Article: SWI/SNF-deficient tumors of the central nervous system: An update.
2023 Volume 43, Issue 1, Page(s) 2–9
Abstract: Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor of the central nervous system characterized by biallelic inactivation of SWI/SNF chromatin remodeling complex members SMARCB1/INI1 or (rarely) SMARCA4/BRG1. Most high-grade central ... ...
Abstract | Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor of the central nervous system characterized by biallelic inactivation of SWI/SNF chromatin remodeling complex members SMARCB1/INI1 or (rarely) SMARCA4/BRG1. Most high-grade central nervous system lesions showing loss of nuclear SMARCB1 or SMARCA4 protein expression can indeed be categorized as AT/RT. However, some high-grade lesions have been identified, whose clinical and/or molecular features justify separation from AT/RT. Furthermore, other recently described tumor types such as desmoplastic myxoid tumor, SMARCB1-mutant, and low-grade diffusely infiltrative tumor, SMARCB1-mutant, may even manifest as low-grade lesions. Here, we review recent developments in the definition of the molecular landscape of AT/RT and give an update on other rare high- and low-grade SWI/SNF-deficient central nervous system tumors. |
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MeSH term(s) | Humans ; SMARCB1 Protein/genetics ; Rhabdoid Tumor/genetics ; Rhabdoid Tumor/pathology ; Neoplasms, Neuroepithelial ; Central Nervous System/pathology ; DNA Helicases/genetics ; Nuclear Proteins/genetics ; Transcription Factors/genetics |
Chemical Substances | SMARCB1 Protein ; SMARCA4 protein, human (EC 3.6.1.-) ; DNA Helicases (EC 3.6.4.-) ; Nuclear Proteins ; Transcription Factors |
Language | English |
Publishing date | 2023-11-16 |
Publishing country | Germany |
Document type | Review ; Journal Article |
ZDB-ID | 603167-5 |
ISSN | 0722-5091 |
ISSN | 0722-5091 |
DOI | 10.5414/NP301594 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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