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[头颈部颅外神经鞘瘤:基于20例病例的临床治疗方法]

[Extracranial schwannomas of the Head and Neck: Clinical -approach based on 20 cases].

作者信息

Höing Benedikt, Stuck Boris A, Kansy Benjamin, Hussain Timon, Schlüter Anke, Holtmann Laura, Ringelstein Adrian, Mattheis Stefan, Lang Stephan, Dominas Nina

机构信息

Universitätsklinikum Essen, Klinik für Hals-Nasen-Ohrenheilkunde, Kopf- und Halschirurgie.

Universitätsklinikum Gießen und Marburg, Standort Marburg, Hals- Nasen- und Ohrenklinik.

出版信息

Laryngorhinootologie. 2017 Sep;96(9):607-614. doi: 10.1055/s-0043-110860. Epub 2017 Jul 6.

Abstract

Schwannomas are rare benign tumors originating from the perineural cells forming the myelin layer in the peripheral nervous system (PNS). While well established therapeutic concepts exist for intracranial schwannomas, there is a lack of consistent clinical standards for extracranial schwannomas. This retrospective study describes the clinical pathway of 20 patients with histologically proven extracranial schwannomas of the head and neck. The diagnostic and therapeutic strategies for schwannomas are discussed with special emphasis on localization and functional outcome. Extracranial schwannomas of the head and neck region mostly originated from the facial nerve (n = 4), vagal nerve (n = 4) or sympathetic chain (n = 3). Most common symptoms were swelling (n = 12) and pain (n = 3). Preoperative imaging included MRI (n = 13), ultrasound (n = 12) and CT (n = 3). Surgical intervention was performed in 18 cases (n = 14 complete extirpation, n = 3 partial extirpation, n = 1 unknown). Regarding completely extirpated schwannomas of motor nerves (n = 10) severing the nerve of origin was more often required in patients with a preexisting functional deficit (3 out of 4 = 75 %) than in patients without preexisting deficits (2 out of 6 = 33 %). Representing rare tumors of the head and neck region mostly originating from the facial nerve, sympathetic chain or caudal cranial nerves extracranial schwannomas require a systematic diagnostic and therapeutic approach. Postoperative functional deficits after complete extirpation must especially be anticipated in patients with a preexisting functional deficit.

摘要

施万细胞瘤是起源于外周神经系统(PNS)中形成髓鞘层的神经周细胞的罕见良性肿瘤。虽然颅内施万细胞瘤已有成熟的治疗理念,但颅外施万细胞瘤缺乏统一的临床标准。这项回顾性研究描述了20例经组织学证实的头颈部颅外施万细胞瘤患者的临床路径。讨论了施万细胞瘤的诊断和治疗策略,特别强调了定位和功能结局。头颈部颅外施万细胞瘤大多起源于面神经(n = 4)、迷走神经(n = 4)或交感神经链(n = 3)。最常见的症状是肿胀(n = 12)和疼痛(n = 3)。术前影像学检查包括MRI(n = 13)、超声(n = 12)和CT(n = 3)。18例患者接受了手术干预(n = 14例完全切除,n = 3例部分切除,n = 1例情况不明)。对于运动神经完全切除的施万细胞瘤(n = 10),术前已有功能缺陷的患者(4例中有3例 = 75%)比无功能缺陷的患者(6例中有2例 = 33%)更常需要切断起源神经。头颈部颅外施万细胞瘤是大多起源于面神经、交感神经链或尾侧颅神经的罕见肿瘤,需要系统的诊断和治疗方法。对于术前已有功能缺陷的患者,尤其要预计到完全切除术后的功能缺陷。

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