title: Orthotopic Wilms tumor xenografts derived from cell lines reflect limited aspects of tumor morphology and clinical characteristics.
abstract: BACKGROUND
  Wilms tumor (WT) is a pediatric tumor of the kidney, the treatment of which includes heavy chemotherapy. Affected children would likely benefit from more targeted therapies with limited side effects. Establishment of relevant orthotopic WT xenografts is important to better understand mechanisms of WT growth and for preclinical drug testing.
  
  
  METHODS
  Here we established and characterized orthotopic xenografts from WT cell lines WiT49, CCG-99-11, and WT-CLS1 to ascertain in what aspects each of them recapitulated WT histology, immunophenotype, invasion, and metastatic spread.
  
  
  RESULTS
  WiT49 xenografts recapitulated near triphasic WTs with clear WT1 staining and anaplastic features, but with tumor restricted to the kidney. On the contrary both CCG-99-11 and WT-CLS1 xenografts conveyed metastatic disease. CCG-99-11 showed a blastemal phenotype whereas WT-CLS1 xenografts did not properly reflect any specific WT subtype.
  
  
  CONCLUSIONS
  From the three tested cell lines, orthotopic WiT49 xenografts best reflect the triphasic pattern of classical WT.
authors.affiliation: Department of Clinical Genetics, Lund University, University and Regional Laboratories, Lund, Sweden.
authors.forename: Linda Holmquist
authors.initials: LH
authors.lastname: Mengelbier
authors.forename: Daniel
authors.initials: D
authors.lastname: Bexell
authors.forename: Daniel
authors.initials: D
authors.lastname: Sehic
authors.forename: Cristina D
authors.initials: CD
authors.lastname: Ciornei
authors.forename: David
authors.initials: D
authors.lastname: Gisselsson
mesh_terms: D000818:Animals; D045744:Cell Line, Tumor; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007680:Kidney Neoplasms / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D051379:Mice; D009361:Neoplasm Invasiveness; D009368:Neoplasm Transplantation; D014183:Transplantation, Heterologous; D025721:WT1 Proteins / Q000032:analysis; D009396:Wilms Tumor / Q000276:immunology / Q000401:mortality / Q000473:pathology*
chemical_list: D025721:WT1 Proteins; C583751:WT1 protein, human
keywords: CCG-99-11; WT-CLS1; WiT49; Wilms tumor; Wilms tumor cell line; orthotopic; xenograft