|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Fusions of ETS with the EWSR1 partner gene define many members of the Ewing family of tumors, including primitive neuroectodermal tumor (PNET).
|
31831298 |
2020 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Chromosomal translocation t(11;22)(q24:q12) results in the formation of EWS/FLI1 gene fusion, which is detected in approximately 90% of tumors of the Ewing family.
|
29581854 |
2018 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Fluorescence in situ hybridization was successful in all cases and confirmed EWSR1 rearrangement in 2 of 4 tumors demonstrating morphologic features of Ewing sarcoma/peripheral PNET and concurrent CD99 and Fli-1 expression.
|
28296680 |
2017 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Cytopathological findings of primary pulmonary Ewing family of tumors with EWSR1 translocation: A case report.
|
27766786 |
2016 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Ewing sarcoma breakpoint region 1 (EWSR1) gene is known to fuse with various partner genes to promote the development of the Ewing sarcoma family of tumors and other sarcomas.
|
27627705 |
2016 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
133 (66.5%) ESFT displayed one of the above EWSR1-ETS translocations.
|
27180056 |
2016 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
An origin in the head and neck and the presence of the typical EWS/FLI1, in conjunction with an opportunity for immediate treatment, may predict a relatively better prognosis for EFT in our case.
|
25755803 |
2015 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Ewing's Sarcoma Oncogene (ews) on chromosome 22q12 is encoding a ubiquitously expressed RNA-binding protein (EWS) with unknown function that is target of tumor-specific chromosomal translocations in Ewing's sarcoma family of tumors.
|
25688366 |
2015 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
EWS/FLI1 inhibition results in a novel adaptive response and suggests that targeting the IL6/STAT3 signaling pathway may increase the efficacy of ESFT therapies.
|
25092916 |
2014 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
The aims of this study were (1) to present the diverse clinicopathological and molecular profile of EFTs in our settings, (2) to identify a pragmatic approach for diagnosing EFTs, especially for application of ancillary techniques, namely RT-PCR for specific transcripts (EWS-FLI1, EWS-ERG) and FISH for EWSR1 gene rearrangement, in certain cases and (3) to show the utility of tissue microarray in establishing a new FISH test.
|
24293381 |
2014 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Using whole transcriptome sequencing, we find that 11% of tumors pathologically diagnosed as EFT lack a typical EWSR1 fusion oncogene and that these tumors do not have a characteristic Ewing sarcoma gene expression signature.
|
25010205 |
2014 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The Ewing sarcoma family of tumors (ESFT) is defined by cell surface expression of CD99 and a translocation involving EWS and an ETS partner.
|
23760780 |
2013 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
No association was found between drug resistance and the expression of EWS/ETS regulated genes in the EFT cell lines.
|
24312454 |
2013 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Nine different EWSR1-FLI1 fusion transcripts and one EWSR1-ERG fusion transcript were identified in 21 out of 23 fresh frozen EFT tissue samples.
|
23494411 |
2013 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults.
|
22420726 |
2012 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Ewing's sarcoma family of tumors (EFT) is characterized by the presence of chromosomal translocations leading to the expression of oncogenic transcription factors such as, in the majority of cases, EWS/FLI1.
|
22323082 |
2012 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Together, our findings offer a strong preclinical rationale to target the EWS-FLI1:PARP1 intersection as a therapeutic strategy to improve the treatment of ESFTs.
|
22287547 |
2012 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Using quantitative expression analysis in 16 ESFT and seven alveolar rhabdomyosarcomas (ARMS), we were able to validate the four genes previously described as direct targets of the EWSR1-FLI1 oncoprotein, showing overexpression of CAV1 and NR0B1 and underexpression of IGFBP3 and TGFBR2 in ESFT as compared to ARMS.
|
23185447 |
2012 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
The creation of a fusion between EWSR1 and an ETS family gene consecutive to a chromosomal translocation is characteristic of the Ewing family of tumors (EFT).
|
22429598 |
2012 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
In this study, we examined EWS-FLI-1 antigens for their capacity to induce immunity against a range of ESFT types.
|
22879388 |
2012 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
We show that ET-743 reverses a gene signature of induced downstream targets of EWS-FLI1 in two different ESFT cell lines (P = .001).
|
21403840 |
2011 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Ewing sarcoma family of tumors (ESFTs) are characterized by the t(11;22)(q24;q12) translocation that generates the Ewing sarcoma breakpoint region 1 and Friend leukemia virus integration 1 (EWS-FLI1) fusion transcription factor responsible for the highly malignant phenotype of this tumor.
|
21653923 |
2011 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The EWS/FLI1 translocations were detected in 7/8 (87.5%) ESFTs cases, whereas non of 8 cPNET cases were detected with this translocation.
|
21267687 |
2011 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
More than 90% of all Ewing's Sarcoma Family of Tumors (ESFT) exhibit specific chromosomal rearrangements between the EWS gene on chromosome 22 and various members of the ETS gene family of transcription factors.
|
20473914 |
2011 |
|
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
We conclude that in CNS PNET: (i) routine application of INI1 immunohistochemistry helps rule out AT/RT, particularly in infants; (ii) MYC gene amplifications (especially MYCN) are common; (iii) involvement of CNS parenchyma by Ewing sarcoma/peripheral PNET is rare enough that EWS gene testing is not necessary unless significant dural involvement is present; and (iv) both anaplastic/large cell features and polysomies of 2 and 8 are associated with more aggressive clinical behavior.
|
19725831 |
2010 |