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原发性肾滑膜肉瘤的临床病理特征及预后分析

Clinicopathological features and prognosis of patients with primary renal synovial sarcoma
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摘要 目的探讨原发性肾滑膜肉瘤(PRSS)的临床病理特征及预后影响因素。方法回顾性分析2012年2月-2021年2月郑州大学第一附属医院收治的19例PRSS患者的临床、影像、病理资料。其中男性11例,女性8例,平均年龄(35.4±11.2)岁。治疗方式包括手术、化疗等,并对患者进行定期随访。采用单因素和多因素分析探讨影响PRSS无复发生存期(RFS)及总生存期(OS)的因素。结果17例接受手术治疗,2例接受化疗,所有患者平均随访(36.0±4.5)个月,治疗后1、3年总生存率分别为89.5%和52.7%。病理结果显示肿瘤由梭形细胞构成并呈束状排列,部分细胞呈上皮样分化;荧光原位杂交结果显示19例均可见SYT-SSX基因融合。单因素分析显示远处转移、肿瘤最大径、pT分期、静脉癌栓、吸烟史与RFS及OS均显著相关,而性别、淋巴结转移、是否手术仅与RFS相关。多因素分析显示远处转移(HR=6.114,95%CI:1.647~22.700,P=0.007)和吸烟史(HR=4.297,95%CI:1.222~16.455,P=0.033)是患者OS的独立危险因素,性别(HR=7.112,95%CI:1.183~42.764,P=0.032)和肿瘤最大径(HR=6.737,95%CI:1.186~38.263,P=0.031)是患者RFS的独立危险因素。结论PRSS临床罕见且预后差,治疗应首选根治性肾切除术。远处转移和吸烟史是PRSS预后的独立危险因素。 Objective To explore the clinicopathological features and prognosis of primary renal synovial sarcoma(PRSS).Methods The clinical,imaging and pathological data of 19 patients with PRSS admitted to our hospital during Feb.2012 and Feb.2021 were retrospectively analyzed,including 11 males and 8 females.The average age was(35.4±11.2)years.Treatment included surgery and chemotherapy.All patients were regularly followed up.Factors affecting recurrence-free survival(RFS)and overall survival(OS)were analyzed with univariate and multivariate analysis.Results Of all patients,17 received surgery and 2 chemotherapy.The mean follow-up was(36.0±4.5)months.The 1-year and 3-year OS were 89.5%and 52.7%,respectively.Histopathological results showed that the tumor was composed of spindle cells in bundles,and some tumor cells showed epithelioid differentiation.SYT-SSX gene fusion was detected by fluorescence in situ hybridization in all cases.Univariate analysis showed that distant metastasis,maximum tumor diameter,pathological T stage,venous tumor thrombus and smoking history were significantly correlated with RFS and OS,while gender,lymph node metastasis and surgery were correlated with RFS.Multivariate analysis showed that distant metastasis(HR=6.114,95%CI:1.647~22.700,P=0.007)and smoking history(HR=4.297,95%CI:1.222~16.455,P=0.033)were independent risk factors affecting OS,while gender(HR=7.112,95%CI:1.183~42.764,P=0.032)and maximum tumor diameter(HR=6.737,95%CI:1.186~38.263,P=0.031)were independent factors affecting RFS.Conclusion PRSS is a rare tumor with poor prognosis.The preferred treatment is radical nephrectomy.Distant metastasis and smoking history are independent risk factors of prognosis.
作者 罗文坚 张婷 张瑜 韩灵 崔林刚 魏寅生 李腾 杨治立 韩彦通 孟庆军 LUO Wenjian;ZHANG Ting;ZHANG Yu;HAN Ling;CUI Lin'gang;WEI Yinsheng;LI Teng;YANG Zhili;HAN Yantong;MENG Qingjun(Department of Urology,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Surgical Intensive Care Unit,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)
出处 《现代泌尿外科杂志》 CAS 2022年第8期656-661,共6页 Journal of Modern Urology
关键词 肾肿瘤 滑膜肉瘤 临床病理特征 预后 原发性肾滑膜肉瘤 renal tumor synovial sarcoma clinicopathological features prognosis primary renal synovial sarcoma
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