Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Furthermore, molecular detection of EWSR1-WT1 fusion gene conclusively confirmed the diagnosis of DSRCT in this uncommon location.
|
31103034 |
2019 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
<b>Purpose:</b> Desmoplastic small round cell tumor (DSRCT), which harbors EWSR1-WT1 t(11;22)(p13:q12) chromosomal translocation, is an aggressive malignancy that typically presents as intra-abdominal sarcomatosis in young males.
|
29871905 |
2018 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Desmoplastic Small Round Cell Tumor (DSRCT) is a rare sarcoma tumor of adolescence and young adulthood, which harbors a recurrent chromosomal translocation between the Ewing's sarcoma gene (EWSR1) and the Wilms' tumor suppressor gene (WT1).
|
29626752 |
2018 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Twenty-six specimens (from 25 patients) histologically diagnosed as DSRCT were assessed for EWSR1 rearrangement and EWSR1-WT1 fusion transcripts.
|
28748349 |
2017 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
Biomarker
|
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Using JN-DSRCT-1, a cell line derived from DSRCT expressing the EWS-WT1 fusion protein, we investigated the ability of trabectedin to modify the function of the chimeric protein, as in other sarcomas expressing fusion proteins.
|
28166781 |
2017 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
In these challenging cases, detection of an EWSR1-WT1 rearrangement and selective WT1 carboxy-terminus immunoreactivity (characteristic of DSRCT) or dual immunoreactivity for the WT1 amino-terminus and carboxy-terminus (characteristic of WT) remain the most discriminating diagnostic tools.
|
24832162 |
2014 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
We found disagreement in only three samples: one ES/pPNET and one embryonal rhabdomyosarcoma harbor a PAX3-FOXO1 translocation (for ARMS), and one neuroepithelioma harboring a EWS-WT1 (for DSRCT).
|
24486246 |
2014 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The presence of the fusion gene EWSR1-WT 1 transcript was identified in all, which confirmed the diagnosis of DSRCT.
|
24639098 |
2014 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Fluorescence in situ hybridization studies using an EWSR1 break-apart probe confirmed the presence of a rearrangement involving the EWSR1 locus and RT-PCR demonstrated the presence of an EWSR1-WT1 fusion transcript associated with the t(11;22) rearrangement, which supported a diagnosis of DSRCT.
|
24953786 |
2014 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
These data provide novel insights into the function of the EWS/WT1 fusion protein which characterize DSRCT.
|
24321497 |
2013 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
The molecular hallmark of DSRCT is the EWS-WT1 fusion protein.
|
22215508 |
2012 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Detection of the EWS-WT1-GFT plays a major role in the diagnosis of DSRCT.
|
22162435 |
2012 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Diagnosis of a DSRCT was confirmed on molecular analysis with positive -RT-PCR and sequencing results for EWS-WT1 transcript and negativity for EWS-FL1.
|
20739766 |
2010 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Herein, we describe two additional examples, fulfilling the morphologic, immunohistochemical, and molecular criteria (EWS/WT1 translocation) of DSRCT.
|
19263077 |
2009 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
Our detailed analyses of a novel target of EWS/WT1 in DSRCT reveal an insight into the oncogenic mechanism of EWS-fusion chromosomal translocation gene products and provide a new marker for DSRCT.
|
18523561 |
2008 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
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 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Desmoplastic small cell tumor of soft tissue: molecular variant of EWS-WT1 chimeric fusion.
|
16930335 |
2006 |
Desmoplastic Small Round Cell Tumor
|
0.400 |
Biomarker
|
disease |
BEFREE |
A case of desmoplastic small round cell tumour (DSRCT) expressing only an unusual EWS-WT1 treated by us is reported here.
|
17021139 |
2006 |