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Matti Tapio Seppälä, Markku Alarik Sainio, Matti Jouko Johannes Haltia, Jaakko Jyri Kinnunen, Kirsi Hannele Setälä, and Juha Erik Jääskeläinen

M ost schwannomas are single sporadic benign neoplasms. Bilateral vestibular schwannomas are the classic hallmark of neurofibromatosis type 2 (NF2), which predisposes patients to multiple schwannomas on cranial, spinal, and peripheral nerves and to intracranial and intraspinal meningiomas and intramedullary ependymomas. 1, 2, 7, 17 The term schwannomatosis 4, 14, 16, 20 or neurilemmomatosis 25 has been used to describe patients with multiple nonvestibular schwannomas with no other signs

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Leonard I. Malis

Neurofibromatosis type 2 (NF2) is a rare disease, affecting only approximately 1000 patients in the entire United States. The diagnosis requires the presence of bilateral acoustic neuromas, but many other tumors of the nervous system are also present. It is a very different disease from von Recklinghausen's neurofibromatosis, NF1. The remarkable genetic research in recent years has defined the origin of NF2 to be the lack of a specific suppressor protein, known as Merlin. While we await a method to replace this protein, the neurosurgical care of these patients is a formidable problem. The author reviews his personal series of 41 patients with NF2 treated during the past 30 years and presents 10 cases in detail to demonstrate their considerable range of differences and the treatment problems they have posed.

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Fu Zhao, Shi-wei Li, Shun Zhang, Peng Li, Chi Zhao, Xiao-bin Zhao, Chun-Hong Wang, Jing Zhang, Bo Wang, and Pi-nan Liu

DM , Butman JA , Neurofibromatosis type 2 . Lancet . 2009 ; 373 ( 9679 ): 1974 – 1986 . 10.1016/S0140-6736(09)60259-2 21968382 3 Baser ME , Makariou EV , Parry DM . Predictors of vestibular schwannoma growth in patients with neurofibromatosis Type 2 . J Neurosurg . 2002 ; 96 ( 2 ): 217 – 222 . 10.3171/jns.2002.96.2.0217 4 Samii M , Gerganov V , Samii A . Microsurgery management of vestibular schwannomas in neurofibromatosis type 2: indications and results . Prog

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Nasser Mohammed, Yi-Chieh Hung, Zhiyuan Xu, Tomas Chytka, Roman Liscak, Manjul Tripathi, David Arsanious, Christopher P. Cifarelli, Marco Perez Caceres, David Mathieu, Herwin Speckter, Gautam U. Mehta, Gregory P. Lekovic, and Jason P. Sheehan

( 6429 ): 515 – 521 . 10.1038/363515a0 2 Evans DG , Huson SM , Donnai D , A clinical study of type 2 neurofibromatosis . Q J Med . 1992 ; 84 ( 304 ): 603 – 618 . 1484939 3 Goutagny S , Bah AB , Henin D , Long-term follow-up of 287 meningiomas in neurofibromatosis type 2 patients: clinical, radiological, and molecular features . Neuro Oncol . 2012 ; 14 ( 8 ): 1090 – 1096 . 22711605 10.1093/neuonc/nos129 4 Baser ME , Friedman JM , Aeschliman D , Predictors of

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Samiya Abi Jaoude, Matthieu Peyre, Vincent Degos, Stéphane Goutagny, Béatrice Parfait, and Michel Kalamarides

family genetic register service . Am J Med Genet A . 2010 ; 152A ( 2 ): 327 – 332 . 10.1002/ajmg.a.33139 20082463 2 Evans DG , Sainio M , Baser ME . Neurofibromatosis type 2 . J Med Genet . 2000 ; 37 ( 12 ): 897 – 904 . 10.1136/jmg.37.12.897 11106352 3 Asthagiri AR , Parry DM , Butman JA , Neurofibromatosis type 2

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Usman A. Khan, Jillian H. Plonsker, Rick A. Friedman, and Marc S. Schwartz

, Wilkinson EP . The combined petrosal approach to the petroclival region . In: Friedman RA , ed. Lateral Skull Base Surgery: The House Clinic Atlas . Thieme Medical Publishers ; 2012 . 10.1055/b-0034-84483 2 Schwartz MS , Lekovic GP . Microsurgery for neurofibromatosis type 2 focusing on vestibular schwannoma . In: Carlson ML , Link MJ , Driscoll CLW , eds. Comprehensive Management of Vestibular Schwannoma . Thieme Medical Publishers ; 2019 : 535 . 3 Schwartz MS , Otto

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Paul D. Sawin, Nicholas Theodore, and Harold L. Rekate

. Mautner VF , Tatagiba M , Guthoff R , et al : Neurofibromatosis 2 in the pediatric age group. Neurosurgery 33 : 92 – 96 , 1993 Mautner VF, Tatagiba M, Guthoff R, et al: Neurofibromatosis 2 in the pediatric age group. Neurosurgery 33: 92–96, 1993 11. Mautner VF , Tatagiba M , Lindenau M , et al : Spinal tumors in patients with neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety [published erratum

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Jung Won Choi, Ji Yeoun Lee, Ji Hoon Phi, Kyu-Chang Wang, Hyun-Tai Chung, Sun Ha Paek, Dong Gyu Kim, Sung-Hye Park, and Seung-Ki Kim

th year after GKS ( Fig. 2 ). The facial nerve function was unconverted after GKS. F ig . 2. Kaplan-Meier plot representing the survival function for useful hearing preservation after GKS. G-R = Gardner-Robertson. Discussion Clinical Features of Pediatric NF2 Neurofibromatosis Type 2 usually occurs in young adulthood, and patients present with vestibular symptoms such as hearing difficulty, tinnitus, and dizziness, which are caused by VSs. Previous studies on pediatric NF2

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Melanie Lang-Orsini, Julian Wu, Carl B. Heilman, Alina Kravtsova, Gene Weinstein, Neel Madan, and Knarik Arkun

of exclusion. This distinction is important, however, because of differences in prognosis and treatment modalities between the two tumors. We report the case of a 41-year-old man with primary meningeal melanomatosis masquerading as neurofibromatosis type 2. This case illustrates the above-mentioned clinical, radiological, and pathological diagnostic difficulties that may arise when evaluating these neoplasms. In addition, we review the recent literature concerning primary meningeal melanocytic

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Takehiko Harada, Richard M. Irving, John H. Xuereb, David E. Barton, David G. Hardy, David A. Moffat, and Eamonn R. Maher

M eningioma is seen relatively frequently in neurological practice, and it accounts for 13% to 19% of all primary brain tumors. 20 The etiology of meningioma is unknown. Most cases are sporadic, but meningioma occurs in as many as half of the patients with the dominantly inherited familial syndrome of neurofibromatosis type 2 (NF2). 7 This disorder is characterized by the development of multiple tumors of the central nervous system (CNS), among which the most common feature is vestibular