|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion, resembling extraskeletal myxoid chondrosarcoma: Case report with a review of Literature.
|
23252871 |
2012 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
In conclusion, strong nuclear ERG immunoreactivity is specific for Ewing sarcomas with EWSR1-ERG rearrangement.
|
22766791 |
2012 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
A t(11;22)(p13;p12) chromosomal translocation, juxtaposing the Wilms' tumor (WT1) and Ewing's sarcoma (EWS) genes, is the cytogenetic hallmark of desmoplastic small round cell tumor (DSRCT), a primitive multiphenotypic sarcoma arising in serosal tissues.
|
9071577 |
1997 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
The EWSR1 and FLI1 genes are involved in balanced translocation t(11;22)(q24;q12), which is present in more than 85% of Ewing sarcomas.
|
16837212 |
2006 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
From the molecular point of view, these neoplasias are grouped into two main types: (a) sarcomas showing specific genetic alterations and relatively simple karyotypes, and translocations which originate gene fusions (e.g., EWS-FLI1 in Ewing tumour); or specific genetic mutations (e.g., c-kit in the gastrointestinal stromal tumour), and (b) sarcomas showing unspecific gene alterations and very complex karyotypes, and very numerous gains and losses.
|
17403624 |
2007 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Gene rearrangements involving the Ewing sarcoma breakpoint region 1 (EWSR1) gene are seen in a broad range of sarcomas and some nonmesenchymal neoplasms.
|
24993903 |
2014 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion: a new tumor entity.
|
21997693 |
2011 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
The current version now includes PEComatous tumors, myoepithelial tumors, and pulmonary myxoid sarcomas with <i>EWSR1-CREB1</i> translocation as new disease entities.
|
30775024 |
2019 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Desmoplastic small round cell tumor (DSRCT) is a primitive sarcoma characterized by a recurrent chromosomal translocation, t(11;22)(p13;q12), which fuses the 5' exons of the EWS gene to the 3' exons of the WT1 gene.
|
16730884 |
2007 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
In the present study, we determined the genomic breakpoint and characterized the chimeric transcript of the EWSR1-ETV4 fusion gene in two t(17;22) Ewing sarcomas.
|
9858836 |
1998 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Our findings therefore expand the spectrum of gene fusions that characterize LGFMS and suggest that the EWSR1 gene may substitute for the function of FUS in gene fusions of sarcoma.
|
23588368 |
2013 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
This paper reviews the molecular pathology and related morphological and clinical features of sarcomas with non-EWS translocations.
|
19396640 |
2010 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
The EWS/Fli-1 fusion gene, a product of the translocation t(11;22, q24;q12), is detected in 85% of Ewing sarcomas and primitive neuroectodermal tumors.
|
20648560 |
2011 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
The observation that the different translocation partners of the EWS gene are specifically associated with several distinct types of primitive sarcomas suggests a model in which the translocation partner supplying the DNA-binding domain confers the target specificity of the transcriptional activation mediated by these chimeric proteins, whereas the partner supplying the N-terminal domain and promoter region determines their transactivation potential and expression level.
|
7493135 |
1995 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Among the genes that can be rearranged, the EWSR1 gene has been identified as a partner in a wide variety of clinically and pathologically diverse sarcomas as well as some non-mesenchymal tumours.
|
24320889 |
2014 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
No EWS/FLI-1 fusion transcripts were detected in five cell lines derived from cases of pediatric sarcomas having a histological diagnosis other than Ewing's sarcoma/PNET.
|
8238248 |
1993 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
From a molecular point of view, these neoplasias are grouped into 2 main types: (a) sarcomas showing relatively simple karyotypes and translocations, which originate gene fusions (eg, EWS-FLI1 in Ewing sarcoma) or point mutations (eg, c-kit in the gastrointestinal tumors) and (b) sarcomas showing unspecific gene alterations, very complex karyotypes, and no translocations.
|
20418671 |
2010 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Study of alternative molecular alterations in EWSR1-negative undifferentiated small round cell sarcomas is clinically relevant, since CIC-DUX4 sarcomas seem to be a very aggressive subset with poor response to the presently used therapeutic regimens.
|
26386605 |
2015 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
We analyzed the status of SMARCB1/INI1 protein expression in 93 cases of sarcomas associated with chromosomal translocation involving EWS, comprising 52 Ewing's sarcoma/primitive neuroectodermal tumors, 24 extraskeletal myxoid chondrosarcomas (EMCS), 14 clear cell sarcomas of soft tissue, 2 desmoplastic small round cell tumors, and 1 myxoid/round cell liposarcoma.
|
18580682 |
2008 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Although we acknowledge that the present molecular classification of soft tissue tumours is much more complex than two decades ago, when EWSR1 gene rearrangements had been described as the hallmark of Ewing sarcoma, we make the strong argument that with very few exceptions, the prevalence of fusion transcripts in most sarcomas is such that they come to define these entities and can be used as highly specific molecular diagnostic markers in the right clinical and pathological context.
|
24378390 |
2014 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
This chapter will focus on the unique genetic and epigenetic changes which characterize these cancers, with an emphasis on translocation-associated sarcomas involving primary gene fusions with the RNA chaperone protein EWSR1.
|
27865462 |
2016 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
This constitutes a novel example of the high frequency of EWSR1 rearrangements in various types of sarcomas as well as of its ability to fuse with a large variety of partner genes.
|
19837262 |
2009 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
All 15 (100%) Ewing sarcomas/PNET and 4 clear cell sarcomas (4/4) harbored EWSR1 translocations.
|
25446247 |
2014 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
These findings are consistent with previous cytogenetic reports of a recurrent t(9;22)(q22-31;q11-12) in the myxoid variant of chondrosarcoma and reveal involvement of the EWS gene in a fourth type of human sarcoma.
|
7539287 |
1995 |
|
Malignant neoplasm of soft tissue
|
0.100 |
GeneticVariation
|
group |
BEFREE |
We propose to classify tumors with the appropriate phenotype and molecularly confirmed NR4A3/EWSR1 rearrangements as myxochondroid sarcoma, either osseous or extraskeletal variants.
|
23588370 |
2013 |