Table 1 Summary of clinicopathological features of the cohort
Case | Age (years) | Sex | H3K27me3 | NFKB | L1CAM | Molecular analysis | 1q gain | Methylation class (calibrated score) | Resection | Metastasis at diagnosis | Adjuvant therapies | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 10 | M | Preserved | Negative | Negative | NA | Absent | EPN-PFB (0.90) SUBCLASS 3 (0.64) | TR | No | No | Relapse-free. Alive at 79 months |
2 | 11 | M | Preserved | Negative | Negative | NA | Absent | EPN-PFB (0.30) | STR | No | RT | Relapse-free. Alive at 29 months |
3 | 15 | F | Preserved | Negative | Negative | NA | Absent | EPN-PFB (0.99) SUBCLASS 2 (0.86) | TR | No | RT | Relapse-free. Alive at 76 months |
4 | 17 | F | Preserved | Negative | Negative | NA | Absent | EPN-PFB SUBCLASS 1 (0.98) | TR | No | RT | Relapse-free. Alive at 22 months |
5 | 11 | M | Preserved | Negative | Negative | NA | Absent | EPN-PFB SUBCLASS 1 (0.99) | TR | No | RT | Relapse-free. Alive at 7 months |
6 | 14 | F | Preserved | Negative | Negative | NA | Absent | EPN-PFB SUBCLASS 2 (0.96) | TR | Yes, dural | RT | Relapse-free. Alive at 25 months |
7 | 11 | F | Preserved | Negative | Negative | NA | Absent | EPN-PFB SUBCLASS 4 (0.99) | TR | No | RT | Relapse-free. Alive at 126 months |
8 | 11 | F | Preserved | Negative | Negative | NA | Absent | EPN-PFB SUBCLASS 4 (0.99) | TR | No | RT | Relapse-free. Alive at 90 months |
9 | 13 | M | Preserved | Negative | Negative | NA | Absent | EPN-PFB SUBCLASS 4 (0.99) | TR | No | RT | Relapse-free. Dead at 127 months after rhabdomyosarcoma and melanoma |
10 | 5 | M | Preserved | Negative | Negative | NA | Absent | EPN-PFA SUBCLASS 1A (0.25) | TR | No | No | Local relapse at 14 months. Alive at 14 months |
11 | 15 | F | Heterogeneous | Negative | Negative | NA | Present | EPN-PFA SUBCLASS 1C (0.53) | PR | No | RT | Supratentorial relapse at 16 months. Alive at 16 months |
12 | 15 | F | Preserved | Negative | Negative | NF2 | Absent | SPINAL EPN (0.99) | TR | No | No | Relapse-free. Alive at 63 months |
13 | 12 | F | Preserved | Negative | Negative | NF2 | Absent | SPINAL EPN (0.99) | TR | No | CT + RT | Local relapse at 22 months. Alive at 98 months |
14 | 3 | F | Preserved | Positive | Positive | ZFTA::RELA | Absent | ST-EPN ZFTA-RELA SUBCLASS A (0.97) | STR | No | RT | Distant leptomeningeal relapse at 12 months. Alive at 39 months |
15 | 1 | M | Preserved | Positive | Positive | ZFTA::RELA | Absent | ST-EPN ZFTA-RELA SUBCLASS A (0.99) | PR | No | CT + RT | Local relapse at 8 months. Alive at 114 months |