Purpose

Less than 5% of chordomas occur in pediatric patients. While many studies have explored the treatment and outcomes of skull base chordomas, few have focused on the differences between pediatric and adult populations. The aim of this study is to analyze the epidemiological variables and clinical outcomes between pediatric and adult skull base chordomas using a large-sample, population-based cancer database.

Methods

The National Cancer Database was queried between 2004 and 2015 for skull base chordomas. We stratified patients as pediatric (<18 years) and adults (≥18 years). We compared several clinical covariates between the two groups.

Results

Our cohort consisted of 658 patients, 61 pediatric (9.3%), and 597 adults (90.7%). Pediatric patients were more likely to have larger tumor size (41.4 ± 15.7 mm versus 34.1 ± 15.8 mm, p < 0.01) and universally treated at academic facilities. There was no significant difference in overall survival.

Conclusions

Pediatric skull base chordomas are rare tumors that are managed with aggressive surgical resection, followed by radiation. While there may be difference between tumor presentation, outcomes between pediatric and adult patients are similar.

中文翻译：

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儿童与成人颅底脊索瘤的特征和总生存期：一项基于人群的研究

目的

不到 5% 的脊索瘤发生在儿科患者中。虽然许多研究探索了颅底脊索瘤的治疗和结果，但很少有人关注儿童和成人人群之间的差异。本研究的目的是使用基于人群的大样本癌症数据库分析儿童和成人颅底脊索瘤之间的流行病学变量和临床结果。

方法

2004 年至 2015 年间，在国家癌症数据库中查询了颅底脊索瘤。我们将患者分为儿童（<18 岁）和成人（≥18 岁）。我们比较了两组之间的几个临床协变量。

结果

我们的队列由 658 名患者、61 名儿科 (9.3%) 和 597 名成人 (90.7%) 组成。儿科患者更有可能具有更大的肿瘤尺寸（41.4 ± 15.7 mm 与 34.1 ± 15.8 mm，p  < 0.01）并且在学术机构普遍接受治疗。总生存期没有显着差异。

结论

小儿颅底脊索瘤是一种罕见的肿瘤，通过积极的手术切除和放射治疗。虽然肿瘤表现之间可能存在差异，但儿童和成人患者之间的结果相似。