|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
In recent years, a novel small round cell sarcoma harboring EWSR1-NFATC2 translocation with immunomorphologic overlap with Ewing sarcoma (ES), myoepithelial tumors, and extraskeletal myxoid chondrosarcoma has emerged.
|
30994538 |
2019 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
To gain insights on this issue, a series of EMC samples (7 EWSR1-NR4A3 and 5 TAF15-NR4A3) were transcriptionally profiled.
|
31020999 |
2019 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Systemic manifestations of extraskeletal myxoid chondrosarcoma associated with a novel t(2;22)(q34;q12) EWS translocation in a child and a review of the literature.
|
30776935 |
2019 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
EMC is characterized by the rearrangement of the NR4A3, which, in most cases (about 62-75%), is fused with EWSR1 and less frequently with other partners, including TAF15 (27%), TCF12 (4%), TFG, and FUS.
|
28383167 |
2017 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
EWSR1 rearrangements were first identified in Ewing sarcoma, but the spectrum of EWSR1-rearranged neoplasms now includes many soft tissue tumour subtypes including desmoplastic small round cell tumour (DSRCT), myxoid liposarcoma (MLPS), extraskeletal myxoid chondrosarcoma (EMC), angiomatoid fibrous histiocytoma (AFH), clear cell sarcoma (CCS) and myoepithelial neoplasms.
|
28141799 |
2017 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The EWSR1 rearrangement was detected in 20 of 51 (39%) cases of CCMC, in 5 of 21 (24%) cases of CCMCexPA, in 1 of 11 (9%) cases of EMC, and in 4 of 5 (80%) cases of hyalinizing clear cell carcinoma.
|
25581728 |
2015 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Extraskeletal myxoid chondrosarcoma with non-EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology.
|
24746215 |
2014 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Extraskeletal myxoid chondrosarcoma is characterized by the reciprocal chromosomal translocation t(9;22) and the resultant fused gene EWS RNA-binding protein 1 and nuclear receptor subfamily 4, group A, member 3 (EWSR1-NR4A3).
|
24508382 |
2014 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Extraskeletal myxoid chondrosarcoma (EMC) is a rare mesenchymal neoplasm with a characteristic translocation usually involving NR4A3 and EWSR1.
|
23588370 |
2013 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
This is the first reported case of a remarkable soft tissue sarcoma that exhibits overlapping morphologic features between SS and EMC and that also harbors a combination of SS18-SSX2 and EWS-NR4A3 gene fusions.
|
22743288 |
2012 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Most, although not all, examples of EMC possess a unique balanced chromosomal translocation [t(9;22)(q22;q12)] between the EWSR1 and NR4A3 (previously termed TEC) genes.
|
22804337 |
2012 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
We have performed microarray analyses of these cells and identified several genes overexpressed in the presence of EWS/NR4A3 which are also overexpressed in EMC tumors.
|
22592656 |
2012 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The SEER database was queried to find cases of mesenchymal tumors associated with EWSR1 translocations: Ewing sarcoma; clear cell sarcoma; extraskeletal myxoid chondrosarcoma; myxoid liposarcoma; desmoplastic small round cell tumor; and myoepithelial tumor.
|
21212061 |
2011 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
The NR4A3 nuclear receptor is implicated in the development of extraskeletal myxoid chondrosarcoma (EMC), primitive sarcoma unrelated to conventional chondrosarcomas, through a specific fusion with EWSR1 resulting in an aberrant fusion protein that is thought to disrupt the transcriptional regulation of specific target genes.
|
18855877 |
2009 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
FusionGene
|
disease |
ORPHANET |
These results suggest that the overall expression of PPARG in EMC may be regulated in part by the balance between EWSR1/NR4A3 and NR4A3, and that PPARG may play a crucial role in the development of these tumours.
|
18855877 |
2009 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
To that end, dual-color, break-apart fluorescence in situ hybridization (FISH) probes spanning the genomic regions of EWSR1 (22q12), DDIT3 (12q13), and FUS (16p11) (Vysis, Downer's Grove, IL) were evaluated in formalin-fixed, paraffin-embedded tissues from myxoid neoplasms, including intramuscular myxoma (n=10), myxoid liposarcoma (n=18), low-grade fibromyxoid sarcoma (n=10), extraskeletal myxoid chondrosarcoma (n=13), and myxofibrosarcoma (n=8).
|
18162764 |
2008 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
We examined the diagnostic utility of fluorescence in situ hybridization for extraskeletal myxoid chondrosarcoma using the LSI EWSR1 break-apart probe (Abbott Molecular/Vysis, Des Plaines, IL, USA).
|
18587326 |
2008 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
SMARCB1/INI1 protein expression in round cell soft tissue sarcomas associated with chromosomal translocations involving EWS: a special reference to SMARCB1/INI1 negative variant extraskeletal myxoid chondrosarcoma.
|
18580682 |
2008 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Human extraskeletal myxoid chondrosarcoma (EMC) is caused by a chromosomal translocation that involves TEC (translocated in extraskeletal myxoid chondrosarcoma), and either EWS (Ewing's sarcoma) or hTAF(II)68 (human TATA-binding protein-associated factor II 68), which generates EWS-TEC or hTAF(II)68-TEC fusion proteins, respectively.
|
18330902 |
2008 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Several observations suggest that one role of EWS/NOR1 in EMC may be to deregulate the expression of specific genes involved in the tumoral process.
|
16112421 |
2005 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Significant structural homology of EWS or TAF2N to TLS (or FUS) prompted us to investigate a potential novel gene fusion of NOR1 to TLS in EMCs without detectable known NOR1 fusions.
|
15188455 |
2004 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
These results support the hypothesis that the role of EWS/NOR-1 in EMC may be to disrupt the proliferation properties of cells involved in chondrogenesis.
|
15212958 |
2004 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Extraskeletal myxoid chondrosarcoma (EMC) is a rare mesenchymal tumor cytogenetically characterized by reciprocal translocations, such as t(9;22)(q22;q12) and t(9;17)(q22;q11), which result in EWSR1/NR4A3 and TAF15/NR4A3 fusion genes (alias EWS/NOR1, TAF2N/NOR1), respectively.
|
15262426 |
2004 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
NOR-1 is normally involved in the balance between cell proliferation and cell death, and is implicated in oncogenesis as part of the EWS/NOR-1 fusion protein found in human extraskeletal myxoid chondrosarcoma (EMC) tumors.
|
12543801 |
2003 |
|
Extraskeletal Myxoid Chondrosarcoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
The EWS/TEC fusion protein encoded by the t(9:22) chromosomal translocation in human extraskeletal myxoid chondrosarcoma tumors is thought to participate in the tumoral process at least in part by deregulating the expression of specific target genes involved in the control of cell proliferation.
|
12049818 |
2002 |