2型神经纤维瘤病的手术策略及疗效分析  

Surgical strategy and outcome analysis of neurofibromatosis type 2

作　　者：王兴朝[1] 李仕维[1] 李朋[1] 王博[1] 杨智君[1] 王振民[1] 赵赋[1] 张俊廷[1] 刘丕楠[1] Wang Xingchao;Li Shivei;Li Peng;Wang Bo;Yang Zhijun;Wang Zhenmin;Zhao Fu;Zhang Junting;Liu Pinan(Neurosurgery Center,Bejing Tiantan Hospital,Capital Medical University,Bejing 100070,China)

机构地区：[1]首都医科大学附属北京天坛医院神经外科学中心,北京100070

出　　处：《中华神经外科杂志》2023年第6期546-550,共5页Chinese Journal of Neurosurgery

基　　金：国家自然科学基金(81974387,82003023)。

摘　　要：目的探讨2型神经纤维瘤病(NF2)的手术策略,分析影响预后的相关因素.方法回顾性分析2009年1月至2018年12月首都医科大学附属北京天坛医院神经外科学中心手术治疗的87例NF2患者的临床资料.采用以优先挽救视听觉功能、精准解除主体肿瘤压迫、个体化手术干预时机的手术策略.采用Karnofsky功能状态评分(KPS)评估患者的预后,KPS较术前不变或增高为预后良好,下降为预后不良.采用单因素和多因素logistic回归分析法分析影响患者预后的因素.结果87例患者的中位随访时间为98个月(36~144个月).至末次随访,54例(62.1%)预后良好;33例(37.9%)预后不良,其中死亡15例,二次手术3例,新发或神经功能障碍加重15例.单因素logistic回归分析结果显示,发病年龄≤30岁、确诊至手术时间≥36个月、肿瘤最大径≥30 mm、术前KPS低、术前有视力和吞咽功能障碍的患者发生预后不良的风险高(均P<0.05).多因素logistic回归分析结果显示,发病年龄≤30岁(OR=3.31,95%CI:1.06~10.39,P=0.040)、术前视力障碍(OR=3.32,95%CI:1.08~10.25,P=0.037)与患者的长期预后差有关.结论采用优化手术治疗策略治疗NF2,可提高患者的远期疗效.发病年龄≤30岁和术前视力障碍是患者长期预后差的重要影响因素.Objective To discuss the surgical strategies of neurofibromatosis type2(NF2)and to analyze the prognostic factors.Methods The clinical data of 87 NF2 patients who underwent surgical treatment at the Neurosurgery Center of Bejing Tiantan Hospital,Capital Medical University from January 2009 to December 2018 were retrospectively analyzed.All patients received optimal surgical strategies that were aimed to protect visual and auditory functions,alleviate key tumor compression,and individualize the timing of surgical intervention.The Karnofsky functional status score(KPS)was used to evaluate the patient's outcome.An unchanged or incereased KPS compared to the preoperative level indicated favorable outcome,while a decreased KPS indicated poor outcome.Univariate and multivariate logistic regression analyses were performed to identify the prognostic factors.Results At the last follow-up,the median follow-up time of the 87 patients was 98 months(36-144 months).A total of 54 cases(62.1%)had favorable outcomes,while 33 cases(37.9%)had poor outcomes,including 15 cases of death,3 cases of a second operation and 15 cases of newly-onset or aggravated neurological dysfunction.Univariate logistic regression analysis showed that the patient's age of onset≤30 years,diagnosis-to-surgery interval of≥36 months,maximum tumor diameter of≥30 mm,low preoperative KPS,and preoperative vision and swallowing dysfunction were risk factors of poor outcomes(all P<0.05).Multivariate logistic regression analysis indicated that age of onset≤30 years(OR=3.31,95%CI:1.06-10.39,P=0.040)and preoperative visual impairment(OR=3.32,95%CI:I.08-10.25,P=0.037)were related to the patients'long-term poor outcomes.Conclusions Optimal surgical treatment strategies for NF2 can improve the long-term clinical outcomes of patients.Age of onset≤30 year and preoperative visual impairment are important risk factors of the patients'long-term poor outcomes.

关 键 词：神经纤维瘤病2型 神经外科手术 治疗结果 随访研究 治疗策略 

分 类 号：R739.4[医药卫生—肿瘤]