腹部Desmoplastic小圆细胞瘤1例报告及文献复习

N. Chai, Lin Chen, Zebin Jiang, Peng Gao, Xiaojun Yang
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摘要

目的探讨腹部促结缔组织增生性小圆细胞瘤的病理特点、诊断、治疗决策及近期疗效。方法回顾性分析2018年4月甘肃省人民医院收治的1例促结缔组织增生性小圆细胞瘤的临床资料。对促结缔组织增生性小圆细胞瘤的临床表现、病理特点、诊断与鉴别诊断、治疗及预后进行了总结和分析。结果患者成功地进行了最大限度的肿瘤缩小治疗。手术时间为360分钟。术中估计失血量为200毫升,术中未输血。术后第8天取下腹腔引流管,肝功能恢复良好。术后病理:(腹膜后)小圆细胞恶性肿瘤。结合临床及免疫组化染色结果:高度重视:促结缔组织增生性小圆细胞瘤。患者于术后第16天出院。患者随访4个月,肿瘤复发,肝转移。随访期至2018年10月。结论Desmoplastic小圆细胞瘤是一种罕见、恶性程度高、预后差的软组织小细胞瘤。影像学检查和肿瘤标志物的检测没有特异性,诊断困难。肿瘤完全切除联合化疗可以改善患者的预后,但预后仍不尽如人意,更有效的治疗决策仍有待探索。关键词:Desmoplastic小圆细胞瘤;化疗;组织病理学
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A case report and literature review of Abdominal Desmoplastic Small Round Cell Tumor
Objective To investigate the pathological characteristics, diagnosis, treatment decision and short-term curative effect of abdominal desmoplastic small round cell tumor. Methods The clinical data of a case of desmoplastic small round cell tumor admitted to Gansu provincial people′s Hospital in April 2018 were analyzed retrospectively.The clinical manifestations, pathological features, diagnosis and differential diagnosis, treatment and prognosis of desmoplastic small round cell tumor were summarized and analyzed. Results The patient was successfully treated with maximum tumor reduction.The operation time was 360 minutes.The estimated blood loss during operation was 200 ml, and no blood was transfused during operation.The abdominal drainage tube was removed on the 8th day after operation and the liver function recovered well.Postoperative pathology: (retroperitoneal) small round cell malignant tumor.Combined with clinical and immunohistochemical staining results: highly considered: desmoplastic small round cell tumor.The patient was discharged on the 16th day after operation.The patient was followed up for 4 months and the tumor recurred and liver metastasis.The follow-up period is now up to October 2018. Conclusion Desmoplastic small round cell tumor is a rare and highly malignant soft tissue small cell tumor with poor prognosis.Imaging examination and detection of tumor markers have no specificity and diagnose of it is difficult.Complete resection of the tumor and combined chemotherapy can improve the prognosis of the patients, but the prognosis is still not satisfactory, and more effective treatment decisions still need to be explored. Key words: Desmoplastic Small Round Cell Tumor; Chemotherapy; Histopathology
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来源期刊
CiteScore
0.10
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0.00%
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16855
期刊介绍: Clinical Medicine of China is an academic journal organized by the Chinese Medical Association (CMA), which mainly publishes original research papers, reviews and commentaries in the field. Clinical Medicine of China is a source journal of Peking University (2000 and 2004 editions), a core journal of Chinese science and technology, an academic journal of RCCSE China Core (Extended Edition), and has been published in Chemical Abstracts of the United States (CA), Abstracts Journal of Russia (AJ), Chinese Core Journals (Selection) Database, Chinese Science and Technology Materials Directory, Wanfang Database, China Academic Journal Database, JST Japan Science and Technology Agency Database (Japanese) (2018) and other databases.
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