Liu D, Wang J Z, Sun J B, Li Z, Zhang T, Sai N, Zhu Y H, Shen W D, Huang D L, Dai P, Yang S M, Han D Y, Han W J
College of Otorhinolaryngology Head and Neck Surgery, Chinese PLA General Hospital, National Clinical Research Center for Otorhinolaryngology Disease, Key Lab of Hearing Science, Ministry of Education, Beijing Key Lab of Hearing Impairment for Prevention and Treatment, Beijing 100853, China.
Department of Pathology, Chinese PLA General Hospital, Beijing 100853, China.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2023 Jun 7;58(6):544-551. doi: 10.3760/cma.j.cn115330-20220607-00334.
To explore the diagnosis, surgical management and outcome of jugular foramen chondrosarcoma (CSA). 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. 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. 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.
探讨颈静脉孔软骨肉瘤（CSA）的诊断、手术治疗及预后。回顾性收集2002年12月至2020年2月在中国人民解放军总医院耳鼻咽喉头颈外科住院的15例颈静脉孔CSA患者，其中男性2例，女性13例，年龄22至61岁。分析其临床症状和体征、影像学特征、鉴别诊断、手术方式、面神经及Ⅸ至Ⅻ对脑神经功能以及手术预后。颈静脉孔CSA患者主要表现为面瘫、听力下降、声音嘶哑、咳嗽、耳鸣及局部肿块。计算机断层扫描（CT）和磁共振成像（MR）可为诊断提供重要信息。CT显示颈静脉孔骨质边缘不规则破坏。MR表现为T1WI等信号或低信号、T2WI高信号及不均匀强化。根据肿瘤大小和范围选择手术方式。采用颞下窝A入路12例，颞下窝B入路2例，乳突联合腮腺入路1例。5例面神经受累患者接受了耳大神经移植。采用House Brackmann（H-B）分级量表评估面神经功能。术前面神经功能4例为Ⅴ级，1例为Ⅵ级。术后面神经功能2例改善至Ⅲ级，3例为Ⅵ级。5例患者出现Ⅸ和Ⅹ对脑神经麻痹。2例患者术后声音嘶哑和咳嗽症状改善，另3例未改善。所有患者均经组织病理学和免疫组化确诊为CSA，免疫组化染色显示肿瘤细胞波形蛋白和S-100阳性，但细胞角蛋白阴性。所有患者在28至234个月的随访期内存活。2例患者术后7年肿瘤复发并接受了翻修手术。术后未发生脑脊液漏、颅内感染等并发症。颈静脉孔CSA缺乏特征性症状和体征。影像学有助于鉴别诊断。手术是颈静脉孔CSA的主要治疗方法。面瘫患者应及时手术以恢复面神经功能。术后需要长期随访以防复发。
