title: Classic and desmoplastic medulloblastoma: complete case reports and characterizations of two new cell lines.
abstract: Medulloblastoma (MB) is the most common type of brain tumor affecting children. These tumors are a significant cause of childhood mortality and morbidity, and more effective and less invasive treatment options are urgently required. To achieve these aims, it will be critical to develop a more comprehensive understanding of the molecular pathogenesis of MB. At present, there are relatively few well-characterized MB cell lines available to the research community for the study of MB molecular and cellular biology. Here we present the case reports of two children diagnosed with classic and desmoplastic MB, and describe the characteristics of two new MB cell lines derived from these individuals. A number of genes encoding components of the sonic hedgehog (SHH) and WNT pathways were up-regulated in the desmoplastic relative to the classic MB cell line consistent with aberrant activation of these pathways in desmoplastic MB. These cell lines represent an additional resource for the analysis of diverse aspects of MB biology.
authors.affiliation: Telethon Institute for Child Health Research and Centre for Child Health Research, University of Western Australia, Perth, Australia.
authors.forename: David J
authors.initials: DJ
authors.lastname: Holthouse
authors.forename: Peter B
authors.initials: PB
authors.lastname: Dallas
authors.forename: Jette
authors.initials: J
authors.lastname: Ford
authors.forename: Vicki
authors.initials: V
authors.lastname: Fabian
authors.forename: Ashleigh R
authors.initials: AR
authors.lastname: Murch
authors.forename: Marcia
authors.initials: M
authors.lastname: Watson
authors.forename: George
authors.initials: G
authors.lastname: Wong
authors.forename: Cornelia
authors.initials: C
authors.lastname: Bertram
authors.forename: Simone
authors.initials: S
authors.lastname: Egli
authors.forename: David L
authors.initials: DL
authors.lastname: Baker
authors.forename: Ursula R
authors.initials: UR
authors.lastname: Kees
mesh_terms: D045744:Cell Line, Tumor; D002528:Cerebellar Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D014407:Tumor Cells, Cultured