title: Trisomy 19 ependymoma, a newly recognized genetico-histological association, including clear cell ependymoma.
abstract: Ependymal tumors constitute a clinicopathologically heterogeneous group of brain tumors. They vary in regard to their age at first symptom, localization, morphology and prognosis. Genetic data also suggests heterogeneity. We define a newly recognized subset of ependymal tumors, the trisomy 19 ependymoma. Histologically, they are compact lesions characterized by a rich branched capillary network amongst which tumoral cells are regularly distributed. When containing clear cells they are called clear cell ependymoma. Most trisomy 19 ependymomas are supratentorial WHO grade III tumors of the young. Genetically, they are associated with trisomy 19, and frequently with a deletion of 13q21.31-31.2, three copies of 11q13.3-13.4, and/or deletions on chromosome 9. These altered chromosomal regions are indicative of genes and pathways involved in trisomy 19 ependymoma tumorigenesis. Recognition of this genetico-histological entity allows better understanding and dissection of ependymal tumors.
authors.affiliation: Laboratory of Pathology, Université catholique de Louvain, Bruxelles, Belgium. e.rousseau@chmouscron.be
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Rousseau
authors.forename: Thomas
authors.initials: T
authors.lastname: Palm
authors.forename: Francesco
authors.initials: F
authors.lastname: Scaravilli
authors.forename: Marie-Magdeleine
authors.initials: MM
authors.lastname: Ruchoux
authors.forename: Dominique
authors.initials: D
authors.lastname: Figarella-Branger
authors.forename: Isabelle
authors.initials: I
authors.lastname: Salmon
authors.forename: David
authors.initials: D
authors.lastname: Ellison
authors.forename: Catherine
authors.initials: C
authors.lastname: Lacroix
authors.forename: Françoise
authors.initials: F
authors.lastname: Chapon
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Mikol
authors.forename: Miikka
authors.initials: M
authors.lastname: Vikkula
authors.forename: Catherine
authors.initials: C
authors.lastname: Godfraind
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics*; D002899:Chromosomes, Human, Pair 9 / Q000235:genetics; D004806:Ependymoma / Q000235:genetics / Q000473:pathology*; D005260:Female; D015894:Genome, Human; D006801:Humans; D007223:Infant; D008297:Male; D018895:Microsatellite Repeats / Q000235:genetics; D008875:Middle Aged; D009693:Nucleic Acid Hybridization / Q000379:methods; D020641:Polymorphism, Single Nucleotide; D046888:Tissue Array Analysis / Q000379:methods; D016707:Tissue Fixation / Q000379:methods; D014314:Trisomy