title: Neuronal differentiation distinguishes supratentorial and infratentorial childhood ependymomas.
abstract: Ependymomas are glial neoplasms occurring in any location throughout the central nervous system and supposedly are derived from radial glia cells. Recent data suggest that these tumors may have different biological and clinical behaviors according to their location. Pediatric supratentorial and infratentorial ependymoma (SE and IE) were compared with respect to clinical and radiological parameters and immunohistochemistry (IHC). Neuronal markers were specifically assessed by IHC and quantitative PCR (qPCR). No single morphological or radiological characteristic was associated with location or any neuronal marker. However, there was a significant overexpression of neuronal markers in SE compared with IE: neurofilament light polypeptide 70 (NEFL)-positive tumor cells were found in 23 of 34 SE and in only 4 of 32 IE (P < .001). Among SE, 10 of 34 exhibited high expression of NEFL, defined as more than 5% positive cells. qPCR confirmed the upregulation of neuronal markers (NEFL, LHX2, FOXG1, TLX1, and NPTXR) in SE compared with IE. In addition, strong NEFL expression in SE was correlated with better progression-free survival (P = .007). Our results support the distinction of SE and IE. SEs are characterized by neuronal differentiation, which seems to be associated with better prognosis.
authors.affiliation: INSERM UMR 8203 "Vectorology and Anticancer Therapies," Institut Gustave Roussy, 39 Rue Camille Desmoulins, Villejuif 94805, France. felipe.andreiuolo@igr.fr
authors.forename: Felipe
authors.initials: F
authors.lastname: Andreiuolo
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Puget
authors.forename: Matthieu
authors.initials: M
authors.lastname: Peyre
authors.forename: Carmela
authors.initials: C
authors.lastname: Dantas-Barbosa
authors.forename: Nathalie
authors.initials: N
authors.lastname: Boddaert
authors.forename: Cathy
authors.initials: C
authors.lastname: Philippe
authors.forename: Audrey
authors.initials: A
authors.lastname: Mauguen
authors.forename: Jacques
authors.initials: J
authors.lastname: Grill
authors.forename: Pascale
authors.initials: P
authors.lastname: Varlet
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D002454:Cell Differentiation; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004806:Ependymoma / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D015192:Infratentorial Neoplasms / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D053208:Kaplan-Meier Estimate; D008297:Male; D009474:Neurons / Q000378:metabolism / Q000473:pathology*; D011379:Prognosis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D015173:Supratentorial Neoplasms / Q000378:metabolism / Q000401:mortality / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor