Introduction
Background
Aim of the study
Methods
Study cohort
Clinical analysis
Radiomic features
Prognostic model
Results
Patient characteristics
Characteristics of patients | |||
---|---|---|---|
Reirradiated (N = 18) | Non-reirradiated (N = 25) | p-value | |
Gender | |||
Male | 9 patients (50%) | 14 (56%) | 0.94 |
Female | 9 patients (50%) | 11 (44%) | |
Age | |||
Median age at the diagnosis | 7.5 years | 7.1 years | 0.72 |
Median age at the progression | 9.1 years | 7.8 years | 0.96 |
First-line RT regimen | |||
54 Gy in 1.8-Gy fractions | 18 patients (100%) | 22 patients (88%) | 0.36 |
44.8 Gy in 2.8-Gy fractions | 0 patients (0%) | 3 patients (12%) | |
Neurological symptoms at diagnosis | |||
Ataxia | 12 patients (67%) | 16 patients (64%) | 0.55 |
Long tract signs | 5 patients (28%) | 11 patients (44%) | |
Cerebral neuropathy | 9 patients (50%) | 21 patients (84%) | |
First-line RT effectiveness | |||
Neurological improvement | 18 patients (100%) | 25 patients (100%) | 1 |
Median progression-free survival | 43.7 weeks | 38.3 weeks | 0.18 |
First-line RT toxicity | |||
Mild symptoms of increased intracranial pressure (grade 1) | 2 patients (11%) | 3 patients (12%) | 1 |
Systemic therapy regimen before progression | |||
Temozolomide | 8 patients (44%) | 5 patients (20%) | 0.51 |
Sirolimus | 5 patients (28%) | 8 patients (32%) | |
Nivolumab | 1 patient (6%) | 1 patient (4%) | |
Only RT | 6 patients (33%) | 11 patients (44%) | |
Second-line RT regimen | |||
20 Gy in 2‑Gy fractions | 16 patients (89%) | – | – |
24 Gy in 2‑Gy fractions | 2 patients (11%) | – | |
Neurological symptoms at progression | |||
Ataxia | 12 patients (67%) | 14 patients (64%) | 0.33 |
Long tract signs | 9 patients (50%) | 19 patients (76%) | |
Cerebral neuropathy | 8 patients (44%) | 21 patients (84%) | |
Second-line RT effectiveness | |||
Neurological improvement | 14 patients (78%) | – | – |
Second-line RT toxicity | |||
Mild symptoms of increased intracranial pressure (grade 1) | 4 patients (22%) | – | – |
Survival | |||
Median survival after diagnosis | 79.1 weeks | 46.1 weeks | < 0.01 |
Median survival after progression | 29.2 weeks | 7.1 weeks | < 0.01 |
Genetic alterations | |||
Proven H3K27 mutation | 9 patients (50%) | 11 patients (44%) | 0.94 |
Prognostic factors
Explanatory variable | Hazard ratio (95% CI) | p-value |
---|---|---|
Shape_Flatness | 0.48 (0.26–0.87) | 0.02 |
Glszm_SizeZoneNonUniformityNormalized | 0.30 (0.13–0.69) | < 0.01 |
Explanatory variable | Hazard ratio (95% CI) | p-value |
---|---|---|
Shape_Flatness | 0.61 (0.35–1.06) | 0.08 |
Glszm_SizeZoneNonUniformityNormalized | 0.43 (0.22–0.85) | 0.02 |