Section Editor: Dharam M. Ramnani, MD

Virginia Urology, Richmond, VA, USA

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Molecular Genetics: Metanephric family of tumors, including metanephric adenoma, metanephric stromal tumor, and metanephric adenofibroma show BRAF V600E mutation in 90% of cases. There is corresponding diffuse cytoplasmic immunoreactivity for BRAF.

BRAF mutations stimulate kinase activity and constititively activate ERK signaling pathway which promotes cell survival. Rare cases show BRAF V600D or K601L mutations.
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