高侵袭性特殊分子特征肾细胞癌的外科治疗决策及其价值 (“大家泌尿网”观看手术视频)

Surgical treatment decision and clinical value of molecularly defined renal carcinoma with highly aggressive behavior

特殊分子特征肾细胞癌是2022版世界卫生组织(WHO)泌尿系统及男性生殖器官肿瘤分类中首次提出的1类具有明确驱动基因的肾癌病理亚型。其中以延胡索酸水合酶缺陷型肾细胞癌(FH-RCC)和SMARCB1基因缺陷型肾髓样癌为代表的肾癌亚型具有高侵袭性和高致死性等临床特点,极易出现早期转移,故称为高侵袭性特殊分子特征肾细胞癌。该类肾癌亚型的发病率低且缺乏相关机制研究,目前临床仍无标准治疗方案。针对原发病灶或者转移病灶的减瘤性肾切除手术均是在全身系统化药物治疗下进一步改善转移性肾癌患者生命和生活质量的重要手段。但针对高侵袭性特殊分子特征肾细胞癌,外科治疗的策略及其价值尚不明确,值得深入讨论和思考。本文就上述问题进行讨论,以期为高侵袭性特殊分子特征肾细胞癌患者的预后改善提供线索和参考。

Molecularly defined renal carcinoma is a pathologic subtype of renal cell cancer(RCC)with a definite driver gene,which was first proposed in the 2022 WHO classification of tumors of the urinary system and male reproductive organs.The fumarate hydratase-deficient renal cell carcinoma and SMARCB1-deficient renal medullary carcinoma are highly aggressive and lethal subtypes.Due to the low incidence and lack of research on the mechanism,there is almost no effective treatment for these aggressive RCC subtypes.Cytoreductive nephrectomy or metastasectomy are important methods to improve the survival and quality of life of metastatic RCC patients under effective systemic therapy.However,for the highly aggressive RCC,the clinical value of the above surgical strategies is still unclear.In this review,we will discuss these problems in order to provide reference for the improvement of prognosis for patients with highly aggressive RCC subtypes.