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丛状神经鞘瘤和丛状神经纤维瘤的治疗挑战。

Challenging Management of Plexiform Schwannoma and Plexiform Neurofibroma.

机构信息

Departments of Otorhinolaryngology - Head and Neck Surgery.

Radiology.

出版信息

J Craniofac Surg. 2022 May 1;33(3):803-808. doi: 10.1097/SCS.0000000000008381. Epub 2021 Dec 2.

Abstract

Plexiform variants of neurofibromas and schwannomas are rare and typically arise in superficial soft tissues in the head and neck region. The treatment of these tumors is challenging and no generally accepted guidelines exist for their optimal management. The purpose of this study was to review the management and longterm prognosis of head and neck plexiform neurofibromas and schwannomas at 2 tertiary care academic hospitals in Finland over a 31-year period. The pathology files were searched for plexiform neurofibromas and schwannomas between the years 1990 and 2020. The case notes were reviewed for full management details. Two plexiform schwannomas and 6 plexiform neurofibromas were identified. Five of the 6 plexiform neurofibromas were managed operatively. All patients with a surgically managed plexiform neurofibroma underwent multiple operations. Sclerotherapy abolished 1 patient's cutaneous plexiform neurofibromas. The management of plexiform neurofibromas and plexiform schwannomas remains challenging. Sclerotherapy may offer a promising management option for cutaneous plexiform neurofibromas.

摘要

神经纤维瘤和神经鞘瘤的丛状变异较为罕见,通常发生于头颈部的浅表软组织中。这些肿瘤的治疗具有挑战性,目前尚无针对其最佳治疗的公认指南。本研究的目的是在芬兰的 2 家三级学术医院回顾过去 31 年间头颈部丛状神经纤维瘤和神经鞘瘤的治疗方法和长期预后。在 1990 年至 2020 年期间,通过病理档案检索丛状神经纤维瘤和神经鞘瘤。查阅病例记录以获取完整的治疗细节。共发现 2 例丛状 schwannoma 和 6 例丛状 neurofibroma。6 例丛状神经纤维瘤中有 5 例接受了手术治疗。所有接受手术治疗的丛状神经纤维瘤患者均接受了多次手术。硬化疗法使 1 例患者的皮肤丛状神经纤维瘤消退。丛状神经纤维瘤和丛状 schwannoma 的治疗仍然具有挑战性。硬化疗法可能为皮肤丛状神经纤维瘤提供一种有前景的治疗选择。

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