Current Real-world Status of Treatment and Problems in Neurofibromatosis 1 Patients with Diffuse Plexiform Neurofibroma

Abstract

Patients with neurofibromatosis 1 (NF1) have various clinical symptoms. In particular, diffuse plexiform NF (dNF) negatively influences the quality of life in NF1 patients due to pain, disfigurement and other problems. An epidemiological study showed that dNF accounted for approximately 30% of NF1 patients with skin lesions. This tumor was found mainly in the trunk. Furthermore, a survey of NF1 patients who underwent surgical treatment with cNF or dNF showed that there was no significant difference between the two groups in terms of medical costs related to surgical procedures. DNF is a special tumor, its clinical characteristics need to be well recognized and appropriate treatment should be given.