恶性周围神经鞘瘤患者的临床特征、治疗方式和预后影响因素分析

nalysis of clinical features,treatment methods,and prognostic influence factors in patients with malignant peripheral nerve sheath tumor

目的探讨恶性周围神经鞘瘤(malignant peripheral nerve sheath tumor,MPNST)患者的临床特征、治疗方式及预后影响因素。方法回顾分析2015年1月1日—2021年12月31日收治的96例MPNST患者资料。男46例,女50例;年龄15~87岁,平均48.2岁。肿瘤位于躯干50例,四肢39例,头颈部7例。肿瘤最大径<5 cm 49例,≥5 cm 32例,缺失15例。肿瘤深度:深部77例,表浅19例。法国国家癌症中心联盟(FNCLCC)组织学分级:G19例,G212例,G334例,缺失41例。37例为复发型MPNST,32例合并Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1),26例为Ⅳ期MPNST。25例接受术后辅助放疗,45例接受围术期化疗,30例接受安罗替尼靶向治疗,73例行R0切除。以是否合并NF1分组,比较两组患者上述基线资料。使用Kaplan-Meier法分别绘制每个因素(年龄、性别、是否合并NF1、是否复发型MPNST、是否Ⅳ期MPNST、FNCLCC分级、是否R0切除、肿瘤部位、肿瘤大小、肿瘤深度、是否围术期化疗、是否术后辅助放疗、是否安罗替尼靶向治疗)下各分组人群的1年无疾病生存期(disease-free survival,DFS)和3年总生存期(overall survival,OS)生存曲线,并用Log-Rank检验不同生存曲线间的差异是否有统计学意义。使用多因素COX比例风险模型探索MPNST的独立预后影响因素。结果合并NF1组较未合并NF1组患者肿瘤更多位于表浅、FNCLCC分级较低者更多,差异有统计学意义(P<0.05);其他指标两组间比较差异均无统计学意义(P<0.05)。Kaplan-Meier分析示,是否复发型MPNST、是否Ⅳ期MPNST、FNCLCC分级、是否R0切除、是否围术期化疗以及是否安罗替尼靶向治疗是1年DFS的影响因素(P<0.05),是否Ⅳ期MPNST、FNCLCC分级以及是否围术期化疗是3年OS的影响因素(P<0.05)。多因素COX比例风险模型分析示,对于1年DFS,复发型MPNST和高级别FNCLCC分级(G3)是独立预后影响因素(P<0.05);对于3年OS,Ⅳ期MPNST、肿瘤表浅、>60岁患者、接受术后辅助放疗和接受安罗替尼靶向治疗是独立预后影响因素(P<0.05)。结论合并NF1的MPNST患者肿瘤部位更表浅,FNCLCC分级更低;FNCLCC分级高低、是否R0切除以及是否放化疗与MPNST预后密切相关。建议临床治疗中应优先考虑手术完全切除肿瘤,并结合辅助治疗(如放疗和安罗替尼靶向治疗)以改善患者预后。

Objective To investigate the clinical features,treatment methods,and prognostic influence factors of patients with malignant peripheral nerve sheath tumor(MPNST).Methods A retrospective analysis was conducted on 96 MPNST patients treated between January 1,2015 and December 31,2021.There were 46 males and 50 females,aged between 15 and 87 years(mean,48.2 years).The tumors were located in the trunk in 50 cases,extremities in 39 cases,and head and neck in 7 cases.The maximum tumor diameter was<5 cm in 49 cases,≥5 cm in 32 cases,with 15 cases missing data.Tumor depth was deep in 77 cases and superficial in 19 cases.The Fédération Nationale des Centres de Lutte Contre le Cancer(FNCLCC)histological grading was G1 in 9 cases,G2 in 12 cases,and G3 in 34 cases,with 41 cases missing data.There were 37 recurrent MPNST cases,32 cases with neurofibromatosis type 1(NF1),and 26 cases in stageⅣ.Postoperative adjuvant radiotherapy was administered to 25 patients,perioperative chemotherapy to 45 patients,and anlotinib-targeted therapy to 30 patients.R0 resection was achieved in 73 cases.Patients were divided into groups based on the presence or absence of NF1,and baseline data between the two groups were compared.Kaplan-Meier curves were generated to assess disease-free survival(DFS)and overall survival(OS)based on various factors(age,gender,presence of NF1,recurrent MPNST,stageⅣMPNST,FNCLCC grade,R0 resection,tumor location,tumor size,tumor depth,perioperative chemotherapy,postoperative adjuvant radiotherapy,and anlotinib-targeted therapy),and differences between survival curves were analyzed using the Log-Rank test.Multivariate COX proportional hazards regression was used to identify independent prognostic factors for MPNST.Results Patients with NF1 had a significantly higher proportion of superficial tumors and lower FNCLCC grade compared to those without NF1(P<0.05);no significant difference was found for other variables(P<0.05).Kaplan-Meier analysis showed that recurrent MPNST,stageⅣMPNST,FNCLCC grade,R0 resection,perioperative chemotherapy,and anlotinib-targeted therapy were factors influencing 1-year DFS(P<0.05),while stageⅣMPNST,FNCLCC grade,and perioperative chemotherapy were factors affecting 3-year OS(P<0.05).Multivariate COX proportional hazards regression analysis revealed that recurrent MPNST and high-grade FNCLCC(G3)were independent prognostic factors for 1-year DFS(P<0.05),while stageⅣMPNST,superficial tumor depth,age over 60 years,postoperative adjuvant radiotherapy,and anlotinib-targeted therapy were independent prognostic factors for 3-year OS(P<0.05).Conclusion MPNST patients with NF1 tend to have more superficial tumors and lower FNCLCC grades.FNCLCC grade,R0 resection,and adjuvant therapies,including radiotherapy and anlotinib-targeted therapy,are closely associated with MPNST prognosis.Complete surgical resection should be prioritized in clinical management,along with adjuvant treatments such as radiotherapy and targeted therapy of anlotinib to improve patient outcomes.