title: Neurofibromatosis type 2 service delivery in England.
abstract: Neurofibromatosis type 2 (NF2) is a complex disease characterized by the development of multiple schwannomas, especially vestibular schwannomas, as well as other types of benign tumours including meningioma and spinal ependymoma. Due to its multisystem nature, the management of NF2 requires a multidisciplinary approach. In England, the delivery of care for NF2 patients has been centralized to four-"hub" centres in Manchester, Cambridge, Oxford and London each having associated "spoke" centres. Each centre has a core multidisciplinary team consisting of genetics, otolaryngology, neurosurgery, paediatrics, neurology, audiology, radiology, psychology, physiotherapy, specialist nurses and administrative staff. In addition, the core team has access to plastic surgery, ophthalmology, peripheral nerve surgery and adult and paediatric oncology. There are weekly multidisciplinary clinics each with six to eight patients. Each patient is discussed during a team meeting and the management decisions that are made are then discussed with the patients. All patients are reviewed at least annually and have annual head magnetic resonance imaging (MRI) and three yearly spinal MRI. Annual audiological assessment is performed. Cochlear implantation and auditory brainstem implantation are offered if indicated. Surgery, stereotactic radiosurgery and bevacizumab therapy are available for the management of intracranial and spinal tumours. The integration of the service in England has provided significant benefits to patient care and, in the long term, will provide robust patient outcome data that will provide an evidence base to assist in optimizing management of patients with NF2.
authors.affiliation: Institute of Cancer Science, University of Manchester, Manchester, M13 9PL, UK; Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, M13 9WL, UK. Electronic address: sklloyd@me.com.
authors.forename: S K
authors.initials: SK
authors.lastname: Lloyd
authors.affiliation: Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, M13 9WL, UK; Manchester Centre for Genomic Medicine, University of Manchester, Manchester Academic Health Sciences Centre (MAHSC), Manchester, UK.
authors.forename: D G
authors.initials: DG
authors.lastname: Evans
mesh_terms: D004739:England; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008577:Meningeal Neoplasms / Q000601:surgery; D008579:Meningioma / Q000601:surgery; D016518:Neurofibromatosis 2 / Q000601:surgery*; D009464:Neuroma, Acoustic / Q000601:surgery*; D019635:Neurosurgical Procedures; D005791:Patient Care; D016634:Radiosurgery / Q000379:methods
keywords: Auditory brainstem implantation; Bevacizumab; Bévacizumab; Cochlear implantation; Implant auditif du tronc cérébral; Implantation cochléaire; Neuriniome de l’acoustique; Neurofibromatose de type 2; Neurofibromatosis type 2; Vestibular schwannoma