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儿童室管膜瘤的预后因素和治疗选择。

Prognostic factors and treatment options for paediatric ependymomas.

机构信息

Department of Radiation Oncology, The Prince of Wales Cancer Centre, Level 2, High Street, Randwick, New South Wales 2031, Australi.

出版信息

J Clin Neurosci. 2012 Sep;19(9):1228-35. doi: 10.1016/j.jocn.2012.02.006. Epub 2012 Jul 25.

Abstract

The aim of this study was to determine factors of prognostic relevance for paediatric ependymomas, and evaluate the efficacy of treatment modalities. This is a retrospective study of 43 patients with ependymoma (<18 years) who underwent a combination of surgical excision, chemotherapy, and/or radiotherapy treatment at The Prince of Wales Cancer Centre between 1969 and 2009. Statistical analysis was performed to assess the prognostic relevance of various parameters affecting the two-year and five-year overall survival (OS) and progression-free survival (PFS). The five-year OS and PFS were 50.3% and 44.8% respectively (median follow-up 50 months). Eighteen patients (41.9%) experienced tumour recurrence: 13 had a local recurrence (LR) and five had both LR and distant recurrence. On univariate analysis, a more favourable prognosis in terms of both OS and PFS was evident for supratentorial tumours compared to infratentorial tumours (OS p=0.007, PFS p=0.045), stereotactic radiosurgery/ fractionated stereotactic radiotherapy compared to craniospinal irradiation or local posterior fossa/local brain±boost radiotherapy modalities (OS p=0.047, PFS p=0.031), total radiotherapy dose >50 Gy compared to ≤50 Gy (OS p=0.008, PFS p=0.005), and in patients with no tumour recurrence compared to those with recurrence (OS p=0.03, PFS p<0.001). Although not statistically significant, a more favourable multivariate outcome was evident in patients who underwent complete surgical resection. Chemotherapy treatment and histopathological grade, however, were not relevant to prognosis. This study supports the need to pursue more aggressive treatment for infratentorial and/or recurrent tumours. Ideal treatment involves maximal surgical resection, followed by adjuvant radiotherapy (>50 Gy).

摘要

本研究旨在确定小儿室管膜瘤的预后相关因素,并评估治疗方式的疗效。这是一项回顾性研究,纳入了 1969 年至 2009 年期间在 The Prince of Wales 癌症中心接受手术切除、化疗和/或放疗联合治疗的 43 例室管膜瘤(<18 岁)患者。统计分析用于评估影响两年和五年总生存率(OS)和无进展生存率(PFS)的各种参数的预后相关性。五年 OS 和 PFS 分别为 50.3%和 44.8%(中位随访时间为 50 个月)。18 例患者(41.9%)发生肿瘤复发:13 例为局部复发(LR),5 例为局部和远处复发。单因素分析显示,与幕下肿瘤相比,幕上肿瘤在 OS 和 PFS 方面具有更有利的预后(OS p=0.007,PFS p=0.045),立体定向放射外科/分次立体定向放射治疗与颅脊髓照射或局部后颅窝/局部脑+加量放疗方式相比(OS p=0.047,PFS p=0.031),总放疗剂量>50Gy 与≤50Gy 相比(OS p=0.008,PFS p=0.005),以及无肿瘤复发患者与复发患者相比(OS p=0.03,PFS p<0.001)。尽管无统计学意义,但完全手术切除患者的多因素预后更有利。然而,化疗治疗和组织病理学分级与预后无关。本研究支持对幕下和/或复发性肿瘤采取更积极的治疗。理想的治疗方法是最大限度地进行手术切除,然后进行辅助放疗(>50Gy)。

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