Atypical cellular neurothekeoma – a diagnosis to be aware of

Abstract

We present a rare case of atypical cellular neurothekeoma arising in the area of a previous nose piercing on the ala of a 34-year-old female. Neurothekeoma is a benign tumour of probable nerve sheath origin. The cell of origin for neurothekeoma is still unknown but most ultrastructural and immunohistochemical studies have favoured the Schwann cell perineurium or fibroblasts. A similar but distinct, more cellular variant with a less prominent myxoid component, was termed cellular neurothekeoma and, unlike myxoid neurothekeoma, the cellular variety generally lacks immunoreactivity to most neuronal markers. Atypical cellular neurothekeoma is characterised by the following features: large size of up to 6 cm, penetration into subcutaneous fat and or muscle, diffusely infiltrating borders, vascular invasion, a high mitotic rate and marked cytological pleomorphism. In this report we provide a review of the relevant literature and describe the clinical, histological and immunohistochemical features of an atypical cellular neurothekeoma excised from the ala nasi of a 34-year-old female, a hitherto unreported lesion in the plastic surgery literature.