title: Clinicopathologic assay of 15 tumor resections in a family with neurofibromatosis type 2.
abstract: The objective of this study is the management of multiple family members with multiple neurofibromatosis type 2 (NF2) related tumors of the skull base that can be challenging, on purely technical, decision-making, and ethical levels. These issues are addressed in this manuscript based on an experience treating an unique large family with NF2. A retrospective chart review was performed, reviewing clinical, radiological, surgical, and pathological data. A unique family of 17 siblings, whose father was the proband as a sporadic mutation is reported. Over a 4-month period, five of eight affected siblings underwent 12 procedures for resection of 15 different NF2-related tumors. This single family experience of NF2-related skull base tumors underscores the importance of preservation of function and quality of life as the major determinants of treatment success.
authors.affiliation: Department of Neurological Surgery, University of Washington, Harborview Medical Center, Seattle, Washington.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Di Maio
authors.forename: Goran
authors.initials: G
authors.lastname: Mrak
authors.forename: Gordana
authors.initials: G
authors.lastname: Juric-Sekhar
authors.forename: Donald
authors.initials: D
authors.lastname: Born
authors.forename: Alessandra
authors.initials: A
authors.lastname: Mantovani
authors.forename: Laligam N
authors.initials: LN
authors.lastname: Sekhar
keywords: NF2 associated tumors; ethics; meningioma; neurofibromatosis type 2; quality of life; vestibular schwannoma
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