›› 2012, Vol. 32 ›› Issue (11): 1490-.doi: 10.3969/j.issn.1674-8115.2012.11.020

• 论著(临床研究) • 上一篇    下一篇


张凤春1,3, 唐 雷2, 马 越2, 任 芳1, 王红霞2, 徐迎春2   

  1. 上海交通大学 1.医学院附属苏州九龙医院肿瘤科, 苏州 215021; 2.医学院附属仁济医院肿瘤科, 上海 200127; 3.医学院附属瑞金医院, 上海 200025
  • 出版日期:2012-11-28 发布日期:2012-11-30
  • 通讯作者: 王红霞, 电子信箱: whx365@126.com;徐迎春, 电子信箱: xiaoxu2384@163.com。
  • 作者简介:张凤春(1957—), 男, 主任医师, 博士, 博士生导师;电子信箱: fczhang2004@163.com。

Analysis of clinical features and prognosis in 126 patients with peripheral primitive neuroectodermal tumor

ZHANG Feng-chun1,3, TANG Lei2, MA Yue2, REN Fang1, WANG Hong-xia2, XU Ying-chun2   

  1. 1.Department of Oncology, Kowloon Hospital, Shanghai Jiaotong University School of Medicine, Suzhou 215021, China;2.Department of Oncology, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China;3.Department of Oncology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200025, China
  • Online:2012-11-28 Published:2012-11-30


目的 探讨外周性原始神经外胚层瘤(pPNET)的临床特点及影响预后的因素。方法 收集临床上9例pPNET患者及文献报道的117例pPNET患者的临床资料,分析pPNET 患者影像学、病理组织学、免疫组织化学、临床特征及治疗状况。应用Kaplan-Meier法计算患者1年、3年、5年生存率;分析影响预后的因素,单因素分析采用Log-rank检验,多因素分析采用COX风险回归模型。结果 126例pPNET患者中,男性65例,女性61例,平均年龄(29.90±16.06)岁;其中,Ⅰ期患者35例(27.8%),Ⅱ期患者53例(42.1%),Ⅲ期患者11例(8.7%),Ⅳ期患者27例(21.4%);共110例接受手术治疗,肿瘤完全切除者88例,12例患者在新辅助化疗后行手术治疗;32例(25.4%)患者接受辅助放疗;61例(48.4%)接受辅助化疗,47例患者接受一线化疗,其中37例进行二线及二线以上挽救治疗。平均随访时间为19.0个月,复发52例,死亡38例;1年、3年、5年生存率分别为54.8%、15.9%和3.2%。单因素分析结果显示,肿瘤直径、淋巴结转移、远处转移和分期是影响预后的因素;多因素分析结果显示,完全性手术切除是影响预后的独立因素。结论 综合治疗是pPNET的主要治疗方法,完全性手术切除是影响预后的重要因素。

关键词: 外周性原始神经外胚层瘤, 治疗, 预后


Objective To investigate the clinical characteristics and factors of prognosis for peripheral primitive neuroectodermal tumors (pPNET). Methods The clinical data of 9 patients in clinics and medical records of 117 patients with pPNET reported by literatures were collected. The data of imaging, histopathology, immunohistochemistry, clinical features and treatment of patients with pPNET were analysed. Kaplan-Meier method was employed to calculate the 1-year, 3-year and 5-year survival rates of patients, and the factors of prognosis were analysed. Log-rank tests were used for univariate analysis, and Cox proportional hazards was adopted for multivariate analysis. Results There were 65 males and 61 females, with the average age of (29.90±16.06) years. There were 35 cases (27.8%) with stage I, and the percentages of stageⅡ, Ⅲ, and Ⅳ were 42.1%, 8.7% and 21.4%, respectively. One hundred and ten patients received surgical treatment, radical surgery was performed in 88 patients, and 12 patients underwent surgery after neoadjuvant chemotherapy. Thirty-two patients (25.4%) received adjuvant radiotherapy, and 61 patients (48.4%) underwent adjuvant chemotherapy. Forty-seven patients received first-line chemotherapy, among whom 37 received second-line or more than second-line salvage therapy. The mean follow-up duration was 19.0 months. Relapse occurred in 52 patients, and there were 38 cases of death. The 1-year, 3-year and 5-year survival rates were 54.8%, 15.9% and 3.2%, respectively. Univariate analysis revealed that tumor size, lymph node status, distant metastasis and stage were significant prognostic factors, and multivariate analysis indicated that complete surgery was an independent prognostic factor. Conclusion Comprehensive therapy is the main treatment for pPNET, and complete surgery is an important prognosis factor.

Key words: peripheral primitive neuroectodermal tumors, treatment, prognostic