Abstract
Background
Methods
Results
Conclusion
Notes
Author Contributions:
Conceptualization: Sangjoon Chong.
Data curation: Sun Woo Jang.
Formal analysis: Sun Woo Jang.
Funding acquisition: Sangjoon Chong.
Investigation: Sun Woo Jang, Sangjoon Chong.
Methodology: Sangjoon Chong.
Project administration: Jeong Hoon Kim, Young-Shin Ra, Sangjoon Chong.
Resources: Sang Woo Song, Young-Hoon Kim, Young Hyun Cho, Seok Ho Hong, Jeong Hoon Kim, Young-Shin Ra, Sangjoon Chong.
Software: Sun Woo Jang, Sangjoon Chong.
Supervision: Sang Woo Song, Young-Hoon Kim, Young Hyun Cho, Seok Ho Hong, Jeong Hoon Kim, Young-Shin Ra, Sangjoon Chong.
Validation: Sangjoon Chong.
Visualization: Sun Woo Jang, Sangjoon Chong.
Writing—original draft: Sun Woo Jang, Sangjoon Chong.
Writing—review & editing: Sang Woo Song, Young-Hoon Kim, Young Hyun Cho, Seok Ho Hong, Jeong Hoon Kim, Young-Shin Ra, Sangjoon Chong.
Availability of Data and Material
References
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Fig. 1
Age distribution of the patients. Green, diffuse (from thalamus–pons–cerebellum); skyblue, basal ganglia; orange, thalamus; purple, midbrain; pink, pons; dark pink, medulla; blue, spinal cord; yellow, cerebellum.
![btrt-10-255-g001](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-g001.jpg)
Fig. 2
Kaplan-Meier survival curves. A: Overall survial curves of the whole cohort (median=10.4 months). B-F: Survival analysis between groups of patients according to tumor locations (p=0.275) (B), surgical treatments (p=0.570) (C), Ki-67 LI of the specimens (p=0.272) (D), tumor sizes and (p=0.054) (E), and adjuvant treatments (p=0.175) (F) reveals no significant differences between the groups (log-rank test). LI, labeling index, CCRT, concurrent chemoradiation therapy; RT, radiation therapy.
![btrt-10-255-g002](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-g002.jpg)
Fig. 3
Kaplan-Meier survival curves. A: Progression-free survival curves for the whole cohort (median=3.9 months). B: Analysis of progression-free survial between brainstem group and non-brainstem group shows no significant difference (log-rank test, p=0.275).
![btrt-10-255-g003](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-g003.jpg)
Fig. 4
Genomic landscape of DMG patients obtained by targeted NGS. DMG, diffuse midline glioma; NGS, next generation sequencing; SNV, single-nucleotide variant; CNV, copy number variation; INS, insertion; DEL, deletion.
![btrt-10-255-g004](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-g004.jpg)
Fig. 5
Illustrative case (Case 12). Preoperative MRI and CT images (A-C) and postoperative MRI and CT images (D-F). A 4-year-old female patient presented with left side weakness (grade 3). The day before surgery, motor weakness aggravated to grade 2 and diplopia occurred. Images revealed pontine tumor with tumor bleeding. The tumor was removed partially and the patient was diagnosed as diffuse midline glioma, H3 K27M altered. After resection, diplopia disappeared and weakness also improved to nearly normal for 9 months till progression.
![btrt-10-255-g005](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-g005.jpg)
Fig. 6
Illustrative case (Case 18). Preoperative MRI and CT images (A-C) and postoperative MRI and CT images (D-F). A 20-year-old female patient presented with progressive left side weakness (grade 3). Images reveals right thalamic mass with hydrocephalus. The tumor was removed subtotally and the patient was diagnosed as diffuse midline glioma, H3 K27M altered. Note that hydrocephalus is resolved after resection. After resection, weakness improved to grade 4 and hydrocephalus remains resolved for 4 months till progression.
![btrt-10-255-g006](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-g006.jpg)
Table 1
Clinical features and prognosis of diffuse midline glioma of the 24 patients
![btrt-10-255-i001](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-i001.jpg)
Ki-67 LI, Ki-67 labeling index; HC, hydrocephalus; PFS, progression free survival; FU, follow up; N/A, not available; CCRT, concurrent chemoradiation therapy; RTx, radiation therapy; ETV, endosopic third ventriculostomy; VPS, ventriculoperitoneal shunt; STR, subtotal resection; RT, radiotherapy; PR, partial resection
Table 2
Factors contributing to OS and PFS on DMG
![btrt-10-255-i002](/upload/SynapseXML/0212btrt/thumb/btrt-10-255-i002.jpg)