El-Hajj Victor Gabriel, Ranganathan Sruthi, Rajjoub Rami, Ghaith Abdul Karim, Theodore Nicholas, Elmi-Terander Adrian, Lubelski Daniel
Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Department of Medicine, University of Cambridge, Cambridge, UK.
J Neurooncol. 2025 Apr;172(2):397-405. doi: 10.1007/s11060-024-04921-x. Epub 2025 Jan 2.
Spinal chordomas are aggressive tumors that rarely occur in the pediatric population. Demographics and post-treatment outcomes in this select group of patients is poorly studied. We hence aimed to analyze the clinical characteristics, demographics, and survival outcomes of pediatric patients with spinal chordomas, in contrast to the adult population. To address this, the literature was reviewed to evaluate the coverage on spinal chordomas of the pediatric population, and the National Cancer Database (NCDB) was analyzed to provide insights into the US experience over the past two decades.
A search of the literature was performed leveraging the MEDLINE and Web of Science electronic databases from inception until March 2024, using the keywords "spinal," "chordoma," and "pediatric". Additionally, the NCDB was queried for pediatric patients (≤ 21 years) with chordoma treated between 2004 and 2017. Baseline characteristics, tumor specifics, treatment details, and survival outcomes were collected and analyzed.
From the literature, 45 pediatric chordoma patients were identified, with a median age of 7 years. Most chordomas were in the cervical spine (40%), and 93% of the patients received surgical treatment. Gross total resection was achieved in 59% of cases, and 49% received adjuvant radiotherapy. Recurrence, metastasis, and mortality rates were 7%, 18%, and 24%, respectively at a median follow-up of 12 months. In the NCDB cohort, 53 pediatric patients (≤ 21 years) and 980 adults (> 21 years) were compared. Despite having smaller tumors in size, pediatric patients presented with more advanced tumors with a higher proportion of stage 4 tumors. They had more mobile spine chordomas (83% vs. 51%) and traveled further for treatment (57 vs. 27 miles). Pediatric patients also received higher radiation doses (5420 vs. 5049 cGy). Surgical resection and adjuvant radiotherapy were common treatments in both groups. After matching, outcomes, including survival rates and early mortality, were similar between age groups. Kaplan-Meier analysis showed no difference in overall survival probabilities between the age groups both prior to and after matching.
While pediatric patients with spinal chordomas present with more advanced stage tumors, they demonstrate similar overall survival outcomes when compared to adults. The current literature is mainly composed of single cases and other reports of low evidence levels.
脊柱脊索瘤是侵袭性肿瘤，在儿科人群中很少见。对这一特定患者群体的人口统计学和治疗后结果研究较少。因此，我们旨在分析小儿脊柱脊索瘤患者的临床特征、人口统计学和生存结果，并与成人进行对比。为解决此问题，我们回顾了文献以评估儿科人群脊柱脊索瘤的研究情况，并分析了国家癌症数据库（NCDB），以了解美国过去二十年的相关经验。
利用MEDLINE和科学网电子数据库，从建库至2024年3月进行文献检索，关键词为“脊柱”“脊索瘤”和“儿科”。此外，查询NCDB中2004年至2017年期间接受治疗的儿科（≤21岁）脊索瘤患者。收集并分析基线特征、肿瘤细节、治疗细节和生存结果。
从文献中确定了45例儿科脊索瘤患者，中位年龄为7岁。大多数脊索瘤位于颈椎（40%），93%的患者接受了手术治疗。59%的病例实现了大体全切，49%的患者接受了辅助放疗。中位随访12个月时，复发率、转移率和死亡率分别为7%、18%和24%。在NCDB队列中，对53例儿科患者（≤21岁）和980例成人（>21岁）进行了比较。尽管儿科患者肿瘤较小，但呈现出更晚期的肿瘤，IV期肿瘤比例更高。他们的脊柱脊索瘤活动度更高（83%对51%），就医距离更远（57英里对27英里）。儿科患者接受的辐射剂量也更高（5420 cGy对5049 cGy）。手术切除和辅助放疗是两组的常见治疗方法。匹配后，各年龄组的生存结果，包括生存率和早期死亡率相似。Kaplan-Meier分析显示，匹配前后各年龄组的总生存概率无差异。
虽然小儿脊柱脊索瘤患者呈现出更晚期的肿瘤，但与成人相比，他们的总生存结果相似。目前的文献主要由单病例和其他低证据水平的报告组成。
