title: Region Specific Differences of Claudin-5 Expression in Pediatric Intracranial Ependymomas: Potential Prognostic Role in Supratentorial Cases.
abstract: Ependymomas are common pediatric brain tumors that originate from the ependyma and characterized by poor prognosis due to frequent recurrence. However, the current WHO grading system fails to accurately predict outcome. In a retrospective study, we analyzed 54 intracranial pediatric ependymomas and found a significantly higher overall survival in supratentorial cases when compared to infratentorial tumors. Next we performed region-specific immunohistochemical analysis of the ependyma in neonatal and adult ependyma from the central canal of spinal cord to the choroid plexus of lateral ventricles for components of cell-cell junctions including cadherins, claudins and occludin. We found robust claudin-5 expression in the choroid plexus epithelia but not in other compartments of the ependyma. Ultrastructural studies demonstrated distinct regional differences in cell-cell junction organization. Surprisingly, we found that 9 out of 20 supratentorial but not infratentorial ependymomas expressed high levels of the brain endothelial tight junction component claudin-5 in tumor cells. Importantly, we observed an increased overall survival in claudin-5 expressing supratentorial ependymoma. Our data indicates that claudin-5 expressing ependymomas may follow a distinct course of disease. The assessment of claudin-5 expression in ependymoma has the potential to become a useful prognostic marker in this pediatric malignancy.
authors.affiliation: 2nd Department of Pediatrics, Semmelweis University, Budapest, Hungary.
authors.forename: József
authors.initials: J
authors.lastname: Virág
authors.affiliation: Institute of Neuropathology, Medical University of Vienna, Vienna, Austria.
authors.forename: Christine
authors.initials: C
authors.lastname: Haberler
authors.affiliation: 1st Department of Anatomy, Semmelweis University, Budapest, Hungary.
authors.forename: Gábor
authors.initials: G
authors.lastname: Baksa
authors.affiliation: 2nd Department of Pathology, Semmelweis University, Üllői út 93, Budapest, H-1091, Hungary.
authors.forename: Violetta
authors.initials: V
authors.lastname: Piurkó
authors.affiliation: 2nd Department of Pathology, Semmelweis University, Üllői út 93, Budapest, H-1091, Hungary.
authors.forename: Zita
authors.initials: Z
authors.lastname: Hegedüs
authors.affiliation: 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary.|MTA-SE NAP, Brain Metastasis Research Group, Hungarian Academy of Sciences - Semmelweis University, Budapest, Hungary.
authors.forename: Lilla
authors.initials: L
authors.lastname: Reiniger
authors.affiliation: 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary.
authors.forename: Katalin
authors.initials: K
authors.lastname: Bálint
authors.affiliation: Department of Pediatrics, Medical University of Vienna, Vienna, Austria.
authors.forename: Monika
authors.initials: M
authors.lastname: Chocholous
authors.affiliation: 2nd Department of Pathology, Semmelweis University, Üllői út 93, Budapest, H-1091, Hungary.
authors.forename: András
authors.initials: A
authors.lastname: Kiss
authors.affiliation: 2nd Department of Pathology, Semmelweis University, Üllői út 93, Budapest, H-1091, Hungary.
authors.forename: Gábor
authors.initials: G
authors.lastname: Lotz
authors.affiliation: 2nd Department of Pathology, Semmelweis University, Üllői út 93, Budapest, H-1091, Hungary.
authors.forename: Tibor
authors.initials: T
authors.lastname: Glasz
authors.affiliation: 2nd Department of Pathology, Semmelweis University, Üllői út 93, Budapest, H-1091, Hungary.
authors.forename: Zsuzsa
authors.initials: Z
authors.lastname: Schaff
authors.affiliation: 2nd Department of Pediatrics, Semmelweis University, Budapest, Hungary.
authors.forename: Miklós
authors.initials: M
authors.lastname: Garami
authors.affiliation: 2nd Department of Pathology, Semmelweis University, Üllői út 93, Budapest, H-1091, Hungary. balazs.hegedus@meduniwien.ac.at.|Department of Thoracic Surgery, Medical University of Vienna, Vienna, Austria. balazs.hegedus@meduniwien.ac.at.|Molecular Oncology Research Group, Hungarian Academy of Sciences - Semmelweis University, Budapest, Hungary. balazs.hegedus@meduniwien.ac.at.
authors.forename: Balázs
authors.initials: B
authors.lastname: Hegedűs
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000378:metabolism* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D062507:Claudin-5 / Q000378:metabolism*; D004806:Ependymoma / Q000378:metabolism* / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D015192:Infratentorial Neoplasms / Q000378:metabolism* / Q000473:pathology*; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000378:metabolism / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D062507:Claudin-5
keywords: Choroid plexus epithelium; Claudin-5; Ependymoma; Prognosis; Supratentorial; Tight junction