New progress in the treatment of diffuse midline glioma with H3K27M alteration

Abstract

Diffuse midline glioma with H3K27 M alteration is a primary malignant tumor located along the linear structure of the brain, predominantly manifesting in children and adolescents. The mortality rate is exceptionally high, with a mere 1 % 5-year survival rate for newly diagnosed patients. Beyond conventional surgery, radiotherapy, and chemotherapy, novel approaches are imperative to enhance patient prognosis. This article comprehensively reviews current innovative treatment modalities and provides updates on the latest research advancements in preclinical studies and clinical trials focusing on H3K27M-altered diffuse midline glioma. The goal is to contribute positively to clinical treatment strategies.

Keywords: DIPG; H3K27 M alteration DMG; H3K27 M mutant DMG; Research progress; Therapy.

Fig. 1

Therapeutic strategies for H3K27 M mutation DMG. CAR T-cells can precisely target and kill tumor cells. HDACi exerts anticancer effects by inducing differentiation, cell cycle arrest, apoptosis and inhibiting angiogenesis. EZH2 inhibitors inhibit tumor growth by a mechanism dependent on the induction of tumor suppressor proteins. ONC201 and its derivatives exert their effects by antagonizing the dopamine receptor D2 (DRD2) and/or over-activating the protein lytic activity of caseinolytic peptidase P(ClpP). TTFields directly target proteins essential for the cell cycle, arresting mitosis and causing cell death. Tumor vaccines attack tumors by inducing an immune response. Oncolytic viruses selectively infect and kill tumor cells.