title: Esthesioneuroblastoma is not a member of the primitive peripheral neuroectodermal tumour-Ewing's group.
abstract: Esthesioneuroblastoma (ENB) is a rare, site-specific, locally aggressive neuronal malignancy so far thought to belong to primitive peripheral neuroectodermal tumour-Ewing's tumour (pPNETs-ETs). Its anatomical location, in addition to morphologic, immunophenotypic and ultrastructural features, suggests its origin in the neuronal or neuroendocrine cells of the olfactory epithelium. However, the cytogenetic and molecular data currently available appear controversial on the presence of the typical translocation t(11;22)(q24;q12) and of trisomy 8, chromosomal changes that characterize the tumours belonging to the pPNETs-ETs. Herein we have analysed five ENB tumour specimens for trisomy 8 by fluorescence in situ hybridization (FISH), for the presence of EWS gene rearrangements by FISH, reverse transcription polymerase chain reaction and Southern blot analyses, as well as for the expression of the Ewing sarcoma-associated MIC2 antigen by immunohistochemistry. Neither EWS/FLI-I, EWS/ERG and EWS/FEV fusion genes nor MIC2 expression were found in any tumour, whereas trisomy 8 was found in one case only. Moreover, DNA from three cases analysed by Southern blot did not show EWS gene rearrangements. Our results support the evidence that ENB is not a member of the pPNETs-ETs.
authors.affiliation: Division of Pathology and Cytology, Istituto Nazionale per lo Studio e la Cura dei Tumori, Milan, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Mezzelani
authors.forename: S
authors.initials: S
authors.lastname: Tornielli
authors.forename: F
authors.initials: F
authors.lastname: Minoletti
authors.forename: M A
authors.initials: MA
authors.lastname: Pierotti
authors.forename: G
authors.initials: G
authors.lastname: Sozzi
authors.forename: S
authors.initials: S
authors.lastname: Pilotti
mesh_terms: D000328:Adult; D000368:Aged; D002869:Chromosome Aberrations; D018304:Esthesioneuroblastoma, Olfactory / Q000737:chemistry / Q000145:classification* / Q000235:genetics; D005260:Female; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008297:Male; D008875:Middle Aged; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000145:classification*; D034802:RNA-Binding Protein EWS; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012261:Ribonucleoproteins / Q000235:genetics
chemical_list: D034441:Heterogeneous-Nuclear Ribonucleoproteins; D034802:RNA-Binding Protein EWS; D012261:Ribonucleoproteins