title: Expression of connective tissue growth factor (CCN2) in desmoplastic small round cell tumour.
abstract: BACKGROUND
  Desmoplastic small round cell tumour (DSRCT) is a rare and often fatal abdominal tumour that is distinguished by well defined islands of cells, surrounded by prominent desmoplastic stroma. As in certain other tumours, the function of the Wilms's tumour protein (WT1) in repressing gene transcription is lost in DSRCT.
  
  
  OBJECTIVE
  To assess the expression and localisation of connective tissue growth factor (CCN2) in DSRCT because this protein is transcriptionally repressed by WT1 and is associated with the production of abundant extracellular matrix.
  
  
  METHODS
  CCN2 was assessed by in situ hybridisation and immunohistochemistry.
  
  
  RESULTS
  CCN2 mRNA and protein were colocalised to the tumour cells themselves, in addition to stromal fibroblasts and vascular endothelial cells.
  
  
  CONCLUSIONS
  These data show that CCN2 is produced in high amounts by several cell types in DSRCT, and highlight a potential role for this factor in the autocrine and paracrine regulation of tumour cell growth, matrigenesis, and angiogenesis.
authors.affiliation: Center for Cell and Vascular Biology, Children's Research Institute, Columbus Ohio 43205, USA.
authors.forename: A W
authors.initials: AW
authors.lastname: Rachfal
authors.forename: M H
authors.initials: MH
authors.lastname: Luquette
authors.forename: D R
authors.initials: DR
authors.lastname: Brigstock
mesh_terms: D000293:Adolescent; D002648:Child; D055513:Connective Tissue Growth Factor; D042783:Endothelial Cells / Q000737:chemistry; D004730:Endothelium, Vascular / Q000737:chemistry; D005347:Fibroblasts / Q000737:chemistry; D006801:Humans; D017874:Immediate-Early Proteins / Q000032:analysis* / Q000235:genetics; D007150:Immunohistochemistry / Q000379:methods; D017403:In Situ Hybridization / Q000379:methods; D036341:Intercellular Signaling Peptides and Proteins / Q000032:analysis* / Q000235:genetics; D008297:Male; D009363:Neoplasm Proteins / Q000032:analysis* / Q000235:genetics; D010534:Peritoneal Neoplasms / Q000737:chemistry*; D012333:RNA, Messenger / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: C526317:CCN2 protein, human; D017874:Immediate-Early Proteins; D036341:Intercellular Signaling Peptides and Proteins; D009363:Neoplasm Proteins; D012333:RNA, Messenger; D055513:Connective Tissue Growth Factor