Abstract：
BACKGROUND: Despite their precarious behavioral classification (benign and low grade on histopathology yet behaviorally malignant), great strides have been taken to improve prognostication and treatment paradigms for patients with skull base chordoma. With respect to surgical techniques, lateral transcranial (TC) approaches have traditionally been used, however endoscopic endonasal approaches (EEA) have been advocated for midline lesions. Nonetheless, due to the rarity of this pathology (0.2% of all intracranial neoplasms), investigations within the literature remain limited to small retrospective series. Furthermore, radiotherapeutic treatments investigated to date have proven largely ineffective.
METHODS: Accordingly, we performed a systematic review in order to profile surgical and survival outcomes for skull base chordoma. Fixed and random-effect meta-analyses were performed for categorical variables including GTR, STR, 5-year OS, 10-year OS, 5-year PFS, and 10-year PFS. Additionally, we pooled eligible studies for formal meta-analysis to compare outcomes by surgical approach (lateral versus midline). Statistical analyses were performed using R Studio \'metafor\' package or Cochrane Review Manager. Furthermore, meta-analysis of pooled mortality rates and sub-analyses of operative margin and surgical complications were used to compare midline versus lateral approaches via the Mantel-Haenszel method. We considered all p-values < 0.05 to be statistically significant.
RESULTS: Following the systematic search and screen, 55 studies published between 1993 and 2022 reporting data for 2453 patients remained eligible for analysis. Sex distribution was comparable between males and females, with a slight predominance of male-identifying patients (0.5625 [95% CI: 0.5418; 0.3909]). Average age at diagnosis was 42.4 ± 12.5 years, while average age of treatment initiation was 43.0 ± 10.6 years. Overall, I2 value indicated notable heterogeneity across the 55 studies [I2 = 56.3% (95%CI: 44.0%; 65.9%)]. With respect to operative margins, the rate of GTR was 0.3323 [95% CI: 0.2824; 0.3909], I2 = 91.9% [95% CI: 90.2%; 93.4%], while the rate of STR was significantly higher at 0.5167 [95% CI: 0.4596; 0.5808], I2 = 93.1% [95% CI: 91.6%; 94.4%]. The most common complication was CSF leak (5.4%). In terms of survival outcomes, 5-year OS rate was 0.7113 [95% CI: 0.6685; 0.7568], I2 = 91.9% [95% CI: 90.0%; 93.5%]. 10-year OS rate was 0.4957 [95% CI: 0.4230; 0.5809], I2 = 92.3% [95% CI: 89.2%; 94.4%], which was comparable to the 5-year PFS rate of 0.5054 [95% CI: 0.4394; 0.5813], I2 = 84.2% [95% CI: 77.6%; 88.8%] and 10-yr PFS rate of 0.4949 [95% CI: 0.4075; 0.6010], I2 = 14.9% [95% CI: 0.0%; 87.0%]. There were 55 reported deaths for a perioperative mortality rate of 2.5%. The relative risk for mortality in the midline group versus the lateral approach group did not indicate any substantial difference in survival according to laterality of approach (-0.93 [95% CI: -1.03, -0.97], I2 = 95%, (p < 0.001).
CONCLUSIONS: Overall, these results indicate good 5-year survival outcomes for patients with skull base chordoma; however, 10-year prognosis for skull base chordoma remains poor due to its radiotherapeutic resistance and high recurrence rate. Furthermore, mortality rates among patients undergoing midline versus lateral skull base approaches appear to be equivocal.
METHODS: Accordingly, we performed a systematic review in order to profile surgical and survival outcomes for skull base chordoma. Fixed and random-effect meta-analyses were performed for categorical variables including GTR, STR, 5-year OS, 10-year OS, 5-year PFS, and 10-year PFS. Additionally, we pooled eligible studies for formal meta-analysis to compare outcomes by surgical approach (lateral versus midline). Statistical analyses were performed using R Studio \'metafor\' package or Cochrane Review Manager. Furthermore, meta-analysis of pooled mortality rates and sub-analyses of operative margin and surgical complications were used to compare midline versus lateral approaches via the Mantel-Haenszel method. We considered all p-values < 0.05 to be statistically significant.
