Article
BRAF fusions in pediatric histiocytic neoplasms define distinct therapeutic responsiveness to RAF paradox breakers.
Pediatric blood & cancer - 1 Jun 2021
Jain Payal, Surrey Lea F, Straka Joshua, Russo Pierre, Womer Richard, Li Marilyn M, Storm Phillip B, Waanders Angela J, Hogarty Michael D, Resnick Adam C, Picarsic Jennifer
Abstract excerpt
Pediatric histiocytic neoplasms are hematopoietic disorders frequently driven by the BRAF-V600E mutation. Here, we identified two BRAF gene fusions (novel MTAP-BRAF and MS4A6A-BRAF) in two aggressive histiocytic neoplasms. In contrast to previously described BRAF fusions, MTAP-BRAF and MS4A6A-BRAF do not respond to the paradox breaker RAF inhibitor (RAFi) PLX8394 due to stable fusion dimerization mediated by the...
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