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Intracerebral small round cell tumor: an unusual case with EWS-WT1 translocation
Karim Bouchireb1, Nathalie Auger, Ranjeev Bhangoo
1Department of Pediatric and Adolescent Oncology, Institut Gustave Roussy, Villejuif, France.
Pediatric Blood & Cancer
|June 19, 2008
Abstract:
Desmoplastic small round cell tumor (DSRCT) is a rare tumor, seen both in children and young adults with a marked predilection for the peritoneal cavity. Histology showed a small round cell tumor with a fibromyxoïd stroma and immunohistochemistry indicated neural and mesenchymal differentiation, and diagnosis was made by molecular detection of the EWS-WT1 fusion gene product. DSRCT should be considered in the differential diagnosis of intracranial small round cell tumors.