2022年发布的世界卫生组织泌尿生殖肿瘤分类对肾细胞癌（RCC）进行了重大修订，与2016年版相比有显著改进。虽然最常见的组织类型基本保持不变，但一些主要创新涉及乳头状RCC和成瘤细胞瘤。主要变化是引入了一类新的分子定义的RCC，其中包括TFE3重排RCC、TFEB重排和TFEB扩增RCC、FH缺陷RCC、SDH缺陷RCC、ALK重排RCC、ELOC（原TCEB1）突变RCC、SMARCB1（INI1）缺陷RCC。在本文中，我们分析了新出现的实体和分子定义的RCC的当前知识，以评估当前的病理分类是否为肿瘤学家提供了选择更具体和个性化治疗的可能性，包括当前可用的和即将推出的治疗。版权所有©2023 Elsevier Ltd.。

The new WHO classification of urogenital tumours published in 2022, contains significant revisions upon the previous 2016 version regarding Renal Cell Carcinoma (RCC). While the most common histotype remains almost untouched, some of the main novelties concerns papillary RCC and oncocytic neoplasms. The main change is the introduction of a new category of molecularly-defined RCC, which includes TFE3-rearranged RCC, TFEB-rearranged, and TFEB-amplified RCC, FH-deficient RCC, SDH-deficient RCC, ALK-rearranged RCC, ELOC (formerly TCEB1)-mutated RCC, SMARCB1 (INI1)-deficient RCC. In this paper we analyze the current knowledge on emerging entities and molecularly-defined RCC to assess whether the current pathological classification offers the oncologist the possibility of selecting more specific and personalized treatments, from both those currently available, as well as those that will soon be available.Copyright © 2023 Elsevier Ltd. All rights reserved.