Wang Binshuai, Qiu Min, Zhang Qianjin, Tian Maofeng, Liu Lei, Wang Guoliang, Lu Min, Tian Xiaojun, Zhang Shudong
Department of Urology, Peking University Third Hospital, Beijing 100191, China.
Department of Urology, The Affiliated Suqian First People's Hospital of Nanjing Medical University, Suqian 223800, Jiangsu, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2024 Dec 18;56(4):636-639. doi: 10.19723/j.issn.1671-167X.2024.04.015.
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.
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.
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 (=0.031).
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.
回顾分析肾尤文肉瘤合并静脉瘤栓的临床诊断与治疗情况，随访患者生存及预后，为该病的诊治提供帮助。
收集2016年6月至2022年6月在北京大学第三医院确诊为肾尤文肉瘤合并静脉瘤栓患者的临床资料（包括一般资料、手术资料及术后病理资料），对患者预后进行随访，分析诊治过程对疾病预后的影响。
共6例患者，其中男性1例，女性5例。左肾肿瘤4例，右肾肿瘤2例。确诊时的中位年龄为28岁（16 - 52岁）。影像学表现均为有内部坏死组织及出血的外生性肿瘤。肿瘤平均最大直径为12.6 cm，肿瘤栓子平均长度为7.8 cm。4例患者接受开放手术，2例患者接受腹腔镜手术。术后病理结果为肾尤文肉瘤。免疫组化结果显示CD99（+）3例，FLI - 1（+）2例，CD99、FLI - 1（-）1例。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例患者接受化疗联合放疗，2例患者未接受辅助治疗。6例患者的平均总生存期（OS）为37个月，接受化疗的4例患者（47个月）的平均OS显著高于未接受化疗的2例患者（16个月）（P = 0.031）。
肾尤文肉瘤合并静脉瘤栓临床少见，好发于年轻女性患者。手术难度大，预后差。手术切除、辅助放疗及化疗可提高患者的总生存率。
