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Expression of connective tissue growth factor (CCN2) in desmoplastic small round cell tumour
  1. A W Rachfal1,
  2. M H Luquette2,
  3. D R Brigstock1
  1. 1Center for Cell and Vascular Biology, Children’s Research Institute, Columbus Ohio 43205, USA
  2. 2Department of Pathology, Children’s Hospital, Columbus OH 43205, USA
  1. Correspondence to:
 Dr D R Brigstock
 Center for Cell and Vascular Biology, Children’s Research Institute, Room NA 2022, 700 Children’s Drive, Columbus Ohio 43205, USA;


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.

Aims: 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.

  • fibrosis
  • stroma
  • connective tissue growth factor
  • desmoplasia
  • tumour
  • CCN2, connective tissue growth factor
  • DSRCT, desmoplastic small round cell tumour
  • EWS, Ewing sarcoma
  • IGF, insulin-like growth factor
  • PDGF, platelet derived growth factor
  • TGFβ, transforming growth factor β
  • RT-PCR, reverse transcription polymerase chain reaction
  • WT1, Wilms’s tumour protein

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