恶性外周神经鞘膜瘤的预后相关因素分析  

Evaluation of prognostic factors for malignant peripheral nerve sheath tumor

作　　者：刘万舜 贡其星[2] 李菊明[1] 韦永中[1] LIU Wanshun;GONG Qixing;LI Juming;WEI Yongzhong.(Department of Oethopedics,the First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院骨科,南京210029 [2]南京医科大学第一附属医院病理科,210029

出　　处：《临床肿瘤学杂志》2022年第3期238-243,共6页Chinese Clinical Oncology

摘　　要：目的探讨影响恶性外周神经鞘膜瘤(MPNST)患者预后的相关因素。方法回顾性分析2010年9月至2019年12月33例MPNST患者的临床病理资料,其中男性15例,女性18例;中位年龄42岁(范围:3~69岁)。神经纤维瘤病1(NF-1)型11例,散发型22例;四肢部位20例,躯干部位13例;手术切缘阴性28例,手术切缘阳性5例。33例患者均接受手术治疗,接受辅助放疗6例,辅助化疗9例。1例ROS1突变患者接受克唑替尼治疗。以无进展生存期(PFS)和总生存期(OS)作为预后评价指标,采用Cox多因素分析评价影响预后的因素。结果全组随访6~96个月,随访期间复发8例,其中四肢部位4例,躯干部位4例;转移14例,其中四肢部位6例,躯干部位8例。全组5年无进展生存率为38.3%,5年生存率为53.3%。单因素分析显示,肿瘤大小、S-100和H3K27me3表达及手术切缘与PFS有关(P<0.05),S-100和H3K27me3表达、手术切缘、放疗与OS有关(P<0.05)。Cox多因素分析结果显示,S-100和手术切缘为影响MPNST患者PFS的独立因素,而手术切缘是影响MPNST患者OS的独立因素(P<0.05)。结论S-100和手术切缘是影响MPNST患者预后的重要因素,应结合患者临床病理特征及基因检测结果制定个性化治疗方案。Objective To investigate the relevant prognostic factors of malignant peripheral nerve sheath tumors(MPNST).Methods The data of 33 patients with MPNST from September 2010 to December 2019 were reviewed.The 33 patients included 15 males and 18 females,and the median age was 42 years(ranged from 3 to 69 years).Eleven cases were neurofibromatosis type 1(NF-1)-associated MPNST,and 22 were sporadic MPNST.The distribution of lesion location included 20 cases of extremities and 13 cases of trunk.There were 28 cases with negative surgical margin and 5 cases with positive surgical margin.All patients underwent surgery.Five patients were treated with adjuvant radiotherapy and 9 patients with adjuvant chemotherapy.One patient with ROS1 mutation received crizotinib.Progression-free survival(PFS)and overall survival(OS)were analyzed as clinical outcomes.Prognostic factors were analyzed by Cox multivariate analysis.Results The follow-up time was 6-96 months.Eight patients had local recurrence(4 of extremities,4 of trunk),and 14 patients were companied by metastasis(6 of extremities,8 of trunk).The 5-year progression-free survival rate was 38.3%and 5-year overall survival rate was 53.3%.Univariate analysis showed that the tumor size,the expression of S-100 and H3K27me3,and margin status were related with PFS(P<0.05),while the expression of S-100 and H3K27me3,margin status and radiotherapy were related with OS(P<0.05).Multivariate Cox regression analysis showed that S-100 and margin status were the independent factors affecting PFS(P<0.05),while margin status was the independent prognostic factor affecting OS(P<0.05).Conclusion S-100 and surgical margin are important factors affecting the prognosis of patients with MPNST.Individualized treatment should be made according to the clinicopathological features and gene detection results of patients.

关 键 词：恶性外周神经鞘膜瘤 手术 预后 

分 类 号：R739.43[医药卫生—肿瘤]