Shu Hui-Kuo G, Sall Walter F, Maity Amit, Tochner Zelig A, Janss Anna J, Belasco Jean B, Rorke-Adams Lucy B, Phillips Peter C, Sutton Leslie N, Fisher Michael J
Department of Radiation Oncology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 30322, USA.
Cancer. 2007 Jul 15;110(2):432-41. doi: 10.1002/cncr.22782.
Because few large studies of pediatric ependymoma treatment are available, the authors believed that a retrospective review of treatment outcomes from a single institution would yield potentially valuable information regarding potential prognostic factors. In this article, they report their 20-year institutional experience with this disease.
Medical records were reviews of patients with intracranial ependymoma who received their initial treatment at the Children's Hospital of Philadelphia (CHOP)/Hospital of the University of Pennsylvania (HUP) between January 1980 and December 2000. Of the 61 patients who were identified, 49 patients underwent primary therapy at CHOP/HUP and formed the basis for the study. Actuarial overall survival (OS) and progression-free survival (PFS) were determined by the Kaplan-Meier method. Univariate and multivariate analyses were performed using the log-rank test and Cox proportional-hazards models.
With median follow-up of 110.2 months, the 5-year OS and PFS rates were 66.2% and 40.7%, respectively. Older age and higher radiation dose significantly predicted for improved OS. Anaplastic histology predicted for decreased PFS. Cervical spinal cord extension resulted in decreased OS primarily caused by failures outside the primary site. Patients who had a favorable prognosis (aged >/=3 years, no dissemination or cord extension, complete resection, and radiation dose >/=54 grays [Gy]) had 5-year OS and PFS rates of 83.1% and 60.6%, respectively.
In this study of patients with pediatric intracranial ependymoma, OS and PFS rates were concordant with the rates published in other modern series. The finding of a dose response up to 54 Gy supported the current trend toward dose escalation. Tumor extension to the cervical spine was identified as a predictor for failure outside of the primary site. Although the survival rates were encouraging, there is still significant room for improvement in the management of this disease.
由于关于小儿室管膜瘤治疗的大型研究较少，作者认为对单一机构的治疗结果进行回顾性分析将产生有关潜在预后因素的潜在有价值信息。在本文中，他们报告了该机构20年来对这种疾病的治疗经验。
回顾了1980年1月至2000年12月在费城儿童医院（CHOP）/宾夕法尼亚大学医院（HUP）接受初始治疗的颅内室管膜瘤患者的病历。在确定的61例患者中，49例在CHOP/HUP接受了初始治疗，并构成了本研究的基础。采用Kaplan-Meier法确定精算总生存率（OS）和无进展生存率（PFS）。使用对数秩检验和Cox比例风险模型进行单变量和多变量分析。
中位随访110.2个月，5年OS率和PFS率分别为66.2%和40.7%。年龄较大和放疗剂量较高显著预测OS改善。间变组织学预测PFS降低。颈髓延伸导致OS降低，主要是由原发部位以外的失败引起的。预后良好（年龄≥3岁，无播散或脊髓延伸，完全切除，放疗剂量≥54格雷[Gy]）的患者5年OS率和PFS率分别为83.1%和60.6%。
在这项小儿颅内室管膜瘤患者的研究中，OS和PFS率与其他现代系列报道的率一致。高达54 Gy的剂量反应的发现支持了当前剂量递增的趋势。肿瘤延伸至颈椎被确定为原发部位以外失败的预测因素。尽管生存率令人鼓舞，但这种疾病的管理仍有很大的改进空间。
