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WHO classification of mesenchymal tumors involving the CNS

WHO classification of mesenchymal tumors involving the CNS
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.
Adapted from: WHO Classification of Tumours Editorial Board. Central nervous system tumours. Lyon (France): International Agency for Research on Cancer; 2021. (WHO classification of tumours series, 5th ed.; vol. 6).
Graphic 111774 Version 2.0

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