促结缔组织增生性小圆细胞肿瘤的临床多学科综合诊治
Multidisciplinary diagnosis and combined treatment of desmoplastic small round-cell tumor
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摘要: 目的：探讨促结缔组织增生性小圆细胞肿瘤(DSRCT)单中心临床多学科综合诊治经验。方法：分析复旦大学附属中山医院肿瘤内科2010年1月至2018年12月收治的经病理确诊为DSRCT患者的临床资料及生存时间。共纳入11例DSRCT患者，其中男性7例（64%），女性4例（36%），男女发病比例为1.75∶1；中位发病年龄为27(12～49)岁。结果：研究截止时存活4例，死亡7例。接受手术+化疗+放疗者2例，至研究截止生存时间分别为27、60个月；接受手术+化疗者5例，其中4例生存时间分别为24、25、25、34个月，1例至研究截止时生存48个月；仅接受化疗者4例，生存时间分别为6（至研究截止时）、12、13、15个月。结论：手术+化疗+放疗多学科综合治疗模式能提高DSRCT患者的生存获益。
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关键词:
- 促结缔组织增生性小圆细胞肿瘤 /
- 诊断 /
- 治疗
Abstract: Objective：To summarize the experience of diagnosis and treatment of desmoplastic small round-cell tumor (DSRCT) in our single clinical center. Methods：The clinical data and survival time of patients with pathologically confirmed DSRCT admitted to the Department of Oncology, Zhongshan Hospital, Fudan University between January 2010 and December 2018 were analyzed. A total of 11 patients with DSRCT were selected, including 7 males (64%) and 4 females (36%). The male/female ratio of DSRCT was 1.75∶1. The median age of onset of patients was 27 (12-49) years. the end of study, there were 4 survivors and 7 deaths. Two patients who were still alive received surgery, chemotherapy. Results：By radiotherapy, and the survival time was 27 and 60 months, respectively. Five patients received surgery plus chemotherapy, and the survival time was 24, 25, 25, and 34 months, respectively, and the other one survived for 48 months by the end of the study. Four patients only received chemotherapy, and the survival time was 6(by the end of study), 12, 13, and 15 months, respectively. Conclusions：Multidisciplinary treatments using surgery, chemotherapy, and radiotherapy would improve the survival of DSRCT patients.-
Keywords:
- desmoplastic small round-cell tumor /
- diagnosis /
- treatment
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