Vol 42, No 4 (2004)
Original paper
Submitted: 2011-12-19
Published online: 2005-02-12
Heterogeneity of extraparenchymal primitive neuroectodermal tumors within the craniospinal axis.
E Izycka-Swieszewska, W Kloc, K Plata-Nazar, J Stefanowicz, E Drozyńska, A Woźniak, D Gasecki, M Debiec-Rychter
Folia Histochem Cytobiol 2004;42(4):229-234.
Vol 42, No 4 (2004)
ORIGINAL PAPERS
Submitted: 2011-12-19
Published online: 2005-02-12
Abstract
Four cases of primitive neuroectodermal tumors (PNETs) with unusual localization (three intraspinal extramedullary and one pontocerebellar) are reviewed. Histologically, they were small round blue cell tumors with diverse patterns. Immunohistochemically, all tumors were positive for at least two neuronal markers, two cases were Mic-2 positive and one showed glial differentiation. The paraffin-embedded tumor specimens were examined by interphase FISH using dual-color probes specific for EWS, HER-2 and BCR loci. Molecular cytogenetic study revealed the presence of EWS rearrangement in two cases and the presence of i(17q) in one tumor. Three tumors exhibited 22 disomy and one was 22 polyploid. Extraparenchymal PNETs within craniospinal axis are heterogeneous from the clinical, histological, immunohistochemical and molecular point of view. These PNETs can be of a central or peripheral type. Multidisciplinary approach is of a basic importance in differential diagnosis of such cases.
Abstract
Four cases of primitive neuroectodermal tumors (PNETs) with unusual localization (three intraspinal extramedullary and one pontocerebellar) are reviewed. Histologically, they were small round blue cell tumors with diverse patterns. Immunohistochemically, all tumors were positive for at least two neuronal markers, two cases were Mic-2 positive and one showed glial differentiation. The paraffin-embedded tumor specimens were examined by interphase FISH using dual-color probes specific for EWS, HER-2 and BCR loci. Molecular cytogenetic study revealed the presence of EWS rearrangement in two cases and the presence of i(17q) in one tumor. Three tumors exhibited 22 disomy and one was 22 polyploid. Extraparenchymal PNETs within craniospinal axis are heterogeneous from the clinical, histological, immunohistochemical and molecular point of view. These PNETs can be of a central or peripheral type. Multidisciplinary approach is of a basic importance in differential diagnosis of such cases.
Title
Heterogeneity of extraparenchymal primitive neuroectodermal tumors within the craniospinal axis.
Journal
Folia Histochemica et Cytobiologica
Issue
Vol 42, No 4 (2004)
Article type
Original paper
Pages
229-234
Published online
2005-02-12
Page views
1021
Article views/downloads
1010
Bibliographic record
Folia Histochem Cytobiol 2004;42(4):229-234.
Authors
E Izycka-Swieszewska
W Kloc
K Plata-Nazar
J Stefanowicz
E Drozyńska
A Woźniak
D Gasecki
M Debiec-Rychter