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Clear cell sarcoma of the kidney expresses insulinlike growth factor-II but not WT1 transcripts
1Department of Pathology, University of Otago Medical School, Dunedin, New Zealand.
Abstract:
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.
Insights
Clear cell sarcoma of the kidney (CCSK) is distinct from Wilms' tumors (WTs). CCSK may arise from undifferentiated blastemal cells, suggesting it be renamed stromal cell sarcoma of the kidney.
Area of Science:
- Pediatric Oncology
- Molecular Biology
- Renal Pathology
Background:
- Clear cell sarcoma of the kidney (CCSK) and Wilms' tumors (WTs) are pediatric renal malignancies with distinct clinical behaviors.
- The histogenesis of CCSK remains unclear, differentiating it from the well-characterized WT.
Purpose of the Study:
- To investigate the cellular origin and histogenesis of CCSK.
- To compare the molecular profiles of CCSK, WTs, and fetal kidneys.
Main Methods:
- Molecular hybridization techniques, including Northern blot and in situ hybridization.
- Analysis of gene expression patterns for insulin-like growth factor-II (IGF-II) and WT1 in tumor and fetal kidney samples.
Main Results:
- Both CCSKs, WTs, and fetal kidneys expressed abundant IGF-II transcripts.
- WTs and fetal kidneys showed significant WT1 gene expression, but CCSKs did not.
- CCSK cells exhibited hybridization patterns similar to primitive metanephrogenic blastemal and early stromal cells.
Conclusions:
- CCSK is molecularly and developmentally distinct from WT.
- CCSK likely originates from undifferentiated metanephrogenic blastemal cells with stromal differentiation potential.
- The findings support renaming CCSK as stromal cell sarcoma of the kidney.