Internal carotid artery sympathetic plexus neurofibroma - A case report

Neurofibromas are benign peripheral nerve sheath tumors most associated with neurofibromatosis type 1 (NF1 ). NF1 is an autosomal dominant disorder resulting from mutations in the NF1 gene located on chromosome 17. While neurofibromas frequently occur in cutaneous or plexiform forms, neurofibromas arising from the sympathetic plexus of the internal carotid artery (ICA) are rare. Only one case of an intracranial sympathetic plexus neurofibroma has been reported in the literature to date. Since most nerve sheath tumors arising from this region are schwannomas, and given the intimate association with the Internal carotid artery (ICA) and surrounding neurovascular structures of the carotid sympathetic plexus (CSP), precise diagnosis can be challenging but essential for surgical planning and optimizing patient outcomes. In this case report, we present the second documented case of an intracranial ICA sympathetic plexus neurofibroma and the first documented case in a patient with NF1. We highlight the unique clinical presentation, representing imaging features, and appropriate surgical management.

The authors report a case of a 28-year-old woman with NF1 presenting with intermittent pain behind the right eye and progressive vision deterioration. On clinical examination, the patient had a cranial nerve 3 palsy with decreased right eye opening and lateral/downward displacement of the right pupil. The medical history was notable for multiple neurofibroma resections in various locations, including the scalp, anterior neck, and the area around the hypoglossal nerve. Magnetic resonance imaging and diagnostic angiography revealed a right cavernous sinus mass encasing the right ICA at the branching of the ophthalmic artery. Surgical intervention achieved a gross total resection, with pathology confirming a CSP (carotid sympathetic plexus) neurofibroma.

The authors present the second documented case of an intracranial CSPcarotid sympathetic plexus neurofibroma and the first reported case of a CSPcarotid sympathetic plexus (carotid sympathetic plexus) neurofibroma and the first reported case of a CSP (carotid sympathetic plexus) neurofibroma in an NF1 patient, discussing relevant literature and key lessons learned.