Lloyd S K, Evans D G
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.
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.
Neurochirurgie. 2018 Nov;64(5):375-380. doi: 10.1016/j.neuchi.2015.10.006. Epub 2016 Jan 27.
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.
2型神经纤维瘤病（NF2）是一种复杂的疾病，其特征是多发性神经鞘瘤，尤其是前庭神经鞘瘤的发生，以及包括脑膜瘤和脊髓室管膜瘤在内的其他类型的良性肿瘤。由于其多系统性质，NF2的管理需要多学科方法。在英国，NF2患者的护理已集中到曼彻斯特、剑桥、牛津和伦敦的四个“中心”，每个中心都有相关的“分支”中心。每个中心都有一个核心多学科团队，由遗传学、耳鼻喉科、神经外科、儿科、神经内科、听力学、放射学、心理学、物理治疗、专科护士和行政人员组成。此外，核心团队还可获得整形外科、眼科、周围神经外科以及成人和儿童肿瘤学方面的服务。每周有多学科门诊，每次有六至八名患者。在团队会议上讨论每个患者的情况，然后与患者讨论做出的管理决策。所有患者至少每年接受一次复查，并进行年度头部磁共振成像（MRI）检查和每三年一次的脊柱MRI检查。进行年度听力学评估。如有指征，可提供人工耳蜗植入和听觉脑干植入。手术、立体定向放射外科和贝伐单抗治疗可用于治疗颅内和脊髓肿瘤。英国该服务的整合为患者护理带来了显著益处，从长远来看，将提供有力的患者预后数据，为优化NF2患者的管理提供证据基础。
