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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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
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author Chang Zhang
Hao Li
author_facet Chang Zhang
Hao Li
author_sort Chang Zhang
collection DOAJ
description 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.
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spelling doaj.art-deb8dc379d9b45548ac4cf373ec0c5e32022-12-22T02:38:51ZengWileyPediatric Investigation2574-22722022-06-016211112210.1002/ped4.12325Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumorsChang Zhang0Hao Li1Department of Neurosurgery Children's Hospital of Fudan University Shanghai ChinaDepartment of Neurosurgery Children's Hospital of Fudan University Shanghai ChinaABSTRACT 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.https://doi.org/10.1002/ped4.12325Atypical teratoid/rhabdoid tumorsSMARCB1SMARCA4SWI/SNF complexTargeted molecular therapy
spellingShingle Chang Zhang
Hao Li
Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors
Pediatric Investigation
Atypical teratoid/rhabdoid tumors
SMARCB1
SMARCA4
SWI/SNF complex
Targeted molecular therapy
title Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors
title_full Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors
title_fullStr Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors
title_full_unstemmed Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors
title_short Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors
title_sort molecular targeted therapies for pediatric atypical teratoid rhabdoid tumors
topic Atypical teratoid/rhabdoid tumors
SMARCB1
SMARCA4
SWI/SNF complex
Targeted molecular therapy
url https://doi.org/10.1002/ped4.12325
work_keys_str_mv AT changzhang moleculartargetedtherapiesforpediatricatypicalteratoidrhabdoidtumors
AT haoli moleculartargetedtherapiesforpediatricatypicalteratoidrhabdoidtumors