- Classifier list
- Reference groups (Classifier 11b4)
- HGNET, BCOR
- Name: Methylation class CNS high grade neuroepithelial tumor with BCOR alteration
- Description: The methylation class "CNS high grade neuroepithelial tumor with BCOR alteration" is mainly comprised of tumors with the histological diagnosis CNS embryonal tumor, NOS; anaplastic ependymoma and glioblastoma, IDH wildtype. Tumors can be located supratentorially or in the posterior fossa. The age at diagnosis is typically under 18 years with rare exceptions; median age is 5 years (range 1 to 26). Molecularly, most cases harbor an internal tandem duplication in the BCOR gene. Rare cases harbor BCOR mutations. Up to 20% of cases show a gain of chromosome 1q, otherwise no recurrent copy number changes are observed and most cases show a flat profile in copy number analysis.
- Histological diagnosis: CNS embryonal tumour, NOS (12; 54.5%); Anaplastic ependymoma (4; 18.2%)
- Localisation: supra (12; 75%), PF (4; 25%)
Sample count |
Ratio male/female |
Age [median (min-max)] |
23 |
0.92 |
5.0 (1.0 - 26.0) |