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Abstract：
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).
CONCLUSIONS: 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.
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).
CONCLUSIONS: 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.
摘要：
目的：回顾分析肾尤文氏肉瘤合并静脉癌栓的临床诊断和治疗。随访患者的生存和预后，并为该病的诊断和治疗提供帮助。
方法：临床数据（包括一般数据，收集2016年6月至2022年6月北京大学第三医院诊断为肾尤因肉瘤伴静脉癌栓的患者的手术资料和术后病理资料),并对患者的预后进行随访,分析诊疗过程对疾病预后的影响。
结果：有6例患者，包括1名男性和5名女性。左肾肿瘤4例,右肾肿瘤2例。诊断时的中位年龄为28岁（16-52岁）。影像学表现均为外源性肿瘤伴内部坏死组织及出血。平均最大肿瘤直径为12.6cm，平均肿瘤血栓长度为7.8cm。4例患者行开腹手术，2例患者行腹腔镜手术。术后病理结果为肾Ewing肉瘤。免疫组化结果显示3例CD99(+),2例FLI-1(+),1例CD99、FLI-1(-)。3例患者接受化疗（环磷酰胺，阿霉素,长春新碱/异环磷酰胺,依托泊苷)，1例化疗联合放疗，2例未接受辅助治疗。6例患者的平均总生存期(OS)为37个月,接受化疗的4例（47个月）患者的平均OS明显高于未接受化疗的2例（16个月）（P=0.031）。
结论：肾尤文氏肉瘤伴静脉癌栓临床少见，这在年轻女性患者中很常见。手术困难，预后差。手术切除,辅助放化疗可以提高患者的总体生存率。
方法：临床数据（包括一般数据，收集2016年6月至2022年6月北京大学第三医院诊断为肾尤因肉瘤伴静脉癌栓的患者的手术资料和术后病理资料),并对患者的预后进行随访,分析诊疗过程对疾病预后的影响。
结果：有6例患者，包括1名男性和5名女性。左肾肿瘤4例,右肾肿瘤2例。诊断时的中位年龄为28岁（16-52岁）。影像学表现均为外源性肿瘤伴内部坏死组织及出血。平均最大肿瘤直径为12.6cm，平均肿瘤血栓长度为7.8cm。4例患者行开腹手术，2例患者行腹腔镜手术。术后病理结果为肾Ewing肉瘤。免疫组化结果显示3例CD99(+),2例FLI-1(+),1例CD99、FLI-1(-)。3例患者接受化疗（环磷酰胺，阿霉素,长春新碱/异环磷酰胺,依托泊苷)，1例化疗联合放疗，2例未接受辅助治疗。6例患者的平均总生存期(OS)为37个月,接受化疗的4例（47个月）患者的平均OS明显高于未接受化疗的2例（16个月）（P=0.031）。
结论：肾尤文氏肉瘤伴静脉癌栓临床少见，这在年轻女性患者中很常见。手术困难，预后差。手术切除,辅助放化疗可以提高患者的总体生存率。
