Table 2 Clinical and molecular characteristics of histone H3 K27-mutant diffuse midline glioma patients in Kansai Network (n = 93)
Total | Location | ||||||
|---|---|---|---|---|---|---|---|
Thalamus | Brainstem | Spinal cord | Others | p value | |||
Number | 93 | 47 (51%) | 24 (26%) | 12 (13%) | 10 (11%) | ||
Clinical characteristics | |||||||
Age (years) | Median (range) | 31 (4–78) | 28 (4–76) | 21 (6–75) | 30 (12–78) | 45 (36–71) | 0.041* |
Sex | 0.809 | ||||||
Male | 55 (59%) | 27 (57%) | 16 (67%) | 7 (58%) | 5 (50%) | ||
Female | 38 (41%) | 20 (43%) | 8 (33%) | 5 (42%) | 5 (50%) | ||
MR images (Gd enhancement) | 0.016* | ||||||
High grade features | 68 (73%) | 36 (76%) | 14 (58%) | 11 (92%) | 7 (70%) | ||
Low grade featrues | 18 (19%) | 6 (13%) | 10 (42%) | 0 (–) | 2 (20%) | ||
Unknown | 7 (8%) | 5 (11%) | 0 (–) | 1 (8%) | 1 (10%) | ||
Histopathology (CNS WHO 2021) | 0.019* | ||||||
LGG | 16 (17%) | 9 (19%) | 6 (25%) | 0 (–) | 1 (10%) | ||
HGG without GBM features | 36 (39%) | 12 (26%) | 13 (54%) | 5 (42%) | 6 (60%) | ||
GBM features | 40 (43%) | 26 (55%) | 5 (21%) | 6 (50%) | 3 (30%) | ||
Unknown | 1 (1%) | 0 (–) | 0 (–) | 1 (8%) | 0 (–) | ||
Preoperative KPS score | 0.568 | ||||||
80–100 | 46 (49%) | 26 (55%) | 11 (46%) | 4 (33%) | 5 (50%) | ||
-70 | 47 (51%) | 21 (45%) | 13 (54%) | 8 (67%) | 5 (50%) | ||
Extent of surgical resection (EOR) | 0.05 | ||||||
GTR | 5 (5%) | 3 (6%) | 0 (–) | 0 (–) | 2 (20%) | ||
STR | 11 (12%) | 10 (21%) | 1 (4%) | 0 (–) | 0 (–) | ||
PR | 18 (19%) | 9 (19%) | 4 (17%) | 4 (33%) | 1 (10%) | ||
Biopsy | 59 (63%) | 25 (53%) | 19 (79%) | 8 (67%) | 7 (70%) | ||
Adjuvant treatment | 0.588 | ||||||
RT + TMZ + BEV | 35 (38%) | 17 (36%) | 11 (46%) | 3 (25%) | 4 (40%) | ||
RT + TMZ | 37 (40%) | 23 (49%) | 6 (25%) | 6 (50%) | 2 (20%) | ||
RT + ACNU | 1 (1%) | 1 (2%) | 0 (–) | 0 (–) | 0 (–) | ||
RT + BEV | 4 (4%) | 1 (2%) | 1 (4%) | 1 (8%) | 1 (10%) | ||
RT alone | 5 (5%) | 1 (2%) | 3 (13%) | 0 (–) | 1 (10%) | ||
TMZ alone | 4 (4%) | 1 (2%) | 2 (8%) | 0 (–) | 1 (10%) | ||
BEV alone | 0 (–) | 0 (–) | 0 (–) | 0 (–) | 0 (–) | ||
None | 7 (8%) | 3 (6%) | 1 (4%) | 2 (17%) | 1 (10%) | ||
Radiation dose (Gy) | < 0.001* | ||||||
50–60 | 74 (80%) | 41 (87%) | 20 (83%) | 4 (33%) | 7 (70%) | ||
40–49 | 8 (9%) | 1 (2%) | 0 (–) | 5 (42%) | 1 (10%) | ||
< 40 (range 35–39) | 4 (4%) | 1 (2%) | 1 (4%) | 1 (8%) | 0 (–) | ||
None | 7 (8%) | 4 (9%) | 3 (13%) | 2 (17%) | 2 (20%) | ||
Bevacizumab | (Adjuvant + Recurrent) | 53 (57%) | 25 (53%) | 16 (67%) | 7 (58%) | 5 (50%) | 0.943 |
Repeat surgical resection | 7 (12%) | 6 (20%) | 1 (7%) | 0 (–) | 0 (–) | 0.398 | |
Genetic status | |||||||
Histone mutation | 0.572 | ||||||
HIST1H3B p.K27M | 2 (2%) | 2 (4%) | 0 (–) | 0 (–) | 0 (–) | ||
H3F3A p.K27M | 91 (98%) | 45 (96%) | 24 (100%) | 12 (100%) | 10 (100%) | ||
IDH1/2 | – | ||||||
Wild | 93 (100%) | 47 (100%) | 24 (100%) | 12 (100%) | 10 (100%) | ||
Mut | 0 (–) | 0 (–) | 0 (–) | 0 (–) | 0 (–) | ||
TERT promoter | 0.386 | ||||||
Wild | 90 (97%) | 44 (94%) | 24 (100%) | 12 (100%) | 10 (100%) | ||
C228T/C250T | 3 (3%) | 3 (6%) | 0 (–) | 0 (–) | 0 (–) | ||
MGMT promoter | 0.304 | ||||||
Methylated | 8 (9%) | 5 (11%) | 1 (4%) | 0 (–) | 2 (20%) | ||
Unmethylated | 85 (91%) | 42 (89%) | 23 (96%) | 12 (100%) | 8 (80%) | ||
TP53 | 0.207 | ||||||
Wild | 39 (42%) | 16 (34%) | 15 (63%) | 6 (50%) | 2 (20%) | ||
Mutation | 53 (57%) | 30 (64%) | 9 (38%) | 6 (50%) | 8 (80%) | ||
Unknown | 1 (1%) | 1 (2%) | 0 (–) | 0 (–) | 0 (–) | ||
BRAF | 0.92 | ||||||
Wild | 91 (98%) | 45 (96%) | 24 (100%) | 12 (100%) | 10 (100%) | ||
p.V600E | 1 (1%) | 1 (2%) | 0 (–) | 0 (–) | 0 (–) | ||
Unknown | 1 (1%) | 1 (2%) | 0 (–) | 0 (–) | 0 (–) | ||
FGFR1 | 0.619 | ||||||
Wild | 78 (84%) | 38 (81%) | 22 (92%) | 9 (75%) | 9 (90%) | ||
Mutation | 13 (14%) | 8 (17%) | 1 (4%) | 3 (25%) | 1 (10%) | ||
Unknown | 2 (2%) | 1 (2%) | 1 (4%) | 0 (–) | 0 (–) | ||
EGFR | 0.692 | ||||||
Wild | 89 (96%) | 45 (96%) | 22 (92%) | 12 (100%) | 10 (100%) | ||
Mutation | 3 (3%) | 1 (2%) | 2 (8%) | 0 (–) | 0 (–) | ||
Unknown | 1 (1%) | 1 (2%) | 0 (–) | 0 (–) | 0 (–) | ||