Atypical Teratoid Rhabdoid Tumour : From Tumours to Therapies
Journal of Korean Neurosurgical Society
;
: 302-311, 2018.
Artigo
em Inglês
| WPRIM
| ID: wpr-788691
ABSTRACT
Atypical teratoid rhabdoid tumours (ATRTs) are the most common malignant central nervous system tumours in children ≤1 year of age and represent approximately 1–2% of all pediatric brain tumours. ATRT is a primarily monogenic disease characterized by the bi-allelic loss of the SMARCB1 gene, which encodes the hSNF5 subunit of the SWI/SNF chromatin remodeling complex. Though conventional dose chemotherapy is not effective in most ATRT patients, high dose chemotherapy with autologous stem cell transplant, radiotherapy and/or intrathecal chemotherapy all show significant potential to improve patient survival. Recent epigenetic and transcriptional studies highlight three subgroups of ATRT, each with distinct clinical and molecular characteristics with corresponding therapeutic sensitivities, including epigenetic targeting, and inhibition of tyrosine kinases or growth/lineage specific pathways.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Fosfotransferases
/
Radioterapia
/
Células-Tronco
/
Tirosina
/
Encéfalo
/
Neoplasias Encefálicas
/
Proteínas Tirosina Quinases
/
Sistema Nervoso Central
/
Montagem e Desmontagem da Cromatina
/
Tratamento Farmacológico
Tipo de estudo:
Estudo prognóstico
Limite:
Criança
/
Humanos
Idioma:
Inglês
Revista:
Journal of Korean Neurosurgical Society
Ano de publicação:
2018
Tipo de documento:
Artigo
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