Luksik Andrew S, Garzon-Muvdi Tomas, Yang Wuyang, Huang Judy, Jallo George I
Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland; and.
Institute for Brain Protection Sciences, Johns Hopkins All Children's Hospital, St. Petersburg, Florida.
J Neurosurg Pediatr. 2017 Jun;19(6):711-719. doi: 10.3171/2017.1.PEDS16528. Epub 2017 Mar 31.
OBJECTIVE Intramedullary spinal cord tumors comprise 1%-10% of all childhood central nervous system neoplasms, with astrocytomas representing the most common subtype. Due to their rarity and poor prognosis, large population-based studies are needed to assess the epidemiology and survival risk factors associated with these tumors in the hope of improving outcome. The authors undertook this retrospective study to explore factors that may influence survival in pediatric patients with spinal cord astrocytomas. METHODS Utilizing the Surveillance, Epidemiology, and End Results (SEER) database, a prospective cancer registry, the authors retrospectively assessed survival in histologically confirmed, primary spinal cord astrocytomas in patients 21 years of age and younger. Survival was described with Kaplan-Meyer curves, and a multivariate regression analysis was used to assess the association of several variables with survival while controlling for confounding variables. RESULTS This analysis of 348 cases showed that age (hazard ratio [HR] 1.05, 95% CI 1.01-1.09, p = 0.017), nonwhite race (HR 1.74, 95% CI 1.11-2.74, p = 0.014), high-grade tumor status (HR 14.67, 95% CI 6.69-32.14, p < 0.001), distant or invasive extension of the tumor (HR 2.37, 95% CI 1.02-5.49, p = 0.046), and radiation therapy (HR 3.74, 95% CI 2.18-6.41, p < 0.001) were associated with decreased survival. Partial resection (HR 0.37, 95% CI 0.16-0.83, p = 0.017) and gross-total resection (HR 0.39, 95% CI 0.16-0.95, p = 0.039) were associated with improved survival. CONCLUSIONS Younger age appears to be protective, while high-grade tumors have a much worse prognosis. Early diagnosis and access to surgery appears necessary for improving outcomes, while radiation therapy has an unclear role. There is still much to learn about this disease in the hope of curing children with the misfortune of having one of these rare tumors.
目的 脊髓髓内肿瘤占儿童中枢神经系统肿瘤的1% - 10%,其中星形细胞瘤是最常见的亚型。由于其罕见性及预后较差,需要开展基于大人群的研究来评估这些肿瘤的流行病学特征及生存风险因素,以期改善治疗结果。作者进行了这项回顾性研究,以探索可能影响小儿脊髓星形细胞瘤患者生存的因素。方法 作者利用监测、流行病学和最终结果(SEER)数据库(一个前瞻性癌症登记系统),回顾性评估21岁及以下组织学确诊的原发性脊髓星形细胞瘤患者的生存情况。采用Kaplan - Meyer曲线描述生存情况,并使用多变量回归分析在控制混杂变量的同时评估多个变量与生存的相关性。结果 对348例病例的分析表明,年龄(风险比[HR] 1.05,95%置信区间[CI] 1.01 - 1.09,p = 0.017)、非白人种族(HR 1.74,95% CI 1.11 - 2.74,p = 0.014)、高级别肿瘤状态(HR 14.67,95% CI 6.69 - 32.14,p < 0.001)、肿瘤远处或浸润性扩展(HR 2.37,95% CI 1.02 - 5.49,p = 0.046)以及放射治疗(HR 3.74,95% CI 2.18 - 6.41,p < 0.001)与生存率降低相关。部分切除(HR 0.37,95% CI 0.16 - 0.83,p = 0.017)和全切除(HR 0.39,95% CI 0.16 - 0.95,p = 0.039)与生存率提高相关。结论 较年轻的年龄似乎具有保护作用,而高级别肿瘤的预后要差得多。早期诊断和获得手术治疗似乎对改善治疗结果很有必要,而放射治疗的作用尚不清楚。对于治愈患有这些罕见肿瘤之一的儿童,仍有许多需要了解的地方。