Newly Diagnosed Childhood Ependymoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The present finding indicates that the TP53 p.R337H germline mutation is uncommon in patients with EPN in Brazil and screening of pediatric patients RELA fusion EPN may be informative to better understand the role of TP53 germline mutations in the development and prognosis of these tumors.
|
31728854 |
2020 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
By DNA methylation profiling, tumors with MN1 or RELA rearrangement clustered with high-grade neuroepithelial tumor with MN1 alteration (HGNET-MN1) and RELA-fusion ependymoma, respectively.
|
30876455 |
2019 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Ependymoma with RELA fusion has been defined as a novel entity of the revised World Health Organization 2016 classification of tumors of the central nervous system (CNS), characterized by fusion transcripts of the RELA gene and consequent pathological activation of the NFkB pathway.
|
30325077 |
2019 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Molecular characteristics are also important for the diagnosis of several other CNS tumors, such as RELA fusion-positive subtype of ependymoma, atypical teratoid rhabdoid tumor (AT/RT), embryonal tumor with multilayered rosettes, and solitary fibrous tumor/hemangiopericytoma.
|
31124566 |
2019 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Role of Immunohistochemistry in the Identification of Supratentorial C11ORF95-RELA Fused Ependymoma in Routine Neuropathology.
|
29266023 |
2019 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We describe a novel RELA-fusion composed by a chimeric transcript C11orf95-LOC-RELA in a supratentorial WHO grade II EPN occurring in a 4-year-old child.
|
30631904 |
2019 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Furthermore, treatment of the RELA-fused EPN cell line with the Notch inhibitors impaired the Notch signaling expression and revealed that Notch axis is not essential for cell proliferation and survival in this setting.
|
31308481 |
2019 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
A De Novo Mouse Model of C11orf95-RELA Fusion-Driven Ependymoma Identifies Driver Functions in Addition to NF-κB.
|
29949764 |
2018 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Their model shows RELA-fusion-based de novo ependymoma tumorigenesis in the forebrain derived from neural stem cells.
|
29949754 |
2018 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Stable knockdown of LDOC1 in EPN cell lines resulted in a significant increase in gene transcription of v-rel avian reticuloendotheliosis viral oncogene homolog A, which correlated to an increase in NF-κB target genes.
|
28510691 |
2017 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Array-comparative genomic hybridization showed copy number abnormalities consistent with chromosomal instability without evidence of RELA- or YAP1-fusion-features most often seen in posterior fossa ependymoma group B.
|
28943417 |
2017 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Among the p53-positive ependymomas, the vast majority exhibited a RELA fusion leading to the hypothesis that p53 inactivation might be linked to RELA positivity.In order to assess the potential of p53 reactivation through MDM2 inhibition in ependymoma, we evaluated the effects of Actinomycin-D and Nutlin-3 treatment in two preclinical ependymoma models representing the high-risk subtypes PF-EPN-A and ST-EPN-RELA.
|
27556362 |
2016 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
C11orf95-RELA fusion present in a primary intracranial extra-axial ependymoma: Report of a case with literature review.
|
27121356 |
2016 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We here describe a case of a sarcoma developing in a patient previously treated with chemotherapy and radiation whose original ependymoma and recurrent sarcoma were both shown to carry the type 1 C11orf95-RELA fusion transcript indicating a monoclonal origin for both tumors.
|
25388523 |
2015 |
Newly Diagnosed Childhood Ependymoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
C11orf95-RELA fusion proteins translocated spontaneously to the nucleus to activate NF-κB target genes, and rapidly transformed neural stem cells--the cell of origin of ependymoma--to form these tumours in mice.
|
24553141 |
2014 |