In the cases of cNF and pNF, no additional pathogenic mutation is recurrently found in neurofibroma except the one located in NF1. 7,8 Clinical management largely comprises surgical resection, and no effective pharmacological agent is yet available for effective treatment or cure, although some agents have shown promising results in preclinical or clinical trials, and will be discussed in detail in this review. This evidence concerns the gene NF1 and plexiform neurofibroma.