NCIt related terms : Neurofibromatosis, NOS; Neurofibromatosis Syndrome;
NCIt definition : An autosomal dominant hereditary neoplastic syndrome. Two distinct clinicopathological
entities are recognized: neurofibromatosis type 1 and neurofibromatosis type 2. Neurofibromatosis
type 1 is associated with the presence of cafe-au-lait cutaneous lesions, multiple
neurofibromas, malignant peripheral nerve sheath tumors, optic nerve gliomas, and
bone lesions. Neurofibromatosis type 2 is associated with the presence of schwannomas,
meningiomas, and gliomas.;