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Recent genomic and epigenomic analyses have pointed out the heterogeneity of tumors from a same histopathological group and have identified key oncogenic alterations that enabled the description of novel tumor entities. Thus, pediatric high-grade gliomas (HGG) comprise a heterogeneous group of tumors, including H3 K27M-mutant, H3 G34-mutant, IDH-mutant and H3/IDH-wildtype HGG. Furthermore, the H3/IDH-wildtype HGG group has recently been divided into three molecular entities based on their DNA methylation profile: Receptor tyrosine kinase type I (RTK I) and II (RTKI I), andMYCN-amplified, the latter representing the most frequent subgroup (41% of cases, 36/87) [4]. However, the current 2016 WHO classification does not discriminate between them. In addition, data on these entities came from large series collectively deciphering molecular landscape of HGG. Therefore, the HGG-MYCN subgroup remains poorly characterized and clinical, imaging and pathological data are scarce (Supplementary Table S1). We investigated data from five pediatric supratentorial HGG-MYCN diagnosed by DNA methylation profiling at our institution (one case included in [7]) and we pooled them with methylation class pediatric HGG-MYCN of the literature (n = 59) [4, 5, 7]. Therefore, we analyzed clinical, histopathological and molecular data of pediatric supratentorial HGG-MYCN and compared them to their pontine counterparts [9] and did a systematic review of four groups of supratentorial pediatric HGG (including 62 H3 K27M-mutant gliomas, 31 H3-G34 mutant gliomas, 44 HGG-RTKI and 16 HGG-RTKII) [1–8, 10]. Clinical data of our cases are summarized in Table S2. The median age of pediatric HGG-MYCN (published cases and our own) was 9.0 years (range from 2 to 18) which was lower than H3 K27M (11.0 years) and H3 G34-mutant (13.0 years), RTKI (10.0 years) and RTKII subgroups (10.0 years) [1–8, 10]. This difference was only significant between HGGMYCN and H3-G34 mutant gliomas (p < 0.001) [4, 5, 7, 8]. The sex ratio male/female for HGG-MYCN was 1.3 and 3.4, 0.6, 1.2 and 1.3 respectively for H3 G34-mutant gliomas, H3 K27M-mutant gliomas, HGG-RTKI and RTK2 (but without significant difference) [1–8, 10]. HGG-MYCN were mostly located in the hemispheres (31/37 cases with available data, 83.8%), but 5 (13.5%) were thalamic and one arose from the sellar area [4, 5, 7]. There was a slight predilection for temporal lobes (16/37 cases, 43.2%), which was significantly higher than in other subgroups (p < 0.001). By imaging, no calcifications were observed and only one tumor was hemorrhagic (Case 5). They were wellcircumscribed with meningeal attachment (except for thalamic tumors). They appeared as solid hypercellular masses with a restricted apparent diffusion coefficient (ADC) in the main part of the tumors. They displayed slight peri-lesional edema and homogeneous enhancement after contrast injection (Fig. 1). These imaging

acta neuropathologica communications

The pediatric supratentorial MYCN-amplified high-grade gliomas methylation class presents the same radiological, histopathological and molecular features as their pontine counterparts