TY - JOUR
T1 - Expression of connective tissue growth factor (CCN2) in desmoplastic small round cell tumour
AU - Rachfal, A. W.
AU - Luquette, M. H.
AU - Brigstock, D. R.
PY - 2004/4
Y1 - 2004/4
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=1842507136&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=1842507136&partnerID=8YFLogxK
U2 - 10.1136/jcp.2003.012344
DO - 10.1136/jcp.2003.012344
M3 - Article
C2 - 15047749
AN - SCOPUS:1842507136
SN - 0021-9746
VL - 57
SP - 422
EP - 425
JO - Journal of Clinical Pathology
JF - Journal of Clinical Pathology
IS - 4
ER -