalize the revisions. This review summarizes the most significant differences between the newly published classification and the prior version for renal, penile, and testicular tumours. Newly recognized epithelial renal tumours are hereditary leiomyomatosis and renal cell carcinoma(RCC)syndrome-associated RCC, succinate dehydrogenase-deficient RCC, tubulocystic RCC, acquired cystic disease-associated RCC, and clear cell papillary RCC. The WHO/International Society of Urological Pathology renal tumour grading system was recommended,and the definition of renal papillary adenoma was modified. The new WHO classification of penile squamous cell carcinomas is based on the presence of human papillomavirus and defines histologic subtypes accordingly. Germ cell neoplasia in situ(GCNIS)of the testis is the WHO-recommended term for precursor lesions of invasive germ cell tumours,and testicular germ cell tumours are now separated into two fundamentally different groups: those derived from GCNIS and those unrelated to CNIS. Spermatocytic seminoma has been designated as a spermatocytic tumour and placed within the group of non-GCNIS-related tumours in the 2016 WHO classification.