Neurofibromatosis type 1

Neurofibromatosis (NF) has perhaps been the most notorious of the neurocuta neous disorders. Both Quasimoto of Victor Hugo's The Hunchback Of Notre Dam e and John Merrick, known as the Elephant Man (who subsequently has been mo re properly classified as having Proteus syndrome, a quite different disord er), are 2 infamous examples that have shaped many popular misconceptions a bout this disease. Neurofibromatosis is now understood as a hamartomatous d isorder on the basis of molecular genetic studies. Studies on NF have been pivotal to understanding the functions of oncogenes in rumorigenesis.