What causes rhabdoid renal cell carcinoma (RRCC)?

Updated: Mar 11, 2019
  • Author: Ronald J Cohen, MB, BCh, PhD, FRCPA, FFPATH; Chief Editor: Liang Cheng, MD  more...
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Answer

Answer

Cells with rhabdoid differentiation arise through transformation of malignant renal epithelial cells, analogous to the process of sarcomatoid change. [8, 9, 10, 11] One study has demonstrated loss of chromosome 3p and/or mutation of the VHL gene in both the rhabdoid and clear cell components of 2 rhabdoid renal cell carcinomas (RRCCs), confirming an epithelial derivation for the rhabdoid cells. [9]


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