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metanephric renal tumors

Wednesday 19 October 2016

Definition: The metanephric family of renal tumors encompasses metanephric adenofibroma , metanephric adenoma and metanephric stromal tumor ( in children).


- metanephric adenoma

Molecular pathology

- BRAF exon 15 mutations have been observed in 88% of metanephric adenomas, , in metanephric stromal tumors and in in metanephric adenofibroma.

- BRAF exon 15 mutation in metanephric stromal tumors

  • found in 11/17 cases (65%) of metanephric stromal tumors, all corresponding to a thymidine to adenine substitution at codon 600 (BRAF V600E).
  • All other renal stromal tumors tested were negative for BRAF exon 15 mutations. -* BRAF V600E mutations are encountered in the majority of metanephric stromal tumors, supporting a link with other metanephric tumors and suggesting a clonal event possibly affecting primordial renal cells.
  • BRAF V600E mutations have been associated with oncogene-induced senescence in other benign tumors, providing clues to the pathogenesis of these neoplasms in keeping with their overall benign behavior.
  • Results also suggest a potential diagnostic use for BRAF exon 15 mutations in differentiating metanephric stromal tumors from other pediatric renal stromal tumors, particularly in limited samples.