Barrett A W, Suhr M
Department of Oral Pathology, Eastman Dental Institute for Oral Healthcare Sciences, University College London, 256 Grays Inn Road, London WC1X 8LD, UK.
Oral Oncol. 2001 Dec;37(8):660-4. doi: 10.1016/s1368-8375(00)00117-2.
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.
细胞性神经鞘黏液瘤是一种罕见的良性肿瘤，尽管其名称如此，但其起源尚不确定。本报告描述了一例颊黏膜的细胞性神经鞘黏液瘤，这是该部位的首例病例。该肿瘤发生于一名29岁男性，表现为黏膜局限性增厚。组织学检查显示，肿瘤通常边界清晰，但无包膜，为实性肿瘤，取代了整个固有层，并浸润至黏膜下层的小唾液腺和横纹肌束之间。上皮下有一无细胞区。肿瘤由淡染的上皮样细胞结节组成，这些结节被梭形细胞束分隔，表面有较小的条索和巢状结构。细胞核呈泡状，虽然主要为良性，但偶尔也有非典型性。间质有混合性慢性炎症细胞中度浸润。在病变周边可见明显的神经和血管，在完整的横纹肌束内可见肿瘤细胞。免疫组化显示，所有肿瘤细胞均强烈表达NKI/C3、突触素、神经元特异性烯醇化酶和波形蛋白，部分表达平滑肌肌动蛋白和PGP 9.5，但均不表达S100、因子ⅩⅢa、CD-34、CD-56、CD-57、CD-68、嗜铬粒蛋白A、结蛋白、上皮膜抗原和血管性血友病因子。因此，该病变的起源具有推测性。然而，肿瘤已被完全切除，12个月来未见复发。
