摘要：

目的 总结颅内侵袭性嗅神经母细胞瘤的临床特点、手术策略和治疗效果。方法 纳入2005年1月至2020年12月在首都医科大学附属北京同仁医院诊断与治疗的24例颅内侵袭性嗅神经母细胞瘤患者，均行双侧扩大经基底入路联合内镜经鼻入路手术+颅底重建术。结果 24例患者肿瘤全切除22例（91.67%）、近全切除2例（8.33%），均经术后病理证实为嗅神经母细胞瘤。术后2例（8.33%）发生短暂性脑脊液漏，2例（8.33%）出现颅内感染，均经对症治疗治愈；1例视力下降加重，2例眼动障碍加重。20例完成随访，平均随访54.60个月，均未发生远期手术相关并发症，12例（60%）肿瘤复发，5年生存率为45%（9/20）。结论 扩大经基底入路联合内镜经鼻入路手术切除颅内侵袭性嗅神经母细胞瘤安全、有效，术者应同时具备显微神经外科和神经内镜技术，值得临床推广。

关键词: 神经母细胞瘤, 嗅神经, 显微外科手术, 神经内窥镜

Abstract:

Objective To investigate the clinical characteristics, neurosurgical strategy and curative effect of intracranial invastive olfactory neuroblastoma. Methods A total of 24 patients with intracranial invasive olfactory neuroblastoma diagnosed and treated in Beijing Tongren Hospital, Capital Medical University from January 2005 to December 2020 were included. All patients underwent bilateral extended transbasal approach combined with endoscopic transnasal approach and skull base reconstruction. Results Among the 24 patients, total resection was performed in 22 cases (91.67%) and nearly total resection in 2 cases (8.33%), all of which were confirmed as olfactory neuroblastoma by postoperative pathological examination. Transient cerebrospinal fluid leakage occurred in 2 cases (8.33%) and intracranial infection occurred in 2 cases (8.33%), which were cured by symptomatic treatment. Visual acuity was aggravated in one case and eye movement disorder in 2 cases. Twenty patients were followed up for an average of 54.60 months, and no long-term operation related complications occurred. During the follow-up period, 12 patients (60%) had tumor recurrence, and the 5-year survival rate was 45% (9/20). Conclusions Surgical resection of intracranial invasive olfactory neuroblastoma via extended transbasal approach combined with endoscopic transnasal approach is safe and effective. The operative surgeon should have both microneurosurgery and neuroendoscopy techniques, which is worthy of clinical promotion.

Key words: Neuroblastoma, Olfactory nerve, Microsurgery, Neuroendoscopes