| 孙 鹏,徐玉清.促纤维增生性小圆细胞肿瘤的诊断和治疗[J].肿瘤学杂志,2019,25(1):67-70. |
| 促纤维增生性小圆细胞肿瘤的诊断和治疗 |
| Diagnosis and Treatment of Desmoplastic Small Round Cell Tumor |
| 投稿时间：2017-08-12 |
| DOI：10.11735/j.issn.1671-170X.2019.01.B016 |
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| 中文关键词: 促纤维增生性小圆细胞肿瘤 诊断 治疗 |
| 英文关键词:desmoplastic small round cell tumor diagnosis treatment |
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| 中文摘要: |
| 摘 要：促纤维增生性小圆细胞肿瘤（desmoplastic small round cell tumor，DSRCT）是一种极罕见的、高度恶性软组织肉瘤，它起源未定，好发于青少年男性的盆腹腔。临床表现无特异性，诊断依赖于临床病理特征、免疫表型和遗传分子学改变，特征性的t（11，22）（p13；q12）染色体异位产生EWS-WT1融合基因，可通过RT-PCR、FISH、Southern blot技术检测这一融合基因来确诊。该疾病预后极差，中位生存期17~25个月。DSRCT最优的治疗目前尚无共识，最大限度地减瘤手术、化疗、放疗、腹腔热灌注化疗等多学科综合治疗虽在一定程度上延长生存期，但总体疗效欠佳。靶向治疗、免疫治疗等新兴治疗措施尚需进一步研究探索。 |
| 英文摘要: |
| Abstract：Desmoplastic small round cell tumor(DSRCT) is a very rare，highly aggressive malignant soft tissue sarcoma usually occurring in abdominal and pelvic cavity of male adolescents. The specific organs or tissue types of origin are not identified. The disease is lack of characteristic clinical manifestations. Its diagnosis is based on clinicopathological features，immune phenotype and molecular genetics. DSRCT is associated with a unique chromosomal translocation t(11，22)(P13；q12)，generating EWS-WT1 fusion gene. Reverse transcriptase-polymerase chain reaction(RT-PCR)，fluorescence in situ hybridization and Southern blot are used to detect the fusion gene and to indentify the DSRCT. The prognosis of DSRCT is very poor，and the median survival time ranges from 17 to 25 months. The optimal management of DSRCT at present is not consentient. Multi-disciplinary comprehensive treatment including debulking surgery，chemotherapy，radiation，and hyperthermal intraperitoneal chemotherapy(HIPEC) may prolong survival time to a certain extent，however，the overall effect is poor. Emerging therapeutic measures such as targeted therapy and immune therapy need to be further studied. |
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