English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

BACKGROUND H3 G34-mutant high-grade gliomas occur primarily in children but can also be encountered in adults. The aim of the present study was to describe the characteristics of H3 G34-mutant high-grade gliomas in adults. MATERIAL AND METHODS We analysed the characteristics of 17 adult H3 G34-mutant high-grade gliomas and compared them with those of adult grade IV gliomas with H3 K27M mutation (n=21), IDH mutation (n=42) and TERT promoter (pTERT) mutation (n=88). RESULTS Median age at diagnosis in H3 G34-mutant gliomas was 25 years (range: 19–33 y). All tumors had a hemispheric location. The radiological presentation was suggestive of a high-grade glioma in 6 patients but in 10 patients it initially suggested another diagnosis due to absent or faint contrast enhancement (n=9) or the presence of an initial intratumoral haemorrhage (n=1). In non- or faint contrast-enhancing cases, diffusion-weighted imaging (DWI) was more helpful to suspect an aggressive tumor than MR spectroscopy and perfusion MRI. All H3 G34-mutant gliomas were classified as IDH wildtype glioblastomas. PNET-like foci were observed in 8 cases and most cases were immunonegative for ATRX (92%) and Olig2 (81%) and demonstrated MGMT promoter methylation (82%). The clinical, radiological and histological presentation of H3 G34-mutant gliomas was different from that of midline H3 K27M-mutant diffuse gliomas and from that of IDH-mutant and IDH-wildtype pTERT-mutant glioblastomas. Median overall survival of G34-mutant gliomas was 23.9 months compared to 19.6 months (p=0.16), 13.7 months (p=0.45) and 58 months (p=2.3x10-10) in H3 K27M-mutant diffuse gliomas, IDHwt pTERT-mutant and IDH-mutant glioblastomas, respectively. CONCLUSION Adults H3 G34-mutant high-grade gliomas are associated with distinct characteristics and a poor prognosis. DWI seems helpful to identify cases that present as non-contrast enhancing lesions and can wrongly suggest another diagnosis in these young patients.

OS5.5 Characteristics of high-grade gliomas with H3 G34 mutation in adults