Giant intra/extracranial facial nerve schwannoma: A case report and review of the literature

IF 0.4 Q4 CLINICAL NEUROLOGY
Bo Tan, Xiaohong Yin
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引用次数: 0

Abstract

Background

Facial nerve schwannomas (FNSs) are rare, benign neoplasms arising from Schwann cells. While most FNSs are confined to the temporal bone or internal auditory canal, giant schwannomas extending across both intracranial and extracranial segments of the facial nerve are exceptionally rare and pose significant diagnostic and surgical challenges.

Case presentation

We report a 51-year-old woman with a 10-year history of progressive right-sided facial paralysis (House-Brackmann grade VI) and profound hearing loss. Imaging revealed a large, heterogeneous mass involving the right middle cranial fossa, external auditory canal, and mastoid, with destruction of adjacent bony structures. Electrophysiological studies confirmed severe facial nerve dysfunction. A combined subtemporal and mastoid approach enabled total tumor resection. Histopathology confirmed a WHO grade I schwannoma with strong S100, SOX10, and H3K27me3 positivity.

Conclusion

This case highlights the importance of integrating advanced neuroimaging, electrophysiological studies, and meticulous surgical planning in the management of extensive FNSs. Early diagnosis and timely intervention contribute to favorable outcomes, although functional recovery may require prolonged rehabilitation.
颅内/外巨大面神经分裂瘤:病例报告和文献综述
背景面神经分裂瘤(FNS)是由许旺细胞引起的罕见良性肿瘤。虽然大多数 FNS 都局限于颞骨或内耳道,但横跨颅内和颅外面神经节段的巨大分裂瘤却异常罕见,给诊断和手术带来了巨大挑战。影像学检查发现一个巨大的异质性肿块,累及右中颅窝、外耳道和乳突,并伴有邻近骨性结构的破坏。电生理检查证实患者有严重的面神经功能障碍。颞下和乳突联合入路使肿瘤得以完全切除。组织病理学证实这是一个 WHO I 级的裂隙瘤,S100、SOX10 和 H3K27me3 呈强阳性。虽然功能恢复可能需要长时间的康复治疗,但早期诊断和及时干预有助于取得良好的疗效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
1.00
自引率
0.00%
发文量
236
审稿时长
15 weeks
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