Immunotherapy for Diffuse Midline Glioma: From Preclinical Modeling to Clinical Translation

Diffuse midline glioma, H3K27-altered (DMG) is a group of central nervous system (CNS) tumors with no curative treatment options. First described in the early 20th century, prognosis and clinical outcome for patients have changed very little, and the only standard treatment modality remains radiation therapy. The development of targeted therapies against DMG has long been hindered due to the paucity of tumor tissue and disease models for laboratory research. However, access to patient-derived tumor tissue and the subsequent establishment of DMG preclinical models in recent years has led to a deeper understanding of underlying disease mechanisms and aided the development of novel therapeutic agents. Here, we provide an overview of newly established DMG modeling systems in the preclinical setting leading toward immunotherapy clinical trials for patients with DMG.