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原发性颅内 gliosarcoma 的临床、影像学和免疫组化特征及转归:一项回顾性单中心研究。

The clinical, radiological, and immunohistochemical characteristics and outcomes of primary intracranial gliosarcoma: a retrospective single-centre study.

机构信息

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, No. 119 South 4th Ring West Road, Fengtai District, Beijing, 100070, China.

China National Clinical Research Centre for Neurological Diseases, Beijing, China.

出版信息

Neurosurg Rev. 2021 Apr;44(2):1003-1015. doi: 10.1007/s10143-020-01285-4. Epub 2020 Mar 18.

Abstract

Primary intracranial gliosarcoma is a rare malignant brain tumour, and the most effective treatment for gliosarcoma remains unclear. This study aimed to identify risk factors for progression-free survival (PFS) and overall survival (OS) in these cases. This retrospective single-centre study evaluated 103 patients (median age, 51 years; 67 men [65%]) with primary intracranial gliosarcoma between 2006 and 2017. Treatments included surgery (GTR, 63 patients; STR, 39 patients; biopsy, 1 patient), radiotherapy (adjuvant, 76 patients; exclusive treatment, 1 patient), and chemotherapy (adjuvant temozolomide, 52 patients; adjuvant nimustine/teniposide, 19 patients; adjuvant bevacizumab, 1 patient; exclusive nimustine/teniposide treatment, 1 patient). The median OS was 13.3 months, and the median PFS was 9.1 months. In the multivariate analyses, the poor prognostic factors were ependymal lining enhancement of the lateral ventricle (PFS, HR 2.406, p = 0.005; OS, HR 2.946, p = 0.009) and enhancement in the motor functional cortex (PFS, HR 2.892, p = 0.002; OS, HR 2.639, p = 0.009). Good OS was predicted by adjuvant radiotherapy alone (HR 0.071, p < 0.001), adjuvant temozolomide-based chemotherapy alone (HR 0.063, p = 0.005), adjuvant temozolomide-based chemotherapy with concurrent radiotherapy (HR 0.056, p < 0.001), and salvage surgery at recurrence (HR 0.449, p = 0.031). The present study revealed that, in patients with primary intracranial gliosarcoma, enhancement in the functional motor cortex and ependymal lining enhancement of the lateral ventricle were both poor prognostic factors. Survival was optimized in cases treated using maximal safe resection followed by adjuvant temozolomide-based chemotherapy with concurrent radiotherapy. Furthermore, salvage surgery provided meaningful therapeutic benefits for recurrent gliosarcoma.

摘要

原发性颅内胶质肉瘤是一种罕见的恶性脑瘤,胶质肉瘤的最有效治疗方法仍不清楚。本研究旨在确定这些病例无进展生存期(PFS)和总生存期(OS)的预后因素。这项回顾性单中心研究评估了 2006 年至 2017 年间 103 例原发性颅内胶质肉瘤患者(中位年龄 51 岁;67 名男性[65%])。治疗包括手术(最大限度肿瘤切除术[GTR],63 例;次最大限度肿瘤切除术[STR],39 例;活检,1 例)、放疗(辅助放疗,76 例;单纯放疗,1 例)和化疗(辅助替莫唑胺,52 例;辅助尼莫司汀/依托泊苷,19 例;辅助贝伐单抗,1 例;单纯尼莫司汀/依托泊苷治疗,1 例)。中位 OS 为 13.3 个月,中位 PFS 为 9.1 个月。在多变量分析中,预后不良的因素包括侧脑室室管膜衬里增强(PFS:风险比[HR]2.406,p=0.005;OS:HR 2.946,p=0.009)和运动功能皮层增强(PFS:HR 2.892,p=0.002;OS:HR 2.639,p=0.009)。单独辅助放疗(HR 0.071,p<0.001)、单独替莫唑胺为基础的辅助化疗(HR 0.063,p=0.005)、替莫唑胺为基础的辅助化疗联合放疗(HR 0.056,p<0.001)和复发时的挽救性手术(HR 0.449,p=0.031)良好的 OS 预测因素。本研究表明,在原发性颅内胶质肉瘤患者中,功能运动皮层增强和侧脑室室管膜衬里增强均为不良预后因素。最大安全切除后,联合替莫唑胺为基础的化疗和放疗的辅助治疗优化了生存。此外,挽救性手术为复发性胶质肉瘤提供了有意义的治疗益处。

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