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- W2912287816 abstract "Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive neoplasm with poor prognosis. It has a predilection towards adolescent young males with estimated incidence between 0.2 and 0.5 per million per year. DSRCT usually presents as multiple widespread peritoneal and omental implants within the abdomen and pelvis. Peritoneal metastasis is almost always present at diagnosis and concurrent extraperitoneal metastases have been found in 47% of patients. Four good prognostic factors include absence of extra peritoneal metastasis, macroscopic resection of peritoneal disease, whole abdominopelvic radiotherapy and postoperative chemotherapy. We report a 25-year-old male who was admitted for constipation, abdominal distension, pain and a 20 lb unintentional weight loss. Physical examination was significant for a hard mass palpated in the left upper quadrant and distension of the abdomen. Vitals were stable and laboratory results were within normal limits. CT scan imaging revealed moderate ascites and multiple intraperitoneal solid tumors with a large mass compressing the sigmoid colon [Figure 1]. Following abdominal biopsy, he was diagnosed with desmoplastic small round cell tumor. Immunohistochemical stains showed expression of cytokeratin (Cam5.2 and AE1/AE3), desmin, CD99 and FLI1, and negative for WT1, S100, synaptophysin and chromogranin [Figure 2]. Though negative staining of WT1 is unusual for DSRCT, fluorescence in-situ hybridization (FISH) assay for EWS-WT1 rearrangement was positive, consistent with DSRCT. Since the number of variants reported is low, information on prognosis and therapeutic response is limited. Furthermore, the extent of tumor invasion in the abdomen of our patient led to extra luminal bowel obstruction.Figure: Sigmoid colon compression by extra luminal mass, denoted.Figure: (A) Tumor cells are immuno positive for AE1/AE3 (40x). (B) Tumor cells are immuno positive for desmin (40x). (C) Tumor cells are immuno positive for CD99 (40x). (D) Tumor cells are immuno positive for FLI 1 (40x)." @default.
- W2912287816 created "2019-02-21" @default.
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- W2912287816 date "2017-10-01" @default.
- W2912287816 modified "2023-09-25" @default.
- W2912287816 title "WT1 Negative Variant Desmoplastic Small Round Cell Tumor" @default.
- W2912287816 doi "https://doi.org/10.14309/00000434-201710001-02602" @default.
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