33例外周原始神经外胚瘤临床分析

Peripheral Primitive Neuroectodermal Tumor:Clinical Analysis of 33 Cases

目的:探讨各种临床因素及治疗方法对外周原始神经外胚瘤(pPNET)预后的影响。方法:回顾性分析天津医科大学附属肿瘤医院2001年1月至2009年11月间收治的33例病理诊断为pPNET患者的临床资料,其中:男15例,女18例;中位年龄17岁;肿瘤原发于躯干部位21例,非躯干部位12例(10例原发于四肢,2例原发于头皮)。25例患者接受了手术治疗,其中19例接受根治性手术,6例接受姑息性手术,8例根治术后患者及1例姑息术后患者接受了术后放疗;8例未手术患者中3例接受了局部放疗。手术治疗病例中17例接受了术后化疗,5例接受了术前化疗;未手术病例中5例接受了全身化疗。采用Kaplan-Meier法及Cox回归模型分析影响生存的因素,组间比较采用x^2检验。结果:全组中位生存期为24个月(范围10～38个月),2年生存率为36.4%;根治术后局部复发率为31.6%;全组主要转移部位为肺和骨,转移率均为24.2%。单因素分析显示肿瘤原发部位(P=0.005)、手术方式(P=0.001)可以影响全组患者的总生存期,术后放疗(P=0.039)有可能提高术后惠者的生存期;多因素分析显示肿瘤原发部位(P=0.037)、手术方式(P=0.017)是影响患者生存的独立预后因素。结论:pPNET临床上恶性程度高,预后差,肿瘤原发部位、手术方式可以影响pPNET患者的总生存期,术后放疗有可能提高术后患者的生存期,肿瘤原发部位及手术方式是影响患者生存的独立预后因素。

Objective： To investigate the effects of clinical factors ripheral primitive neuroectodermal tumor （ pPNET ）. Methods： Clinical and treatment modalities on the prognosis of patients with pedata of 33 patients with pPNET who underwent surgery or regional radiotherapy in our hospital were included in the retrospective study. Among the patients, 15 were male and 18 were female, with a median age of 17 years. Up to 21 of the 33 patients had primary foci in the trunk, whereas the other 12 patients suffered from primary tumors outside the trunk, specifically, 10 in the extremities and 2 in the scalp. Surgery was conducted on 25 of the patients, among which, 19 underwent radical operations and 6 received palliative surgery. Postoperative radiotherapy was performed on 8 of the 19 patients after radical surgery and on 1 of the 6 patients after the palliative surgery. Regional radiotherapy was performed in 3 of the 8 patients who did not undergo surgery. Of the 25 patients who underwent the surgery, 17 patients received postoperative chemotherapy and 5 patients received preoperative neoadjuvant chemotherapy. Systemic chemotherapy was conducted in 5 of the patients who failed to undergo the surgery. The Kaplan-Meier method and Cox regression were used for survival analysis, and a Chi-square test was used to analyze the incidence in the different groups. Results： The median survival time was 24 months （ range, 10-38 months ）, and the 2-year survival rate was 36.4% for all patients. The local relapse rate was 31.6% for patients who underwent the radical surgery. The most common metastatic sites were the lungs and bones, with a 24.2% metastatic rate for all the groups. Univariate analysis demonstrated that both the primary lesion of the tumor （ P = 0.005 ） and the mode of surgery （ P = 0.001 ） affected overall survival, and postoperative radiotherapy （ P = 0.039 ） prolonged the survival time of patients after surgery. Multivariate analysis suggested that both the primary lesion of tumor （ P = 0.037 ） and the mode of operation （ P = 0.017 ） were the independent prognostic factors for survival. Conclusion： pPNETs have a very high degree of malignancy. The prognosis of the tumor is poor. The primary site of the tumors and the mode of operation are significantly correlated with overall survival. Postoperative radiotherapy may extend the survival time of patients who undergo surgery. Multivariate analysis suggests that the primary lesion and the mode of surgery are the independent prognostic factors for the patient survival.