Ⅰ型神经纤维瘤病的神经外科治疗进展  

Progress in neurosurgical treatment of neurofibromatosis type 1

作　　者：李策 刘炳含 王延俊 于台飞 郑志明 王国栋 LI Ce;LIU Binghan;WANG Yanjun;YU Taifei;ZHENG Zhiming;WANG Guodong(Department of Neurosurgery,Shandong Provincial Hospital Affiliated to Shandong First Medical University,Ji’nan Shandong,250021,P.R.China;Department of Radiology,Shandong Provincial Hospital Affiliated to Shandong First Medical University,Ji’nan Shandong,250021,P.R.China)

机构地区：[1]山东第一医科大学附属省立医院神经外科,济南250021 [2]山东第一医科大学附属省立医院医学影像科,济南250021

出　　处：《中国修复重建外科杂志》2024年第10期1171-1179,共9页Chinese Journal of Reparative and Reconstructive Surgery

基　　金：齐鲁卫生与健康领军人才培育工程(2020-2025);山东省自然科学基金青年基金(ZR2021QH227)。

摘　　要：目的总结Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1)的神经外科治疗进展,探讨治疗策略,以期为临床治疗提供参考。方法回顾近年国内外NF1领域的相关文献和临床案例,从NF1相关的主要神经系统病变类型及其治疗等方面进行深入总结,分析神经外科手术未来研究方向。结果NF1易发生复杂多样的中枢和周围神经系统病变,尤其是脑内低级别胶质瘤、椎管与椎旁神经纤维瘤,其治疗方案应由多学科诊疗团队共同制定。对有症状的丛状神经纤维瘤、有恶性肿瘤影像学证据的肿瘤需要神经外科手术治疗,手术目的是缩小肿瘤、缓解疼痛和改善外观。术后功能康复训练、多学科长期随访和心理社会干预是改善患者生活质量的重要措施。先进的影像引导系统和人工智能技术有助于提高肿瘤切除率和降低疾病复发率。结论神经外科手术是药物治疗无效且病变进展快速的丛状神经纤维瘤和恶性周围神经鞘瘤的首要治疗方式,术前多学科评估、术中电生理监测以及手术辅助设备能提高手术有效性和安全性。未来应继续探索新的手术技术和改进术后管理策略,实现对NF1患者更精确和个性化的治疗。Objective To summarize the latest developments in neurosurgical treatments for neurofibroma-tosis type 1(NF1)and explore therapeutic strategies to provide comprehensive treatment guidelines for clinicians.Methods The recent domestic and international literature and clinical cases in the field of NF1 were reviewed.The main types of neurological complications associated with NF1 and their treatments were thorough summarized and the future research directions in neurosurgery was analyzed.Results NF1 frequently results in complex and diverse lesions in the central and peripheral nervous systems,particularly low-grade gliomas in the brain and spinal canal and paraspinal neurofibromas.Treatment decisions should be made by a multidisciplinary team.Symptomatic plexiform neurofibromas and tumors with malignant imaging evidence require neurosurgical intervention.The goals of surgery include reducing tumor size,alleviating pain,and improving appearance.Postoperative functional rehabilitation exercises,long-term multidisciplinary follow-up,and psychosocial interventions are crucial for improving the quality of life for patients.Advanced imaging guidance systems and artificial intelligence technologies can help increase tumor resection rates and reduce recurrence.Conclusion Neurosurgical intervention is the primary treatment for symptomatic plexiform neurofibromas and malignant peripheral nerve sheath tumors when medical treatment is ineffective and the lesions progress rapidly.Preoperative multidisciplinary assessment,intraoperative electrophysiological monitoring,and advanced surgical assistance devices significantly enhance surgical efficacy and safety.Future research should continue to explore new surgical techniques and improve postoperative management strategies to achieve more precise and personalized treatment for NF1 patients.

关 键 词：Ⅰ型神经纤维瘤病 低级别胶质瘤 丛状神经纤维瘤 恶性周围神经鞘瘤 神经外科治疗 多学科 

分 类 号：R739.4[医药卫生—肿瘤]