Table 3.
Clinical features of eleven SNUH patients with NTRK-fused gliomas
| No | Sex | Age (Year) | Site | Grade | Histopathological classification | Gene fusion | Additional genetic alterations | Post-OP Tx | F-U | Outcomes |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 14 | Rt P | LG | DLGG | HOOK3::NTRK2 | Absent | None | 2 yrs | NET |
| 2 | F | 15 | Rt LV & 3rd V | LG | MGNT-like | KIF5A::NTRK2 | Absent | None | 1 mo | Stable |
| 3 | F | 3 | Rt Thal, 3rd V | LG | DLGG | GKAP1::NTRK2 | Absent | Larotrectinib | 10 mos | Stable |
| 4 | M | 1 | Lt TO | LG | DIG | TPR::NTRK1 | Absent | OP (× 2), CCRT | 25 yrs | NET |
| 5 | F | 27 | Lt T | LG | Astrocytoma with Neuropil-like islands-like | LHFPL2::NTRK2 | Absent | None | 13 yrs | NET |
| 6 | F | 31 | Rt F | LG | DLGG | SLMAP-NTRK2 |
Absent except BRCA1 (Q206*) |
None | 5 mos | NET |
| 7 | F | 2 | Lt FT | HG | IHG | ZBTB43::NTRK2 | CDKN2A/2B HemiD |
Proton Tx, 2nd OP, Repotrectinib |
3 yrs | NET |
| 8 | F | 2 | C7-L1 | HG | GBM-like | TPM3::NTRK1 |
CDKN2A/2B HoD MDM4/AKT3 amp, PTEN/FGFR2 loss |
CCRT-TMZ #2, 2nd OP, PCV #4, 3rd OP, 4th OP, Larotrectinib | 4.3 yrs | Progressive, Death (OS post–larotrectinib: 22 mos) |
| 9 | M | 64 | Cerebellar tonsil | HG | GBM-like | SPECC1L::NTRK2 |
CDKN2A HoD CDKN2B HemiD |
GTR-CCRT-TMZ #6 | 27 mos | Stable |
| 10 | M | 67 | Lt cerebellum | HG | GBM-like | FKBP15::NTRK2 |
CDKN2A/2B HoD, PDGFRA/KIT amp, PTEN/NF1 loss, SMARCA2 HoD |
CCRT-TMZ #6, Entrectinib | 21.5 mos |
Progression, Death |
| 11 | M | 72 | Rt T | HG | GBM-like | KANK1::NTRK2 |
CDKN2A/2B HoD TERTp (C228T), PTEN & TP53 mut PTEN loss, 7 trisomy, loss of 10, 14q, 22q |
None, due to poor general condition | 4 mos |
Death (OS: 4 mos) |
| 12 | M | 54 | Lt Thal, Bilat. F | HG | GBM-like | BCR::NTRK2 |
CDKN2A/2B HoD TERTp (C228T), PTEN mut |
None refused treatment | 13 mos |
Death (OS: 13 mos) |
P Parietal; LV Lateral ventricle; 3rd V Third ventricle; Thal Thalamus; T Temporal; PO Parieto-occipital lobe; FT Frontotemporal; Bilat. F Bilateral frontal; IHG, Infant-type hemispheric glioma; LG Low-grade; HG High-grade; DLGG Diffuse low-grade glioma; MGNT Myxoid glioneuronal tumour; DIG Desmoplastic infantile ganglioglioma; IHG Infant-type hemispheric glioma; DHGG Diffuse high-grade glioma; GBM IDH-wt Glioblastoma, IDH-wildtype; HemiD Hemizygous deletion, HoD Homozygous deletion; loss, 1 copy loss; Mut Mutation; OP Operation; CCRT-TMZ Concurrent chemoradiotherapy with temozolomide; PCV Procarbazine + lomustine + vincristine chemotherapy; mos Months; NET No evidence of tumour; OS Overall survival