Surgical Management of an Unresectable Trigeminal Nerve (V2) Plexiform Neurofibroma

C Neurofibromatosis is an autosomal dominant condition which affects 1 in 4,000 births, known to be caused by a genetic defect on chromosome 17. Clinically, this disease process is characterized by numerous cutaneous or subcutaneous neurofibromas, optic gliomas, and cafe au lait spots.1 Plexiform neurofibromas are slow growing, infiltrative tumors associated with Type 1 Neurofibromatosis (von Recklinghausen’s disease). Plexiform neurofibromas are most often noted in the head, neck, and face while less commonly seen in the spine, mediastinum, and abdomen. They are general slow growing tumors, however can undergo rapid growth during periods of hormonal changes including infancy, puberty as well as pregnancy. Plexiform neurofibromas are histologically characterized by a proliferation of Schwann cells within a nerve sheath and involve multiple nerve fasicles.2 They are known to be locally invasive benign tumors, however have the potential to undergo malignant change. Approximately 5% of these tumors undergo transformation into a malignant peripheral nerve sheath tumor (MPNST).3 The management of plexiform neurofibromas is surgical excision, as these infiltrating tumors are radioresistant and given their slow growth, chemotherapy is not beneficial.4,5 Unfortunately, because these tumors are infiltrating, sacrificing vital structures may be required in order to improve function and prevent further progression of this debilitating disease process.6