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Review
. 2018 Feb;38(1):73-85.
doi: 10.1055/s-0038-1627471. Epub 2018 Mar 16.

Neurofibromatosis and Schwannomatosis

Affiliations
Review

Neurofibromatosis and Schwannomatosis

Scott R Plotkin et al. Semin Neurol. 2018 Feb.

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.

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