Rhabdoid tumors

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Rhabdoid tumors : clinical approaches and molecular targets for innovative therapy. / Kerl, Kornelius; Holsten, Till; Frühwald, Michael C.

in: PEDIATR HEMAT ONCOL, Jahrgang 30, Nr. 7, 10.2013, S. 587-604.

Publikationen: SCORING: Beitrag in Fachzeitschrift/ZeitungSCORING: ReviewForschung

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@article{0828c10bda0044d9b5984514779c7a51,
title = "Rhabdoid tumors: clinical approaches and molecular targets for innovative therapy",
abstract = "Rhabdoid tumors are rare but highly aggressive tumors with a predilection for infants and young children. The majority of these tumors harbor biallelic mutations in SMARCB1/INI1/hSNF5. Rather rare cases with mutations in other SWI/SNF core members such as BRG1 are on record. Rhabdoid tumors have only recently been registered and treated according to specifically designed treatment recommendations and in the framework of clinical trials. Within the last decade, prognosis has improved significantly but at least 50% of patients still relapse and subsequently almost inevitably succumb to their disease. This review summarizes past and current clinical approaches and presents an overview of the rationales for targeted therapy with potential for future clinical treatment trials for rhabdoid tumors. ",
keywords = "Chromosomal Proteins, Non-Histone/genetics, Clinical Trials as Topic, DNA Helicases/genetics, DNA-Binding Proteins/genetics, Humans, Mutation, Nuclear Proteins/genetics, Rhabdoid Tumor/genetics, SMARCB1 Protein, Transcription Factors/genetics",
author = "Kornelius Kerl and Till Holsten and Fr{\"u}hwald, {Michael C}",
year = "2013",
month = oct,
doi = "10.3109/08880018.2013.791737",
language = "English",
volume = "30",
pages = "587--604",
journal = "PEDIATR HEMAT ONCOL",
issn = "0888-0018",
publisher = "informa healthcare",
number = "7",

}

RIS

TY - JOUR

T1 - Rhabdoid tumors

T2 - clinical approaches and molecular targets for innovative therapy

AU - Kerl, Kornelius

AU - Holsten, Till

AU - Frühwald, Michael C

PY - 2013/10

Y1 - 2013/10

N2 - Rhabdoid tumors are rare but highly aggressive tumors with a predilection for infants and young children. The majority of these tumors harbor biallelic mutations in SMARCB1/INI1/hSNF5. Rather rare cases with mutations in other SWI/SNF core members such as BRG1 are on record. Rhabdoid tumors have only recently been registered and treated according to specifically designed treatment recommendations and in the framework of clinical trials. Within the last decade, prognosis has improved significantly but at least 50% of patients still relapse and subsequently almost inevitably succumb to their disease. This review summarizes past and current clinical approaches and presents an overview of the rationales for targeted therapy with potential for future clinical treatment trials for rhabdoid tumors.

AB - Rhabdoid tumors are rare but highly aggressive tumors with a predilection for infants and young children. The majority of these tumors harbor biallelic mutations in SMARCB1/INI1/hSNF5. Rather rare cases with mutations in other SWI/SNF core members such as BRG1 are on record. Rhabdoid tumors have only recently been registered and treated according to specifically designed treatment recommendations and in the framework of clinical trials. Within the last decade, prognosis has improved significantly but at least 50% of patients still relapse and subsequently almost inevitably succumb to their disease. This review summarizes past and current clinical approaches and presents an overview of the rationales for targeted therapy with potential for future clinical treatment trials for rhabdoid tumors.

KW - Chromosomal Proteins, Non-Histone/genetics

KW - Clinical Trials as Topic

KW - DNA Helicases/genetics

KW - DNA-Binding Proteins/genetics

KW - Humans

KW - Mutation

KW - Nuclear Proteins/genetics

KW - Rhabdoid Tumor/genetics

KW - SMARCB1 Protein

KW - Transcription Factors/genetics

U2 - 10.3109/08880018.2013.791737

DO - 10.3109/08880018.2013.791737

M3 - SCORING: Review article

C2 - 23848359

VL - 30

SP - 587

EP - 604

JO - PEDIATR HEMAT ONCOL

JF - PEDIATR HEMAT ONCOL

SN - 0888-0018

IS - 7

ER -