An unusual localization of neurofibromas in a patient with neurofibromatosis type-1: The great auricular nerve

Abstract

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by café au lait macules, axillary freckling, neurofibromas, Lisch nodules, skeletal abnormalities, and a tendency to develop neoplasms. Neurofibromas are benign peripheral nerve sheath tumors that occur commonly in individuals with NF-1. Neurofibromas originating from the vagus nerve and spinal nerves are rarely reported in the literature. Here, we report an interesting case of NF-1 with a striking cutaneous presentation and bilateral localization in the great auricular nerve with bilateral vagal and multiple spinal neurofibromas. This is the first report of an NF-1 case with bilateral symmetric localization in the great auricular nerve.