Expanding the spectrum of "mesenchymal" tumors of the central nervous system

Cristina Pizzimenti; Francesca Gianno; Marco Gessi

doi:10.32074/1591-951X-826

Expanding the spectrum of "mesenchymal" tumors of the central nervous system

Pathologica. 2022 Dec;114(6):455-464. doi: 10.32074/1591-951X-826.

Authors

Cristina Pizzimenti¹, Francesca Gianno², Marco Gessi³

Affiliations

¹ Department of Biomedical, Dental, Morphological and Functional Imaging Sciences, University of Messina, Italy.
² Department of Radiological, Oncological and Pathological sciences, University of Rome "la Sapienza" Rome Italy.
³ Neuropathology Unit, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.

Abstract

In this review, we summarize the clinical, histopathological, and molecular features of central nervous system (CNS) tumors with BCOR internal tandem duplication, intracranial mesenchymal tumor with FET/CREB fusion, CNS CIC-rearranged sarcomas and primary intracranial sarcoma DICER1-mutant, now included in the 2021 WHO classification of CNS tumors. Possible relationships between tumors occurring in the CNS and their systemic counterparts are discussed.

Keywords: CNS CIC-rearranged sarcomas; CNS tumor with BCOR internal tandem duplication; central nervous system; intracranial mesenchymal tumor with FET/CREB fusion; primary intracranial sarcoma DICER1-mutant.

Publication types

Review

MeSH terms

Biomarkers, Tumor
Brain Neoplasms*
Central Nervous System / pathology
Central Nervous System Neoplasms*
DEAD-box RNA Helicases
Humans
Neoplasms, Connective and Soft Tissue*
Proto-Oncogene Proteins
Repressor Proteins
Ribonuclease III
Sarcoma* / pathology

Substances

Proto-Oncogene Proteins
Repressor Proteins
Biomarkers, Tumor
DICER1 protein, human
Ribonuclease III
DEAD-box RNA Helicases