Neurofibromatosis and schwannomatosis

SR Plotkin, A Wick - Seminars in neurology, 2018 - thieme-connect.com
SR Plotkin, A Wick
Seminars in neurology, 2018thieme-connect.com
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.
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.
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