Abstract
Neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis are a group of related
classically inherited but often times sporadic tumor suppressor syndromes. Neuro-oncologists
should recognize these syndromes, initiate necessary tests in patients with a clinical
suspicion, and support genetic counseling of patients and families. In this review,
clinical presentation, diagnostic criteria, day-to-day management including supportive
care as well as updates on genetics, and experimental treatment strategies are discussed.
Keywords
neurofibromatosis - schwannomatosis - neurofibroma - schwannoma - meningioma - ependymoma