A 54-year-old African-American male patient underwent removal of several cutaneous neurofibromas. Histopathologic examination revealed a nonencapsulated, haphazardly arranged proliferation of slender spindle cells in a myxoid stroma consistent with neurofibroma. Interestingly, each specimen exhibited numerous, large, multinucleate giant cells with nuclei arranged in a wreath-like or linear pattern. Immunoperoxidase staining revealed these cells to be negative with S-100 and CD-34. It was subsequently learned that this patient has neurofibromatosis type 1 (NF1). A retrospective review of all lesions coded as neurofibroma from our institution between 1 June 2006 and 28 August 2006 was performed. Biopsies of 53 cutaneous neurofibromas from 51 patients were reviewed. In these, multinucleate giant cells were present in only three (5.7%), all in patients with single lesions. Although the incidence of floret-like giant cells in neurofibromas of patients with NF1 is unknown, our findings suggest that the presence of these cells might be a clue to the presence of NF1.
Read full abstract