Primary Peripheral PNET/Ewing's Sarcoma of the Dura: a Clinicopathologic Entity Distinct from Central PNET

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...

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Veröffentlicht in:Modern pathology 2002-06, Vol.15 (6), p.673-678
Hauptverfasser: Dedeurwaerdere, Franceska, Giannini, Caterina, Sciot, Raf, Rubin, Brian P, Perilongo, Giorgio, Borghi, Laura, Ballotta, Maria Luisa, Cornips, Erwin, Demunter, Anouk, Maes, Brigitte, Dei Tos, Angelo P
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container_title Modern pathology
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creator Dedeurwaerdere, Franceska
Giannini, Caterina
Sciot, Raf
Rubin, Brian P
Perilongo, Giorgio
Borghi, Laura
Ballotta, Maria Luisa
Cornips, Erwin
Demunter, Anouk
Maes, Brigitte
Dei Tos, Angelo P
description 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.
doi_str_mv 10.1038/modpathol.3880585
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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.</abstract><cop>New York</cop><pub>Elsevier Inc</pub><pmid>12065782</pmid><doi>10.1038/modpathol.3880585</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record>
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subjects 12E7 Antigen
Antigens, CD - analysis
case-report
CD99
Cell Adhesion Molecules - analysis
Child
Chromosome Aberrations
Chromosomes, Human, Pair 22 - genetics
Diagnosis, Differential
Dura Mater - chemistry
Dura Mater - metabolism
Dura Mater - pathology
Ewing's sarcoma
Humans
Immunohistochemistry
In Situ Hybridization, Fluorescence
Laboratory Medicine
Male
Medicine
Medicine & Public Health
Meningeal Neoplasms - genetics
Meningeal Neoplasms - metabolism
Meningeal Neoplasms - pathology
Meninges
Neuroectodermal Tumors, Primitive, Peripheral - genetics
Neuroectodermal Tumors, Primitive, Peripheral - metabolism
Neuroectodermal Tumors, Primitive, Peripheral - pathology
Neurofilament Proteins - analysis
Pathology
Peripheral primitive neuroectodermal tumor
S100 Proteins - analysis
Sarcoma, Ewing - genetics
Sarcoma, Ewing - metabolism
Sarcoma, Ewing - pathology
Synaptophysin - analysis
t(11
22)
title Primary Peripheral PNET/Ewing's Sarcoma of the Dura: a Clinicopathologic Entity Distinct from Central PNET
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