From the Radiology (S.V., T.Y.P.), Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts
DF/HCC Tumor Imaging Metrics Core (D.B.), Massachusetts General Hospital, Boston, Massachusetts.
AJNR Am J Neuroradiol. 2020 Apr;41(4):718-724. doi: 10.3174/ajnr.A6499. Epub 2020 Apr 2.
Diffuse intrinsic pontine glioma is a lethal childhood brain cancer with dismal prognosis and MR imaging is the primary methodology used for diagnosis and monitoring. Our aim was to determine whether advanced diffusion, perfusion, and permeability MR imaging metrics predict survival and pseudoprogression in children with newly diagnosed diffuse intrinsic pontine glioma.
A clinical trial using the poly (adenosine diphosphate ribose) polymerase (PARP) inhibitor veliparib concurrently with radiation therapy, followed by maintenance therapy with veliparib + temozolomide, in children with diffuse intrinsic pontine glioma was conducted by the Pediatric Brain Tumor Consortium. Standard MR imaging, DWI, dynamic contrast-enhanced perfusion, and DSC perfusion were performed at baseline and approximately every 2 months throughout treatment. ADC histogram metrics of T2-weighted FLAIR and enhancing tumor volume, dynamic contrast-enhanced permeability metrics for enhancing tumors, and tumor relative CBV from DSC perfusion MR imaging were calculated. Baseline values, post-radiation therapy changes, and longitudinal trends for all metrics were evaluated for associations with survival and pseudoprogression.
Fifty children were evaluable for survival analyses. Higher baseline relative CBV was associated with shorter progression-free survival (= .02, = 0.089) and overall survival (= .006, = 0.055). Associations of higher baseline mean transfer constant from the blood plasma into the extravascular extracellular space with shorter progression-free survival (= .03, = 0.105) and overall survival (= .03, = 0.102) trended toward significance. An increase in relative CBV with time was associated with shorter progression-free survival (< .001, < 0.001) and overall survival (= .004, = 0.043). Associations of longitudinal mean extravascular extracellular volume fraction with progression-free survival (= .03, = 0.104) and overall survival (= .03, = 0.105) and maximum transfer constant from the blood plasma into the extravascular extracellular space with progression-free survival (= .03, = 0.102) trended toward significance. Greater increases with time were associated with worse outcomes. True radiologic progression showed greater post-radiation therapy decreases in mode_ADC_FLAIR compared with pseudoprogression (means, -268.15 versus -26.11, = .01.) CONCLUSIONS: ADC histogram, perfusion, and permeability MR imaging metrics in diffuse intrinsic pontine glioma are useful in predicting survival and pseudoprogression.
弥漫性内在脑桥神经胶质瘤是一种致命的儿童脑癌，预后不良，磁共振成像（MRI）是诊断和监测的主要方法。我们的目的是确定新诊断的弥漫性内在脑桥神经胶质瘤患儿中，高级弥散、灌注和通透性 MRI 指标是否可预测生存和假性进展。
由儿科脑肿瘤联盟开展了一项临床试验，使用多聚（腺苷二磷酸核糖）聚合酶（PARP）抑制剂维利帕尼联合放射治疗，然后使用维利帕尼联合替莫唑胺进行维持治疗，入组对象为弥漫性内在脑桥神经胶质瘤患儿。在基线时以及整个治疗过程中，大约每 2 个月进行一次标准 MRI、弥散加权成像（DWI）、动态对比增强灌注和 DSC 灌注。计算 T2 加权 FLAIR 上的 ADC 直方图指标（肿瘤体积强化、动态对比增强通透性指标、肿瘤 DSC 灌注 MR 成像的相对脑血容量（CBV）。评估所有指标的基线值、放射治疗后变化和纵向趋势与生存和假性进展的相关性。
50 例患儿可进行生存分析。较高的基线相对 CBV 与较短的无进展生存期（= 0.02，= 0.089）和总生存期（= 0.006，= 0.055）相关。较高的基线血血浆向血管外细胞外空间的平均转移常数与较短的无进展生存期（= 0.03，= 0.105）和总生存期（= 0.03，= 0.102）相关，但无统计学意义。随着时间的推移，相对 CBV 的增加与较短的无进展生存期（< 0.001，< 0.001）和总生存期（= 0.004，= 0.043）相关。血管外细胞外容积分数的纵向平均变化与无进展生存期（= 0.03，= 0.104）和总生存期（= 0.03，= 0.105）和血血浆向血管外细胞外空间的最大转移常数与无进展生存期（= 0.03，= 0.102）相关，但无统计学意义。随时间增加的变化与较差的预后相关。真性放射学进展与假性进展相比，在放射治疗后模式 ADC_FLAIR 上显示出更大的降低（均值，-268.15 比-26.11，= 0.01）。
弥漫性内在脑桥神经胶质瘤的 ADC 直方图、灌注和通透性 MRI 指标可用于预测生存和假性进展。
