title: Cellular neurothekeoma of the oral mucosa.
abstract: Cellular neurothekeoma is an unusual benign neoplasm which, despite its name, is of uncertain origin. This report describes a cellular neurothekeoma of the cheek mucosa, the first at this site. The tumour presented in a 29-year-old man as a discrete mucosal thickening. Histology showed a generally well circumscribed, but unencapsulated, solid tumour which replaced the entire lamina propria and permeated between minor salivary glands and bundles of striated muscle in the submucosa. There was a sub-epithelial Grenz zone. The tumour was composed of nodules of pale, epithelioid cells separated by fascicles of spindle cells, with smaller strands and nests superficially. The nuclei were vesicular and, though mainly bland, occasionally atypical. The stroma was moderately infiltrated by mixed chronic inflammatory cells. Prominent nerves and blood vessels were seen at the periphery of the lesion, and neoplastic cells were noted within intact striated muscle fascicles. With immunohistochemistry, all the neoplastic cells strongly expressed NKI/C3, synaptophysin, neurone-specific enolase and vimentin, some expressed smooth muscle actin and PGP 9.5, but all were negative for S100, factor XIIIa, CD34, CD56, CD57, CD68, chromogranin A, desmin, epithelial membrane antigen and von Willebrand factor. The origin of the lesion is thus speculative. It was, however, completely excised and in 12 months there has been no recurrence.
authors.affiliation: Department of Oral Pathology, Eastman Dental Institute for Oral Healthcare Sciences, University College London, 256 Grays Inn Road, London WC1X 8LD, UK. a.barrett@eastman.ucl.ac.uk
authors.forename: A W
authors.initials: AW
authors.lastname: Barrett
authors.forename: M
authors.initials: M
authors.lastname: Suhr
mesh_terms: D000328:Adult; D003176:Complement C3 / Q000378:metabolism; D006801:Humans; D008297:Male; D009061:Mouth Mucosa; D009062:Mouth Neoplasms / Q000378:metabolism / Q000473:pathology*; D009363:Neoplasm Proteins / Q000378:metabolism; D018321:Neurothekeoma / Q000378:metabolism / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D016708:Synaptophysin / Q000378:metabolism; D014746:Vimentin / Q000378:metabolism
chemical_list: D003176:Complement C3; D009363:Neoplasm Proteins; D016708:Synaptophysin; D014746:Vimentin; D010751:Phosphopyruvate Hydratase