促结缔组织增生性小圆细胞肿瘤的临床特征及预后因素分析

Analysis of clinical features and prognostic factors of desmoplastic small round cell tumor

目的·分析促结缔组织增生性小圆细胞肿瘤(desmoplastic small round cell tumor,DSRCT)的临床特征及预后因素。方法·通过检索Medline和Embase数据库,收集2003年11月—2012年7月发表的140例DSRCT患者临床资料并进行回顾性分析,观察指标为无进展生存期(progression-free survival,PFS)或总生存期(overall survival,OS)。采用Kaplan-Meier法计算生存率,并使用对数秩检验进行组间比较;采用Cox模型进行多变量分析,确定影响预后的因素。结果·患者中位年龄为(23.2±12.7)岁(4~74岁),男女比例3.12:1;最常见的症状是腹痛(35.7%)和腹部包块(20.0%);腹腔或盆腔肿瘤106例,其余34例为腹腔外肿瘤;接受常规化学治疗(简称化疗)、减瘤手术、新辅助化疗、辅助化疗或一线化疗的患者分别占76.4%、52.1%、17.1%、47.9%和38.6%,部分患者接受辅助放射治疗(17.1%)、高温腹腔化疗(4.1%)和骨髓移植(7.3%)。单因素分析结果显示男性、无转移、有效减瘤术、化疗和多模式治疗与OS延长相关(均P<0.05);原发肿瘤部位、腹腔外肿瘤、无转移和有效减瘤手术与PFS改善相关(均P<0.05);Cox回归分析结果显示,有效减瘤手术和化疗是独立的良性预后因素。结论·可通过手术、辅助治疗清除肿瘤的多模式治疗方式来提高DSRCT患者生存水平。

Objective·To analyze the clinical features and prognostic factors of desmoplastic small round cell tumor(DSRCT).Methods·Clinical datum of 140 patients with DSRCT published from Nov.2003 to Jul.2012 were collected and studied retrospectively by searching Medline and Embase databases.The observation indicators were progression-free survival(PFS)or overall survival(OS).Survival rates were calculated using the Kaplan-Meier method and compared between groups using a log-rank test.Multivariate analysis was performed using the Cox model to determine the prognostic factors.Results·Patient median age was(23.2±12.7)years(range 4-74 years,the ratio of male and female was 3.12:1).Frequent symptoms were abdominal pain(35.7%)and evidence of a palpable mass(20.0%).106 cases tumors were in the abdominal or pelvic cavity,the remaining were extra-abdominal tumors.The frequency of patients receiving conventional chemotherapy,cytoreductive surgery,neoadjuvant chemotherapy,adjuvant chemotherapy or first-line chemotherapy was 76.4%,52.1%,17.1%,47.9%and 38.6%,respectively.Some patients received adjuvant radiotherapy(17.1%),hyperthermic intraperitoneal chemotherapy(4.1%)and bone marrow transplantation(7.3%).By univariate analysis,male gender,absence of metastasis,effective cytoreductive surgery,chemotherapy and multimodal therapy were significant prognostic factors for prolonged OS(all P<0.05).Primary tumor site,extraabdominal tumors,absence of metastasis and effective cytoreductive surgery were associated with improved PFS(all P<0.05).Cox regression analysis showed effective cytoreductive surgery and chemotherapy were independent prognostic factors.Conclusion·Multimodal therapeutics that clear tumors by surgery,adjuvant therapy are favorable prognostic factors for improved survival level in DSRCT patients.