Subtotal and total resection of superficial plexiform neurofibromas of face and neck: four case reports

Abstract

Introduction: Plexiform neurofibromas are benign tumours of the peripheral nerves and connective tissue. They develop most often in patients with neurofibromatosis type 1 (NF1) and often grow continuously. Removal of plexiform neurofibromas is usually unsatisfactory because the network-like growth of these tumours often involves multiple nerve fascicles and other adjacent tissues. It has been previously shown that magnetic resonance tomography can distinguish the growth patterns of plexiform neurofibromas into three different categories: superficial, displacing and invasive.

Patients and methods: Three cases are described with successful subtotal resections of superficial plexiform neurofibromas, and one case with total resection following the diagnosis of tumour subtype using magnetic resonance imaging (MRI).

Results: There was a significant, lasting improvement in appearance which demonstrates that surgical intervention in the case of superficial plexiform neurofibroma is valuable.

Conclusion: Careful classification of plexiform neurofibroma by means of MRI provides valuable information for the surgical management of patients. It enables the distinction to be drawn between this subtype and the other two subtypes of plexiform neurofibromas.