Rhabdoid tumors: clinical approaches and molecular targets for innovative therapy.
Pediatr Hematol Oncol
; 30(7): 587-604, 2013 Oct.
Article
em En
| MEDLINE
| ID: mdl-23848359
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.
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Base de dados:
MEDLINE
Assunto principal:
Fatores de Transcrição
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Proteínas Nucleares
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Proteínas Cromossômicas não Histona
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Tumor Rabdoide
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DNA Helicases
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Proteínas de Ligação a DNA
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Mutação
Idioma:
En
Ano de publicação:
2013
Tipo de documento:
Article