Table 1 Comparison of clinical, histopathological and molecular data according to methylation classes and diagnoses
HGNET-BCOR ITD (n = 29) | HGNET-BCOR EP300:BCOR/BCORL1 fusions (n = 3) | GLIOMAS EP300:BCOR fusion (n = 4) | |
|---|---|---|---|
Location | Infratentorial (52%) | Supratentorial (100%) | Supratentorial (100%) |
Age | Median age = 3.5 YO (0;22) | Median age = 27 YO (13;72) | Median age = 12 YO (10;18) |
Sex | Male (54%) | Male (100%) | Male (66%) |
Radiology | Large, well-circumscribed, solid with meningeal attachment; T1 hypointense, T2 hyperintense, low ADC, heterogeneous enhancement | Large, well-circumscribed, solid with meningeal attachment; T1 hypointense, T2 hyperintense, low ADC, heterogeneous enhancement | Limited data: no meningeal attachment, not well circumscribed, T2 hyperintense, mild enhancement |
Histopathology | High-grade solid tumor with perivascular pseudorosettes and microcysts | High-grade solid tumor with perivascular pseudorosettes and microcysts | Infiltrative tumor Variable grade (low in 2 cases, high in 2 cases) |
Immunohistochemistry | GFAP-/Olig2+/EMA-/Neuronal markers+/BCOR+ | GFAP-/Olig2+/EMA-/Neuronal markers+/BCOR- | GFAP+/Olig2+/Neuronal markers-/BCOR+ |
DNA-methylation class | HGNET-BCOR | HGNET-BCOR | LGG-MYB/MYBL1 |
Outcome | 65% recurrences Median PFS = 12.5 months 30% dead at the end of follow-up Median OS = 26 months | 0% recurrences 0% dead at the end of follow-up Median OS = 27 months | 100% recurrences Median PFS = 4.0 months 0% dead at the end of follow-up Median OS = 7 months |