Desmoplastic small round cell tumor of the central nervous system: Report of two cases and review of the literature

Luciano Neder, Bernd W. Scheithauer, Keki E. Turel, Mark A. Arnesen, Rhett P. Ketterling, Long Jin, Timothy J. Moynihan, Caterina Giannini, Fredric B. Meyer

Research output: Contribution to journalArticlepeer-review

23 Scopus citations


Desmoplastic small round cell tumor (DSRCT) is a malignant tumor often involving the abdominal and/or pelvic peritoneum. Only one fully documented example has arisen in the central nervous system (CNS). Herein, we describe two additional examples, fulfilling the morphologic, immunohistochemical, and molecular criteria (EWS/WT1 translocation) of DSRCT. Both arose in the cerebellopontine angle (CPA) and underwent spinal dissemination. Patient 1, a 37-year-old male, underwent a subtotal resection, and 2 years later died of recurrent disease with spinal dissemination. Patient 2, a 39-year-old man, presented with cerebellar and CPA lesions as well as spinal leptomeningeal deposits. After 27 months of adjuvant therapy, he is alive with progressive disease. In conclusion, CNS DSRCT follows a similar aggressive course as do peritoneal examples. Although rare, DSRCT warrants consideration in the differential diagnosis of "malignant small blue cell tumors" of the CNS.

Original languageEnglish (US)
Pages (from-to)431-439
Number of pages9
JournalVirchows Archiv
Issue number4
StatePublished - Apr 2009


  • Central nervous system
  • Desmoplastic small round cell tumor
  • EWS/WT1 translocation
  • Meninges

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology


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