Although the terms neurothekeoma and nerve sheath myxomas were used interchangeably, they are now described as distinct entities, with the later exhibiting positive immunohistochemistry for S100 and Sox-10 and moderate/diffuse reactivity to glial fibrillary acidic protein (GFAP) and CD57 [4,5,6]. Here, B3GAT1 is linked to neurothekeoma.