Description on Clinical Characteristics and Analysis of Prognostic Factors in Patients with Desmoplastic Small Round Cell Tumor
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摘要: 目的 探讨促纤维组织增生性小圆细胞肿瘤（desmoplastic small round cell tumor,DSRCT）的临床特征及影响DSRCT生存预后的因素。 方法 收集1998年1月至2012年2月福建省肿瘤医院收治的5例DSRCT患者的临床资料，结合1998年至2012年国内发表的文献53篇、国外发表的文献37篇，对其中具有完整临床病理及诊疗资料的126例DSRCT患者进行统计分析。单因素分析采用Kaplan-Meier法，组间曲线比较用Log rank检验，多因素分析采用Cox回归分析。 结果 在单因素分析当中，与预后有关的因素有肿瘤发生部位、肿瘤数目、肿瘤大小、有无脏器转移、是否接受根治性手术、是否接受化疗及放射治疗。Cox风险比例回归模型进行多因素分析，是否接受根治性手术［相对危险度(RR)为13.250，95%置信区间(CI)为5.675～30.937，P=0.000］、是否接受化疗(RR=5.714，95%CI为3.165～10.317，P=0.000)及是否接受放射治疗(RR=7.498，95%CI为2.628～21.389，P=0.000)是DSRCT的预后因素。 结论 是否接受根治性手术、化疗及放射治疗是DSRCT的独立预后因素。早期诊断，争取根治性手术及积极接受全身化疗、放射治疗是目前延长生存期的合理途径。
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关键词:
- 促纤维组织增生性小圆细胞肿瘤 /
- 治疗 /
- 预后
Abstract: Objective To investigate the clinical characteristics and prognostic factors affecting the survival of the desmoplastic small round cell tumor (DSRCT). Methods Clinical data of five patients with DSRCT who were diagnosed and treated in Fujian Tumor Hospital from January 1998 to February 2012 were collected．126 cases with complete clinical data were collected and reviewed from 90 published reports.Therefore,totally 131 patients with DSRCT were analysed．Kaplan-Meier method was applied to univariate analysis.Log rank was used to compare curves between these groups.Cox regression was applied to multivariate prognostic analysis.Statistical significance was considered at P<0.05. Results In the univariate analysis,tumor location,number of tumors,size of tumor,with or without metastases,and whether or not to accept radical surgery,whether or not to accept chemotherapy and radiation therapy are the prognostic factors.In the Cox proportional hazards regression model for multivariate analysis,whether or not to accept radical surgery [relative risk (RR) 13.250,95% confidence interval (CI) 5.675-30.937,P=0.000],whether or not to undergo chemotherapy(RR=5.714,95% CI=3.165-10.317,P=0.000) and whether or not to undergo radiation therapy(RR=7.498,95% CI=2.628-21.389,P=0.000) are the prognostic factors in DSRCT. Conclusion Whether or not to accept radical surgery,chemotherapy and radiation therapy are the independent prognostic factors in DSRCT.Currently,early diagnosis,striving for radical surgery,systemic chemotherapy and radiation therapy is the reasonable way to prolong survival.-
Key words:
- Desmoplastic small round cell tumor /
- Treatment /
- Prognosis
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