Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings

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Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Patients typically present with symptoms of abdominal sarcomatosis. Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic stroma. DSRCT is associated w...

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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...

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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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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...

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ژورنال

عنوان ژورنال: Sarcoma

سال: 2012

ISSN: 1357-714X,1369-1643

DOI: 10.1155/2012/714986