Yuan Z N, Xu L B, Zhao Z G, Xu S F, Zhang X X, Liu T, Zhang S G, Yu S J
Department of Orthopaedics, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
Zhonghua Zhong Liu Za Zhi. 2017 Jun 23;39(6):439-444. doi: 10.3760/cma.j.issn.0253-3766.2017.06.008.
To investigate the clinicopathological features and prognosis of malignant peripheral nerve sheath tumors (MPNST). We 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. The 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 <0.05). The tumor location, AJCC stage, S-100, Ki-67 staining, margin status, radiotherapy and chemotherapy affected 3-year LR rate (all <0.05). The tumor location, AJCC stage, S-100, Ki-67 staining and margin status affected 3-year DM rate (all <0.05). Multivariate analysis showed that the tumor location, AJCC stage, S-100 were independent factors for 5-year OS rate (all <0.05). The tumor location, Ki-67 staining and chemotherapy were independent factors for LR (all <0.05) while the AJCC stage, margin status and Ki-67 staining were independent factors for DM (all <0.05). MPSNT 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 AJCC stage, positive margin and Ki-67 staining ≥ 20%.
探讨恶性外周神经鞘瘤（MPNST）的临床病理特征及预后。我们回顾性分析了1999年1月至2016年1月在中国医学科学院肿瘤医院接受治疗的MPNST患者的临床资料。共有140例MPNST患者纳入研究，其中男性66例，女性74例。诊断时的中位年龄为40岁。采用Kaplan-Meier法和Log rank检验进行生存分析，采用Cox比例风险回归模型进行多因素分析。中位随访时间为43.0个月。3年和5年总生存率（OS）分别为56.4%和48.6%。3年局部复发（LR）率和远处转移（DM）率分别为42.9%和49.3%。单因素分析显示，肿瘤位置、美国癌症联合委员会（AJCC）分期、S-100、放疗和切缘状态影响5年总生存率（均<0.05）。肿瘤位置、AJCC分期、S-100、Ki-67染色、切缘状态、放疗和化疗影响3年局部复发率（均<0.05）。肿瘤位置、AJCC分期、S-100、Ki-67染色和切缘状态影响3年远处转移率（均<0.05）。多因素分析显示，肿瘤位置、AJCC分期、S-100是5年总生存率的独立影响因素（均<0.05）。肿瘤位置、Ki-67染色和化疗是局部复发的独立影响因素（均<0.05），而AJCC分期、切缘状态和Ki-67染色是远处转移的独立影响因素（均<0.05）。MPNST是一种侵袭性肿瘤，预后较差。本研究确定了多个影响因素。肿瘤位于头颈部、AJCC分期晚期且S-100阴性的患者5年总生存率通常较低。肿瘤位于头颈部、Ki-67染色≥20%且未接受化疗的患者局部复发倾向较高。远处转移的预后不良因素为AJCC分期晚期、切缘阳性和Ki-67染色≥20%。
