Purpose

Pediatric primary high-grade spinal glioma (p-HGSG) is an extremely rare disease process, with little data within the current literature. Akin to primary high-grade gliomas, this cancer has been exemplified by dismal prognosis and poor response to modern treatment paradigms. This study seeks to investigate the current trends affecting overall survival using the National Cancer Database (NCDB).

Methods

The NCDB was queried for p-HGSG between 2004 and 2016, by utilizing the designated diagnosis codes. Kaplan-Meier curves were generated, and log-rank testing was performed to analyze factors affecting overall survival. In addition, a Cox proportional-hazards model was used to perform multivariate regression analysis of survival outcomes.

Results

A cohort of 97 patients was identified with a histologically confirmed p-HGSG. The overall incidence of p-HGSG in all pediatric spinal cord tumors is 7.5%, with a mean survival time of 25.3 months (SD, 21.0) and 5-year overall survival of 17.0%. The majority of patients underwent surgery (n = 87, 89.7%), radiotherapy (n = 73, 75.3%), and chemotherapy (n = 60, 61.9%). Univariate, multivariate, and Kaplan-Meier log-rank testing failed to demonstrate an association between performing surgery, extent of resection, radiotherapy, or chemotherapy with improved survival outcomes.

Conclusions

The current study constitutes the largest retrospective analysis of p-HGSGs to date, finding that current treatment options of surgery, radiotherapy, and chemotherapy have unclear benefit. This disease process has a poor prognosis without a current modality of treatment that conclusively alters survival. The risks and side effects of these treatment modalities must be carefully considered in such a highly aggressive disease process, especially given potentially limited survival benefits.

中文翻译：

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儿科原发性高级脊柱神经胶质瘤：美国国家癌症数据库对当前治疗方式和预后的分析。

目的

小儿原发性高级别脊柱神经胶质瘤（p-HGSG）是一种极为罕见的疾病过程，目前的文献资料很少。类似于原发性高级神经胶质瘤，这种癌症的预后令人沮丧，并且对现代治疗模式的反应较差。本研究旨在使用国家癌症数据库（NCDB）调查影响总体生存的当前趋势。

方法

通过使用指定的诊断代码，在2004年至2016年期间向NCDB查询了p-HGSG。产生Kaplan-Meier曲线，并进行对数秩检验以分析影响总体存活的因素。此外，使用Cox比例风险模型对生存结果进行多元回归分析。

结果

经组织学确认的p-HGSG鉴定出了97名患者。在所有小儿脊髓肿瘤中，p-HGSG的总发生率为7.5％，平均生存时间为25.3个月（SD，21.0），五年总生存率为17.0％。大多数患者接受了手术（n  = 87，89.7％），放疗（n  = 73，75.3％）和化学疗法（n  = 60，61.9％）。单变量，多变量和Kaplan-Meier对数秩检验未能证明进行手术，切除范围，放疗或化疗与改善生存结果之间的关联。

结论

当前的研究构成了迄今为止对p-HGSGs的最大的回顾性分析，发现目前的手术，放疗和化学治疗方案尚无明显获益。如果没有目前能最终改变生存率的治疗方法，该疾病的预后就很差。在这种高度侵袭性的疾病过程中，必须谨慎考虑这些治疗方式的风险和副作用，特别是考虑到可能有限的生存收益。