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2016年WHO正式将透明细胞乳头状肾细胞癌作为一种肾细胞癌新类型.该肿瘤可以为散发性也可以发生于终末期肾病或Von Hippel-Lindau综合征(VHL综合征),具有相对特征性的病理组织学形态、免疫表型和分子遗传学特点,诊断时应与透明细胞肾细胞癌、乳头状肾细胞癌以及Xp11.2易位/TFE3基因融合相关性肾癌相鉴别.鉴于透明细胞乳头状肾细胞癌生物学行为相对惰性或低度恶性,因此准确诊断对指导临床治疗和判断预后具有十分重要的意义.“,”WHO officially identified clear cell papillary renal cell carcinoma (CCPRCC) as a new type of renal cell carcinoma in 2016.This tumor type can be encountered in a sporadic setting or can occur in end-stage renal disease or even VHL syndrome, with relatively characteristic histopathological morphology, immunophenotype and molecular genetics.It needs to be identified with clear cell renal cell carcinoma, papillary renal cell carcinoma or renal carcinomas associated with Xp 11.2 translocations.In view of the biological behavior of CCPRCC is relatively inert or low-grade malignant, therefore accurate diagnosis plays an important role in clinical treatment and judging prognosis.