title: Establishment and molecular characterization of five cell lines derived from renal and extrarenal malignant rhabdoid tumors.
abstract: Malignant rhabdoid tumor (MRT) is a rare, enigmatic childhood cancer characterized by extreme aggressiveness and resistance to chemotherapy. To understand better the origin of the tumor and the mechanisms by which it develops and resists treatment, five cell lines were established from patients presenting with MRT (two renal and three extrarenal tumors). All of the cell lines display the light microscopic and ultrastructural features, as well as the variable immunohistochemical profile, characteristic of MRT. All are capable of forming tumors in nude mice. Three of the cell lines have detectable abnormalities of chromosome 22: one a t(22, 22) unbalanced translocation and two others a loss of heterozygosity of polymerase chain reaction-based microsatellite markers. Northern blot analysis showed that overexpression of the c-myc message was a consistent characteristic of the five MRTs evaluated. Although mutations of the p53 gene were not detectable by sequence analysis, all of the cell lines showed nuclear accumulation of the p53 protein by an immunocytochemical analysis in a minority of the cells. This result suggests that dysfunction in a p53-dependent apoptotic pathway might play a role in the multiple drug resistance phenotype of these tumors.
authors.affiliation: Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston 29425, USA.
authors.forename: G B
authors.initials: GB
authors.lastname: Rosson
authors.forename: D J
authors.initials: DJ
authors.lastname: Hazen-Martin
authors.forename: J A
authors.initials: JA
authors.lastname: Biegel
authors.forename: M C
authors.initials: MC
authors.lastname: Willingham
authors.forename: A J
authors.initials: AJ
authors.lastname: Garvin
authors.forename: B W
authors.initials: BW
authors.lastname: Oswald
authors.forename: L
authors.initials: L
authors.lastname: Wainwright
authors.forename: N A
authors.initials: NA
authors.lastname: Brownlee
authors.forename: C F
authors.initials: CF
authors.lastname: Wright
mesh_terms: D000293:Adolescent; D000818:Animals; D015152:Blotting, Northern; D002467:Cell Nucleus / Q000648:ultrastructure; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D016158:Genes, p53 / Q000235:genetics; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D007231:Infant, Newborn; D007621:Karyotyping; D007680:Kidney Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D019656:Loss of Heterozygosity; D008297:Male; D051379:Mice; D008807:Mice, Inbred BALB C; D008819:Mice, Nude; D009368:Neoplasm Transplantation; D016271:Proto-Oncogene Proteins c-myc / Q000378:metabolism; D018335:Rhabdoid Tumor / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D014407:Tumor Cells, Cultured
chemical_list: D016271:Proto-Oncogene Proteins c-myc