| 纵隔促纤维增生性小圆细胞肿瘤1例并文献复习 |
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| 引用本文： | 陈 猛1,' target='_blank'>2,金大成1,' target='_blank'>2,王 兵1,' target='_blank'>2,董信春2. 纵隔促纤维增生性小圆细胞肿瘤1例并文献复习[J]. 现代肿瘤医学, 2020, 0(7): 1133-1136. DOI: 10.3969/j.issn.1672-4992.2020.07.017 |
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| 作者姓名： | 陈 猛1 ' target='_blank'>2 金大成1 ' target='_blank'>2 王 兵1 ' target='_blank'>2 董信春2 |
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| 作者单位： | 1.甘肃中医药大学，甘肃 兰州 730000;2.甘肃省人民医院胸外一科，甘肃 兰州 730000 |
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| 基金项目： | 甘肃省卫生行业计划（编号:GSWSKY2017-56）；甘肃省人民医院院内科研基金（编号:16GSSY3-1） |
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| 摘 要： | 目的:探讨纵隔促纤维增生性小圆细胞肿瘤（DSRCT）的诊断、治疗及预后。方法:报告1例我科收治的纵隔DSRCT，结合以往报道的文献探讨纵隔DSRCT的诊断方法，以减瘤术、放化疗及靶向治疗等综合治疗为主的原则及预后。结果:回顾分析1例纵隔DSRCT患者，行诊断性穿刺检查，从影像学、病理学等确诊为纵隔DSRCT，并给予相应化疗及放疗方案，患者预后较差且病情进展迅速，结合以往文献分析并讨论DSRCT的确诊方法、治疗策略及预后情况。结论:DSRCT是一种罕见的间质组织恶性肿瘤，影像学无特异性表现，确诊多依靠组织学及基因检测结果，该疾病具有侵袭性高，进展快，复发率高等特征，有效的减瘤术及综合性联合治疗可达到部分缓解或提高患者生存时间。
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| 关 键 词： | 纵隔 促纤维增生性小圆细胞肿瘤 化疗 |
Desmoplastic small round cell tumor of the mediastinum:A case report and literature review |
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| Chen Meng1,' target='_blank'>2,Jin Dacheng1,' target='_blank'>2,Wang Bing1,' target='_blank'>2,Dong Xinchun2. Desmoplastic small round cell tumor of the mediastinum:A case report and literature review[J]. Journal of Modern Oncology, 2020, 0(7): 1133-1136. DOI: 10.3969/j.issn.1672-4992.2020.07.017 |
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| Authors: | Chen Meng1 ' target='_blank'>2 Jin Dacheng1 ' target='_blank'>2 Wang Bing1 ' target='_blank'>2 Dong Xinchun2 |
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| Affiliation: | 1.Gansu University of Traditional Chinese Medicine，Gansu Lanzhou 730000，China；2.The First Department of Thoracic Surgery，Gansu Province People's Hospital，Gansu Lanzhou 730000，China. |
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| Abstract: | Objective:To investigate the diagnosis，treatment and prognosis of desmoplastic small round cell tumor of the mediastinum (DSRCT).Methods:A case of mediastinal DSRCT admitted to our department was reported.Combined with previous reports，the diagnostic method of mediastinal DSRCT，the principle and prognosis of comprehensive treatment including tumor reduction surgery，radiotherapy and chemotherapy，and targeted therapy were discussed.Results:A case of mediastinal DSRCT was retrospectively analyzed with diagnostic puncture examination.It was diagnosed as mediastinal DSRCT by imaging and pathology，and was given corresponding chemotherapy and radiotherapy.The prognosis of the patient was poor and the disease progressed rapidly.Conclusion:DSRCT is a rare mesenchymal malignancy，with no specific imaging manifestations.The diagnosis of this disease mainly depends on the histological and genetic test results.It is characterized by high invasiveness，rapid progress and high recurrence rate. |
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| Keywords: | mediastinum DSRCT chemotherapy |
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