Although telomeres are maintained in most @entity5 by telomerase activation, a subset of @entity5 utilize @entity10205 ( @entity10205 ) to sustain self-renewal capacity. In order to study the prevalence and significance of @entity10205 in childhood @entity1313 we screened 517 pediatric @entity1313 using the novel C-circle assay. We examined the association of @entity10205 with alterations in genes found to segregate with specific histological phenotypes and with clinical outcome. @entity10205 was detected almost exclusively in @entity5 (p  =  0.001). @entity10205 was highly enriched in primitive @entity4969 (12  %), @entity9204 (23  %) and high-grade @entity11 (22  %). Furthermore, in contrast to @entity11 , pediatric low grade @entity11 which progressed to high-grade @entity5 did not exhibit the @entity10205 phenotype. Somatic but not germline @entity876 mutations were highly associated with @entity10205 (p  =  1.01     10(-8)). Of the other alterations examined, only @entity8171 point mutations and reduced expression were associated with the @entity10205 phenotype (p  =  0.0005). Interestingly, @entity10205 attenuated the poor outcome conferred by @entity876 mutations in specific pediatric @entity1313 . Due to very poor prognosis, one year overall survival was quantified in @entity11 , while in @entity1 with @entity9204 , five year overall survival was investigated. For @entity1 with @entity876 mutant @entity11 , one year overall survival was 63      12 and 23      10  % for @entity10205 positive and negative @entity5 , respectively (p  =  0.03), while for @entity1 with @entity876 mutant @entity9204 , 5  years overall survival was 67      19 and 27      13  % for @entity10205 positive and negative @entity5 , respectively (p  =  0.07). These observations suggest that the presence of @entity10205 is limited to a specific group of @entity1313 which harbor somatic @entity876 mutations and may influence the outcome of these @entity1 . Analysis of @entity10205 may contribute to risk stratification and targeted therapies to improve outcome for these @entity1 .

Alternative lengthening of telomeres is enriched in, and impacts survival of XXXX mutant pediatric @entity1313 .

@entity1 :: ('9606', 'Species') :: ['children', 'patients'] @entity10205 :: ('MESH:C536801', 'Disease') :: ['alternative lengthening of telomeres', 'ALT'] @entity8171 :: ('546', 'Gene') :: ['ATRX'] @entity4969 :: ('MESH:D017599', 'Disease') :: ['neuroectodermal tumors'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumors', 'cancers', 'malignant tumors'] @entity1313 :: ('MESH:D001932', 'Disease') :: ['brain tumors', 'childhood brain cancers'] @entity11 :: ('MESH:D005910', 'Disease') :: ['adult gliomas', 'malignant gliomas', 'gliomas'] @entity876 :: ('7157', 'Gene') :: ['TP53'] @entity9204 :: ('MESH:C562943', 'Disease') :: ['choroid plexus carcinomas', 'choroid plexus carcinoma']
@entity876 :: (7157,Gene) :: ['TP53']
