Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET.

Dedeurwaerdere, Franceska; Giannini, Caterina; Sciot, Raf; Rubin, Brian P; Perilongo, Giorgio; Borghi, Laura; Ballotta, Maria Luisa; Cornips, Erwin et al. · Mod Pathol · 2002

case_report · Level V

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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.

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