咽旁间隙恶性副神经节细胞瘤的临床分析

目的 总结咽旁间隙恶性副神经节细胞瘤的临床特点，探讨其诊断及治疗方法。方法 回顾性分析解放军总医院耳鼻咽喉头颈外科自2010年1月—2021年12月收治并经病理确诊的咽旁间隙恶性副神经节细胞瘤8例的临床资料，其中男3例，女5例，年龄21~57岁。患者的临床症状无特异性，所有患者术前均行增强CT/MRI及数字减影血管造影(DSA)等影像学检查，其特点是咽旁间隙富血供病变，与颈部大血管关系密切。结果 所有患者均经颈外入路或颅颈联合入路肿瘤切除术及区域性淋巴结清扫术，其中5例患者行1次手术，2例患者行2次手术，1例患者行3次手术。所有患者均无脑血管损伤并发症。7例患者发生局部淋巴结转移，1例患者发生远处转移。1例患者术后行放射治疗，1例行术后化疗。所有患者均无死亡。结论 咽旁间隙恶性副神经节细胞瘤发病少，其治疗是以手术为主，同时需结合肿瘤特点及患者具体情况制定综合治疗方案。

Clinical analysis of malignant paraganglioma of the parapharyngeal space

Objective To analyze the clinical characteristics of malignant paraganglioma of the parapharyngeal space, and to explore its diagnosis and treatment.Methods Clinical data of 8 patients with pathologically confirmed malignant paraganglioma of the parapharyngeal space hospitalized in Department of Otolaryngology Head and Neck Surgery, the Chinese PLA General Hospital between Jan 2010 and Dec 2021 were retrospectively analyzed. There were 3 males and 5 females, aged from 21 to 57 years old. Without specific clinical symptoms, preoperative enhanced computerized tomography/magnetic resonance imaging and digital angiography in all the patients revealed imaging features of parapharyngeal space hypervascularized tumors with close relations to the large vessels of the neck.Results Cervical or combined craniovertebral approaches were adopted in all patients for tumor resection and regional lymph node dissection. Of them, 5 underwent one operation, 2 had two operations and 1 received three operations. There were no cerebrovascular complications in all patients. Local lymph node metastasis occurred in 7 patients and distant metastasis in one. One patient underwent postoperative radiotherapy and one underwent postoperative chemotherapy. None of the patients died.Conclusions Malignant paraganglioma of the parapharyngeal space is rare and its main treatment is surgery. At the same time, a comprehensive treatment plan needs to be developed according to the characteristics of the tumor and specific situation of the patient.