Background: To assess anatomical and quantitative diffusion-weighted MR imaging features in a recently classified lethal neoplasm, H3 K27M histone-mutant diffuse midline glioma [World Health Organization (WHO) IV].Methods: Fifteen untreated gliomas in teenagers and adults (median age 19, range, 14-64) with confirmed H3 K27M histone-mutant genotype were analysed at a national referral centre. Morphological characteristics including tumour epicentre(s), T2/FLAIR and Gadolinium enhancement patterns, calcification, haemorrhage and cyst formation were recorded. Multiple apparent diffusion coefficient (ADC min , ADC mean ) regions of interest were sited in solid tumour and normal appearing white matter (ADC NAWM ) using post-processing software (Olea Sphere v2.3, Olea Medical). ADC histogram data (2 nd , 5 th , 10 th percentile, median, mean, kurtosis, skewness) were calculated from volumetric tumour segmentations and tested against the regions of interest (ROI) data (Wilcoxon signed rank test).
Results:The median interval from imaging to tissue diagnosis was 9 (range, 0-74) days. The structural MR imaging findings varied between individuals and within tumours, often featuring signal heterogeneity on all MR sequences. All gliomas demonstrated contact with the brain midline, and 67% exhibited rimenhancing necrosis. The mean ROI ADC min value was 0.84 (±0.15 standard deviation, SD) ×10 −3 mm 2 /s. In the largest tumour cross-section (excluding necrosis), an average ADC mean value of 1.12 (±0.25)×10 −3 mm 2 /s was observed. The mean ADC min/NAWM ratio was 1.097 (±0.149), and the mean ADC mean/NAWM ratio measured 1.466 (±0.299). With the exception of the 2 nd centile, no statistical difference was observed between the regional and histogram derived ADC results.Conclusions: H3 K27M-mutant gliomas demonstrate variable morphology and diffusivity, commonly featuring moderately low ADC values in solid tumour. Regional ADC measurements appeared representative of volumetric histogram data in this study.