Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, 101 Daehangno, Jongno-gu, Seoul, 110-744, Republic of Korea.
J Neurooncol. 2012 Feb;106(3):619-26. doi: 10.1007/s11060-011-0699-x. Epub 2011 Aug 24.
Pediatric infratentorial ependymomas are difficult to cure. Despite the availability of advanced therapeutic modalities for brain tumors, total surgical resection remains the most important prognostic factor. Recently, histological grade emerged as an independent prognostic factor for intracranial ependymoma. We retrospectively reviewed the treatment outcome of 33 pediatric patients with infratentorial ependymoma. Progression-free survival (PFS) and overall survival (OS) rates were calculated and relevant prognostic factors were analyzed. Fourteen patients (42%) were under the age of 3 at diagnosis. Gross total resection was achieved in 16 patients (49%). Anaplastic histology was found in 13 patients (39%). Adjuvant therapies were delayed until progression in 12 patients (36%). Actuarial PFS rates were 64% in the first year and 29% in the fifth year. Actuarial OS rates were 91% in the first year and 71% in the fifth year. On univariate analysis, brainstem invasion (P = 0.047), anaplastic histology (P = 0.004), higher mitotic count (P = 0.001), and higher Ki-67 index (P = 0.004) were significantly related to a shorter PFS. Gross total resection (P = 0.029) and a greater age at diagnosis (P = 0.033) were significantly related to a longer PFS. On multivariate analysis, anaplastic histology alone was significantly related to a shorter PFS (P = 0.023). Gross total resection (P = 0.039) was significantly related to a longer overall survival (OS) on multivariate analysis. Anaplastic histology and gross total resection were the most important clinical factors affecting PFS and OS, respectively. Anaplastic histology, mitotic count, and Ki-67 index can be used as universal and easily available prognostic parameters in infratentorial ependymomas.
儿童小脑幕下室管膜瘤难以治愈。尽管有多种治疗脑肿瘤的先进方法，但完全手术切除仍然是最重要的预后因素。最近，组织学分级已成为颅内室管膜瘤的独立预后因素。我们回顾性分析了 33 例儿童小脑幕下室管膜瘤患者的治疗结果。计算了无进展生存期（PFS）和总生存期（OS），并分析了相关的预后因素。14 例患者（42%）在诊断时年龄小于 3 岁。16 例患者（49%）实现了大体全切除。13 例患者（39%）为间变组织学。12 例患者（36%）在进展后才开始辅助治疗。第 1 年和第 5 年的估计 PFS 率分别为 64%和 29%。第 1 年和第 5 年的估计 OS 率分别为 91%和 71%。单因素分析显示，脑干侵犯（P=0.047）、间变组织学（P=0.004）、较高的有丝分裂计数（P=0.001）和较高的 Ki-67 指数（P=0.004）与较短的 PFS 显著相关。大体全切除（P=0.029）和较大的诊断年龄（P=0.033）与较长的 PFS 显著相关。多因素分析显示，仅间变组织学与较短的 PFS 显著相关（P=0.023）。多因素分析显示，大体全切除（P=0.039）与总生存时间（OS）显著相关。间变组织学和大体全切除是影响 PFS 和 OS 的最重要的临床因素。间变组织学、有丝分裂计数和 Ki-67 指数可作为小脑幕下室管膜瘤普遍且易于获得的预后参数。
