TABLE.
Epidemiology of the previously reported genetically confirmed angiocentric gliomas
| References | No. | Age | Sex | Clinical features | DX | Site | Genetics | Op and adjuvant Tx | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Zhang et al, 2013 (1) | 1 | 3–8 | M | NM | GG | Cerebral cortex | MYB rearrangement | NM | NM |
| 2 | >8 | M | NM | AG | Cerebral cortex | MYB rearrangement | NM | NM | |
| Ramkissoon et al, 2013 (19) | 3 | <2 | F | NM | AG | NM | focal 6q23.3/MYB del | NM | NM |
| 4 | 2–10 | F | NM | AG | NM | focal 6q23.3/MYB del | NM | NM | |
| 5 | 2–10 | M | NM | AG | NM | focal 6q23.3/MYB del | NM | NM | |
| Qaddoumi et al, 2016 (18) | 6 | 3 | M | NM | AG | Parietal | MYB::QKI fusion | NM | NM |
| 7 | 11 | M | NM | AG | Parietal | MYB::QKI fusion | NM | NM | |
| 8 | 5 | F | NM | AG | Frontal | MYB::QKI fusion | NM | NM | |
| 9 | 11 | M | NM | AG | Temporal | MYB::QKI fusion | NM | NM | |
| 10 | 37 | M | NM | AG | Temporal | MYB::QKI+BRAF V600E | NM | NM | |
| 11 | 15 | F | NM | AG | Temporal | MYB::QKI fusion | NM | NM | |
| 12 | 3 | M | NM | AG | Frontoparietal | MYB::QKI fusion | NM | NM | |
| 13 | 5 | M | NM | AG | Temporal | MYB::QKI fusion | NM | NM | |
| 14 | 7 | M | NM | AG | Parietal | MYB::QKI fusion | NM | NM | |
| 15 | 10 | F | NM | AG | Temporal | MYB::QKI fusion | NM | NM | |
| 16 | 17 | M | NM | AG | Occipital | MYB::QKI+BRAF V600E | NM | NM | |
| 17 | 19 | F | NM | AG | Frontal | MYB::QKI fusion | NM | NM | |
| 18 | 41 | F | NM | AG | Temporal | MYB::QKI fusion | NM | NM | |
| 19 | 7 | M | NM | AG | Temporal | MYB::QKI fusion | NM | NM | |
| 20 | 3 | F | NM | AG | Temporal | QKI | NM | NM | |
| Bandopadhayay et al, 2016 (2) | 21 | NM | NM | NM | AG | NM | MYB::QKI fusion | NM | NM |
| 22 | NM | NM | NM | AG | NM | MYB::QKI fusion | NM | NM | |
| 23 | NM | NM | NM | AG | NM | MYB::QKI fusion | NM | NM | |
| 24 | NM | NM | NM | AG | NM | MYB::QKI fusion | NM | NM | |
| 25 | NM | NM | NM | AG | NM | MYB::QKI fusion | NM | NM | |
| 26 | NM | NM | NM | AG | NM | MYB::QKI fusion | NM | NM | |
| 27 | NM | NM | NM | AG | NM | Other MYB mutation | NM | NM | |
| Chan et al, 2017 (7) | 28 | 7 | M | Sixth nerve palsy | AG | Inferior Pons | MYB::QKI fusion | Biopsy only | NM |
| D’Aronco et al, 2017 (8) | 29 | 7 | M | Developmental delay | AG | Pons and medulla | MYB::QKI fusion | Biopsy only, Unresectable, Carboplatin, and Vincristine+Bevacizumab | Initially progressed but stable size in 12 months |
| 30 | 3 | F | Seventh nerve palsy | AG | Brainstem | MYB::QKI fusion | Biopsy only, Unresectable, carboplatin, and vincristine+Bevacizumab, mTOR inhibitor | Initially progressed but stable in 4 years | |
| Lake et al, 2020 (17) | 31 | 5 | M | NM | AG | Thalamus | MYB::QKI fusion | Radiotherapy | NM |
| 32 | 2 | M | NF1 patient | AG | Lt frontal | MYB::QKI fusion | Chemotherapy | NM | |
| Suh et al, Present case | 33 | 11 | M | Tonic seizure | HGG | Lt frontal | MYB::QKI fusion | CCRT+trial | One recurs 20 months after surgery and no further recurs 40 months after the initial surgery |
| Suh et al, Present case | 34 | 46 | F | Visual impairment | AG | Rt hemisphere CC and Thalamus | MYBL1::QKI fusion | GTR only | No recur for 52 months F-U |
GG, gangliogloma; AG, angiocentric glioma, CNS WHO grade 1; NM, not mentioned; CC, corpus callosum; CCRT, concurrent chemo-radiotherapy; GTR, gross total resection; F-U, follow-up.