Abtract

Background

This study describes imaging features of diffuse intrinsic pontine glioma (DIPG) and correlates with overall survival (OS) and histone mutation status in the International DIPG Registry (IDIPGR).

Methods

Four hundred cases submitted to the IDIPGR with a local diagnosis of DIPG and baseline MRI were evaluated by consensus review of 2 neuroradiologists; 43 cases were excluded (inadequate imaging or alternative diagnoses). Agreement between reviewers, association with histone status, and univariable and multivariable analyses relative to OS were assessed.

Results

On univariable analysis imaging features significantly associated with worse OS included: extrapontine extension, larger size, enhancement, necrosis, diffusion restriction, and distant disease. On central review, 9.5% of patients were considered not to have DIPG. There was moderate mean agreement of MRI features between reviewers. On multivariable analysis, chemotherapy, age, and distant disease were predictors of OS. There was no difference in OS between wild-type and H3 mutated cases. The only imaging feature associated with histone status was the presence of ill-defined signal infiltrating pontine fibers.

Conclusions

Baseline imaging features are assessed in the IDIPGR. There was a 9.5% discordance in DIPG diagnosis between local and central review, demonstrating need for central imaging confirmation for prospective trials. Although several imaging features were significantly associated with OS (univariable), only age and distant disease were significant on multivariable analyses. There was limited association of imaging features with histone mutation status, although numbers are small and evaluation exploratory.

中文翻译：

---

弥漫性内源性桥骨胶质瘤（DIPG）的MR成像特征及其与总体生存率的关系：国际DIPG注册中心的报告。

抽象

背景

这项研究描述了弥漫性桥脑神经胶质瘤（DIPG）的影像学特征，并与国际DIPG注册中心（IDIPGR）中的总体生存率（OS）和组蛋白突变状态相关。

方法

通过两名神经放射科医生的共识评估，评估了400例提交给IDIPGR且局部诊断为DIPG和基线MRI的病例。排除了43例（影像学或其他诊断不足）。评价者之间的一致性，与组蛋白状态的关联以及相对于OS的单变量和多变量分析被评估。

结果

在单变量分析中，与OS恶化显着相关的影像学特征包括：桥外延伸，更大的尺寸，增强，坏死，扩散受限和远处的疾病。在中央检查中，有9.5％的患者被认为没有DIPG。审阅者之间的MRI特征存在中等程度的平均一致性。在多变量分析中，化学疗法，年龄和远处疾病是OS的预测指标。野生型和H3突变病例之间的OS没有差异。与组蛋白状态有关的唯一影像学特征是存在信号模糊的桥脑纤维浸润。

结论

基线成像功能在IDIPGR中进行评估。在局部和中央评价之间，DIPG的诊断差异为9.5％，这表明前瞻性试验需要中央影像学确认。尽管多种影像学特征与OS（单变量）显着相关，但在多变量分析中只有年龄和远处的疾病显着。影像学特征与组蛋白突变状态之间的关联有限，尽管数量很少且评估探索性。