These novel entities differ in their anatomical location, age at incidence and histological features, and are marked by distinct pathognomonic structural variants, and are described as CNS neuroblastoma with FOXR2 activation (CNS NB-FOXR2), CNS Ewing sarcoma family tumour with CIC alteration (CNS EFT-CIC), CNS high-grade neuroepithelial tumour with BCOR alteration (CNS HGNET-BCOR), and CNS high-grade neuroepithelial tumour with MN1 alteration (CNS HGNET-MN1). Here, FOXR2 is linked to Ewing sarcoma/peripheral primitive neuroectodermal tumor.