Intracerebral small round cell tumor: an unusual case with EWS-WT1 translocation.
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.
Department of Pediatric and Adolescent Oncology, Institut Gustave Roussy, Villejuif, France.
Karim
K
Bouchireb
Nathalie
N
Auger
Ranjeev
R
Bhangoo
Federico
F
Di Rocco
Nicole
N
Brousse
Olivier
O
Delattre
Pascale
P
Varlet
Jacques
J
Grill
D001932:Brain Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D002648:Child; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism*
C106032:EWS1-WT1 fusion protein, human; D015514:Oncogene Proteins, Fusion