Figure.

Hepatic endothelial gain-of-function KRAS^G12D or BRAFV^600E mutations cause cavernous vascular malformations via activation of KRAS-BRAF-MAP2K-MAPK1 signaling pathway. Contiguous expression of adherens junctional proteins in KRAS or BRAF mutant hepatic endothelium promote cavernous sinusoidal expansion instead of branching. Concurrent administration of Food and Drug Administration-approved dabrafenib and trametinib inhibits growth of BRAF^V600E-driven cavernous sinusoidal malformations. Adapted from Janardhan HP et al.¹³