Case Report
Mod Pathol 2002;15(6):673–678
Primary Peripheral PNET/Ewing's Sarcoma of the Dura: a Clinicopathologic Entity Distinct from Central PNET
Franceska Dedeurwaerdere M.D.1, Caterina Giannini M.D., PhD2, Raf Sciot M.D., PhD1, Brian P Rubin M.D.3, Giorgio Perilongo M.D.4, Laura Borghi M.D.5, Maria Luisa Ballotta M.D.5, Erwin Cornips M.D.6, Anouk Demunter M.D.1, Brigitte Maes M.D.1 and Angelo P Dei Tos M.D.7
- 1Department of Pathology, Katholieke Universiteit Leuven, Leuven, Belgium
- 2Department of Pathology, Mayo Clinic, Rochester, Minnesota
- 3Department of Pathology, University of Washington, Seattle, Washington
- 4Division of Pediatric Oncology, University of Padua Medical School, Padua, Italy
- 5Department of Pathology, Hospital of Rovigo, Rovigo, Italy
- 6Department of Neurosurgery, Academisch Ziekenhuis Maastricht, the Netherlands
- 7Department of Pathology, Regional Hospital Treviso, Italy
Correspondence: Catrina Giannini, M.D., Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 S.W. First Street, Rochester, MN; e-mail: giannini.caterina@mayo.edu; fax: 507-284-1599.
Accepted 22 January 2002.
Abstract
We describe two cases of peripheral primitive neuroectodermal tumor–Ewing's sarcoma (PNET-ES) arising intracranially in the leptomeninges. Both tumors exhibited a primitive undifferentiated round-cell morphology. Immunohistochemical stains revealed strong membrane expression of CD99 in both cases. A t(11;22)(q24;q12) could be demonstrated with reverse transcriptase–polymerase chain reaction in one case, whereas fluorescence in situ hybridization analysis performed in the second case showed a rearrangement of the EWS gene. The occurrence of PNET-ES at this site is very unusual. Immunophenotypical as well as genetic analysis play a key role in the diagnosis and the distinction from central PNET.
Keywords:
CD99, Ewing's sarcoma, Meninges, Peripheral primitive neuroectodermal tumor, t(11;22)
