DHH and plexiform neurofibroma: The first neurofibroma mouse model was established by depletion of the Nf1 gene in Krox20 lineage Schwann cells.116 Subsequently, similar neurofibroma mouse models have been successfully created by ablation of Nf1 under various Schwann cell promotor Cre lines, such as Dhh,117P0A118 and Plp. 99 Strikingly, these mice exclusively developed pNFs as no strong evidence of cNFs was reported.