RESULTS: Following the systematic search and screen, 55 studies published between 1993 and 2022 reporting data for 2453 patients remained eligible for analysis. Sex distribution was comparable between males and females, with a slight predominance of male-identifying patients (0.5625 [95% CI: 0.5418; 0.3909]). Average age at diagnosis was 42.4 ± 12.5 years, while average age of treatment initiation was 43.0 ± 10.6 years. Overall, I2 value indicated notable heterogeneity across the 55 studies [I2 = 56.3% (95%CI: 44.0%; 65.9%)]. With respect to operative margins, the rate of GTR was 0.3323 [95% CI: 0.2824; 0.3909], I2 = 91.9% [95% CI: 90.2%; 93.4%], while the rate of STR was significantly higher at 0.5167 [95% CI: 0.4596; 0.5808], I2 = 93.1% [95% CI: 91.6%; 94.4%]. The most common complication was CSF leak (5.4%). In terms of survival outcomes, 5-year OS rate was 0.7113 [95% CI: 0.6685; 0.7568], I2 = 91.9% [95% CI: 90.0%; 93.5%]. 10-year OS rate was 0.4957 [95% CI: 0.4230; 0.5809], I2 = 92.3% [95% CI: 89.2%; 94.4%], which was comparable to the 5-year PFS rate of 0.5054 [95% CI: 0.4394; 0.5813], I2 = 84.2% [95% CI: 77.6%; 88.8%] and 10-yr PFS rate of 0.4949 [95% CI: 0.4075; 0.6010], I2 = 14.9% [95% CI: 0.0%; 87.0%]. There were 55 reported deaths for a perioperative mortality rate of 2.5%. The relative risk for mortality in the midline group versus the lateral approach group did not indicate any substantial difference in survival according to laterality of approach (-0.93 [95% CI: -1.03, -0.97], I2 = 95%, (p < 0.001).
CONCLUSIONS: Overall, these results indicate good 5-year survival outcomes for patients with skull base chordoma; however, 10-year prognosis for skull base chordoma remains poor due to its radiotherapeutic resistance and high recurrence rate. Furthermore, mortality rates among patients undergoing midline versus lateral skull base approaches appear to be equivocal.
摘要：
背景：尽管它们的行为分类不稳定（组织病理学上的良性和低度，但行为恶性），在改善颅底脊索瘤患者的预后和治疗模式方面取得了长足的进步。关于手术技术，传统上使用外侧经颅(TC)方法，然而，内镜经鼻内镜入路（EEA）已被提倡用于中线病变。尽管如此，由于这种病理的罕见性（占所有颅内肿瘤的0.2％），文献中的调查仍然限于小型回顾性系列。此外，迄今为止研究的放射治疗已被证明在很大程度上是无效的。
