1Department of Neurology and Neurosurgery, Universidade Federal de São Paulo; and.
Divisions of2Neurosurgery.
J Neurosurg Pediatr. 2020 Dec 11;27(2):160-169. doi: 10.3171/2020.7.PEDS20389. Print 2021 Feb 1.
Tumors of the CNS are the main causes of childhood cancer and have an incidence that exceeds that of leukemia. In addition, they are the leading causes of cancer-related death in childhood. High-grade gliomas account for 11% of such neoplasms and are characterized by aggressive clinical behavior and high morbidity and mortality. There is a lack of studies focusing on the factors that can prolong survival in these patients or guide therapeutic interventions. The authors aimed to investigate the factors related to longer survival durations, with a focus on reoperation for gross-total resection (GTR).
In this retrospective cohort study, the authors analyzed 78 patients diagnosed with high-grade gliomas occurring across all CNS locations except diffuse intrinsic pontine gliomas. Patients 0 to < 19 years of age were followed up at the Pediatric Oncology Institute. Overall survival (OS) and progression-free survival (PFS) were analyzed in the context of various prognostic factors, such as age, sex, histology, extent of tumor resection, reoperation for GTR, adjuvant treatment, and treatment initiation from 2010 onward.
With a mean age at diagnosis of 8.7 years, 50% of the patients were female and approximately 39% underwent GTR at some point, which was already achieved in approximately 46% of them in the first surgery. The median OS was 17 months, and PFS was 10 months. In terms of median OS, the authors found no significant difference between those with reoperation for GTR and patients without GTR during treatment. Significant differences were observed in the OS in terms of the extent of resection in the first surgery, age, sex, Ki-67 expression, adjuvant treatment, and treatment initiation from 2010 onward. Furthermore, the PFS values significantly differed between those with GTR in the first surgery and Ki-67 expression ≥ 50%.
This study demonstrates the importance of GTR for these neoplasms, highlights the role of surgeons in its achievement in the first attempt, and questions the role of reoperation for this purpose. Finally, this study further supports the use of combined adjuvant treatment for the improvement of OS and PFS.
中枢神经系统肿瘤是儿童癌症的主要病因,其发病率超过白血病。此外,它们也是儿童癌症相关死亡的主要原因。高级别神经胶质瘤占此类肿瘤的 11%,其临床行为具有侵袭性,发病率和死亡率均较高。目前缺乏研究关注能够延长此类患者生存时间的因素,或指导治疗干预的因素。作者旨在研究与生存时间延长相关的因素,重点是再次手术行大体全切除(GTR)。
在这项回顾性队列研究中,作者分析了在除弥漫性内在脑桥胶质瘤外的所有中枢神经系统部位发生的高级别神经胶质瘤患者。0 至<19 岁的患者在儿科肿瘤研究所接受随访。在各种预后因素的背景下,如年龄、性别、组织学、肿瘤切除范围、再次手术行 GTR、辅助治疗以及 2010 年以后开始治疗,分析总生存期(OS)和无进展生存期(PFS)。
诊断时平均年龄为 8.7 岁,50%的患者为女性,约 39%的患者在某一时间点行 GTR,其中约 46%的患者在首次手术中即已实现 GTR。中位 OS 为 17 个月,中位 PFS 为 10 个月。在中位 OS 方面,作者发现再次手术行 GTR 的患者与治疗过程中未行 GTR 的患者之间没有显著差异。在首次手术切除范围、年龄、性别、Ki-67 表达、辅助治疗以及 2010 年以后开始治疗等方面,OS 存在显著差异。此外,在首次手术行 GTR 与 Ki-67 表达≥50%的患者中,PFS 值存在显著差异。
本研究表明 GTR 对这些肿瘤很重要,突出了外科医生在首次尝试时实现 GTR 的作用,并对再次手术的作用提出了质疑。最后,本研究进一步支持采用联合辅助治疗来提高 OS 和 PFS。