Clinical Features and Prognosis of Diffuse Midline Glioma: A Series of 24 Cases

Sun Woo Jang; Sang Woo Song; Young-Hoon Kim; Young Hyun Cho; Seok Ho Hong; Jeong Hoon Kim; Young-Shin Ra; Sangjoon Chong

doi:10.14791/btrt.2022.0035

Clinical Features and Prognosis of Diffuse Midline Glioma: A Series of 24 Cases

Brain Tumor Res Treat. 2022 Oct;10(4):255-264. doi: 10.14791/btrt.2022.0035.

Authors

Sun Woo Jang¹, Sang Woo Song¹, Young-Hoon Kim¹, Young Hyun Cho¹, Seok Ho Hong¹, Jeong Hoon Kim¹, Young-Shin Ra¹, Sangjoon Chong²

Affiliations

¹ Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
² Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. neurosj@gmail.com.

Abstract

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.

Keywords: Biopsy; Chemoradiotherapy; Diffuse intrinsic pontine glioma; Histone H3; Sequence analysis; Survival.