Diagnostic and treatment challenges in neurofibroma concerning the external nose.

[BACKGROUND] This paper discusses clinical features and management aspects of an extremely rare entity, neurofibroma of the external nose.

[METHODS] Database searches were performed using PubMed, Embase and Google Scholar to identify previously published articles.

[RESULTS] Twelve articles comprising 13 patients with neurofibroma of the external nose were included. The mean age of presentation was 31 years. Sixty-nine per cent of patients were diagnosed at final histopathology. External approach rhinoplasty was performed in 76.9 per cent of patients, while the intranasal approach was used in 15.3 per cent of patients. There was a 15.3 per cent association with neurofibromatosis type 1. Recurrence was noted in 23 per cent of patients.

[CONCLUSION] It may be challenging to diagnose this entity clinically because of its rarity and striking features on histopathology. Neurofibroma of the external nose should be kept in mind as a differential diagnosis for any soft progressive swelling over the nose. Management requires complete excision, with cosmesis and restoration of functions.