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颈部良性周围非颅神经鞘肿瘤。

Benign Peripheral Non-cranial Nerve Sheath Tumors of the Neck.

机构信息

Instituto de Investigación Sanitaria del Principado de Asturias, Avenida de Roma s/n, 33011, Oviedo, Spain.

Department of Otolaryngology, Hospital Universitario Central de Asturias, Instituto Universitario de Oncología del Principado de Asturias, University of Oviedo, CIBERONC, Avenida de Roma s/n, 33011, Oviedo, Spain.

出版信息

Adv Ther. 2022 Aug;39(8):3449-3471. doi: 10.1007/s12325-022-02191-5. Epub 2022 Jun 11.

DOI:10.1007/s12325-022-02191-5
PMID:
35689724
Abstract

Benign peripheral non-cranial nerve sheath tumors are rare lesions, including both schwannomas and neurofibromas. These tumors arise from Schwann cells, and may originate from any peripheral, cranial, or autonomic nerve. Most of them are localized and sporadic but multifocal systemic forms can occur. Cervical sympathetic chain, brachial plexus, cervical plexus and spinal roots and nerves are the major nerve systems commonly affected. Dumbbell-shaped intra- and extradural tumors occur most commonly in the cervical spine, as well as purely extradural and paravertebral tumors. The management of these tumors has improved greatly owing to the developments in imaging techniques and surgical innovations such as endoscopically assisted approaches and robotic surgery. Microsurgical intracapsular excision of the tumor helped by the use of intraoperative fluorescent dyes and intraoperative neurophysiological monitoring minimize postoperative neural deficit, since most schwannomas are encapsulated. Most tumors can be removed with a low rate of complications and recurrence. Radiotherapy should be considered for growing lesions that are not amenable to surgery. In asymptomatic patients, observation and serial scans is an option for elderly infirm patients.

摘要

良性周围非颅神经鞘肿瘤是罕见的病变,包括神经鞘瘤和神经纤维瘤。这些肿瘤来源于施万细胞,可能起源于任何周围、颅神经或自主神经。它们大多数是局限性和散发性的,但也可能发生多灶性系统性形式。颈椎交感神经链、臂丛、颈丛和脊神经根和神经是最常受影响的主要神经系统。哑铃形的硬膜内和硬膜外肿瘤最常见于颈椎,也有单纯的硬膜外和椎旁肿瘤。由于成像技术和手术创新(如内窥镜辅助方法和机器人手术)的发展,这些肿瘤的治疗得到了极大的改善。术中荧光染料和术中神经生理监测有助于微外科囊内切除肿瘤,最大限度地减少术后神经缺损,因为大多数神经鞘瘤是有包膜的。大多数肿瘤可以通过低并发症和低复发率切除。对于无法手术的生长性病变,应考虑放疗。对于无症状患者,对于年老体弱的患者,观察和连续扫描是一种选择。

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J Craniofac Surg. 2022 May 1;33(3):803-808. doi: 10.1097/SCS.0000000000008381. Epub 2021 Dec 2.
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Trametinib therapy for children with neurofibromatosis type 1 and life-threatening plexiform neurofibroma or treatment-refractory low-grade glioma.曲美替尼治疗伴有危及生命的丛状神经纤维瘤或治疗抵抗性低级别胶质瘤的 1 型神经纤维瘤病患儿。
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外周神经鞘瘤术中钠荧光成像——一种新的有前景的辅助诊断工具。
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Genetic basis of neurofibromatosis type 1 and related conditions, including mosaicism.1型神经纤维瘤病及相关病症(包括嵌合体)的遗传基础。
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