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成星形细胞瘤患者的治疗模式与生存结局:152例患者的个体患者数据分析

Patterns of care and survival outcomes in patients with astroblastoma: an individual patient data analysis of 152 cases.

作者信息

Mallick Supriya, Benson Rony, Venkatesulu Bhanuprasad, Melgandi Wineeta, Rath Goura K

机构信息

Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Childs Nerv Syst. 2017 Aug;33(8):1295-1302. doi: 10.1007/s00381-017-3410-5. Epub 2017 May 5.

DOI:10.1007/s00381-017-3410-5
PMID:28477040
Abstract

BACKGROUND

Astroblastoma (AB) is a rare tumor with significant dilemma regarding diagnostic criteria, behavior, and optimum treatment.

MATERIALS AND METHODS

We searched PubMed, Google Search, and Cochrane Library for eligible studies with the following search words: astroblastoma, high-grade astroblastoma, and anaplastic astroblastoma till July 1, 2016, published in English language and collected data regarding age, sex, site of disease, pathological grade, treatment received, and survival.

RESULTS

Data of 152 patients were retrieved from 63 publications. Median age was 16 years (range 0-71). Females were affected twice more frequently than male (70.3 vs. 29.7%). Tumors were most commonly located in the frontal (39%) followed by parietal lobe (26.7%). Fifty-two and 25% of the patients had headache and seizure at presentation, 76.3% of the patients underwent a gross total resection, 41 out of 89 had a high-grade tumor, and 56 patients received adjuvant radiation with a median dose of 54 Gy (range 20-72). Adjuvant chemotherapy was used in 23 patients. Temozolomide was the most common drug used in 30% of the patients. A combination of cisplatin, etoposide with vincristine, or ifosfamide was used in 17%. Median follow-up duration was 37 months (range 1-238). Median progression-free survival and OS were 36 and 184 months, respectively. Patients with a higher-grade tumor had significantly worse OS with HR 5.260 and p = 0.001. Forty patients experienced local progression. Sixty-five percent patients underwent surgery while 50% underwent radiation as salvage.

CONCLUSION

AB has two distinct grades with higher-grade tumors having significantly poor survival. Maximal safe surgery followed by adjuvant radiation and temozolomide should be advocated for these tumors.

摘要

背景

成星形细胞瘤(AB)是一种罕见肿瘤,在诊断标准、生物学行为及最佳治疗方面存在重大难题。

材料与方法

我们在PubMed、谷歌搜索和考克兰图书馆中检索符合条件的研究,检索词如下:成星形细胞瘤、高级别成星形细胞瘤和间变性成星形细胞瘤,检索截至2016年7月1日以英文发表的研究,并收集有关年龄、性别、疾病部位、病理分级、接受的治疗及生存情况的数据。

结果

从63篇出版物中检索到152例患者的数据。中位年龄为16岁(范围0 - 71岁)。女性受影响的频率是男性的两倍多(70.3%对29.7%)。肿瘤最常见于额叶(39%),其次是顶叶(26.7%)。52%和25%的患者在就诊时出现头痛和癫痫发作,76.3%的患者接受了全切除,89例中有41例为高级别肿瘤,56例患者接受了辅助放疗,中位剂量为54 Gy(范围20 - 72)。23例患者使用了辅助化疗。替莫唑胺是30%的患者中最常用的药物。17%的患者使用顺铂、依托泊苷联合长春新碱或异环磷酰胺。中位随访时间为37个月(范围1 - 238个月)。中位无进展生存期和总生存期分别为36个月和184个月。高级别肿瘤患者的总生存期明显更差,风险比为5.260,p = 0.001。40例患者出现局部进展。65%的患者接受了手术,50%的患者接受了挽救性放疗。

结论

AB有两个不同级别,高级别肿瘤的生存率明显较差。对于这些肿瘤,应提倡最大限度的安全手术,随后进行辅助放疗和替莫唑胺治疗。

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Morphological and molecular features of astroblastoma, including BRAFV600E mutations, suggest an ontological relationship to other cortical-based gliomas of children and young adults.成星形细胞瘤的形态学和分子特征,包括BRAFV600E突变,提示其与儿童和年轻成人的其他基于皮质的胶质瘤存在本体论关系。
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