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.
(c) 2008 Wiley-Liss, Inc.