cerebral desmoplastic small round cell tumor
Desmoplastic small round cell tumor (DSRCT) is a malignant tumor often involving the abdominal and/or pelvic peritoneum. Some fully documented examples have arisen in the central nervous system (CNS).
Although rare, DSRCT warrants consideration in the differential diagnosis of "malignant small blue cell tumors" of the CNS.
Synopsis
small round cell tumor
fibromyxoïd stroma
Immunohistochemistry
neural and mesenchymal differentiation
Molecular biology
EWS-WT1 fusion gene
Differential diagnosis
DSRCT should be considered in the differential diagnosis of cerebral small round cell tumors.
References
Desmoplastic small round cell tumor of the central nervous system: report of two cases and review of the literature. Neder L, Scheithauer BW, Turel KE, Arnesen MA, Ketterling RP, Jin L, Moynihan TJ, Giannini C, Meyer FB. Virchows Arch. 2009 Apr;454(4):431-9. PMID: #19263077#
Intracerebral small round cell tumor: an unusual case with EWS-WT1 translocation. Bouchireb K, Auger N, Bhangoo R, Di Rocco F, Brousse N, Delattre O, Varlet P, Grill J. Pediatr Blood Cancer. 2008 Oct;51(4):545-8. PMID: #18561179#