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目的:探讨小脑脑桥角(CPA)区血管母细胞瘤的临床特点、治疗及其预后。方法:回顾性分析2003年1月至2019年1月四川大学华西医院神经外科收治的31例CPA区血管母细胞瘤患者的临床资料。31例患者均行肿瘤切除术，10例患者术前行血管内栓塞治疗。术后每3个月对患者进行1次门诊随访，1年后每6个月门诊随访1次。随访内容包括患者临床症状的变化及复查头颅增强MRI检查明确是否有肿瘤复发。结果:31例患者中，男20例，女11例；中位病程为15.3个月(0.5~72.0个月)。临床表现以头痛(25例，80.6%)、听力丧失(19例，61.3%)及耳鸣(13例，41.9%)为主。10例(32.3%)被诊断为Von Hippel-Lindau(VHL)综合征。术前头颅增强MRI表现为实性肿瘤18例(58.1%)，囊实性肿瘤13例(41.9%)。10例术前行血管内栓塞治疗。手术资料完整的27例患者中，22例(81.5%)采用经乙状窦后入路，5例(18.5%)采用远外侧入路；1例肿瘤切除术中出血严重、肿瘤与脑神经粘连紧密，仅行次全切除术。术后复查头颅增强MRI显示，30例(96.8%)患者肿瘤全切除，1例(3.2%)肿瘤次全切除。术后新出现面瘫4例(12.9%)、后组脑神经麻痹4例(12.9%)、三叉神经麻痹和外展神经麻痹各2例(6.5%)；发生颅内出血、脑脊液漏以及假性脑膜膨出各1例(3.2%)。出院时，22例患者的术前症状改善，6例未改善，3例加重。31例患者的随访时间为(39.1±23.4)个月(3~144个月)。至末次随访，术前症状改善25例，未改善3例，加重3例；除2例术后新发面瘫患者的面神经功能未改善外，余术后新发脑神经功能障碍的患者神经功能均恢复正常；5例(16.1%)患者在随访中出现肿瘤原位复发，其中3例为VHL综合征患者。结论:CPA区血管母细胞瘤临床少见，男性患者略多，常以头痛、听力下降及耳鸣为首发症状。手术是其有效治疗方法；术前血管内栓塞有助于降低手术风险、减少术中出血。尽管其为良性肿瘤，术后仍可能复发，需长期随访。“,”Objective:To explore the clinical features, treatment and outcomes of hemangioblastomas in the cerebellopontine angle (CPA) region.Methods:A retrospective analysis was conducted on the clinical data of 31 patients with hemangioblastoma in the CPA region admitted to the Department of Neurosurgery, West China Hospital of Sichuan University from January 2003 to January 2019. All 31 patients underwent tumor resection, and 10 patients underwent preoperative endovascular embolization. Outpatient follow-up was performed every 3 months after surgery, and every 6 months after 1 year. Follow-up content included changes in clinical symptoms of patients and re-examination of enhanced brain MRI to determine whether there was tumor recurrence.Results:Among the 31 patients, 20 were males and 11 were females; the median disease duration was 15.3 months (0.5-72.0 months). The main clinical manifestations were headache (25 cases, 80.6%), hearing loss (19 cases, 61.3%) and tinnitus (13 cases, 41.9%). Ten patients (32.3%) were diagnosed with Von Hippel-Lindau (VHL) syndrome. Preoperative contrast-enhanced MRI showed solid tumors in 18 cases (58.1%) and cystic tumors in 13 cases (41.9%). Ten cases underwent preoperative endovascular embolization. Among the 27 patients with complete surgical data, 22 patients (81.5%) underwent surgery via retrosigmoid approach, and 5 patients (18.5%) underwent surgery via the far lateral approach. One patient had severe bleeding during tumor resection, the tumor was tightly adhered to the cranial nerves, and only subtotal resection was performed. Postoperative re-examination of enhanced brain MRI showed that 30 patients (96.8%) underwent total tumor resection, and 1 patient (3.2%) underwent subtotal tumor resection. There were 4 cases (12.9%) of new-onset facial paralysis, 4 cases (12.9%) of posterior cranial nerve palsy, 2 cases (6.5%) of trigeminal nerve damage, 2 cases (6.5%) of abducens nerve palsy, 1 case (3.2%) of intracranial hemorrhage, 1 case (3.2%) of cerebrospinal fluid leakage and 1 case (3.2%) of pseudomeningocele. At discharge, preoperative symptoms improved in 22 patients, remained unchanged in 6 patients, and worsened in 3 patients. The follow-up time of 31 patients was 39.1±23.4 months (3-144 months). By the last follow-up, preoperative symptoms improved in 25 cases, did not improve in 3 cases, and aggravated in 3 cases. Except for 2 patients with new-onset facial paralysis after surgery whose facial nerve function did not improve, the neurological functions of the rest of the patients with new-onset cranial nerve dysfunction after surgery recovered to normal. Five patients(16.1%) had tumor recurrence in situ during follow-up, including 3 patients with VHL syndrome.Conclusions:Hemangioblastomas in the CPA region are clinically rare, and there are slightly more male patients. Headache, hearing loss and tinnitus are often the first symptoms. Surgery is an effective treatment and preoperative endovascular embolization can help reduce surgical risks and intraoperative bleeding. Although it is a benign tumor, long-term follow-up is necessary after surgery.