Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities

Acta Neuropathol Commun. 2023 Feb 3;11(1):22. doi: 10.1186/s40478-023-01522-z.


The fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System (CNS) now includes mesenchymal tumors that occur uniquely or frequently in the CNS. Moreover, this version has aligned the terminology of mesenchymal tumors with their soft tissue counterparts. New tumor types have been added, such as the "intracranial mesenchymal tumor, FET-CREB fusion-positive", the "CIC-rearranged sarcoma", and the "Primary intracranial sarcoma, DICER1-mutant". Other entities (such as rhabdomyosarcoma) have remained in the current WHO classification because these tumor types may present specificities in the CNS as compared to their soft tissue counterparts. Based on an extensive literature review, herein, we will discuss these newly recognized entities in terms of clinical observation, radiology, histopathology, genetics and outcome, and consider strategies for an accurate diagnosis. In light of this literature analysis, we will also introduce some potentially novel tumor types.

Keywords: Central nervous system; Classification; DNA-methylation profiling; Mesenchymal.

Publication types

  • Review

MeSH terms

  • Brain Neoplasms* / diagnosis
  • Brain Neoplasms* / genetics
  • Central Nervous System / pathology
  • Central Nervous System Neoplasms* / diagnosis
  • Central Nervous System Neoplasms* / genetics
  • DEAD-box RNA Helicases
  • Humans
  • Ribonuclease III
  • Sarcoma* / pathology


  • DICER1 protein, human
  • Ribonuclease III
  • DEAD-box RNA Helicases