1Department of Radiation Oncology, Gachon University Gil Hospital, Incheon, Korea
2Department of Radiation Oncology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
3Department of Radiation Oncology, Kyung Hee University Medical Center, Kyung Hee University School of Medicine, Seoul, Korea
4Department of Radiation Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
5Department of Radiation Oncology, School of Medicine, Kyungpook National University, Daegu, Korea
6Department of Radiation Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
7Department of Radiation Oncology, Seoul National University Bundang Hospital, Seongnam, Korea
8Department of Radiation Oncology, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea
9Department of Radiation Oncology, Chonnam National University Medical School, Gwangju, Korea
10Proton Therapy Center, National Cancer Center, Goyang, Korea
11Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea
Copyright © 2023 by the Korean Cancer Association
This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Ethical Statement
This study was approved by the KROG and institutional review boards of each participating center, according to the ethical standards of the Declaration of Helsinki. The requirement for written informed consent was waived due to the retrospective study design.
Author Contributions
Conceived and designed the analysis: Kim HJ, Yoon HI, Suh CO.
Collected the data: Kim HJ, Lee JH, Kim Y, Lim DH, Park SH, Ahn SD, Kim IA, Im JH, Chung JW, Kim JY, Kim IH, Yoon HI.
Contributed data or analysis tools: Kim HJ, Kim JY, Yoon HI, Suh CO.
Performed the analysis: Kim HJ, Yoon HI, Suh CO.
Wrote the paper: Kim HJ, Yoon HI, Suh CO.
Conflicts of Interest
Conflict of interest relevant to this article was not reported.
Clinical and radiologic information
No. of patients (%) | |
---|---|
Age (yr) | |
Mean±SD | 10.0±7.0 |
Median (IQR) | 7.0 (5.0–12.3) |
Sex | |
Male | 89 (54.9) |
Female | 73 (45.1) |
Lansky/Karnofsky performance status | |
Median (IQR) | 70 (60–80) |
< 80 | 89 (54.9) |
≥ 80 | 72 (44.5) |
Unknown | 1 (0.6) |
Symptoms | |
Cerebral nerve palsy | 103 (63.6) |
Cerebellar signs | 108 (66.7) |
Pyramidal signs | 52 (32.1) |
Symptom duration, median (IQR, wk) | 4.0 (2.0–8.0) |
Pathologic confirmation | |
No | 118 (72.8) |
Biopsy only | 30 (18.5) |
Surgical resection | 14 (8.7) |
Histopathology | |
Diffuse midline glioma, H3K27M-mutant | 18 (11.1) |
Glioblastoma | 8 (4.9) |
Anaplastic astrocytoma | 14 (8.6) |
Diffuse astrocytoma | 4 (2.4) |
MRI features | |
Extrapontine extension | 124 (76.5) |
Enhancement | 103 (63.6) |
Infiltrative margin | 94 (58.0) |
Cyst or necrosis | 61 (37.7) |
Hydrocephalus | 23 (14.2) |
IQR, interquartile range; SD, standard deviation; MRI, magnetic resonance imaging.
Treatment information
No. of patients (%) | |
---|---|
Treatment period | |
2000–2009 | 51 (31.4) |
2010–2015 | 61 (37.7) |
2016–2018 | 50 (30.9) |
RT modality | |
2D-RT | 8 (4.9) |
3D-CRT | 63 (38.9) |
IMRT | 69 (42.6) |
Proton | 22 (13.6) |
CTV volume | |
GTV without margin | 15 (9.9) |
GTV+margin < 1 cm | 67 (41.4) |
GTV+margin 1–2 cm | 68 (42) |
GTV+margin ≥ 2 cm | 2 (1.2) |
RT dose, median (IQR, Gy) | |
GTV | 54 (54–54) |
CTV | 52.2 (46–54) |
Concurrent chemotherapy | |
No | 78 (48.1) |
Yes | 84 (51.9) |
Adjuvant chemotherapy | |
No | 92 (56.8) |
Yes | 70 (43.2) |
Salvage treatment | |
No | 84 (51.9) |
Chemotherapy only | 45 (27.8) |
Re-RT+chemotherapy | 2 (1.2) |
Surgery | 1 (0.6) |
Surgery+chemotherapy | 1 (0.6) |
Unknown | 29 (17.9) |
2D, 2-dimensional; 3D-CRT, 3-dimensional conformal radiotherapy; CTV, clinical target volume; GTV, gross tumor volume; IMRT, intensity-modulated radiotherapy; IQR, interquartile range; RT, radiotherapy.
