Department of Paediatric and Adolescent Medicine, Universitätsmedizin Mannheim, Medical Faculty Mannheim, Heidelberg University, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany.
Br J Cancer. 2013 Aug 20;109(4):844-51. doi: 10.1038/bjc.2013.404. Epub 2013 Jul 18.
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.
小脑高级别胶质瘤(HGG)仅占儿童 HGG 的 5%。由于对这些肿瘤知之甚少,本研究旨在进一步对其进行表征。
从 HIT-GBM/HIT-HGG 数据库中确定了 29 名经中心审查的小脑 HGG 患儿。将临床和流行病学数据与 180 名皮质 HGG 患儿的数据进行比较。
小脑肿瘤患儿年龄更小(中位数年龄为 7.6 岁 vs 11.7 岁,P=0.028),但两组在性别、肿瘤易患综合征、继发性 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无显著差异。除小脑亚组中间变毛细胞型星形细胞瘤(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,肿瘤分级对生存有预后意义。与皮质 HGG 相比,小脑部位的总生存率明显更差(中位总生存率:0.92 ± 0.02 岁 vs 2.03 ± 0.32 岁;P=0.0064),并且肿瘤位置在小脑是通过 Cox 回归分析显示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,与小脑部位的生存明显更差相关。