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目的:分析Xp11. 2易位性肾癌病例的临床特点、治疗及预后.方法:回顾性分析2008年1月至2018年6月于四川大学华西医院病理确诊为Xp11. 2易位性肾癌的15例患者的临床资料.Ⅰ~Ⅲ期的病例中,4例行保留肾单位手术,7例行根治性肾癌切除术,术后均未行辅助治疗. 4例Ⅳ期的患者中2例行细胞减灭性肾切除术联合靶向药物治疗,1例行原发灶和转移灶联合切除术,1例给予最佳支持治疗.应用Kaplan-Meier法分析总生存率,Log-rank检验进行单因素分析.结果:全组病例中男性6例,女性9例;年龄5~60岁,中位年龄28岁;18岁以下5例,小于46岁者共13例(86. 7% ).本组Ⅰ~Ⅲ期的11例患者随访中均未见复发及转移,其中1例随访中出现第二原发肿瘤. Log-rank检验分析得出T4／Ⅳ期的患者较非T4／Ⅳ期患者的生存率低、肿瘤最大径>7cm者较≤7cm者生存率低.结论:Xp11. 2易位性肾癌发病率女性多于男性,临床症状不典型,晚期患者易出现骨、肺转移.手术为该病最为重要的治疗手段,原发病灶和转移病灶联合切除可以提高晚期患者生存时间. MSKCC评分可能作为Ⅳ期患者评价预后的指标.肿瘤最大径>7cm、T4／Ⅳ期可能是影响预后的因素.“,”Objective: To analyze the clinical features, treatment and prognosis of Xp11. 2 translocation renal cell carci-noma. Methods: Clinical data of 15 patients with Xp11. 2 translocation renal cell carcinoma diagnosed in the pathology de-partment of West China Hospital from January 2008 to June 2018 were retrospectively analyzed. In patients in stage I~III, nephron-sparing surgery was performed in 4 patients, and radical nephrectomy in 7 patients, and no adjuvant therapy was performed after operation. Of the 4 patients in stageⅣ, 2 underwent cytoreductive nephrectomy combined with targeted drug therapy, 1 underwent combined resection of primary and metastatic lesions, and 1 underwent optimal adjuvant therapy. The overall survival rate was analyzed by Kaplan-Meier analysis and single factor analysis was performed. The Log-rank test was used to compare the difference of survival rate between groups. Results: Of all the cases, there were 6 males and 9 females; the age was 5 to 60 years and the median age was 28 years; There were 5 cases under 18 years old and 13 cases less than 46 years old (86. 7% ). No recurrence or metastasis was found in patients in stage I~III during the follow-up. One of them had a second primary tumor during follow-up. Kap-lan-Meier analysis showed that the survival rate of patients in the T4／IV phase was lower than that of patients in the non-T4／IV phase, and the survival rate of patients with the tumor’s maximum diameter greater than 7cm was lower than that of pa-tients with the tumor’s maximum diameter smaller than 7cm. Conclusion: The incidence of Xp11. 2 translocation renal cell carcinoma is higher in females than in males. The clinical symptoms of Xp11. 2 translocation renal cell carcinoma are atypi-cal, and bone or lung metastasis is prone to occur in advanced patients. Surgery is the most important treatment for this dis-ease. Combined resection of primary and metastatic lesions can improve the survival time of advanced patients. The MSKCC score may be used as an indicator of prognosis in patients in stage IV. Tumor maximum diameter greater than 7cm and the T4／IV phase were probably potential prognostic factors.