140例恶性外周神经鞘瘤患者的临床病理特征与预后分析  

Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor： a retrospective study of 140 cases

作　　者：袁振南[1] 徐立斌[1] 赵振国[1] 许宋锋[1] 张鑫鑫[1] 刘婷[1] 张曙光[1] 于胜吉[1] 

机构地区：[1]国家癌症中心中国医学科学院北京协和医学院肿瘤医院骨科,100021

出　　处：《中华肿瘤杂志》2017年第6期439-444,共6页Chinese Journal of Oncology

摘　　要：目的探讨恶性外周神经鞘瘤（MPNST）患者的临床病理特征及其预后因素。方法回顾性分析1999年1月至2016年1月中国医学科学院肿瘤医院收治的140例MPNST患者的临床资料，其中男性66例，女性74例，中位发病年龄40岁。生存分析采用Kaplan-Meier法，并行Log rank检验，多因素分析采用Cox比例风险回归模型。结果全组患者的中位随访时间为43个月，3年和5年生存率分别为56.4%和48.6%，3年局部复发率为42.9%，3年远处转移率为49.3%。单因素分析显示，MPNST患者的5年生存率与肿瘤部位、美国癌症联合委员会（AJCC）分期、S-100的表达、Ki-67指数、手术切缘状态以及是否放疗有关（均P〈0.05）。MPNST患者的3年局部复发率与肿瘤部位、AJCC分期、S-100表达、Ki-67指数、手术切缘状态、是否放疗、是否化疗有关（均P〈0.05）。MPNST患者的3年远处转移率与肿瘤部位、AJCC分期、S-100表达、Ki-67指数、手术切缘状态有关（均P〈0.05）。多因素分析显示，肿瘤部位、AJCC分期、S-100表达是影响MPNST患者5年总生存的独立因素（均P〈0.05）。肿瘤部位、Ki-67指数、是否化疗是影响MPNST患者局部复发的独立因素（均P〈0.05）。AJCC分期、手术切缘状态和Ki-67指数是影响MPNST患者发生远处转移的独立因素（均P〈0.05）。结论MPNST患者的预后较差。影响MPNST患者预后的因素较多，肿瘤位于头颈、AJCC分期较晚、S-100阴性的患者5年生存率较低；肿瘤位于头颈、Ki-67指数≥20%、未行化疗的患者易复发；AJCC分期较晚、手术切缘阳性、Ki-67指数≥20%的患者易发生远处转移。ObjectiveTo investigate the clinicopathological features and prognosis of malignant peripheral nerve sheath tumors （MPNST）.MethodsWe retrospectively reviewed the clinical data of MPNST patients who were treated at Cancer Institute ＆ Hospital, Chinese Academy of Medical Science from January 1999 to January 2016. A total of 140 patients with 66 male and 74 female with MPNST were enrolled in the study. The median age was 40 at the time of diagnosis. Survival analysis were estimated by Kaplan-Meier method and Log rank test. Multivariate analysis were estimated by Cox proportional hazards regression model.ResultsThe median follow-up time was 43.0 months. The 3- and 5-year overall survival （OS） rates were 56.4% and 48.6%, respectively. The 3-year local recurrence （LR） rate and distant metastasis （DM） rates were 42.9% and 49.3%, respectively. Univariate analysis showed that the tumor location, AJCC stage, S-100, radiotherapy and margin status affected 5-year OS rate （all P〈0.05）. The tumor location, AJCC stage, S-100, Ki-67 staining, margin status, radiotherapy and chemotherapy affected 3-year LR rate （all P〈0.05）. The tumor location, AJCC stage, S-100, Ki-67 staining and margin status affected 3-year DM rate （all P〈0.05）. Multivariate analysis showed that the tumor location, AJCC stage, S-100 were independent factors for 5-year OS rate （all P〈0.05）. The tumor location, Ki-67 staining and chemotherapy were independent factors for LR （all P〈0.05） while the AJCC stage, margin status and Ki-67 staining were independent factors for DM （all P〈0.05）.ConclusionsMPSNT is an aggressive tumor with poor prognosis. Multiple factors were identified in this study. Patients with the tumor located at head and neck, advanced AJCC stage and negative S-100 usually have a low 5-year overall survival rate. Patients with the tumor located at head and neck, Ki-67 staining ≥ 20% and without chemotherapy had a higher tendency of local recurrence. Poor prognosis factors for DM were advanced

关 键 词：恶性外周神经鞘瘤 临床病理特征 预后 复发 转移 

分 类 号：R739.4[医药卫生—肿瘤]