MSCH are characterized by a diffuse proliferation of S-100 positive spindle cells within the lamina propria and absence of ganglion cells, distinguishing these tumors from other neural lesions such as neurofibromas or mucosal neuromas.1,3,5 Staining is positive for S-100, with one other known documented case of positive for SRY-related HMG-box gene 10.6 Although MSCH shares similarities with Schwannoma, as both are composed of spindle cells, they can be differentiated histologically. This evidence concerns the gene SOX10 and schwannoma.