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低级别少突胶质细胞瘤:一种进展缓慢但无法治愈的疾病?临床文章。

Low-grade oligodendroglioma: an indolent but incurable disease? Clinical article.

作者信息

El-Hateer Hamdy, Souhami Luis, Roberge David, Maestro Rolando Del, Leblanc Richard, Eldebawy Eman, Muanza Thierry, Melançon Denis, Kavan Petr, Guiot Marie-Christine

机构信息

Department of Radiation Oncology, McGill University Health Center, Montreal, Quebec, Canada.

出版信息

J Neurosurg. 2009 Aug;111(2):265-71. doi: 10.3171/2008.11.JNS08983.

Abstract

OBJECT

The authors reviewed their institutional experience with pure low-grade oligodendroglioma (LGO), correlating outcomes with several variables of possible prognostic values.

METHODS

Sixty-nine patients with WHO-classified LGOs were treated between 1992 and 2006 at the McGill University Health Center. Clinical, pathological, and radiological records were carefully reviewed. Demographic characteristics; the nature and duration of presenting symptoms; baseline neurological function; extent of resection; Karnofsky Performance Scale score; preoperative radiological findings including tumor size, location, and absence/presence of enhancement; and pathological data including chromosome arms 1p/19q codeletion and O-methylguanine-DNA methyltransferase promoter gene methylation status were all compiled. The timing and dose of radio- and/or chemotherapy, date of tumor progression, pathological finding at disease progression, treatment at time of disease progression, and status at the last follow-up were also recorded.

RESULTS

The median follow-up period was 6.1 years (range 1.3-16.3 years). The majority (78%) of patients presented with seizures; contrast enhancement was initially seen in 16 patients (25%). All patients had undergone an initial surgical procedure: gross-total resection in 27%, partial resection in 59%, and biopsy only in the remaining 13%. Fifteen patients received adjuvant radiotherapy. Data on O-methylguanine-DNA methyltransferase promoter gene methylation status was available in 47 patients (68%) and in all but 1 patient for 1p/19q status. Survival at 5, 10, and 15 years was 83, 63, and 29%, respectively. Multivariate analysis showed that seizures at presentation and the absence of contrast enhancement were the only independent favorable prognostic factors for survival. The 5-, 10-, and 15-year progression-free survival rates were 46, 7.7, and 0%, respectively.

CONCLUSIONS

This retrospective review confirms the indolent but progressively fatal nature of LGOs. Contrast enhancement was the most evident single prognostic factor. New treatment strategies are clearly needed in the management of this disease.

摘要

目的

作者回顾了其所在机构治疗纯低级别少突胶质细胞瘤(LGO)的经验,将治疗结果与几个可能具有预后价值的变量相关联。

方法

1992年至2006年间,麦吉尔大学健康中心共治疗了69例世界卫生组织分类的LGO患者。仔细回顾了临床、病理和放射学记录。收集了人口统计学特征;首发症状的性质和持续时间;基线神经功能;切除范围;卡诺夫斯基功能状态评分;术前放射学检查结果,包括肿瘤大小、位置以及有无强化;以及病理数据,包括染色体臂1p/19q共缺失和O-甲基鸟嘌呤-DNA甲基转移酶启动子基因甲基化状态。还记录了放疗和/或化疗的时间和剂量、肿瘤进展日期、疾病进展时的病理检查结果、疾病进展时的治疗情况以及最后一次随访时的状态。

结果

中位随访期为6.1年(范围1.3 - 16.3年)。大多数患者(78%)以癫痫发作起病;16例患者(25%)最初可见强化。所有患者均接受了初次手术:27%为全切除,59%为部分切除,其余13%仅行活检。15例患者接受了辅助放疗。47例患者(68%)有O-甲基鸟嘌呤-DNA甲基转移酶启动子基因甲基化状态的数据,除1例患者外,其余患者均有1p/19q状态的数据。5年、10年和15年生存率分别为83%、63%和29%。多因素分析显示,首发癫痫发作和无强化是仅有的独立的生存有利预后因素。5年、10年和15年无进展生存率分别为为46%、7.7%和0%。

结论

这项回顾性研究证实了LGO生长缓慢但逐渐致命的特性。强化是最明显的单一预后因素。显然需要新的治疗策略来管理这种疾病。

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