title: Expression pattern of ISL-1, TTF-1 and PAX5 in olfactory neuroblastoma.
abstract: Olfactory neuroblastoma (ONB) is a rare neoplasm of the sinonasal area with neuroendocrine differentiation. ISL-1, TTF-1 and PAX5 are transcription factors that are frequently upregulated in tumors showing neuroendocrine differentiation. The aim of our study was to evaluate these markers in a group of ONBs. We included 11 ONBs from 4 large university hospitals. Immunohistochemical expression of TTF-1, PAX5 and ISL-1 was evaluated. TTF-1, ISL-1 and PAX5 were expressed in 3/11 cases (27.27%, h-score: 3-45), 7/11 cases (63.64%, h-score: 23-200), and in 3/11 cases (27.77%, h-score 3-85), respectively. The patient with the strongest PAX5 reactivity exhibited an aggressive clinical course with rapid dissemination to the spine and death shortly after the diagnosis. No significant correlation in the expression of PAX5 and TTF-1 ( = 0.43; p = 0.18) was observed. ISL-1 is widely expressed in tumors with neuroendocrine differentiation and therefore of limited value in their differential diagnosis. TTF-1 positivity does not exclude the diagnosis of primary ONB, although usually only a small percentage of cells are positive. PAX5 expression is infrequent (27.27%) in ONB; however, if present it can be associated with a very aggressive clinical course.
authors.affiliation: Piotr Czapiewski, Department of Pathomorphology, Medical University of Gdańsk, Dębinki 7, 80-952 Gdansk, Poland, tel. +48 58 349 27 40, e-mail: czapiewskipiotr@gumed.edu.pl.
authors.forename: P
authors.initials: P
authors.lastname: Czapiewski
authors.forename: A
authors.initials: A
authors.lastname: Gorczyński
authors.forename: J
authors.initials: J
authors.lastname: Haybaeck
authors.forename: K
authors.initials: K
authors.lastname: Okoń
authors.forename: J
authors.initials: J
authors.lastname: Reszeć
authors.forename: W
authors.initials: W
authors.lastname: Skrzypczak
authors.forename: J
authors.initials: J
authors.lastname: Dzierżanowski
authors.forename: M
authors.initials: M
authors.lastname: Kunc
authors.forename: J
authors.initials: J
authors.lastname: Karczewska
authors.forename: W
authors.initials: W
authors.lastname: Biernat
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D004268:DNA-Binding Proteins / Q000032:analysis / Q000096:biosynthesis*; D018304:Esthesioneuroblastoma, Olfactory / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D060850:LIM-Homeodomain Proteins / Q000032:analysis / Q000096:biosynthesis*; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity / Q000473:pathology; D009669:Nose Neoplasms / Q000378:metabolism* / Q000473:pathology; D051757:PAX5 Transcription Factor / Q000032:analysis / Q000096:biosynthesis*; D014157:Transcription Factors / Q000032:analysis / Q000096:biosynthesis*; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; D060850:LIM-Homeodomain Proteins; D051757:PAX5 Transcription Factor; C497281:PAX5 protein, human; C504003:TTF1 protein, human; D014157:Transcription Factors; C063843:insulin gene enhancer binding protein Isl-1