Factors associated with overall survival
Univariate analysis | Multivariate analysis | |||||
---|---|---|---|---|---|---|
|
| |||||
HR | 95% CI | p-value | HR | 95% CI | p-value | |
Age (< 10 yr vs. ≥ 10 yr) | 0.593 | 0.41–0.85 | 0.004 | 0.662 | 0.46–0.76 | 0.029 |
| ||||||
Sex (male vs. female) | 1.158 | 0.83–1.62 | 0.395 | - | - | - |
| ||||||
Karnofsky/Lansky PS (< 80 vs. ≥ 80) | 0.705 | 0.50–0.99 | 0.043 | 0.684 | 0.48–0.97 | 0.032 |
| ||||||
Extrapontine extension (no vs. yes) | 0.599 | 0.40–0.89 | 0.011 | 0.707 | 0.47–1.07 | 0.104 |
| ||||||
Tumor margin (well vs. infiltrative) | 0.976 | 0.70–1.37 | 0.887 | - | - | - |
| ||||||
Tumor enhancement (no vs. yes) | 1.113 | 0.78–1.58 | 0.552 | - | - | - |
| ||||||
Cyst or necrosis (no vs. yes) | 1.413 | 1.01–1.99 | 0.048 | 1.308 | 0.90–1.89 | 0.155 |
| ||||||
Hydrocephalus (no vs. yes) | 1.259 | 0.79–2.01 | 0.333 | - | - | - |
| ||||||
Surgery (no vs. yes) | 0.716 | 0.47–1.09 | 0.121 | - | - | - |
| ||||||
Treatment decades (before 2010 vs. after 2010) | 0.628 | 0.44–0.89 | 0.009 | 0.578 | 0.40–0.84 | 0.004 |
| ||||||
RT modality (3D-CRT+IMRT vs. proton) | 0.573 | 0.33–1.01 | 0.051 | - | - | - |
| ||||||
RT modality (IMRT vs. proton) | 0.615 | 0.34–1.11 | 0.104 | - | - | - |
| ||||||
Post-RT necrosis (other vs. increased) | 1.713 | 1.20–2.44 | 0.003 | 1.499 | 1.03–2.19 | 0.035 |
| ||||||
Concurrent chemotherapy (no vs. yes) | 1.199 | 0.86–1.68 | 0.292 | - | - | - |
| ||||||
Adjuvant chemotherapy (no vs. yes) | 0.966 | 0.69–1.36 | 0.842 | - | - | - |
3D-CRT, 3-dimensional conformal radiotherapy; CI, confidence interval; HR, hazard ratio; IMRT, intensity-modulated radiotherapy; PS, performance status; RT, radiotherapy.
Failure rates according to the CTV volume
Site of progression | CTV volume | |||
---|---|---|---|---|
GTV+no margin | GTV+< 1 cm | GTV+1-2 cm | GTV+> 2 cm | |
Infield intracranial failure | 11 (78.6) | 41 (75.9) | 49 (86.0) | 2 (100) |
Outfield intracranial failure | 3 (21.4) | 9 (16.7) | 8 (14.0) | 0 |
Extracranial failure | 0 | 4 (7.4) | 0 | 0 |
Values are presented as number (%). CTV, clinical target volume; GTV, gross tumor volume.
IQR, interquartile range; SD, standard deviation; MRI, magnetic resonance imaging.
2D, 2-dimensional; 3D-CRT, 3-dimensional conformal radiotherapy; CTV, clinical target volume; GTV, gross tumor volume; IMRT, intensity-modulated radiotherapy; IQR, interquartile range; RT, radiotherapy.
3D-CRT, 3-dimensional conformal radiotherapy; CI, confidence interval; HR, hazard ratio; IMRT, intensity-modulated radiotherapy; PS, performance status; RT, radiotherapy.
Values are presented as number (%). CTV, clinical target volume; GTV, gross tumor volume.