Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors

ABSTRACT Atypical teratoid/rhabdoid tumors (AT/RTs) are lethal central nervous system tumors, which are primarily diagnosed in infants. Current treatments for AT/RTs include surgery, radiotherapy, and chemotherapy; these treatments have poor prognoses and challenging side effects. The pivotal geneti...

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Bibliographic Details
Main Authors: Chang Zhang, Hao Li
Format: Article
Language:English
Published: Wiley 2022-06-01
Series:Pediatric Investigation
Subjects:
Online Access:https://doi.org/10.1002/ped4.12325
Description
Summary:ABSTRACT Atypical teratoid/rhabdoid tumors (AT/RTs) are lethal central nervous system tumors, which are primarily diagnosed in infants. Current treatments for AT/RTs include surgery, radiotherapy, and chemotherapy; these treatments have poor prognoses and challenging side effects. The pivotal genetic event in AT/RT pathogenesis comprises the inactivation of SMARCB1 or SMARCA4. Recent epigenetic studies have demonstrated mutual and subtype‐specific epigenetic derangements that drive tumorigenesis; the exploitation of these potential targets might improve the dismal treatment outcomes of AT/RTs. This review aims to summarize the literature concerning targeted molecular therapies for pediatric AT/RTs.
ISSN:2574-2272