BACKGROUND The HERBY trial evaluated the benefit of the addition of the antiangiogenic agent Bevacizumab (BEV) to radiotherapy/Temozolomide (RT/TMZ) in pediatric patients with newly diagnosed non-brainstem high-grade glioma (HGG). The work presented here aims to correlate imaging characteristics and outcome measures with pathological and molecular data. METHODS Radiological, pathological and molecular data were correlated with trial clinical information to retrospectively re-evaluate event free and overall survival. RESULTS One-hundred thirteen patients were randomized to the RT/TMZ arm (n =54) or the RT/TMZ+BEV (BEV arm; n =59). The tumor arose in the cerebral hemispheres in 68 patients (Cerebral group) and a midline location in 45 cases (Midline group). Pathological diagnosis was available in all cases and molecular data in 86/113. H3 K27M histone mutations were present in 23/32 Midline cases and H3 G34R/V mutations in 7/54 Cerebral cases. Total/near-total resection occurred in 44/68 (65%) Cerebral cases but only 5/45 (11%) Midline cases (p <0.05). Leptomeningeal metastases (27 cases, 13 with subependymal spread) at relapse were more frequent in Midline (17/45) than Cerebral tumors (10/68, p <0.05). Mean OS (14.1 months) and EFS (9.0 months) in Midline tumors were significantly lower than mean OS (20.7 months) and EFS (14.9 months) in Cerebral tumors (p <0.05). Pseudoprogression occurred in 8/111 (6.2%) cases. CONCLUSIONS This study has shown that the poor outcome of midline tumors (compared to cerebral) may be related to 1) lesser surgical resection, 2) H3 K27M histone mutations, and 3) higher leptomeningeal dissemination.

中文翻译：

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HERBY II期试验对新诊断的非脑干儿科高级别神经胶质瘤的放射学评估。

背景HERBY试验评估了在新诊断为非脑干高级别神经胶质瘤（HGG）的儿科患者中，在放疗/替莫唑胺（RT / TMZ）中添加抗血管生成药贝伐单抗（BEV）的益处。本文介绍的工作旨在将影像学特征和结果测量与病理和分子数据相关联。方法将放射，病理和分子数据与临床试验信息相关联，以回顾性地重新评估无事件生存率和总生存期。结果一百三十三名患者被随机分配到RT / TMZ组（n = 54）或RT / TMZ + BEV（BEV组； n = 59）。肿瘤出现在脑半球的68例（大脑组）和中线位置的45例（中线组）。在所有情况下都可以进行病理诊断，在86/113中可以提供分子数据。在23/32中线病例中存在H3 K27M组蛋白突变，在7/54脑病例中存在H3 G34R / V突变。全切除/近全切除在44/68（65％）脑病例中发生，但仅5/45（11％）中线病例发生（p <0.05）。复发时的小脑脑转移（27例，表皮下扩散13例）在中线（17/45）比脑部肿瘤更常见（10/68，p <0.05）。中线肿瘤的平均OS（14.1个月）和EFS（9.0个月）明显低于脑肿瘤的平均OS（20.7个月）和EFS（14.9个月）（p <0.05）。伪进展发生在8/111（6.2％）的情况下。结论这项研究表明，中线肿瘤（与脑部相比）的不良结局可能与1）较少的手术切除，2）H3 K27M组蛋白突变和3）较高的软脑膜扩散有关。