The NF2 gene product, merlin, is a cell membrane protein that functions as a tumor suppressor.5 Merlin is involved in various signaling pathways including Ras/Raf/MEK/ERK, PI3K/Akt/mTORC1, NF-kB, and Hippo signaling pathways (Figure 2).6–9 Mutations in merlin results in changes in its interactions with the actin cytoskeleton.10,11 Welling et al. studied a series of patients with VS and found that the frequency, type, and distribution of NF2 mutations were different between sporadic and familial NF2-associated tumors. Here, NF2 is linked to neoplasm.