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/Zeitung › SCORING: Review › Forschung
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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 -