How is adult rhabdoid renal cell carcinoma (RRCC) differentiated from pediatric malignant rhabdoid rumor (MRT)?

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

RR CC was named for its morphologic resemblance to pediatric malignant rhabdoid tumor (MRT) of the kidney, which is a highly aggressive tumor characterized by cells that resemble rhabdomyoblasts and by genetic alterations involving chromosome 22, particularly the hSNF5/INI1 gene on 22q11.2. [13, 14] MRT usually arises in children younger than 3 years, and the occasional cases previously reported as adult MRT are now considered more likely to be RRCC in which rhabdoid components have overgrown the original histologic tumor type. [8]

Go to Renal Cell Carcinoma and Clear Cell Renal Cell Carcinoma for more complete information on these topics.


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