두피에서 발생한 거대한 신경섬유종 1예

Abstract

Neurofibromatosis 1 (NF-1) is an inherited neurocutaneous disorder arising from the chromosome 17q with a prevalence of one in 4,000. Neurofibroma, Cafe-au-lait spots, freckles in axilla, Lisch nodule and bony dysplasia are features of NF-1. It is diagnosed preoperatively by history taking, physical examination, CT, MRI and intraoperatively by tissue biopsy. Scalp neurofibroma accompanying pain, motor disturbance, rapid growth, and suspicious of malignancy are indications for surgical removal. We present a case of NF-1 with two giant neurofibromas (15×7×4 cm, 3 ×5×2 cm) on the left scalp in a patient suffering from a head injury after falling down. The neurofibromas were successfully removed after diagnosed by MRI and histology.