Table 3 Pathologic and molecular characteristics of patients with histone H3 K27-mutant diffuse non-midline glioma (n = 16) and H3 G34-mutant diffuse hemispheric glioma (n = 9) in the Kansai Network
Characteristic | H3 K27-mutant diffuse non-midline glioma (n = 16) | H3 G34-mutant diffuse hemispheric glioma (n = 9) | p value |
|---|---|---|---|
Histopathologic findings | |||
 Histologic diagnosis (CNS WHO 2021) | 0.289 | ||
  LGG | 1 (6.3%) | 0 (–) |  |
  HGG without GBM features | 12 (75%) | 5 (55.6%) |  |
  GBM features | 3 (18.8%) | 3 (33.3%) |  |
  NA | 0 (–) | 1 (11.1%) |  |
 Microvascular proliferation | 0.0824 | ||
  Present | 1 (6.3%) | 3 (33.3%) |  |
  Absent | 5 (31.3%) | 4 (44.4%) |  |
  NA | 10 (62.5%) | 2 (22.2%) |  |
  Necrosis | 0.0736 | ||
  Present | 2 (12.5%) | 3 (33.3%) |  |
  Absent | 3 (18.8%) | 4 (44.4%) |  |
  NA | 11 (68.8%) | 2 (22.2%) |  |
 Ki-67 (%) | 0.5958 | ||
  ≤ 5 | 1 (6.3%) | 0 (–) |  |
  > 5 | 4 (25.0%) | 3 (33.3%) |  |
  NA | 11 (68.8%) | 6 (66.7%) |  |
Genetic status | |||
 Histone H3 |  < 0.0001* | ||
  K27M | 16 (100%) | 0 (–) |  |
  G34R | 0 (–) | 7 (77.8%) |  |
  G34V | 0 (–) | 2 (22.2%) |  |
 TERT promoter | 0.174 | ||
  Wild | 16 (100%) | 8 (88.9%) |  |
  C228T/C250T | 0 (–) | 1 (11.1%) |  |
 MGMT promoter | 0.053 | ||
  Methylated | 4 (25.0%) | 6 (66.7%) |  |
  Unmethylated | 12 (75.0%) | 3 (33.3%) |  |
 TP53 | 0.093 | ||
  Wild | 7 (43.7%) | 1 (11.1%) |  |
  Mutated | 9 (56.3%) | 8 (88.9%) |  |
 BRAF | 0.444 | ||
  Wild | 15 (93.7%) | 9 (100%) |  |
  p.V600E | 1 (6.3%) | 0 (–) |  |
 FGFR1 | 0.444 | ||
  Wild | 15 (93.7%) | 9 (100%) |  |
  Mutated | 1 (6.3%) | 0 (–) |  |
 EGFR | – | ||
  Wild | 16 (100%) | 9 (100%) |  |
  Mutated | 0 | 0 |  |
 PDGFRA | – | ||
  Wild | 9 (56.3%) | 5 (55.6%) |  |
  Mutated | 0 (–) | 0 (–) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 CDK4/6 | – | ||
  Wild | 9 (56.3%) | 5 (55.6%) |  |
  Mutated | 0 (–) | 0 (–) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 ACVR1 | – | ||
  Wild | 9 (56.3%) | 5 (55.6%) |  |
  Mutated | 0 (–) | 0 (–) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 MYC | – | ||
  Wild | 9 (56.3%) | 5 (55.6%) |  |
  Mutated | 0 (–) | 0 (–) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 ATRX | – | ||
  Wild | 9 (56.3%) | 5 (55.6%) |  |
  Mutated | 0 (–) | 0 (–) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 CDKN2A/B | – | ||
  Wild | 9 (56.3%) | 5 (55.6%) |  |
  Mutated | 0 (–) | 0 (–) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 PIC3CA | 0.788 | ||
  Wild | 9 (56.3%) | 4 (44.4%) |  |
  Mutated | 0 (–) | 1 (11.1%) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 PTEN | 0.733 | ||
  Wild | 8 (50.0%) | 4 (44.4%) |  |
  Mutated | 1 (6.3%) | 1 (11.1%) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |
 APC | 0.757 | ||
  Wild | 8 (50.0%) | 5 (55.6%) |  |
  Mutated | 1 (6.3%) | 0 (–) |  |
  NA | 7 (43.8%) | 4 (44.4%) |  |