Kirkman Matthew A, Hayward Richard, Phipps Kim, Aquilina Kristian
J Neurosurg Pediatr. 2018 Jun;21(6):563-573. doi: 10.3171/2018.1.PEDS17456. Epub 2018 Apr 6.
OBJECTIVE It is relatively unusual for pediatric CNS tumors to be disseminated at presentation, and the literature on the clinical features, management, and outcomes of this specific group is scarce. Surgical management in this population is often challenging, particularly in the presence of hydrocephalus. The authors present their recent experience of treating pediatric CNS tumors that were disseminated at presentation, and they compare these lesions with focal tumors. METHODS The authors performed a retrospective review of prospectively collected data on children presenting to a tertiary center between 2003 and 2016 inclusive. RESULTS Of 361 children with CNS tumors, the authors identified 53 patients with disease dissemination at presentation (male/female ratio 34:19, median age 3.8 years, age range 7 days to 15.6 years) and 308 without dissemination at presentation (male/female ratio 161:147, median age 5.8 years, age range 1 day to 16.9 years). Five tumor groups were studied: medulloblastoma (disseminated n = 29, focal n = 74), other primitive neuroectodermal tumor (n = 8, n = 17), atypical teratoid rhabdoid tumor (n = 8, n = 22), pilocytic astrocytoma (n = 6, n = 138), and ependymoma (n = 2, n = 57). The median follow-up duration in survivors was not significantly different between those with disease dissemination at presentation (64.0 months, range 5.2-152.0 months) and those without it (74.5 months, range 4.7-170.1 months) (p > 0.05). When combining data from all 5 tumor groups, dissemination status at presentation was significantly associated with a higher risk of requiring CSF diversion, a higher surgical complication rate, and a reduced likelihood of achieving gross-total resection of the targeted lesion (all variables p < 0.05). Differences between the 5 tumor groups were evident. No factors that predicted the need for permanent CSF diversion following temporary external ventricular drainage were identified on multivariate analysis, and there was no clear superiority of either ventriculoperitoneal shunt surgery or endoscopic third ventriculostomy as a permanent CSF diversion procedure. CONCLUSIONS Tumor type and dissemination status at initial presentation significantly affect outcomes across a range of measures. The management of hydrocephalus in patients with CNS tumors is challenging, and further prospective studies are required to identify the optimal CSF diversion strategy in this population.
小儿中枢神经系统肿瘤在初诊时出现播散相对少见，关于这一特定群体的临床特征、治疗及预后的文献较少。该人群的手术治疗通常具有挑战性，尤其是在存在脑积水的情况下。作者介绍了他们近期治疗初诊时已播散的小儿中枢神经系统肿瘤的经验，并将这些病变与局灶性肿瘤进行比较。方法：作者对2003年至2016年期间在一家三级中心就诊的儿童的前瞻性收集数据进行了回顾性分析。结果：在361例中枢神经系统肿瘤患儿中，作者确定了53例初诊时疾病已播散的患者（男/女比例为34:19，中位年龄3.8岁，年龄范围7天至15.6岁）和308例初诊时未播散的患者（男/女比例为161:147，中位年龄5.8岁，年龄范围1天至16.9岁）。研究了五个肿瘤组：髓母细胞瘤（播散型n = 29，局灶型n = 74）、其他原始神经外胚层肿瘤（n = 8，n = 17）、非典型畸胎样横纹肌样肿瘤（n = 8，n = 22）、毛细胞型星形细胞瘤（n = 6，n = 138）和室管膜瘤（n = 2，n = 57）。初诊时疾病已播散的幸存者的中位随访时间（64.0个月，范围5.2 - 152.0个月）与未播散的幸存者（74.5个月，范围4.7 - 170.1个月）之间无显著差异（p > 0.05）。综合所有五个肿瘤组的数据时，初诊时的播散状态与需要脑脊液分流的较高风险、较高的手术并发症发生率以及实现目标病变全切的可能性降低显著相关（所有变量p < 0.05）。五个肿瘤组之间的差异明显。多因素分析未发现预测临时脑室外引流后需要永久性脑脊液分流的因素，并且在作为永久性脑脊液分流手术的脑室腹腔分流术和内镜下第三脑室造瘘术之间没有明显的优势。结论：肿瘤类型和初诊时的播散状态在一系列指标上显著影响预后。中枢神经系统肿瘤患者的脑积水管理具有挑战性，需要进一步的前瞻性研究来确定该人群中最佳的脑脊液分流策略。
