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原发性胶质肉瘤患者的现代系列的临床特征和结局。

Clinical characteristics and outcomes for a modern series of primary gliosarcoma patients.

机构信息

Department of Neurological Surgery, University of California, San Francisco, CA, USA.

出版信息

Cancer. 2010 Mar 1;116(5):1358-66. doi: 10.1002/cncr.24857.

Abstract

BACKGROUND

Primary gliosarcoma (PGS) is a rare central nervous system tumor with limited experience reported in the literature. In the current study, the authors present a modern series of confirmed PGS cases treated in the era of magnetic resonance imaging (MRI), after the accepted glioblastoma management of resection, radiation, and temozolomide.

METHODS

Using a retrospective review, patients with confirmed PGS were identified (1996-2008). Cases were determined to be PGS by central pathology review using the 2007 World Health Organization criteria. Extensive chart review was performed to gather clinical and pathologic data on these cases.

RESULTS

All but 1 patient had undergone a preoperative MRI, with 1 patient receiving a computed tomography scan due to a cardiac pacemaker. A total of 10 patients received radiotherapy with concurrent and adjuvant temozolomide chemotherapy, and 8 patients received radiotherapy alone or in combination with other chemotherapeutic agents. In 2 patients, the history of adjuvant treatment could not be confirmed. The overall median survival was 13.9 months (range, 2.2-22.9 months). Patients with gliosarcomas resembling meningioma were found to have a significantly prolonged median survival compared with patients harboring gliosarcoma resembling glioblastoma multiforme (16 months vs 9.6 months; P = .011). However, no difference in survival was noted between patients who received concurrent radiotherapy and temozolomide compared with those who did not (10.4 months vs 13.9 months; P = .946).

CONCLUSIONS

The results of the current study support previous hypotheses that there are 2 distinct types of PGS. The type mimicking the appearance of a meningioma appears to carry a significantly more favorable prognosis, most likely due to an increased chance at achieving macroscopic total resection.

摘要

背景

原发性神经胶质瘤肉瘤(PGS)是一种罕见的中枢神经系统肿瘤,文献报道的经验有限。在目前的研究中,作者介绍了一组在磁共振成像(MRI)时代接受治疗的确诊 PGS 病例,这些病例接受了与胶质母细胞瘤一致的治疗方法,包括手术切除、放疗和替莫唑胺化疗。

方法

通过回顾性研究,确定了经中心病理复查确诊为 PGS 的患者(1996-2008 年)。使用 2007 年世界卫生组织标准,通过中心病理学复查确定病例为 PGS。对这些病例进行了广泛的病历回顾,以收集临床和病理数据。

结果

除 1 例患者外,所有患者均接受了术前 MRI 检查,其中 1 例因心脏起搏器而接受了 CT 扫描。共 10 例患者接受了放疗联合替莫唑胺化疗,8 例患者仅接受了放疗或联合其他化疗药物治疗。有 2 例患者的辅助治疗史无法确认。总的中位生存期为 13.9 个月(范围,2.2-22.9 个月)。具有脑膜瘤样外观的神经胶质瘤肉瘤患者的中位生存期明显长于具有胶质母细胞瘤多形性样外观的患者(16 个月比 9.6 个月;P =.011)。然而,接受同步放疗和替莫唑胺化疗的患者与未接受该治疗的患者的生存无差异(10.4 个月比 13.9 个月;P =.946)。

结论

本研究的结果支持先前的假设,即存在 2 种不同类型的 PGS。类似于脑膜瘤的类型似乎具有更有利的预后,这很可能是由于实现大体全切除的机会增加。

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