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Cellular Neurothekeoma : Introduction & Clinical Features

 
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Introduction: Neurothekeoma (Cellular Neurothekeoma) is a benign tumor of fibrohistiocytic origin, hence the term is clearly a misnomer. However, it is still commonly used because it is well-entrenched in the literature. Neurothekeoma is unrelated to the entity previously called classic myxoid neurothekeoma which is of Schwannian origin and has now renamed as nerve sheath myxoma.

Neurothekeoma is a tumor of young patients with a peak incidence in the 2nd decade of life. It is more common in women (F:M=2:1). The most frequent sites of involvement are head/neck region, upper extremities, and the shoulder. They present as small (usually <1.5 cm), solitary, dermal lesions that may extend into the subcutis (50% of cases) or skeletal muscle (rare).

The image shows variably-sized round to oval nests of spindle and epithelioid cells in the dermis separated by dense collagen bundles.

Image courtesy of: Rami Al-Rohil, MBBS, Department of Pathology, Duke University School of Medicine, Durham, North Carolina, USA; used with permission.

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