ความก้าวหน้าในการรักษา Diffuse Intrinsic Pontine Glioma (DIPG): กลยุทธ์และนวัตกรรมใหม่

Suvichan Phibalwong; Jiraporn Amornfa

Authors

Suvichan Phibalwong Division of Neurosurgery, Department of Surgery, Faculty of Medicine, Chulalongkorn University
Jiraporn Amornfa Division of Neurosurgery, Department of Surgery, Faculty of Medicine, Chulalongkorn University

Keywords:

Cerebral hyperperfusion syndrome, STA-MCA bypass, Direct bypass, Moyamoya disease, Intracranial Internal carotid artery stenos

Abstract

Diffuse intrinsic pontine glioma (DIPG), currently classified within diffuse pediatric-type highgrade gliomas, is one of the most aggressive pediatric brain tumors and is associated with an extremely poor prognosis despite advances in neuro-oncology. This review summarizes current knowledge regarding the molecular pathogenesis, diagnosis, standard treatment, and emerging therapeutic strategies for DIPG. Recent molecular studies have identified H3K27 alterations, including H3.3K27M, H3.1K27M, and EZHIP, together with abnormalities in TP53, PDGFRA, and ACVR1, as key drivers of tumor development and treatment resistance. Magnetic resonance imaging remains the cornerstone of diagnosis, while stereotactic biopsy has become increasingly important for molecular characterization and treatment planning. Radiotherapy remains the standard treatment, providing temporary clinical improvement, whereas the survival benefit of temozolomide remains limited. Novel therapeutic approaches, including chimeric antigen receptor (CAR) T-cell therapy, oncolytic virotherapy, survivin peptide vaccination (SurVaxM), and immune checkpoint inhibitors, have demonstrated encouraging preliminary results in early-phase studies. Although these emerging therapies offer promising opportunities for improving outcomes, further translational research and well-designed clinical trials are required to establish their long-term efficacy and safety and to develop more effective personalized treatment strategies for patients with DIPG.

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