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[1型神经纤维瘤病的神经外科治疗进展]

[Progress in neurosurgical treatment of neurofibromatosis type 1].

作者信息

Li Ce, Liu Binghan, Wang Yanjun, Yu Taifei, Zheng Zhiming, Wang Guodong

机构信息

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.

出版信息

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2024 Oct 15;38(10):1171-1179. doi: 10.7507/1002-1892.202407058.

Abstract

OBJECTIVE

To summarize the latest developments in neurosurgical treatments for neurofibromatosis 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.

摘要

目的

总结1型神经纤维瘤病(NF1)神经外科治疗的最新进展,探索治疗策略,为临床医生提供综合治疗指南。

方法

回顾了NF1领域近期的国内外文献及临床病例。全面总结了与NF1相关的主要神经并发症类型及其治疗方法,并分析了神经外科未来的研究方向。

结果

NF1常导致中枢和周围神经系统出现复杂多样的病变,尤其是脑和椎管内的低级别胶质瘤以及椎旁神经纤维瘤。治疗决策应由多学科团队做出。有症状的丛状神经纤维瘤和有恶性影像学证据的肿瘤需要神经外科干预。手术目标包括缩小肿瘤大小、减轻疼痛和改善外观。术后功能康复锻炼、长期多学科随访及心理社会干预对提高患者生活质量至关重要。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并降低复发率。

结论

当药物治疗无效且病变进展迅速时,神经外科干预是有症状的丛状神经纤维瘤和恶性周围神经鞘膜瘤的主要治疗方法。术前多学科评估、术中电生理监测及先进的手术辅助设备可显著提高手术疗效和安全性。未来研究应继续探索新的手术技术,改进术后管理策略,以实现对NF1患者更精准、个性化的治疗。

相似文献

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[Progress in neurosurgical treatment of neurofibromatosis type 1].[1型神经纤维瘤病的神经外科治疗进展]
Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2024 Oct 15;38(10):1171-1179. doi: 10.7507/1002-1892.202407058.
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Plexiform neurofibromas.丛状神经纤维瘤
Am J Med Genet. 1999 Mar 26;89(1):31-7. doi: 10.1002/(sici)1096-8628(19990326)89:1<31::aid-ajmg7>3.0.co;2-w.
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Cervical cord compression from plexiform neurofibromas in neurofibromatosis 1.1型神经纤维瘤病中丛状神经纤维瘤导致的颈髓压迫
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