Cases reported "Neurothekeoma"

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1/4. Intraoral myxoid nerve sheath tumour.

    A case of an intraoral myxoid nerve sheath tumour of the dorsum of the tongue in a 73-year-old Caucasian male is reported. This case describes the oldest patient with this pathology to date. Immunoperoxidase staining for neuron-specific enolase (NSE) and epithelial membrane antigen (EMA) expression demonstrated the perineural origin of the lesion.
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2/4. Cellular neurothekeoma of the oral mucosa.

    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.
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3/4. Atypical cellular neurothekeoma.

    BACKGROUND: neurothekeoma is a distinctive neoplasm that in the past was often lumped into the broad category of benign peripheral nerve sheath tumors. Atypical cellular neurothekeoma is a rare variant of this neoplasm. This atypical variant is characterized by features that can include the following: large sizes (up to 6 cm), deep penetration extending into skeletal muscle and/or subcutaneous fat, diffusely infiltrative borders, vascular invasion, high mitotic rate, and marked cytologic pleomorphism. methods: In this report, we describe the clinical, histopathologic, and immunohistochemical features of a case of atypical cellular neurothekeoma in a 34-year-old woman. RESULTS: This neoplasm demonstrated both the cellular subtype and intermediate subtype. In the cellular area, cells demonstrated rounded or ovoid nucleus with abundant cytoplasm. Mitotic activity ranged from 8 to 12 per 10 high-powered fields, and few individual cell necroses were present. In the intermediate area, cells were arranged as smaller nests with admixed abundant mucin. Mitotic activity ranged from 1 to 2 per 10 high-powered fields. These cells were negative for S100, epithelial membrane antigen, and smooth muscle actin. CONCLUSION: Dermatologists should be aware of the features of atypical cellular neurothekeoma. Although the atypical features raise concern about the malignant potential of this lesion, previous cases show that complete surgical excision of these lesions is curative.
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4/4. Spinal nerve sheath myxoma (neurothekeoma).

    Nerve sheath myxoma (NSM) is a rare, benign tumor of predominantly cutaneous location. They usually arise from small cutaneous nerves in the head, neck, and extremities, but exceptionally they arise from spinal nerve roots. Only three cases of intraspinal NSM have been reported. Herein is describe two additional cases of spinal NSM. The patients were 64-year-old and 31-year-old men who presented with low back pain. spine magnetic resonance imaging showed small intradural extramedullary masses at the L2-3 level. Both tumors had typical histological features of myxoid-type NSM. The tumors had a strong immunoreactivity for vimentin, S-100 protein, and neuron-specific enolase and focal expression of epithelial membrane antigen and phosphorylated neurofilament. Ultrastructural observation of tumor cells with perineurial, fibroblast-like, and Schwann-cell differentiation suggests an origin from nerve sheath precursor cells.
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