Objective: To explore the diagnosis, surgical management and outcome of jugular foramen chondrosarcoma (CSA). Methods: Fifteen patients with jugular foramen CSA hospitalized in the Department of Otorhinolaryngology Head and Neck Surgery of Chinese PLA General Hospital from December 2002 to February 2020 were retrospectively collected,of whom 2 were male and 13 were female, aging from 22 to 61 years old. The clinical symptoms and signs, imaging features, differential diagnosis, surgical approaches, function of facial nerve and cranial nerves IX to XII, and surgical outcomes were analyzed. Results: Patients with jugular foramen CSA mainly presented with facial paralysis, hearing loss, hoarseness, cough, tinnitus and local mass. Computed tomography (CT) and magnetic resonance (MR) could provide important information for diagnosis. CT showed irregular destruction on bone margin of the jugular foramen. MR demonstrated iso or hypointense on T1WI, hyperintense on T2WI and heterogeneous contrast-enhancement. Surgical approaches were chosen upon the sizes and scopes of the tumors. Inferior temporal fossa A approach was adopted in 12 cases, inferior temporal fossa B approach in 2 cases and mastoid combined parotid approach in 1 case. Five patients with facial nerve involved received great auricular nerve graft. The House Brackmann (H-B) grading scale was used to evaluate the facial nerve function. Preoperative facial nerve function ranked grade Ⅴ in 4 cases and grade Ⅵ in 1 case. Postoperative facial nerve function improved to grade Ⅲ in 2 cases and grade Ⅵ in 3 cases. Five patients presented with cranial nerves Ⅸ and Ⅹ palsies. Hoarseness and cough of 2 cases improved after operation, while the other 3 cases did not. All the patients were diagnosed CSA by histopathology and immunohistochemistry, with immunohistochemical staining showing vimentin and S-100 positive, but cytokeratin negative in tumor cells. All patients survived during 28 to 234 months' follow-up. Two patients suffered from tumor recurrence 7 years after surgery and received revision surgery. No complications such as cerebrospinal fluid leakage and intracranial infection occurred after operation. Conclusions: Jugular foramen CSA lacks characteristic symptoms or signs. Imaging is helpful to differential diagnosis. Surgery is the primary treatment of jugular foramen CSA. Patients with facial paralysis should receive surgery in time as to restore the facial nerve. Long-term follow-up is necessary after surgery in case of recurrence.
目的： 总结分析颈静脉孔区软骨肉瘤的诊断要点、手术方法及治疗效果。 方法： 回顾分析2002年12月至2020年2月在解放军总医院耳鼻咽喉头颈外科接受手术治疗的15例颈静脉孔区软骨肉瘤患者的临床资料，其中男性2例、女性13例，年龄22~61岁。总结分析患者的临床表现、影像学特点、诊断要点、手术入路、面神经及后组颅神经功能、疗效及复发等。 结果： 颈静脉孔区软骨肉瘤患者主要症状和体征包括面神经麻痹、听力下降、声音嘶哑、呛咳、耳鸣、局部包块等。影像学检查是术前诊断和鉴别诊断的重要依据：CT表现为颈静脉孔区骨质边缘不规则破坏；颅脑MRI表现为颈静脉孔区T1WI等或低信号、T2WI高信号、增强后不均匀强化的病变。根据病变范围选择手术入路，15例患者中颞下窝A入路12例，颞下窝B入路2例，乳突及颈部联合入路1例。5例面神经严重受侵患者在手术切除肿瘤的同时行耳大神经移植桥接术重建面神经功能，术前面神经功能House-Brackmann（H-B）分级Ⅴ级4例、Ⅵ级1例，术后面神经功能恢复至Ⅲ级2例、Ⅳ级3例。术前Ⅸ、Ⅹ脑神经功能障碍者5例，术后有2例恢复功能，3例无改善。所有病例均经术后病理及免疫组化检查（波形蛋白及S-100蛋白染色阳性、细胞角蛋白染色阴性）确诊为软骨肉瘤。术后未发生脑脊液漏、颅内感染等并发症。随访28~234个月，15例患者均存活，其中2例复发，复发时间均为术后7年，给予再次手术切除。 结论： 颈静脉孔区软骨肉瘤的症状和体征缺乏特异性，其影像学特点有助于鉴别诊断，最终确诊依靠病理检查。外科手术是首选的治疗方法，伴有面神经麻痹患者应尽早手术切除肿瘤并积极修复面神经。软骨肉瘤术后有复发风险，患者应接受长期随访。.