Kukreja Sunil, Ambekar Sudheer, Sin Anthony Hunkyun, Nanda Anil
Department of Neurosurgery, Louisiana State University Health Sciences Center-Shreveport, Shreveport, Louisiana.
J Neurosurg Pediatr. 2014 Apr;13(4):400-7. doi: 10.3171/2014.1.PEDS13532. Epub 2014 Feb 14.
Reports of myxopapillary ependymomas (MPEs) of the spinal cord in pediatric patients are scarce. In the literature, various authors have shared their experiences with small groups of patients, which makes it difficult to create a consensus regarding the treatment approach for spinal MPEs in young patients. The aim of this study was to perform a survival analysis of patients in the first 2 decades of life whose cases were selected from the published studies, and to examine the influence of various factors on outcomes.
A comprehensive search of studies published in English was performed on PubMed. Patients whose age was ≤ 20 years were included for integrative analysis. Information about age, treatment characteristics, critical events (progression, recurrence, and death), time to critical events, and follow-up duration was recorded. The degree of association of the various factors with the survival outcome was calculated by using Kaplan-Meier estimator and Cox proportional hazard model techniques.
A total of 95 patients were included in the analysis. The overall rate of recurrence (RR) was 34.7% (n = 33), with a median time to recurrence of 36 months (range 2-100 months). Progression-free survival (PFS) and overall survival rates at 5 years were 73.7% and 98.9%, respectively. Addition of radiotherapy (RT) following resection significantly improved PFS (log-rank test, p = 0.008). In patients who underwent subtotal resection (STR), administering RT (STR + RT) improved outcome with the lowest failure rates (10.3%), superior to patients who underwent gross-total resection (GTR) alone (RR 43.1%; log-rank test, p < 0.001). Addition of RT to patients who underwent GTR was not beneficial (log-rank test, p = 0.628). In patients who had disseminated tumor at presentation, adjuvant RT controlled the disease effectively. High-dose RT (≥ 50 Gy) did not change PFS (log-rank test, p = 0.710).
Routine inclusion of RT in the treatment protocol for spinal MPEs in young patients should be considered. Complete resection is always the goal of tumor resection. However, when complete resection does not seem to be possible in complex lesions, RT should be used as an adjunct to avoid aggressive resection and to minimize inadvertent injury to the surrounding neural tissues. High-dose RT (≥ 50 Gy) did not provide additional survival benefits, although this association needs to be evaluated by prospective studies.
小儿脊髓黏液乳头型室管膜瘤（MPE）的报道较少。在文献中，不同作者分享了他们对一小群患者的治疗经验，这使得难以就年轻患者脊髓MPE的治疗方法达成共识。本研究的目的是对从已发表研究中选取的20岁以下患者进行生存分析，并研究各种因素对预后的影响。
在PubMed上对以英文发表的研究进行全面检索。纳入年龄≤20岁的患者进行综合分析。记录有关年龄、治疗特征、关键事件（进展、复发和死亡）、关键事件发生时间以及随访时间的信息。使用Kaplan-Meier估计器和Cox比例风险模型技术计算各种因素与生存结果的关联程度。
共有95例患者纳入分析。总体复发率（RR）为34.7%（n = 33），复发的中位时间为36个月（范围2 - 100个月）。5年无进展生存率（PFS）和总生存率分别为73.7%和98.9%。切除术后加用放疗（RT）显著改善了PFS（对数秩检验，p = 0.008）。在接受次全切除（STR）的患者中，给予RT（STR + RT）可改善预后，失败率最低（10.3%），优于单纯接受全切除（GTR）的患者（RR 43.1%；对数秩检验，p < 0.001）。对接受GTR的患者加用RT并无益处（对数秩检验，p = 0.628）。对于初诊时已有播散性肿瘤的患者，辅助性RT可有效控制疾病。高剂量RT（≥50 Gy）并未改变PFS（对数秩检验，p = 0.710）。
应考虑在年轻患者脊髓MPE的治疗方案中常规加入RT。完整切除始终是肿瘤切除的目标。然而，当复杂病变似乎无法完整切除时，应使用RT作为辅助手段，以避免激进切除并尽量减少对周围神经组织的意外损伤。高剂量RT（≥50 Gy）并未提供额外的生存益处，尽管这种关联需要通过前瞻性研究进行评估。
