摘要/Abstract

摘要： 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。
关键词: &ensp, 促结缔组织增生性小圆细胞瘤；手术；化学治疗；预后因素
Abstract:
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.
Key words: desmoplastic small round cell tumor (DSRCT), surgery, chemotherapy, prognosis factor


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