title: Gene expression profiling of solitary fibrous tumors.
abstract: BACKGROUND
  Solitary fibrous tumors (SFTs) are rare spindle-cell tumors. Their cell-of-origin and molecular basis are poorly known. They raise several clinical problems. Differential diagnosis may be difficult, prognosis is poorly apprehended by histoclinical features, and no effective therapy exists for advanced stages.
  
  
  METHODS
  We profiled 16 SFT samples using whole-genome DNA microarrays and analyzed their expression profiles with publicly available profiles of 36 additional SFTs and 212 soft tissue sarcomas (STSs). Immunohistochemistry was applied to validate the expression of some discriminating genes.
  
  
  RESULTS
  SFTs displayed whole-genome expression profiles more homogeneous and different from STSs, but closer to genetically-simple than genetically-complex STSs. The SFTs/STSs comparison identified a high percentage (∼30%) of genes as differentially expressed, most of them without any DNA copy number alteration. One of the genes most overexpressed in SFTs encoded the ALDH1 stem cell marker. Several upregulated genes and associated ontologies were also related to progenitor/stem cells. SFTs also overexpressed genes encoding therapeutic targets such as kinases (EGFR, ERBB2, FGFR1, JAK2), histone deacetylases, or retinoic acid receptors. Their overexpression was found in all SFTs, regardless the anatomical location. Finally, we identified a 31-gene signature associated with the mitotic count, containing many genes related to cell cycle/mitosis, including AURKA.
  
  
  CONCLUSIONS
  We established a robust repertoire of genes differentially expressed in SFTs. Certain overexpressed genes could provide new diagnostic (ALDH1A1), prognostic (AURKA) and/or therapeutic targets.
authors.affiliation: Département d'Oncologie Moléculaire, Centre de Recherche en Cancérologie de Marseille (CRCM), Institut Paoli-Calmettes (IPC), UMR1068 Inserm, Marseille, France.
authors.forename: François
authors.initials: F
authors.lastname: Bertucci
authors.forename: Corinne
authors.initials: C
authors.lastname: Bouvier-Labit
authors.forename: Pascal
authors.initials: P
authors.lastname: Finetti
authors.forename: Philippe
authors.initials: P
authors.lastname: Metellus
authors.forename: José
authors.initials: J
authors.lastname: Adelaide
authors.forename: Karima
authors.initials: K
authors.lastname: Mokhtari
authors.forename: Dominique
authors.initials: D
authors.lastname: Figarella-Branger
authors.forename: Anne-Valérie
authors.initials: AV
authors.lastname: Decouvelaere
authors.forename: Catherine
authors.initials: C
authors.lastname: Miquel
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Coindre
authors.forename: Daniel
authors.initials: D
authors.lastname: Birnbaum
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000235:genetics* / Q000378:metabolism; D016000:Cluster Analysis; D005260:Female; D020869:Gene Expression Profiling; D015972:Gene Expression Regulation, Neoplastic; D063990:Gene Ontology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D020411:Oligonucleotide Array Sequence Analysis; D054364:Solitary Fibrous Tumors / Q000235:genetics* / Q000378:metabolism; D046888:Tissue Array Analysis; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor