Abstract:Objective: to analyze the clinical characteristics of malignant paraganglioma of the parapharyngeal space, and to explore its diagnosis and treatment .methods:The clinical data of 11 patients with pathologically definite malignant paraganglioma of the parapharyngeal space were retrospectively analyzed between January 2000 and December 2021 in Department of otolaryngology Head and Neck surgery, the PLA general hospital. There were 4 males and 7 females, aged from 21 to 57 years old. All patients underwent enhanced CT/MRI and DSA, the imaging features were tumors with rich blood supply in the parapharyngeal space and the tumor were closely related to the large vessels of the neck. Results: All patients were adopted cervical or combined craniovertebral approach for tumor resection and regional lymph node dissection, 5 underwent one operation, 4 underwent two operations, 1 underwent three operations, and 1 underwent four operations. There were no cerebrovascular complications in all patients. Local lymph node metastasis occurred in nine patients and distant metastasis in two patients. One patient underwent postoperative radiotherapy and one underwent postoperative chemotherapy. None of the patients died. Conclusion: malignant paraganglioma of the parapharyngeal space is rare and its treatment is mainly based on surgery. At the same time, a comprehensive treatment plan needs to be made according to the characteristics of the tumor and specific situation of the patient.