Desmoplastic small round cell tumor is dependent on the EWS-WT1 transcription factor
نویسندگان
چکیده
منابع مشابه
EWS-WT1 Chimeric Protein in Desmoplastic Small Round Cell Tumor is a Potent Transactivator of FGFR4
Desmoplastic small round cell tumor (DSRCT) is a rare but highly aggressive malignant tumor that typically involves the abdominal or pelvic peritoneum in children and young adults [1]. This tumor is characterized histologically by solid nests of small round-cell tumor cells expressing epithelial, muscular, and neural markers, surrounded by a dense reactive stroma. This tumor is also characteriz...
متن کاملFusion of the EWS and WT1 genes in the desmoplastic small round cell tumor.
The desmoplastic small round cell tumor (DSRCT) is a recently recognized type of primitive sarcoma defined by a predilection for young males, aggressive clinical behavior, widespread abdominal serosal involvement, and a primitive histological appearance with prominent desmoplasia and striking divergent, multilineage differentiation. Previous cytogenetic case reports have identified a recurrent ...
متن کاملVariant EWS-WT1 chimeric product in the desmoplastic small round cell tumor.
Chromosome translocations found in neoplasms often result in the creation of hybrid genes encoding chimeric proteins. Desmoplastic small round cell tumor (DSRCT) is a recently described aggressive malignancy associated with a unique chromosomal translocation t(11;22)(p13;q12). This translocation has recently been characterized, revealing the rearrangement and fusion of the WT1 gene on chromosom...
متن کاملMorphoproteomic Profiling of the Mammalian Target of Rapamycin (mTOR) Signaling Pathway in Desmoplastic Small Round Cell Tumor (EWS/WT1), Ewing’s Sarcoma (EWS/FLI1) and Wilms’ Tumor(WT1)
BACKGROUND Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma in adolescents and young adults. The hallmark of this disease is a EWS-WT1 translocation resulting from apposition of the Ewing's sarcoma (EWS) gene with the Wilms' tumor (WT1) gene. We performed morphoproteomic profiling of DSRCT (EWS-WT1), Ewing's sarcoma (EWS-FLI1) and Wilms' tumor (WT1) to better understand the signali...
متن کاملMolecular heterogeneity and function of EWS-WT1 fusion transcripts in desmoplastic small round cell tumors.
Desmoplastic small round cell tumor (DSRCT) is a primitive sarcoma with a consistent cytogenetic abnormality, t(11;22)(p13;q12). This chromosomal translocation generates a chimeric transcript that is formed by fusion of the 5' region of the Ewing's sarcoma gene, EWS, with the 3' DNA-binding segment of WT1, the Wilms' tumor suppressor gene. We collected 14 DSRCT tumor samples and examined the hy...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Oncogenesis
سال: 2020
ISSN: 2157-9024
DOI: 10.1038/s41389-020-0224-1