|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In the new review all the tumors were re-classified as, ES (n=16), Ewing-like tumor with EWSR1 rearrangement and amplification and possible EWSR1-NFATC2 gene fusion (n=1), CIC-rearranged sarcomas or undifferentiated sarcoma, most consistent with CIC-rearranged sarcoma (n=7), sarcoma with BCOR-alteration or undifferentiated sarcoma, consistent with BCOR-associated sarcoma (n=3), neuroblastoma (n=2), unclassifiable neoplasm with neuroblastic differentiation (n=1), malignant rhabdoid tumor (n=2), lymphoblastic lymphoma (n=1), clear cell sarcoma of the gastrointestinal tract (n=1), small cell carcinoma (n=1), sclerosing rhabdomyosarcoma (n=1), desmoplastic small round cell tumor (n=1), malignant peripheral sheath nerve tumor (n=1), poorly-differentiated synovial sarcoma (n=1), Possible gastrointestinal stromal tumor/GIST with predominant round cells (n=1) and possible SMARCA4-deficient-sarcoma (n=1).
|
29661713 |
2018 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Here we present a strategy combining CRISPR-Cas9 technology and homology-directed repair to allow for the selection of human mesenchymal stem cells harboring the oncogenic translocation <i>EWSR1-WT1</i> found in the aggressive desmoplastic small round cell tumor.
|
28325870 |
2017 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Three cases (1.5%) revealed the SYT-SSX transcript for Synovial sarcoma, and one (0.5%) a EWSR1-WT1 transcript for Desmoplastic Small Round Cell tumor.
|
27180056 |
2016 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements.
|
22420726 |
2012 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Herein, we describe a desmoplastic small round cell tumor of soft tissue with an unusual pattern of WT1 expression associated with a novel variant EWS-WT1 fusion transcript.
|
18703217 |
2008 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Adenosine transporter ENT4 is a direct target of EWS/WT1 translocation product and is highly expressed in desmoplastic small round cell tumor.
|
18523561 |
2008 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
A novel EWS-WT1 gene fusion product in desmoplastic small round cell tumor is a potent transactivator of the insulin-like growth factor-I receptor (IGF-IR) gene.
|
16730884 |
2007 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
The current case emphasizes the utility of fluorescence in situ hybridization to demonstrate EWS-WT1 gene fusion in desmoplastic small round cell tumor with nonclassic morphologic and immunohistochemical features to avoid potential misdiagnosis.
|
17425400 |
2007 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
The EWS-WT1 gene fusion in desmoplastic small round cell tumor.
|
15826834 |
2005 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Other targets of the EWS-WT1 transcription factor other than PDGF-A may be directly responsible for the prominent tumor-associated desmoplasia seen in desmoplastic small round cell tumor.
|
15389255 |
2005 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Sequence confirmation of the EWS-WT1 fusion gene transcript in the peritoneal effusion of a patient with desmoplastic small round cell tumor.
|
14648792 |
2003 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Detection of the EWS-WT1 gene fusion is characteristic of desmoplastic small round cell tumor and has been used reliably in tumor diagnosis.
|
12640103 |
2003 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Desmoplastic small round cell tumor is a rare, aggressive neoplasm that mainly affects young male patients and is characterized by a reciprocal translocation t(11;22)(p13;q12) associated with the EWS-WT1 gene fusion transcript.
|
12131150 |
2002 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
WT1 is also expressed in mesothelial cells; a specific oncogenic chromosomal translocation fusing the N-terminal domain of the Ewing sarcoma gene EWS to the three C-terminal zinc fingers of WT1 underlies desmoplastic small round cell tumor, an abdominal tumor thought to arise from the peritoneal lining.
|
11237525 |
2001 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Detection of EWS-WT1 fusion mRNA in ascites of a patient with desmoplastic small round cell tumor by RT-PCR.
|
10029456 |
1999 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Variant EWS-WT1 chimeric product in the desmoplastic small round cell tumor.
|
9949226 |
1999 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Desmoplastic small round-cell tumor of the paratesticular region: report of an adult case with demonstration of EWS and WT1 gene fusion using paraffin-embedded tissue.
|
10430278 |
1999 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Novel breakpoints of the EWS gene and the WT1 gene in a desmoplastic small round cell tumor.
|
9797782 |
1998 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Intraabdominal desmoplastic small round cell tumor with EWS/ERG fusion transcript.
|
9706984 |
1998 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The desmoplastic small round cell tumor t(11;22) translocation produces EWS/WT1 isoforms with differing oncogenic properties.
|
9591781 |
1998 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Molecular variants of the EWS-WT1 gene fusion in desmoplastic small round cell tumor.
|
9646031 |
1998 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The potentially diagnostic chromosomal translocations and associated genes identified in STT include Ewing's/PNET t(11;22)(q24;q12)(FLI1;EWS), t(21;22)(q22;q12)(ERG; EWS); t(7;22)(p22;q12)(ETV1;EWS); desmoplastic small round cell tumor t(11;22)(p13;q12)(WT1;EWS); extraskeletal myxoid chondrosarcoma t(9;22)(q22;q12) (TEC(CHN);EWS); malignant ectomesenchymoma t(11;22)(q24;q12)(FLI1;EWS); alveolar rhabdomyosarcoma t(2;13)(q35;q14)(PAX-3;FKHR); t(1;13) (p36;q14)(PAX-7;FKHR); myxoid and round cell liposarcoma t(12;16)(q13;p11)(CHOP;TLS(FUS)); synovial sarcoma t(X;18)(p11;q11)(SSX1&2;SYT), and others.
|
9344317 |
1997 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
One desmoplastic small round cell tumor was found to have a variant EWS-WT1 chimeric product that included exon 8 of EWS EWS-FLI-1 chimeric RNA was present in all Ewing's sarcoma/primitive neuroectodermal tumor and not identified in any other tumor types, including desmoplastic small round cell tumor.
|
7495283 |
1995 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
These assays detect PAX3-FKHR and PAX7-FKHR chimeric transcripts in alveolar rhabdomyosarcoma, EWS-FLI1 and EWS-ERG chimeric transcripts in Ewing's sarcoma, and EWS-WT1 chimeric transcripts in desmoplastic small round cell tumor.
|
7530783 |
1995 |
|
Adult Desmoplastic Small Round Cell Tumor
|
0.100 |
Biomarker
|
disease |
BEFREE |
Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor.
|
8187063 |
1994 |