TY - JOUR
T1 - Desmoplastic small round cell tumor of the central nervous system
T2 - Report of two cases and review of the literature
AU - Neder, Luciano
AU - Scheithauer, Bernd W.
AU - Turel, Keki E.
AU - Arnesen, Mark A.
AU - Ketterling, Rhett P.
AU - Jin, Long
AU - Moynihan, Timothy J.
AU - Giannini, Caterina
AU - Meyer, Fredric B.
N1 - Copyright:
Copyright 2009 Elsevier B.V., All rights reserved.
PY - 2009/4
Y1 - 2009/4
N2 - 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.
AB - 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.
KW - Central nervous system
KW - Desmoplastic small round cell tumor
KW - EWS/WT1 translocation
KW - Meninges
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U2 - 10.1007/s00428-009-0750-x
DO - 10.1007/s00428-009-0750-x
M3 - Article
C2 - 19263077
AN - SCOPUS:63949084467
SN - 0945-6317
VL - 454
SP - 431
EP - 439
JO - Virchows Archiv
JF - Virchows Archiv
IS - 4
ER -