[Neurofibromatoses]

Abstract

Neurofibromatosis type 1 (NF1) and type 2 (NF2) are hereditary autosomal dominant neurocutaneous disorders, the phacomatoses, characterized by the development of tumors derived from the cells of the peripheral nerve sheath and also includes schwannomatosis. Regarding the clinical and genetic aspects they are, however, two distinct entities which are described separately in the following review. In addition to the typical clinical presentation and diagnostic criteria, characteristic imaging findings are presented especially in terms of the role of imaging in the diagnosis, follow-up and assessment of prognostic aspects in these multisystemic disorders.