Yun K
Department of Pathology, University of Otago Medical School, Dunedin, New Zealand.
Am J Pathol. 1993 Jan;142(1):39-47.
Two cases of clear cell sarcoma of the kidney (CCSK), five Wilms' tumors (WTs), and three fetal kidneys were studied by molecular hybridization to elucidate the histogenesis of CCSK. Northern blot and in situ hybridization demonstrated that all the CCSKs, WTs, and fetal kidneys contained abundant insulinlike growth factor-II (IGF-II) transcripts, whereas WTs and fetal kidneys--but not CCSKs--showed significant expression of WT1 gene, a candidate tumor suppressor gene implicated in the etiology of WTs. Comparative analysis of in situ hybridization of IGF-II and WT1 transcripts in CCSKs and fetal kidneys revealed that CCSK cells showed similar hybridization patterns to primitive metanephrogenic blastemal cells and early stromagenic cells. The data strongly suggest that CCSK is distinct from WT and may be derived from undifferentiated metanephrogenic blastemal cells with potential to differentiate into a stromal cell lineage. The result also suggests that this unique tumor should, more appropriately, be known as stromal--rather than clear--cell sarcoma of the kidney.
为阐明肾透明细胞肉瘤（CCSK）的组织发生，对2例肾透明细胞肉瘤、5例肾母细胞瘤（WTs）和3个胎儿肾脏进行了分子杂交研究。Northern印迹法和原位杂交显示，所有CCSK、WTs和胎儿肾脏均含有丰富的胰岛素样生长因子-II（IGF-II）转录本，而WTs和胎儿肾脏（但CCSK无此现象）显示WT1基因有显著表达，WT1基因是一种候选肿瘤抑制基因，与WTs的病因有关。对CCSK和胎儿肾脏中IGF-II和WT1转录本的原位杂交进行比较分析发现，CCSK细胞显示出与原始后肾胚基细胞和早期基质生成细胞相似的杂交模式。这些数据强烈表明，CCSK与WT不同，可能起源于未分化的后肾胚基细胞，具有分化为基质细胞谱系的潜力。该结果还表明，这种独特的肿瘤更合适的名称应为肾基质细胞肉瘤，而非肾透明细胞肉瘤。
