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Genes (11)
Species: human : 11 | |
Human | SLC29A4 | 222962 | solute carrier family 29 (equilibrative nucleoside transporter), member 4 | identification of ENT4 as a highly expressed transcript in desmoplastic small round cell tumor (DSRCT) may represent an attractive pathway for targeting chemotherapeutic drugs into DSRCT | Human | MSLN | 10232 | mesothelin | In the group of small round blue cell tumors, only desmoplastic small round cell tumors exhibited mesothelin positivity (7 of 12). | Human | WT1 | 7490 | Wilms tumor 1 | 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 | Human | PDGFA | 5154 | platelet-derived growth factor alpha polypeptide | The EWS-WT1 translocation product induces PDGFA in desmoplastic small round-cell tumour. | Human | PAX3 | 5077 | paired box 3 | To investigate the structure and potential diagnostic utility of the detection of EWS-WT1 chimeric RNA in desmoplastic small round cell tumor, 12 examples of this entity and 49 other tumors that enter in its differential diagnosis were studied by reverse transcriptase polymerase chain reaction for the presence of EWS-WT1, EWS-FLI-1, PAX3-FKHR, and PAX7-FKHR chimeric transcripts. | Human | MYOG | 4656 | myogenin (myogenic factor 4) | Although nuclear reactivity of the early myogenic regulatory factors (MyoD, myogenin, Myf5) was not detected, myoglobin immunoreactivity was present in 29% of desmoplastic small round cell tumors. | Human | MYF5 | 4617 | myogenic factor 5 | Although nuclear reactivity of the early myogenic regulatory factors (MyoD, myogenin, Myf5) was not detected, myoglobin immunoreactivity was present in 29% of desmoplastic small round cell tumors. | Human | CD99 | 4267 | CD99 molecule | Desmoplastic small round cell tumors showed reactivity with calretinin in 4/21, desmin in 21/23, myoglobin in 5/17, placental alkaline phosphatase in 17/21, HER2/neu in 7/18 (3+ in 1 and 1+ in 6), c-kit in 2/14, MIC2 in 13/23, WT1 in 16/23, CAM5.2 in 21/23, and AE1/3 in 16/23 cases. Further pathological examination revealed that the membrane of the tumor cells was positive for MIC-2, and negative for epithelial membrane antigen, cytokeratin, and desmin, which are usually positive in intra-abdominal desmoplastic small round-cell tumors. In the case of NB84-positive tumors other than neuroblastoma, their specific reactivity for other markers was useful (Ewings sarcoma CD99 positive, desmoplastic small round cell tumor desmin and keratin positive). | Human | FUS | 2521 | fused in sarcoma | Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewing;s sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). | Human | FLI1 | 2313 | Fli-1 proto-oncogene, ETS transcription factor | FLI-1 expression was seen in 29 of 41 (71%) ES/PNET, 7 of 8 (88%) lymphoblastic lymphomas, 0 of 8 poorly differentiated synovial sarcomas (PDSS), 0 of 32 rhabdomyosarcoma (RMS), 0 of 30 neuroblastomas, 0 of 8 esthesioneuroblastomas, 0 of 3 Wilms tumors, 0 of 1 mesenchymal chondrosarcoma, and in 1 of 1 desmoplastic round cell tumor. Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewings sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). | Human | ATF1 | 466 | activating transcription factor 1 | Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewings sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). Different classes of DNA binding proteins, ATF1, WT1 and CHOP are fused to EWS generating distinct tumor phenotypes: clear cell sarcoma, desmoplastic small round cell tumor, and myxoid liposarcoma, respectively. |
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