神经内镜经鼻蝶入路切除颅底脊索瘤的临床疗效及其影响因素分析

目的探讨神经内镜经鼻蝶入路切除颅底脊索瘤的手术策略、临床疗效及其影响因素。方法回顾性分析2004年1月至2021年3月首都医科大学附属北京天坛医院神经外科采用神经内镜经鼻蝶入路切除的颅底脊索瘤患者的临床资料,共纳入373例患者(共行500例次手术)。其中2004—2019年为早期手术组,采用以肿瘤为中心的手术策略;2020—2021年为近期手术组,依据肿瘤的起源部位及其沿着颅底骨缝、神经和大血管孔道延伸生长的特点进行顺序探查和切除(探查顺序形似"龟背")。比较两种手术策略切除肿瘤程度的差异。随访患者的无进展生存期(PFS)和总生存期(OS),采用Kaplan-Meier法生存分析,采用log-rank检验分析不同临床特征患者间PFS和OS的差异。结果500例次手术中,413例次为早期手术组,肿瘤的全切除率、次全切除率、部分切除率分别为35.1%(145例次)、36.6%(151例次)、28.3%(117例次);87例次为近期手术组,肿瘤的全切除率、次全切除率、部分切除率分别为52.9%(46例次)、37.9%(33例次)、9.2%(8例次),两组肿瘤的切除率差异有统计学意义(P<0.001)。500例次手术,脑脊液漏的发生率为4.6%(23例次)。373例患者中,术后1例(0.3%)死亡,1例(0.3%)发生大面积脑梗死;共325例(87.1%)患者获得完整的随访资料,中位随访时间为31个月(3~193个月)。182例(56.0%)复发,估计总体患者的中位PFS为24个月(95%CI:17.5~30.5);85例(26.2%)死亡,估计总体患者的中位OS为106个月。生存分析显示,与原发性肿瘤、肿瘤体积小、肿瘤全切除、病理学类型为经典型或软骨型肿瘤及术后放疗的患者比较,复发性肿瘤、肿瘤体积大、肿瘤非全切除、病理学类型为去分化或差分化或肉瘤样型肿瘤及术后未行放疗患者的PFS和OS均短,差异均有统计学意义(均P<0.05)。结论依据颅底脊索瘤生长侵袭特点,采用"龟背"形顺序探查、切除肿瘤的手术策略有利于提高肿瘤的切除程度。肿瘤是否原发、体积大小、切除程度、病理学类型、术后是否放疗可能与颅底脊索瘤患者术后的PFS和OS有关。

Clinical outcomes and prognostic factors of endoscopic transnasosphenoidal resection of skull base chordomas

Objective To discuss the surgical strategies,clinical outcomes,and prognostic factors of endoscopic transnasosphenoidal resection of skull base chordomas.Methods A retrospective analysis was conducted on the clinical data of patients with skull base chordomas who underwent endoscopic transnasosphenoidal resection at the Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University from January 2004 to March 2021.A total of 373 patients were enrolled in the analysis and underwent 500 surgical operations.The patients undergoing surgery from 2004 to 2019 were enrolled in the early operation group,in whom the surgical strategy was based on the tumor center;those undergoing surgery from 2020 to 2021 were enrolled in the recent operation group,in whom the surgical strategy was based on the growth pattern of tumor which was to perform sequential exploration and resection according to the origin of the tumor and its characteristics of extension and growth along the cranial sutures,spaces between nerves and vessels(turtle-back-like sequence).The resection rates were compared between the two surgical strategies.Kaplan-Meier survival analysis was performed to calculate the progression-free survival(PFS)and overall survival(OS).The The log-rank test was adopted to analyze the prognostic factors associated with PFS and OS.Results Among the 500 operations,413 cases belonged to the early operation group,in which the tumor total resection rate,subtotal resection rate,and partial resection rate were 35.1%(145 cases),36.6%(151 cases),and 28.3%(117 cases).There were 87 cases were in the recent surgery group,in which the tumor total resection rate,subtotal resection rate,and partial resection rate were 52.9%(46 cases),37.9%(33 cases),and 9.2%(8 cases),respectively.The difference in tumor resection rate between the two groups was statistically significant(P<0.001).Complete follow-up data of 325 patients(87.1%)were achieved.The median follow-up time was 31 months(3-193 months)and 182 cases(56.0%)relapsed.The estimated median PFS of the overall patients was 24 months(95%CI:17.5-30.5 months).There were 85 cases(26.2%)of death,and the median OS of the overall patients was estimated to be 106 months.Survival analysis showed that compared with patients with primary tumor,small tumor size,total tumor resection,classical or cartilaginous tumor,postoperative radiotherapy,those with recurrence tumor,large tumor volume,non-total tumor resection,pathologically dedifferentiated or poorly differentiated tumors,and without postoperative radiotherapy had shorter PFS and OS,and the differences were statistically significant(all P<0.05).Conclusion According to the characteristics of the growth and invasion of skull base chordoma,the surgical strategy of"turtle-back-like"sequential exploration and resection of the tumor is beneficial to improve the degree of tumor resection.Whether the tumor is primary or recurrent,tumor size,degree of resection,pathological type,and postoperative radiotherapy may be related to PFS and OS in patients with skull base chordoma.