Desmoplastic small round cell tumor (DSRCT): emerging therapeutic targets and future directions for potential therapies
نویسندگان
چکیده
منابع مشابه
Desmoplastic Small Round Cell Tumor (DSRCT)
Desmoplastic small round cell tumor (DSRCT) is an aggressive malignant neoplasm that occurs in adolescents and young adults. This tumor can co-express epithelial, neuronal, and mesenchymal markers. Clinical manifestations are often related to widespread abdominal disease. Distant metastases can be present at the time of diagnosis. The molecular hallmark of DSCRT is the EWS-WT1 fusion protein. T...
متن کاملDesmoplastic small round cell tumor (DSRCT) xenografts and tissue culture lines: Establishment and initial characterization
Desmoplastic small round cell tumor (DSRCT) is an extremely rare and aggressive neoplasm, which mainly affects young males and generally presents as a widely disseminated tumor within the peritoneal cavity. Due to the rarity of the tumor, its younger and overall healthier patient population (compared with other tumor types) and the fact that it lacks definitive histological and immunohistologic...
متن کاملIntra-abdominal desmoplastic small round cell tumor
BACKGROUND The desmoplastic small round cell tumour is a rare and aggressive intra-abdominal neoplasia, with only 200 cases reported, and a higher incidence in men and predilection for the second decade of life. Histologically characterized by the presence of small nests of undifferentiated tumour cells, wrapped in fibrous desmoplastic stroma. CLINICAL CASE A 24 year old male started with abd...
متن کاملDesmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations
Desmoplastic small round blue cell tumors (DSRCTs) originate from a cell with multilineage potential. A molecular hallmark of DSRCT is the EWS-WT1 reciprocal translocation. Ewing sarcoma and DSRCT are treated similarly due to similar oncogene activation pathways, and DSRCT has been represented in very limited numbers in sarcoma studies. Despite aggressive therapy, median survival ranges from 17...
متن کاملDesmoplastic Small Round Cell Tumor of Stomach
Desmoplastic small round cell tumor (DSRCT) is an extremely uncommon, highly aggressive, and malignant mesenchymal neoplasm of undetermined histogenesis. Less than 200 case reports have been documented in literature so far. Herein, we report a 26-year-old otherwise healthy female patient who presented with a 1-month history of epigastric pain. On physical examination, a palpable, slightly mobil...
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ژورنال
عنوان ژورنال: Expert Opinion on Therapeutic Targets
سال: 2020
ISSN: 1472-8222,1744-7631
DOI: 10.1080/14728222.2020.1738392