A 45-year-old woman presented with a painless mass in the tongue that had grown gradually over the past 20 years (Figure, arrowheads). She had café-au-lait spots and previous neurofibroma resections. Neurofibromatosis type 1 was also found in her father and two children. Recent speech problems made a resection necessary. Partial removal of the mass immediately improved communication. Pathological analysis showed plexiform neurofibroma without malignant transformation. Neurofibromatosis type 1 is an autosomal dominant disorder characterised by neurofibromas that can potentially affect every site of the body. Malignant transformation is rare and resection is indicated when functional or aesthetic impairment is associated.1
- 1. Hirbe AC, Gutmann DH. Neurofibromatosis type 1: a multidisciplinary approach to care. Lancet Neurol 2014; 13: 834-843.