Malignant trigeminal schwannoma extending into the anterior skull base

Malignant schwannomas are rare tumours that seldom grow in the head and neck and their occurrence is even rarer in patients not affected by neurofibromatosis. The authors report a 40 year old woman with a left trigeminal malignant schwannoma (arising from V2), involving the infratemporal fossa and subsequently extending into the maxillary, sphenoid and ethmoidal sinuses, the orbit, and the intracranial compartment. Given the complex location of this tumour, the patient initially underwent radiation treatment; however due to a poor response to this form of treatment as well as disease progression, surgical resection requiring a combined Neurosurgical, ENT and Plastic Surgery was recommended. The planned operation included sacrificing the internal carotid artery and cavernous sinus, ipsilateral orbit exenteration and reconstruction with a free flap from the latissimus dorsi muscle. In this report, we discuss the sporadic occurrence of these tumours, the histo-pathological features and the management strategy along with a review of the literature.