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Updated: Aug 22, 2025
Live-3D-Cell Immunocytochemistry Assays of Pediatric Diffuse Midline Glioma
Published on: November 11, 2021
Clinical Features and Prognosis of Diffuse Midline Glioma: A Series of 24 Cases
Sun Woo Jang1, Sang Woo Song1, Young-Hoon Kim1
1Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Background:
Diffuse midline glioma (DMG) which occurs in midline structures and characterized by harboring K27M mutation in genes encoding the histone 3 protein is classified as World Health Organization (WHO) grade IV regardless of histological findings and has a poor prognosis. Nevertheless, because of its relatively rare incidence compared with other high-grade gliomas, a comprehensive description encompassing clinical features and genomic profiles of DMG is still lacking.
Methods:
In this study, we analyzed data of 24 patients who were diagnosed as DMG which was confirmed by surgical specimens in both pediatric and adult patients. We described the clinical outcomes of patients with DMG and their genomic profiles through a retrospective analysis of 24 patients with DMG.
Results:
The clinical characteristics of the 24 patients with DMG were analyzed. Ten patients (41%) underwent tumor resection and 14 patients (59%) underwent tumor biopsy. The median overall survival was 10.4 months (95% confidence interval [CI], 8.4 to 12.5) and progression free survival was 3.9 months (95% CI, 2.6 to 5.2). Fifteen patients (62%) were accompanied by hydrocephalus. None of the patient, tumor, or treatment factors had any significant associated with survival. In both immunohistochemistry staining (n=24) and targeted next generation sequencing (n=15), TP53 mutation was the most common genetic mutation (25% and 46%, respectively) found in the patients except alterations in histone 3 protein.
Conclusion:
Although surgical treatment of patient with DMG does not affect the overall survival prognosis, it can help improve the patient's accompanying neurological symptoms in some limited cases. Hydrocephalus is often accompanied with DMG and treatment for hydrocephalus is often also required. Multidisciplinary therapeutic approach is needed.
Insights
Diffuse midline glioma (DMG) is a rare, aggressive brain tumor. This study found TP53 mutations common in DMG and highlighted the need for multidisciplinary care, as survival was not significantly impacted by surgical intervention.
Area of Science:
- Neuro-oncology
- Genomics
- Pediatric and Adult Oncology
Background:
- Diffuse midline glioma (DMG) is a WHO grade IV brain tumor characterized by K27M mutations in histone 3 genes.
- DMG has a poor prognosis and lacks comprehensive clinical and genomic descriptions due to its rarity.
Purpose of the Study:
- To describe the clinical outcomes and genomic profiles of Diffuse midline glioma (DMG) patients.
- To analyze factors influencing survival in DMG.
Main Methods:
- Retrospective analysis of 24 pediatric and adult patients diagnosed with DMG.
- Evaluation of clinical characteristics, surgical interventions (resection vs. biopsy), and genomic profiles (immunohistochemistry and next-generation sequencing).
Main Results:
- Median overall survival was 10.4 months; progression-free survival was 3.9 months.
- TP53 mutations were the most common genetic alterations (25% IHC, 46% NGS) besides histone 3 alterations.
- Hydrocephalus affected 62% of patients; no patient, tumor, or treatment factors significantly impacted survival.
Conclusions:
- Surgical treatment for DMG does not significantly improve overall survival but may alleviate neurological symptoms in some cases.
- Hydrocephalus is a frequent comorbidity requiring management.
- A multidisciplinary therapeutic approach is essential for managing DMG patients.
