10154-PEDT-6 RETROSPECTIVE STUDY OF 12 PEDIATRIC EPENDYMOMAS TREATED AT OUR HOSPITAL

Yoshiki Shiba, J. Yamaguchi, Yuhei Takido, Daiki Shimizu, Y. Kibe, Sachi Maeda, Tomohide Nishikawa, F. Ohka, K. Motomura, R. Saito
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Abstract

Abstract Ependymoma is an important disease in pediatric brain tumors, but reports with a large number of cases are scarce. In this study, we investigated the clinical course of 12 cases of ependymoma in children treated at our hospital.The median age was 2.5 years (0-15 years), and 10 had posterior fossa and 2 had supratentorial onset. Surgery was performed in all cases, and total resection was achieved in 9 cases (75%). Five cases were diagnosed as ependymoma, Grade II, and seven cases were diagnosed as anaplastic ependymoma, Grade III, according to the WHO revised 4th edition. Adjuvant therapy was administered in 8 patients, 6 with radiotherapy and 2 with combination chemotherapy and radiotherapy. Recurrence was seen in 6 cases, 4 of which were local recurrences and 2 of which were dissemination. The 2-year and 5-year progression-free survival rates were both 60%, and the 2-year and 5-year survival rates were 100% and 66.6%. Progression-free survival/overall survival analysis was performed, and the above results were considered to be equivalent to the published data of 27 cases (Peralia et al. Cell 2022). Progression-free survival tended to be favorable in patients with complete resection (HR = 0.18, 95% CI: 0.025-1.28) and patients with supratentorial disease (HR = 0.18, 95% CI: 0.030-1.11). No difference was seen by WHO grade (HR = 1.022, 95% CI: 0.20-5.24). Our cohort also yielded the same results regarding prognostic factors as previously reported, and WHO grade did not correlate with prognosis. The WHO 5th edition classification adopts molecular diagnosis and further subclassifies ependymoma. Since molecular diagnostics require advanced analytical techniques, there is a problem that it is difficult to put them into practical use, but surrogate markers that can be applied clinically have also been reported. This time, we will reclassify pediatric ependymoma using surrogate markers and compare it with the old classification.
10154-pedt-6 对在我院接受治疗的 12 例小儿脑外胚瘤的回顾性研究
室管膜瘤是小儿脑肿瘤中的重要疾病,但病例较多的报道很少。在这项研究中,我们调查了12例在我院治疗的儿童室管膜瘤的临床过程。中位年龄为2.5岁(0-15岁),10例后颅窝发病,2例幕上发病。所有病例均行手术治疗,其中9例(75%)完全切除。根据WHO第4版修订本,5例诊断为ⅱ级室管膜瘤,7例诊断为间变性室管膜瘤ⅲ级。辅助治疗8例,其中放疗6例,放化疗联合治疗2例。复发6例,局部复发4例,播散性2例。2年和5年无进展生存率均为60%,2年和5年生存率分别为100%和66.6%。进行无进展生存期/总生存期分析,认为上述结果与27例(Peralia等)已发表的数据相当。2022细胞)。完全切除患者(HR = 0.18, 95% CI: 0.025-1.28)和幕上疾病患者(HR = 0.18, 95% CI: 0.030-1.11)的无进展生存期倾向于有利。WHO分级无差异(HR = 1.022, 95% CI: 0.20-5.24)。我们的队列也得出了与先前报道的预后因素相同的结果,并且WHO分级与预后无关。WHO第5版分类采用分子诊断,进一步对室管膜瘤进行亚分类。由于分子诊断需要先进的分析技术,存在难以实际应用的问题,但也有可用于临床的替代标记物的报道。这一次,我们将使用替代标记物对儿科室管膜瘤进行重新分类,并与旧的分类进行比较。
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