Cerebellar location may predict an unfavourable prognosis in paediatric high-grade glioma.
Cerebellar location may predict an unfavourable prognosis in paediatric high-grade glioma.
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DOI：
10.1038/bjc.2013.404
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发表时间：
2013-08-20
影响因子：
8.8
通讯作者：
Kramm CM
中科院分区：
文献类型：
--
作者：
Karremann M;Rausche U;Roth D;Kühn A;Pietsch T;Gielen GH;Warmuth-Metz M;Kortmann RD;Straeter R;Gnekow A;Wolff JE;Kramm CM
小脑高级胶质瘤（HGG）仅占儿科HGG的5%。由于对这些肿瘤知之甚少，本研究旨在进一步表征它们。从HIT-GBM/HIT-HGG数据库中确定了29例小脑性HGG患儿。对180例皮质性HGG患儿的临床和流行病学资料进行比较。小脑肿瘤患者更年轻（中位年龄为7.6岁vs 11.7岁，P=0.028），但两组在性别、肿瘤易感综合征、继发HGG、原发性转移、肿瘤分级、肿瘤切除程度、化疗方案或放疗方面没有显著差异。除了在小脑亚群中间变性毛细胞星形细胞瘤（APA）的发生率增加（20.7% vs 3.3% P<0.001）外，两组的组织学实体分布相似。正如预期的那样，肿瘤分级与生存有预后相关性。与皮质HGG相比，小脑部位的总生存期明显较差（中位总生存期：0.92±0.02年vs 2.03±0.32年；P=0.0064）， cox回归分析显示，小脑部位的肿瘤具有独立的不良预后意义（P=0.019）。高级别胶质瘤是一组具有明显部位特异性异质性的肿瘤，其在小脑部位的生存率较差。
High-grade glioma (HGG) of the cerebellum accounts for only 5% of paediatric HGG. Since little is known about these tumours, the present study aimed at their further characterisation. Twenty-nine paediatric patients with centrally reviewed cerebellar HGG were identified from the HIT-GBM/HIT-HGG database. Clinical and epidemiological data were compared with those of 180 paediatric patients with cortical HGG. Patients with cerebellar tumours were younger (median age of 7.6 vs 11.7 years, P=0.028), but both groups did not differ significantly with regard to gender, tumour predisposing syndromes, secondary HGG, primary metastasis, tumour grading, extent of tumour resection, chemotherapy regimen, or radiotherapy. Except for an increased incidence of anaplastic pilocytic astrocytoma (APA) in the cerebellar subset (20.7% vs 3.3% P<0.001), histological entities were similarly distributed in both groups. As expected, tumour grading had a prognostic relevance on survival. Compared with cortical HGG, overall survival in the cerebellar location was significantly worse (median overall survival: 0.92±0.02 vs 2.03±0.32 years; P=0.0064), and tumour location in the cerebellum had an independent poor prognostic significance as shown by Cox-regression analysis (P=0.019). High-grade glioma represents a group of tumours with an obviously site-specific heterogeneity associated with a worse survival in cerebellar location.
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影响因子：
0.4
作者：
Kramm, C.;Rausche, U.;van Gool, S.
通讯作者：
van Gool, S.
影响因子：
45.3
作者：
FINLAY, JL;BOYETT, JM;PACKER, RJ
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PACKER, RJ
影响因子：
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作者：
TOMLINSON, FH;SCHEITHAUER, BW;JACK, CR
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JACK, CR
影响因子：
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作者：
Ganigi, PM;Santosh, V;Kolluri, VRS
通讯作者：
Kolluri, VRS
影响因子：
4.8
作者：
Fernandez, C;Figarella-Branger, D;Lena, G
通讯作者：
Lena, G