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

Virchows Arch 2009 Apr 5;454(4):431-9. Epub 2009 Mar 5.

Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA.

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.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00428-009-0750-xDOI Listing
April 2009
9 Reads

Publication Analysis

Top Keywords

cell tumor
8
central nervous
8
nervous system
8
dissemination patient
8
round cell
8
spinal dissemination
8
desmoplastic small
8
small round
8
recurrent disease
4
died recurrent
4
years died
4
subtotal resection
4
resection years
4
disease spinal
4
patient 39-year-old
4
cerebellar cpa
4
cpa lesions
4
presented cerebellar
4
man presented
4
39-year-old man
4

References

(Supplied by CrossRef)

WL Gerald et al.
Pediatr Pathol 1989

F Chang et al.
Arch Pathol Lab Med 2006

WL Gerald et al.
J Clin Oncol 1998

WL Gerald et al.
Am J Surg Pathol 1991

I Leuschner et al.
Semin Diagn Pathol 1996

CR Antonescu et al.
2002

ME Lae et al.
Am J Surg Pathol 2002

WL Gerald et al.
Proc Natl Acad Sci USA 1995

M Ladanyi et al.
Cancer Res 1994

E Rodriguez et al.
Cancer Genet Cytogenet 1993

Y Bian et al.
Acta Cytol 1993

Similar Publications