方法：因此，我们进行了系统评价,以描述颅底脊索瘤的手术和生存结局.对分类变量进行了固定和随机效应荟萃分析，包括GTR，STR,5年操作系统,十年操作系统,5年PFS,10年PFS此外,我们汇集了符合条件的研究进行正式荟萃分析,以比较手术入路(侧位和中线)的结局.使用RStudio\'metafor\'软件包或CochraneReviewManager进行统计分析。此外，通过Mantel-Haenszel方法,对合并死亡率进行荟萃分析,并对手术切缘和手术并发症进行亚分析,以比较中线入路和外侧入路.我们认为所有P值<0.05具有统计学意义。
结果：经过系统的搜索和筛选，1993年至2022年间发表的55项研究报告了2453名患者的数据，仍有资格进行分析。男女性别分布相当，男性识别患者略有优势(0.5625[95%CI:0.5418;0.3909])。诊断时的平均年龄为42.4±12.5岁，而开始治疗的平均年龄为43.0±10.6岁。总的来说,I2值表明55项研究中存在显著异质性[I2=56.3%(95CI:44.0%;65.9%)]。关于手术裕度，GTR率为0.3323[95%CI：0.2824；0.3909]，I2=91.9%[95%CI：90.2%；93.4%]，而STR的比率显著高于0.5167[95%CI：0.4596;0.5808]，I2=93.1%[95%CI：91.6%；94.4%]。最常见的并发症是脑脊液漏（5.4%）。就生存结果而言，5年OS率为0.7113[95%CI：0.6685；0.7568]，I2=91.9%[95%CI：90.0%；93.5%]。10年OS率为0.4957[95%CI：0.4230；0.5809]，I2=92.3%[95%CI：89.2%；94.4%]，与5年PFS率0.5054[95%CI：0.4394；0.5813]相当，I2=84.2%[95%CI：77.6%;88.8%]，10年PFS率为0.4949[95%CI：0.4075;0.6010]，I2=14.9%[95%CI：0.0%；87.0%]。有55例报告死亡，围手术期死亡率为2.5%。中线组相对于侧方入路组的死亡率相对风险未表明根据入路侧方的生存率有任何实质性差异(-0.93[95%CI:-1.03,-0.97],I2=95%，（p<0.001）。
结论：总体而言，这些结果表明颅底脊索瘤患者的5年生存结局良好;然而,颅底脊索瘤的10年预后仍然很差，由于其放疗耐药性和高复发率。此外，接受中线和外侧颅底入路的患者的死亡率似乎是模棱两可的.
方法：因此，我们进行了系统评价,以描述颅底脊索瘤的手术和生存结局.对分类变量进行了固定和随机效应荟萃分析，包括GTR，STR,5年操作系统,十年操作系统,5年PFS,10年PFS此外,我们汇集了符合条件的研究进行正式荟萃分析,以比较手术入路(侧位和中线)的结局.使用RStudio\'metafor\'软件包或CochraneReviewManager进行统计分析。此外，通过Mantel-Haenszel方法,对合并死亡率进行荟萃分析,并对手术切缘和手术并发症进行亚分析,以比较中线入路和外侧入路.我们认为所有P值<0.05具有统计学意义。
结果：经过系统的搜索和筛选，1993年至2022年间发表的55项研究报告了2453名患者的数据，仍有资格进行分析。男女性别分布相当，男性识别患者略有优势(0.5625[95%CI:0.5418;0.3909])。诊断时的平均年龄为42.4±12.5岁，而开始治疗的平均年龄为43.0±10.6岁。总的来说,I2值表明55项研究中存在显著异质性[I2=56.3%(95CI:44.0%;65.9%)]。关于手术裕度，GTR率为0.3323[95%CI：0.2824；0.3909]，I2=91.9%[95%CI：90.2%；93.4%]，而STR的比率显著高于0.5167[95%CI：0.4596;0.5808]，I2=93.1%[95%CI：91.6%；94.4%]。最常见的并发症是脑脊液漏（5.4%）。就生存结果而言，5年OS率为0.7113[95%CI：0.6685；0.7568]，I2=91.9%[95%CI：90.0%；93.5%]。10年OS率为0.4957[95%CI：0.4230；0.5809]，I2=92.3%[95%CI：89.2%；94.4%]，与5年PFS率0.5054[95%CI：0.4394；0.5813]相当，I2=84.2%[95%CI：77.6%;88.8%]，10年PFS率为0.4949[95%CI：0.4075;0.6010]，I2=14.9%[95%CI：0.0%；87.0%]。有55例报告死亡，围手术期死亡率为2.5%。中线组相对于侧方入路组的死亡率相对风险未表明根据入路侧方的生存率有任何实质性差异(-0.93[95%CI:-1.03,-0.97],I2=95%，（p<0.001）。
结论：总体而言，这些结果表明颅底脊索瘤患者的5年生存结局良好;然而,颅底脊索瘤的10年预后仍然很差，由于其放疗耐药性和高复发率。此外，接受中线和外侧颅底入路的患者的死亡率似乎是模棱两可的.
