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Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients.J Formos Med Assoc. 2007 Oct;106(10):854-60

BACKGROUND/PURPOSE: Desmoplastic small round cell tumor (DSRCT) is a rare but highly aggressive tumor that usually occurs in the abdominal cavity of young males. Immunohistochemical staining plays an important role in the differentiation of DSRCT from other small round cell neoplasms. Recently, a novel translocation of chromosome 11 and 22 [t(11;22)(p13+q12)] was identified in DSRCTs, which could result in the formation of the chimeric EWS-WT1 fusion gene. Reverse transcriptasepolymerase chain reaction (RT-PCR) has been applied to detect the fusion gene in fresh tissue and confirm the diagnosis. Only a few studies using RT-PCR with formalin-fixed and paraffin-embedded (FFPE) tissue sections to detect the specific transcript have been reported. METHODS: We collected four patients who had been diagnosed with DSRCT from the archives of the Department of Pathology, National Taiwan University Hospital, between 1996 and 2006. The clinical information and histologic sections of the patients were reviewed. Panel of primary antibodies including cytokeratin, vimentin, desmin and WT-1 were used for immunohistochemical staining. RNA extracted from the FFPE tissue sections was used for RT-PCR to identify the transcript of EWS-WT1. RESULTS: All four patients were male, with a mean age of 24 years. All of the tumors originated in the abdominal cavity and liver metastases were found in two patients. Two patients died of disease within 2 years, and the other two remained alive with disease. Histologically, the tumors were composed of nests of primitive small round cells within a desmoplastic stroma. Immunohistochemically, all the tumor cells were reactive to cytokeratin, vimentin, desmin and WT-1. The characteristic EWS-WT1 fusion gene could be identified in the FFPE specimens of all three patients who had tissue sections available for RT-PCR analysis. CONCLUSION: DSRCT is an aggressive tumor that usually occurs in the peritoneum of young males. The tumor is characterized by the expression of cytokeratin, vimentin, desmin and WT-1. RT-PCR, using paraffin-embedded tissue sections, can effectively detect the characteristic EWS-WT1 fusion gene transcript.

Desmoplastic small round cell tumour

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Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors.Am J Surg Pathol. 2002 Jul;26(7):823-35

Desmoplastic small round cell tumor is a rare, aggressive neoplasm that mainly affects young male patients and is characterized by a reciprocal translocation t(11;22)(p13;q12) associated with the EWS-WT1 gene fusion transcript. Clinical, histopathologic, immunohistochemical, and molecular genetics features were reviewed for 32 tumors. There were 29 male and three female patients, with ages from 6 to 54 years (mean, 25 years). The main clinical signs and symptoms included abdominal pain (eight patients), weight loss (five patients), and presence of umbilical hernia (four patients). Two tumors primarily involved the ethmoid sinus and the soft tissues of the scalp; the other tumors (mean size, 10 cm) involved the abdominal cavity (88%). One patient presented initially with an axillary lymph node metastasis. Generally, all tumors showed the typical histologic findings of variably sized clusters of small, round, or spindled cells lying in a desmoplastic stroma. The neoplastic cells in formalin-fixed, paraffin-embedded tissue sections were positive for desmin (dot pattern) (81% of the cases), WT1 (91%), keratin (87%), neuron-specific enolase (84%), CD99 (23%), and actin (3%). The EWS-WT1 gene fusion transcript was detected in 29 of 30 tumors. One tumor with typical clinicopathologic and immunohistochemical features did not show the gene fusion. Follow-up for 27 patients showed that 19 patients (70%) died of uncontrolled, local, or widespread metastatic disease 3-46 months (mean, 20 months) after diagnosis, and eight patients were alive with known evidence of disease. Occasionally, desmoplastic small round cell tumor lacks the classic clinical, histologic, and immunohistochemical features. This study emphasizes the utility of analysis of the EWS-WT1 gene fusion transcript, which was performed on paraffin-embedded tissues, to confirm the diagnosis.

 
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