|
Age (years)
|
|
Median: 7.3 years (range 2.3–24)
| | |
|
< 3
|
2
|
7
|
|
3–10
|
16
|
57
|
|
10 +
|
10
|
36
|
|
Gender
|
|
Female
|
15
|
54
|
|
Male
|
13
|
46
|
|
Overall survival (months)
|
|
Median: 11.5 months (range 0–82)
| | |
|
< 12
|
14
|
50
|
|
12–24
|
11
|
39
|
|
> 24
|
3
|
11
|
|
Symptom duration (months)
| | |
|
< 6
|
19
|
68
|
|
6–12
|
2
|
7
|
|
12–24
|
5
|
18
|
|
Not available
|
2
|
7
|
|
Radiotherapy
|
26
|
93
|
|
Photon irradiation
|
24
|
86
|
|
Proton irradiation
|
2
|
7
|
|
Re-irradiation
|
5
|
18
|
|
Systemc therapy
|
25
|
89
|
|
Radiosensitizer
|
11
|
44
|
|
Adjuvant therapy
|
22
|
79
|
|
Specific systemic therapy agent(s) received
| | |
|
Bevacizumab
|
8
|
30
|
|
Vorinostat (HDAC inhibitor)
|
7
|
26
|
|
CDK4/6 inhibitor (ribcoclib [n = 6], palbociclib [n = 1])
|
7
|
26
|
|
Everolimus (mTOR inhibitor)
|
4
|
15
|
|
Irinotecan
|
4
|
15
|
|
Temozolomide
|
3
|
11
|
|
PARP inhibitor
|
3
|
11
|
|
Tyrosine kinase inhibitor (cabozantinib [n = 1], pazopanib [n = 1], dasatinib [n = 1])
|
3
|
11
|
|
WEE-1 inhibitor
|
2
|
7
|
|
Tivantinib (cMET inhibitor)
|
2
|
7
|
|
ONC201
|
1
|
4
|
|
Trametinib (MEK inhibitor)
|
1
|
4
|
|
Imetelstat (Telomerase inhibitor)
|
1
|
4
|
|
Distant metastases at diagnosisa
|
1
|
4
|
|
Distant metastases at autopsy
|
9
|
32
|
|
Cerebral cortex
|
7
|
25
|
|
Cerebellar peduncles and/or cerebellum
|
4
|
14
|
|
Thalamus and/or subthalamus
|
3
|
11
|
|
Basal ganglia
|
3
|
11
|
|
Cervical spinal cord
|
1
|
4
|
|
Histone mutation status
|
|
H3.3
|
20
|
71
|
|
H3.1
|
7
|
25
|
|
WT
|
1
|
4
|
|
Extent of pathological spread (autopsy)
|
|
Extension beyond brainstem
|
22
|
79
|
|
Limited to brainstem
|
5
|
18
|
|
Insuffient information
|
1
|
4
|
|
Location of pathologic spread (autopsy)
|
|
Cerebellar peduncles and/or cerebellum
|
21
|
75
|
|
Thalamus and/or subthalamus
|
15
|
54
|
|
Cervical cord
|
13
|
46
|
|
Basal ganglia
|
8
|
29
|
|
Cortex
|
2
|
7
|
