Semin Neurol 2018; 38(01): 073-085
DOI: 10.1055/s-0038-1627471
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Neurofibromatosis and Schwannomatosis

Scott R. Plotkin
1  Department of Neurology and Cancer Center, Massachusetts General Hospital, Boston, Massachusetts
Antje Wick
2  Department of Neurology, University Hospital of Heidelberg, Heidelberg, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
16 March 2018 (online)


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.