Table 1.
Clinical data for the cohort of thirteen children with bithalamic gliomas.
| Patient | Age (yrs) | Sex | Surgical procedure | Histology | Genetic alterations | Radiation therapy | Initial chemotherapy | Additional radiation | Additional chemotherapy | Outcome | Follow-up (months) |
|---|---|---|---|---|---|---|---|---|---|---|---|
| #1 | 11 | M | biopsy | anaplastic astrocytoma | EGFR ex20 ins, TP53 fs, CDKN2A/B del | localized 59 Gy | temozolomide, bevacizumab, osimertinib | 30 Gy | bevacizumab, afatinib | died of disease | 22 |
| #2 | 11 | M | biopsy | anaplastic astrocytoma | EGFR ex20 ins, TP53 mis, CCND2 amp | localized 54.4 Gy | temozolomide, valproic acid | none | etoposide | died of disease | 14 |
| #3 | 11 | F | biopsy | anaplastic astrocytoma | EGFR ex20 ins, TP53 mis, CDK6 amp, KDM6A amp | localized 59.4 Gy | temozolmide, CCNU | none | trametinib, ribociclib | died of disease | 21 |
| #4 | 16 | F | biopsy | anaplastic astrocytoma | EGFR ex20 ins, TP53 fs, CDK6 amp, TERT promoter | localized 54 Gy | temozolomide, valproic acid | none | none | died of disease | 12 |
| #5 | 3 | M | biopsy | diffuse astrocytoma | EGFR ex20 ins, TP53 indel, CREBBP fs | localized 54 Gy | temozolmide, osimertinib | none | bevacizumab, CCNU | alive with progressive disease | 8 |
| #6 | 3 | M | STR | glioblastoma | EGFR ex20 ins, PDGFRA amp, TP53 mis, GRB2 amp | localized 54 Gy | methotrexate, vincristine, cisplatin, cyclophosphamide | none | none | died of disease | 12 |
| #7 | 4 | M | STR | anaplastic astrocytoma | EGFR ex20 ins, BCOR del, BCORL1 non | localized 54 Gy | temozolomide, carboplatin, vincristine | none | bevacizumab, enzastaurin, CCNU | died of disease | 17 |
| #8 | 6 | M | biopsy | diffuse astrocytoma | EGFR ex20 ins, TERT promoter | declined | carboplatin, vincristine | none | thioguanine, procarbazine, CCNU, vincristine | died of disease | 19 |
| #9 | 2 | M | biopsy | anaplastic astrocytoma | EGFR ex20 ins | localized 46 Gy | methotrexate, vincristine, cisplatin, cyclophosphamide | none | bevacizumab, topotecan, vorinostat | died of disease | 10 |
| #10 | 5 | M | biopsy | anaplastic astrocytoma | EGFR ex7 mis, CDKN2C indel | localized 59 Gy | temozolomide, afatinib | 30 Gy | bevacizumab, CCNU, osimertinib | alive with progressive disease | 19 |
| #11 | 9 | F | biopsy | diffuse astrocytoma | EGFR ex7 mis, HIST1H3B K27M, BCORL1 fs | localized 54 Gy | ONC201 | none | none | alive with stable disease | 6 |
| #12 | 7 | M | biopsy | anaplastic astrocytoma | EGFR amp, TP53 mis, NF1 non+fs, H3F3A K27M | craniospinal 36 Gy + 4 Gy thalamic boost | temozolomide, bevacizumab, cetuximab | none | none | died of disease | 2 |
| #13 | 9 | M | STR | anaplastic astrocytoma | TP53 mis, CDKN2C indel, BCOR fs, BCORL1 non, LZTR1 fs | localized 59 Gy | bevacizumab | none | none | died of disease | 13 |
STR, subtotal resection. ins, small in-frame insertion. fs, frameshift mutation. del, homozygous deletion. mis, missense mutation. amp, focal amplification. indel, small in-frame insertion or deletion, nonsense mutation.
EGFR and MEK small molecule tyrosine kinase inhibitors are in bold.