Management of an incidental malignant peripheral nerve sheath tumour in the parapharyngeal space.

BACKGROUND: Parapharyngeal space tumours are uncommon and represent 0.5-1 per cent of all head and neck neoplasms; 20-30 per cent of these are malignant. Malignant peripheral nerve sheath tumours are rare and mostly encountered in patients with neurofibromatosis type 1. Only four cases of parapharyngeal space tumours have been reported in the English language in patients without neurofibromatosis type 1. CASE REPORT: We report the case of a 64-year-old man with no stigmata of neurofibromatosis type 1, in whom a mass in the left pre-styloid region of the parapharyngeal space was an incidental finding following magnetic resonance imaging for investigation of cervical spine problems. The mass was consequently removed using a transcervical approach. A histological review revealed a low-grade malignant peripheral nerve sheath tumour. DISCUSSION: We consider the pathophysiology of this highly malignant tumour as well as the challenging anatomy of the parapharyngeal space and the surgical and other therapeutic modalities utilised to treat this condition.