目的: 回顾和分析肾尤文肉瘤伴静脉瘤栓患者的临床诊治过程，随访患者的生存预后，为疾病的诊治提供帮助。方法: 收集2016年6月至2022年6月在北京大学第三医院诊断为肾尤文肉瘤伴静脉瘤栓患者的临床资料，包括一般资料、手术资料和术后病理资料，电话随访患者预后情况，分析诊治过程对疾病预后的影响。结果: 患者6例，其中男性1例、女性5例；左肾肿物4例，右肾肿物2例；确诊时中位年龄28岁(16~52岁)；影像学表现均为外生性肿瘤，内部存在坏死组织及出血；平均肿瘤最大直径为12.6 cm，平均瘤栓长度为7.8 cm；4例行开放手术，2例行腹腔镜手术；术后病理结果均为肾尤文肉瘤，免疫组织化学结果中3例CD99(+)，2例FLI-1(+)，1例CD99、FLI-1(-)；术后接受化疗(环磷酰胺、多柔比星、长春新碱/异环磷酰胺、依托泊苷方案)3例，化疗联合放疗1例，未行放化疗2例；术后6例平均总体生存率(overall survival，OS)为37个月，接受化疗的4例患者平均术后OS(47个月)显著高于未行化疗的2例患者(16个月，P=0.031)。结论: 肾尤文肉瘤伴静脉瘤栓临床罕见，年轻女性患者多发，手术难度大，预后不良，手术切除加辅助放化疗可提高患者OS。

Objective: To review and analyze the clinical diagnosis and treatment of renal Ewing's sarcoma with venous tumor embolus, to follow up the survival and prognosis of the patients, and to provide help for the diagnosis and treatment of the disease. Methods: Clinical data (including general data, surgical data and postoperative pathological data) of patients diagnosed with renal Ewing's sarcoma with venous tumor embolus in Peking University Third Hospital from June 2016 to June 2022 were collected, and the prognosis of the patients was followed up to analyze the influence of diagnosis and treatment process on the prognosis of the disease. Results: There were 6 patients, including 1 male and 5 females. There were 4 cases of left renal tumor and 2 cases of right renal tumor. The median age at diagnosis was 28 years (16-52 years). The imaging findings were all exogenous tumors with internal necrotic tissue and hemorrhage. The mean maximum tumor diameter was 12.6 cm, and the mean tumor thrombus length was 7.8 cm. Four patients underwent open surgery and 2 patients underwent laparoscopic surgery. The postoperative pathological results were renal Ewing sarcoma. Immunohistochemical results showed 3 cases of CD99 (+), 2 cases of FLI-1 (+), and 1 case of CD99, FLI-1 (-). 3 patients received chemotherapy (cyclophosphamide, doxorubicin, vincristine/ifosfamide, etoposide), 1 case received chemotherapy combined with radiotherapy, and 2 cases received no adjuvant therapy. The mean overall survival (OS) of the 6 patients was 37 months, and the mean OS of the 4 patients (47 months) who received chemotherapy was significantly higher than that of the 2 patients (16 months) who did not receive chemotherapy (P=0.031). Conclusion: Renal Ewing's sarcoma with venous tumor embolus is rare in clinic, and it is common in young female patients. The operation is difficult and the prognosis is poor. Surgical resection, adjuvant radiotherapy and chemotherapy can improve the overall survival rate of the patients.