High-grade glioma, IDH- and H3-wildtype in young adults: a rare condition with a distinct epigenetic landscape.

IF 3.1 2区医学 Q2 CLINICAL NEUROLOGY

Journal of Neuro-Oncology Pub Date : 2025-10-16 DOI:10.1007/s11060-025-05246-z

Alexandre Roux, Arnault Tauziede-Espariat, Giorgia Antonia Simboli, Angela Elia, Alessandro Moiraghi, Benoit Hudelist, Gonzague Defrance, Alexandre Gehanno, Edouard Dezamis, Thomas Blauwblomme, Volodia Dangouloff-Ros, Nathalie Boddaert, Christelle Dufour, Jacques Grill, Jun Muto, Alice Metais, Raphael Saffroy, Pascale Varlet, Fabrice Chretien, Catherine Oppenheim, Marc Zanello, Johan Pallud

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引用次数: 0

Abstract

Purpose: High-grade glioma, IDH- and H3-wildtype in young adults is a rare and poorly known entity. We compared newly diagnosed cases in young adults (18-39 years) to those in adult patients (> 39 years).

Methods: We performed an observational, retrospective, single-centre cohort study at a tertiary neurosurgical oncology centre between January 2006 and December 2023.

Results: We included 1.139 adult patients with a newly diagnosed high-grade glioma, IDH- and H3-wildtype. Young adults: (1) represent a small proportion of patients with high-grade glioma (n = 33, 2.9%); (2) have a high rate of unclassified cases based on epigenetics (n = 5, 15.2%); (3) have a longer progression-free survival (p = 0.003) and overall survival (p = 0.001) and; (4) do not have higher surgically-related adverse event rates (p = 0.198). Concerning young adults, surgical resection was associated with improved progression-free and overall survival (p < 0.001 and p < 0.001, respectively). The DNA-methylation class significantly impacts the overall survival (p = 0.028), however, the MGMT methylation status is not significantly associated with either progression-free or overall survival (p = 0.320 and p = 0.639, respectively).

Conclusion: High-grade glioma, IDH- and H3-wildtype is a rare histo-molecular subtype in young adults with a better prognosis than older adults. In young adults, DNA-methylation subtypes are different from their adult counterpart and had a significant impact on survival unlike MGMT status. Given the rarity in young adults, a dedicated management in specialized neurosurgical oncology centres is preferred. Further molecular and epigenetic analyses are required to understand the differences in prognosis compared to adult patients.

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年轻人中高级别胶质瘤，IDH-和h3野生型：一种具有独特表观遗传景观的罕见疾病。

目的：青年人中高级别胶质瘤，IDH-和h3野生型是一种罕见且鲜为人知的实体。我们比较了年轻成人（18-39岁）和成年患者（18-39岁）的新诊断病例。方法：我们于2006年1月至2023年12月在一家三级神经外科肿瘤中心进行了一项观察性、回顾性、单中心队列研究。结果：我们纳入了1.139例新诊断的高级别胶质瘤，IDH-和h3 -野生型。年轻人：(1)占高级别胶质瘤患者的一小部分（n = 33, 2.9%）；(2)基于表观遗传学的未分类病例率高（n = 5, 15.2%）；(3)无进展生存期（p = 0.003）和总生存期（p = 0.001）更长；(4)没有较高的手术相关不良事件发生率（p = 0.198）。对于年轻人，手术切除与改善无进展和总生存率相关(p结论：高级别胶质瘤，IDH-和h3 -野生型是年轻人中罕见的组织分子亚型，其预后优于老年人。在年轻人中，dna甲基化亚型与成人不同，与MGMT状态不同，对生存率有显著影响。鉴于罕见的年轻人，一个专门的管理在专门的神经外科肿瘤中心是首选。需要进一步的分子和表观遗传学分析来了解与成年患者相比预后的差异。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Neuro-Oncology 医学-临床神经学

CiteScore

6.60

自引率

7.70%

发文量

277

审稿时长

3.3 months

期刊介绍： The Journal of Neuro-Oncology is a multi-disciplinary journal encompassing basic, applied, and clinical investigations in all research areas as they relate to cancer and the central nervous system. It provides a single forum for communication among neurologists, neurosurgeons, radiotherapists, medical oncologists, neuropathologists, neurodiagnosticians, and laboratory-based oncologists conducting relevant research. The Journal of Neuro-Oncology does not seek to isolate the field, but rather to focus the efforts of many disciplines in one publication through a format which pulls together these diverse interests. More than any other field of oncology, cancer of the central nervous system requires multi-disciplinary approaches. To alleviate having to scan dozens of journals of cell biology, pathology, laboratory and clinical endeavours, JNO is a periodical in which current, high-quality, relevant research in all aspects of neuro-oncology may be found.