Diffuse gliomas with H3F3A point mutations affecting histone H3.3 glycine position 34 are a distinct glioma subtype mostly occurring in the cerebral hemispheres of paediatric and young adult patients. These tumours have a high rate of MGMT promoter hypermethylation, a predictive biomarker for response to the DNA alkylating agent temozolomide (TMZ). Post-treatment TMZ-associated hypermutation is a mechanism for TMZ resistance in recurrent isocitrate dehydrogenase (IDH) wildtype glioblastoma and may be seen in association with tumour progression in lower-grade IDH-mutant diffuse astrocytic gliomas.

中文翻译：

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具有 H3.3 p.G34 突变的复发性弥漫性胶质瘤的治疗后超突变

具有影响组蛋白 H3.3 甘氨酸 34 位的 H3F3A 点突变的弥漫性神经胶质瘤是一种独特的神经胶质瘤亚型，主要发生在儿童和年轻成人患者的大脑半球。这些肿瘤具有很高的 MGMT 启动子超甲基化率，这是对 DNA 烷化剂替莫唑胺 (TMZ) 反应的预测性生物标志物。治疗后 TMZ 相关超突变是复发性异柠檬酸脱氢酶 (IDH) 野生型胶质母细胞瘤中 TMZ 耐药的一种机制，可能与低级别 IDH 突变弥漫性星形胶质细胞瘤的肿瘤进展有关。