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 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.
Dedeurwaerdere
Franceska
F
Department of Pathology, Katholieke Universiteit Leuven, Belgium.
Giannini
Caterina
C
Sciot
Raf
R
Rubin
Brian P
BP
Perilongo
Giorgio
G
Borghi
Laura
L
Ballotta
Maria Luisa
ML
Cornips
Erwin
E
Demunter
Anouk
A
Maes
Brigitte
B
Dei Tos
Angelo P
AP
D000071443:12E7 Antigen; D015703:Antigens, CD / Q000032:analysis; D015815:Cell Adhesion Molecules / Q000032:analysis; D002648:Child; D002869:Chromosome Aberrations; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D003937:Diagnosis, Differential; D004388:Dura Mater / Q000737:chemistry / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008577:Meningeal Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016900:Neurofilament Proteins / Q000032:analysis; D009418:S100 Proteins / Q000032:analysis; D012512:Sarcoma, Ewing / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016708:Synaptophysin / Q000032:analysis
D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D016900:Neurofilament Proteins; D009418:S100 Proteins; D016708:Synaptophysin