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目的 探讨促纤维组织增生性小圆细胞肿瘤(DSRCT)的临床特征和治疗模式.方法 收集中国医学科学院肿瘤医院1999年1月至2009年5月收治的5例DSRCT患者的临床资料,并结合1989年至2007年国内外发表的文献23篇,对其中有完整临床病理资料的48例DSRCT患者进行统计分析.结果 全组53例DSRCT患者的中位年龄为23岁(1.5～66岁),其中男性40例,占75.5%.首诊症状为腹部包块或腹痛41例,占77.4%;病变原发于腹腔或盆腔46例,占86.8%;首诊即出现淋巴结转移或远处转移15例,占28.3%.中位随访时间为1.8年(0.1～10.0年).全组患者的1、3和5年生存率分别为45.8%、20.8%和5.7%.53例患者中,接受手术治疗47例,其中行根治性手术者的1年和3年生存率分别为70.5%和53.7%,明显高于行姑息性手术者的1年和3年生存率(37.2%和4.8%,P=0.0020).接受全身化疗34例,患者的1年和3年生存率分别为60.1%和35.2%,明显高于未接受化疗者的1年和3年生存率(29.7%和12.7%,P=0.0396).接受放射治疗12例,患者的1年和3年生存率分别为75.0%和38.9%,明显高于未接受放疗者的1年和3年生存率(36.9%和14.8%,P=0.0314).进一步分层分析显示,与单一治疗模式相比,采用根治性手术联合化疗或放疗以及化疗联合手术或放疗的患者有生存优势(P=0.0466和P=0.0100).结论 根治性手术、全身化疗或放射治疗均能够延长DSRCT患者的生存期,但采用根治性手术联合化疗或放疗以及化疗联合手术或放疗的疗效更好.

Objective To investigate the clinical characteristics and treatment of desmoplastic small round cell tumor. Methods Five patients with DSRCT were diagnosed and treated in our Hospital from January 1999 to May 2009. Forty-eight cases with complete clinical data were collected and reviewed from 23 published reports. Therefore totally 53 patients with DSRCT were analysed. The survival rate was calculated by Kaplan-Meier method and compared by log-rank test. Results The median age of all cases was 23 (1.5-66) years old at the time of diagnosis. 75.5% of patients were male. The most common presenting complaint was intra-abdominal mass or pain (77.4%). In 46 patients (86.8%), the primary tumor was located in the abdomen or pelvis. Fifteen (28.3% ) had positive lymph nodes or distant parenchymal metastases. The median follow-up was 1.8 years (range, 0.1-10.0 years). The overall 1-,3-and 5-year survivals were 45.8%, 20.8% and 5.7%, respectively. Forty-seven patients underwent surgery. Complete tumor resection was significantly correlated with long survival. The 1- and 3-year survival rates were 70.5% and 53.7% in patients treated with complete tumor resection compared to 37.2% and 4.8% in the incomplete tumor resection cohort (P=0.0020). Thirty-four patients received chemotherapy and the 1- and 3-year survival rates were 60.1% and 35.2%, respectively, however, only 29.7% and 12.7% in patients without chemotherapy (P=0.0396). Twelve patients had radiotherapy and the 1- and 3-year survival rateswere 75.0% and 38.9%, respectively, compared with 36.9% and 14.8% in those without radiotherapy (P=0.0314). Conclusion Complete tumor resection results in improved survival in patients with DSRCT. Chemotherapy and radiotherapy correlate with improved patient outcome. Multimodal therapy mayimprove the survival in patients with DSRCT.