Genomic and Proteomic Alterations in Desmoplastic Small Round Blue-Cell Tumors
نویسندگان
چکیده
منابع مشابه
Desmoplastic 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 tumors in a young man.
A previously healthy 18-year-old man was admitted to our hospital with abdominal pain in September 2010. We performed a percutaneous biopsy of multiple intrahepatic masses. A diagnosis of desmoplastic small round cell tumors was confirmed based on the presence of a polyphenotypic immunoprofile (positivity for EMA, vimentin, cytokeratin, desmin and WT1) and characteristic EWS-WT1 gene fusion. Be...
متن کاملResults of multimodal treatment for desmoplastic small round cell tumors.
PURPOSE Desmoplastic small round cell tumors (DSRCTs) are rare aggressive neoplasms that frequently present with large symptomatic intraabdominal masses. We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT. METHODS Institutional Review Board permission was obtained. Sixty-six patie...
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 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: JCO Precision Oncology
سال: 2018
ISSN: 2473-4284
DOI: 10.1200/po.17.00170