病例报告：咽旁间隙巨大成人型横纹肌瘤：诊断与手术陷阱。

文献信息

中文摘要

背景： 成人型心外横纹肌瘤是一种罕见的良性骨骼肌肿瘤，最常发生于头颈部，但在咽旁间隙极为罕见，可能模仿唾液腺或神经源性肿瘤。病例介绍：一名67岁男性，有6个月进行性吞咽困难、咽异物感和轻度声音嘶哑病史。计算机断层扫描和磁共振成像显示右侧咽旁间隙约9厘米、边界清楚、内部特征相对均匀、轻至中度强化的肿块。由于直接声门暴露不充分，先行气管切开辅助气道管理，然后通过右侧颈侧入路完整切除。组织病理学显示大的多边形细胞，胞质丰富、嗜酸性颗粒状。肿瘤desmin和平滑肌肌动蛋白阳性，局灶性MyoD1和myogenin表达，Ki-67标记指数低，S-100染色阴性，支持成人型横纹肌瘤。恢复顺利，6个月和12个月磁共振成像显示无复发。结论：在老年患者边界清楚的咽旁间隙肿块的鉴别诊断中，应考虑成人横纹肌瘤。综合影像学和病理学评估、仔细的气道规划、完整的保守性切除以及长期监测是管理的核心。

英文摘要

BACKGROUND: Adult-type extracardiac rhabdomyoma is a rare benign skeletal-muscle neoplasm that most often arises in the head and neck but is exceptional in the parapharyngeal space, where it may mimic salivary or neurogenic tumours. Case presentation: A 67-year-old man presented with a 6-month history of progressive dysphagia, globus sensation, and mild hoarseness. Computed tomography and magnetic resonance imaging demonstrated an approximately 9-cm, well-circumscribed right parapharyngeal mass with relatively homogeneous internal characteristics and mild-to-moderate enhancement. Because direct glottic exposure was inadequate, tracheotomy-assisted airway management preceded complete excision through a right lateral transcervical approach. Histopathology showed large polygonal cells with abundant eosinophilic granular cytoplasm. The tumour was positive for desmin and smooth-muscle actin, with focal MyoD1 and myogenin expression, a low Ki-67 labelling index, and negative S-100 staining, supporting adult-type rhabdomyoma. Recovery was uneventful, and magnetic resonance imaging at 6 and 12 months showed no recurrence.
CONCLUSION: Adult rhabdomyoma should be considered in the differential diagnosis of a well-circumscribed parapharyngeal mass in an older adult. Integrated radiological and pathological assessment, careful airway planning, complete conservative excision, and long-term surveillance are central to management.