|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Although β-catenin immunostaining missed 4/6 WNT MBs, CTNNTB mutation analysis confirmed all WNT MB cases with amplifiable DNA.
|
31343993 |
2019 |
|
Adult Medulloblastoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Transient β-catenin transfection led to an increase in the β-catenin gene and protein expression in MB cell lines.
|
30374857 |
2018 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Here, we show that paracrine signals driven by mutant β-catenin in WNT-medulloblastoma, an essentially curable form of the disease, induce an aberrant fenestrated vasculature that permits the accumulation of high levels of intra-tumoral chemotherapy and a robust therapeutic response.
|
27050100 |
2016 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Standard neuropathological evaluation of medulloblastoma samples should include IHC of β-catenin because tumours with high nuclear accumulation of β-catenin most probably belong to the Wnt subgroup of medulloblastomas.
|
24894640 |
2015 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Methylation subgrouping and CTNNB1 mutation status represent robust tools for the risk stratification of medulloblastoma.
|
24791927 |
2014 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Using AGDEX analysis and k-means clustering, we show that the Blbp-cre::Ctnnb1(ex3)(Fl/+)Trp53 (Fl/Fl) mouse model fits well to human WNT medulloblastoma, and that, among various Myc- or Mycn-based mouse medulloblastomas, tumors in Glt1-tTA::TRE-MYCN/Luc mice proved to be most specific for human group 3 medulloblastoma.
|
24871706 |
2014 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Nuclear beta-catenin has been suggested as a marker for MB prognosis.
|
23094051 |
2012 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Several recurrent mutations were identified, both in known medulloblastoma-related genes (CTNNB1, PTCH1, MLL2, SMARCA4) and in genes not previously linked to this tumour (DDX3X, CTDNEP1, KDM6A, TBR1), often in subgroup-specific patterns.
|
22832583 |
2012 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
We identified twelve genes mutated at statistically significant frequencies, including previously known mutated genes in medulloblastoma such as CTNNB1, PTCH1, MLL2, SMARCA4 and TP53.
|
22820256 |
2012 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Recurrent mutations were detected in 41 genes not yet implicated in medulloblastoma; several target distinct components of the epigenetic machinery in different disease subgroups, such as regulators of H3K27 and H3K4 trimethylation in subgroups 3 and 4 (for example, KDM6A and ZMYM3), and CTNNB1-associated chromatin re-modellers in WNT-subgroup tumours (for example, SMARCA4 and CREBBP).
|
22722829 |
2012 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Genes and pathways expressed during embryonic development, including the Notch, Wnt/β-Catenin, TGF-β/BMP, Shh/Patched, and Hippo pathways are mutated, lost, or aberrantly regulated in a wide variety of human cancers, including skin, breast, blood, and brain cancers, including medulloblastoma.
|
21295689 |
2011 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
TP53 mutation status was not associated with unfavorable prognosis (P = .63) and was not linked to 17p allelic loss but was over-represented in the prognostically favorable WNT subgroup of MB as defined by CTNNB1 mutation (seven of 35 TP53-mutated tumors v 14 of 271 TP53 wild-type tumors; P = .005) and in tumors carrying high-level MYCN amplification (seven of 21 TP53-mutated tumors v 14 of 282 TP53 wild-type tumors; P = .001).
|
21060032 |
2010 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Here we provide evidence that a discrete subtype of medulloblastoma that contains activating mutations in the WNT pathway effector CTNNB1 (hereafter, WNT subtype) arises outside the cerebellum from cells of the dorsal brainstem.
|
21150899 |
2010 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Surprisingly, adult MBs with 6q deletion and nuclear beta-catenin activation did not share the excellent prognosis with their pediatric counterparts.
|
20479417 |
2010 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
The WNT/beta-catenin pathway has been implicated in many tumour types, including medulloblastoma.
|
19293793 |
2009 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Further studies are required to test if this could explain the radiosensitivity of MB and the favorable prognostic value of nuclear beta-catenin in this tumor.
|
18688572 |
2008 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
These observations support an important functional role of WNT/beta-catenin pathway in neuronal differentiation in medulloblastoma.
|
17455096 |
2007 |
|
Adult Medulloblastoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
1p/19q codeletion in grade 2 and 3 gliomas, nuclear beta-catenin expression in medulloblastoma) or response to the treatment (e.g. the methyl guanyl methyl transferase promoter methylation status).
|
16988585 |
2006 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
We tested the hypothesis that nuclear immunoreactivity for beta-catenin is a prognostic marker in medulloblastoma, and assessed the relationship between nuclear beta-catenin immunoreactivity and mutations of CTNNB1 and APC.
|
16258095 |
2005 |
|
Adult Medulloblastoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Control of beta-catenin/Tcf-directed transcription in medulloblastoma.
|
15176713 |
2004 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
A subset of cases is associated with colon cancer and APC germline mutations (Turcot syndrome), and APC and beta-catenin point mutations occur in up to 10% of sporadic cases, indicating the involvement of the Wnt pathway in the development of medulloblastoma.
|
12555076 |
2003 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
INTERVENTION OR TECHNIQUE: Immunostaining of tissue blocks for gene products involved in medulloblastoma differed in the two siblings for beta-catenin and was similar with staining for gli.
|
12182422 |
2002 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Disruption of these proteins could result in upregulation of the Wnt signaling and accumulation of beta-catenin, followed by cell proliferation and medulloblastoma oncogenesis.
|
12209999 |
2002 |
|
Adult Medulloblastoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Whereas APC mutations are rare in sporadic MBs, a hot-spot region of beta-catenin (CTNNB1) mutations was identified in a subset of MBs.
|
11585731 |
2001 |
|
Adult Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Nuclear beta-catenin staining was present in 9 of the sporadic tumors (18%) and in the 1 medulloblastoma from a Turcot's patient.
|
10759189 |
2000 |