title: Three new chondrosarcoma cell lines: one grade III conventional central chondrosarcoma and two dedifferentiated chondrosarcomas of bone.
abstract: BACKGROUND
  Chondrosarcoma is the second most common primary sarcoma of bone. High-grade conventional chondrosarcoma and dedifferentiated chondrosarcoma have a poor outcome. In pre-clinical research aiming at the identification of novel treatment targets, the need for representative cell lines and model systems is high, but availability is scarce.
  
  
  METHODS
  We developed and characterized three cell lines, derived from conventional grade III chondrosarcoma (L835), and dedifferentiated chondrosarcoma (L2975 and L3252) of bone. Proliferation and migration were studied and we used COBRA-FISH and array-CGH for karyotyping and genotyping. Immunohistochemistry for p16 and p53 was performed as well as TP53 and IDH mutation analysis. Cells were injected into nude mice to establish their tumorigenic potential.
  
  
  RESULTS
  We show that the three cell lines have distinct migrative properties, L2975 had the highest migration rate and showed tumorigenic potential in mice. All cell lines showed chromosomal rearrangements with complex karyotypes and genotypic aberrations were conserved throughout late passaging of the cell lines. All cell lines showed loss of CDKN2A, while TP53 was wild type for exons 5-8. L835 has an IDH1 R132C mutation, L2975 an IDH2 R172W mutation and L3252 is IDH wild type.
  
  
  CONCLUSIONS
  Based on the stable culturing properties of these cell lines and their genotypic profile resembling the original tumors, these cell lines should provide useful functional models to further characterize chondrosarcoma and to evaluate new treatment strategies.
authors.affiliation: Department of Pathology, Leiden University Medical Center, Albinusdreef 2, 2333, ZA, Leiden, The Netherlands.
authors.forename: Jolieke G
authors.initials: JG
authors.lastname: van Oosterwijk
authors.forename: Danielle
authors.initials: D
authors.lastname: de Jong
authors.forename: Maayke A J H
authors.initials: MA
authors.lastname: van Ruler
authors.forename: Pancras C W
authors.initials: PC
authors.lastname: Hogendoorn
authors.forename: P D Sander
authors.initials: PD
authors.lastname: Dijkstra
authors.forename: Carla S P
authors.initials: CS
authors.lastname: van Rijswijk
authors.forename: Isidro
authors.initials: I
authors.lastname: Machado
authors.forename: Antonio
authors.initials: A
authors.lastname: Llombart-Bosch
authors.forename: Karoly
authors.initials: K
authors.lastname: Szuhai
authors.forename: Judith V M G
authors.initials: JV
authors.lastname: Bovée
mesh_terms: D000818:Animals; D001859:Bone Neoplasms / Q000235:genetics / Q000473:pathology*; D045744:Cell Line, Tumor; D002465:Cell Movement; D002813:Chondrosarcoma / Q000235:genetics / Q000473:pathology*; D055028:Comparative Genomic Hybridization; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D051379:Mice; D008819:Mice, Nude; D008875:Middle Aged; D009154:Mutation; D060787:Neoplasm Grading; D011859:Radiography; D011884:Radius / Q000000981:diagnostic imaging / Q000473:pathology; D014183:Transplantation, Heterologous