Rachfal A W, Luquette M H, Brigstock D R
Center for Cell and Vascular Biology, Children's Research Institute, Columbus Ohio 43205, USA.
J Clin Pathol. 2004 Apr;57(4):422-5. doi: 10.1136/jcp.2003.012344.
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.
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.
CCN2 was assessed by in situ hybridisation and immunohistochemistry.
CCN2 mRNA and protein were colocalised to the tumour cells themselves, in addition to stromal fibroblasts and vascular endothelial cells.
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.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见且通常致命的腹部肿瘤，其特征是细胞岛边界清晰，周围有显著的促结缔组织增生性基质。与某些其他肿瘤一样，Wilms肿瘤蛋白（WT1）在DSRCT中丧失了抑制基因转录的功能。
评估结缔组织生长因子（CCN2）在DSRCT中的表达和定位，因为该蛋白受WT1转录抑制，且与丰富的细胞外基质产生有关。
通过原位杂交和免疫组织化学评估CCN2。
CCN2 mRNA和蛋白除了定位于基质成纤维细胞和血管内皮细胞外，还共同定位于肿瘤细胞本身。
这些数据表明，DSRCT中的几种细胞类型大量产生CCN2，并突出了该因子在肿瘤细胞生长、基质生成和血管生成的自分泌和旁分泌调节中的潜在作用。
