头颈部迷走神经副神经节瘤的诊断与治疗

Management of Vagal Paraganglioma in Head and Neck Region

目的探讨头颈部迷走神经副神经节瘤的临床特点、诊断与治疗方法。方法回顾分析我院于2003年1月至2013年12月诊治的8例迷走神经副神经节瘤患者临床资料,结合文献复习,对该病的临床特点、诊断与治疗经验进行探讨。结果 8例患者主要以声嘶和颈部包块为特点,其中7单发,1为多发副神经节瘤,7例为良性副神经节瘤,1例伴有淋巴结转移。散发患者7例,1例为家族性发病。6例肿瘤位于颈动脉分叉和颈静脉孔之间的上颈部或局限于颈静脉孔区,2例为咽旁的巨大肿瘤且其中1例伴有颅内侵犯,1例多发副神经节瘤患者同时伴有颈动脉体瘤。5例患者进行了琥珀酸脱氢酶相关基因筛查,3例患者携带有突变。结论迷走神经副神经节瘤的临床特点与颈动脉体瘤和颈静脉球瘤有相似之处,需加以鉴别。CTA和增强MRI对定性诊断有价值,增强MRI和颞骨CT则能提供较多的手术所需信息。根据肿瘤位置的不同,选择颈侧入路或辅以Fisch A型颞下窝入路切除肿瘤。迷走神经副神经节瘤有一定恶性倾向,需注意全身筛查和随访以及家系检查。

Objective To discuss the diagnosis and treatment of vagal paraganglioma （VP） in the head and neck region. Methods Eight patients diagnosed with head and neck vagal paraganglioma in our department between 2003 and 2014 were in-cluded in this retrospective study. We carried out analysis of clinical characteristics and genetic screening results in 5 of the 8 patients. The diagnosis and treatment of head and neck vagal paraganglioma were discussed. Results The most common clini-cal symptoms of vagal paraganglioma in these patients were hoarseness caused by vocal cord paralysis and a mass behind the mandibular angle. Seven patients had benign paragangliomas, while the other one had lymph node metastasis. Six of the 8 pa-tients had paragangliomas around the jugular foramen area. In the other 2 cases, the large size tumors were located in the para-pharyngeal space. One patient had both vagal and carotid paragangliomas and was also a familial case. Three patients were found to carry mutations in the coding gene for succinic dehydrogenase subunit D. Conclusions The relationship among the tu-mor, internal carotid, jugular foramen and tympanum on CT and MR images is the key to differential diagnosis of vagal para-ganlioma, glomus jugular tumour and carotid body tumor. Lateral cervical approach or infratemporal fossa approach can be ap-propriate depending on the location of the tumor’s upper pole. Genetic consultation should be recommended to all patients for better understanding of the genotype and familial screening.