Tumor type | CNS WHO grade | Characteristic molecular genetic alterations |
Fibroblastic and myofibroblastic tumors | ||
Solitary fibrous tumor | 1, 2, 3 | NAB2-STAT6 |
Vascular tumors | ||
Cavernous hemangioma | NA | KRIT1 (CCM1), CCM2, PDCD10 (CCM3) |
Capillary hemangioma | NA | |
Arteriovenous malformation | NA | KRAS, BRAF |
Hemangioblastoma | 1 | VHL |
Skeletal muscle tumors | ||
Embryonal rhabdomyosarcoma | NA | Chromosome 8 gain |
Alveolar rhabdomyosarcoma | NA | FOXO1 |
Rhabdomyosarcoma, pleomorphic-type | NA | |
Spindle cell rhabdomyosarcoma | NA | NCOA2, VGLL2, MYOD1 |
Tumors of uncertain differentiation | ||
Intracranial mesenchymal tumor, FET-CREB fusion-positive* | NA | FET-CREB |
CIC-rearranged sarcoma | 4 | CIC |
Primary intracranial sarcoma, DICER1-mutant | NA | DICER1 |
Ewing sarcoma | 4 | FET-ETS |
Chondrogenic tumors | ||
Mesenchymal chondrosarcoma | NA | HEY1-NCOA2 |
Chondrosarcoma | 1, 2, 3 | IDH1, IDH2 |
Dedifferentiated chondrosarcoma | 1, 2, 3 | IDH1, IDH2 |
Notochordal tumors | ||
Chordoma | NA | SMARCB1 |
WHO: World Health Organization; CNS: central nervous system; NA: not assigned; NAB2: NGFI-A binding protein 2; STAT6: signal transducer and activator of transcription 6; CCM: cerebral cavernous malformation; PDCD10: programmed cell death 10; VHL: von Hippel-Lindau tumor suppressor; FOXO1: forkhead box O1; NCOA2: nuclear receptor coactivator 2; VGLL2: vestigial like family member 2; MYOD1: myogenic differentiation 1; CIC: capicua transcriptional repressor; HEY1: hes-related family bHLH transcription factor with YRPW motif 1; IDH: isocitrate dehydrogenase; SMARC: SWI/SNF related matrix-associated actin-dependent regulator of chromatin.
* Provisional tumor.Do you want to add Medilib to your home screen?