The fifth edition of the World Health Organization (WHO) classification of urogenital tumours (WHO “Blue Book”), published in 2022, contains significant revisions. This review summarises the most relevant changes for renal, penile, and testicular tumours. In keeping with other volumes in the fifth edition series, the WHO classification of urogenital tumours follows a hierarchical classification and lists tumours by site, category, family, and type. The section “essential and desirable diagnostic criteria” included in the WHO fifth edition represents morphologic diagnostic criteria, combined with immunohistochemistry and relevant molecular tests. The global introduction of massive parallel sequencing will result in a diagnostic shift from morphology to molecular analyses. Therefore, a molecular-driven renal tumour classification has been introduced, taking recent discoveries in renal tumour genomics into account. Such novel molecularly defined epithelial renal tumours include SMARCB1-deficient medullary renal cell carcinoma (RCC), TFEB-altered RCC, Alk-rearranged RCC, and ELOC-mutated RCC. Eosinophilic solid and cystic RCC is a novel morphologically defined RCC entity. The diverse morphologic patterns of penile squamous cell carcinomas are grouped as human papillomavirus (HPV) associated and HPV independent, and there is an attempt to simplify the morphologic classification. A new chapter with tumours of the scrotum has been introduced. The main nomenclature of testicular tumours is retained, including the use of the term “germ cell neoplasia in situ” (GCNIS) for the preneoplastic lesion of most germ cell tumours and division from those not derived from GCNIS. Nomenclature changes include replacement of the term “primitive neuroectodermal tumour” by “embryonic neuroectodermal tumour” to separate these tumours clearly from Ewing sarcoma. The term “carcinoid” has been changed to “neuroendocrine tumour”, with most examples in the testis now classified as “prepubertal type testicular neuroendocrine tumour”.

世界卫生组织 (WHO) 泌尿生殖肿瘤分类第五版（WHO“蓝皮书”）于 2022 年出版，包含重大修订。本综述总结了与肾脏、阴茎和睾丸肿瘤最相关的变化。与第五版系列中的其他卷保持一致，世界卫生组织泌尿生殖肿瘤分类遵循层次分类，并按部位、类别、家族和类型列出肿瘤。 WHO第五版中的“基本和理想的诊断标准”部分代表了形态学诊断标准，并结合了免疫组织化学和相关分子检测。全球大规模并行测序的引入将导致诊断从形态学分析转向分子分析。因此，考虑到肾肿瘤基因组学的最新发现，引入了分子驱动的肾肿瘤分类。这种新型分子定义的上皮性肾肿瘤包括 SMARCB1 缺陷型肾髓细胞癌 (RCC)、TFEB 改变的 RCC、Alk 重排的 RCC 和 ELOC 突变的 RCC。嗜酸性实性囊性肾细胞癌是一种新的形态学定义的肾细胞癌实体。阴茎鳞状细胞癌的不同形态学模式分为人乳头瘤病毒 (HPV) 相关性和 HPV 独立性，并且有人试图简化形态学分类。介绍了阴囊肿瘤的新章节。保留了睾丸肿瘤的主要命名法，包括使用术语“生殖细胞原位肿瘤”（GCNIS）来表示大多数生殖细胞肿瘤的癌前病变，并将其与非 GCNIS 衍生的肿瘤分开。 命名法的变化包括将术语“原始神经外胚层肿瘤”替换为“胚胎神经外胚层肿瘤”，以将这些肿瘤与尤文肉瘤明确区分开。术语“类癌”已更改为“神经内分泌肿瘤”，睾丸中的大多数例子现在被归类为“青春期前型睾丸神经内分泌肿瘤”。