目的:探讨颅底脊索瘤的CT、MRI表现及治疗措施。方法:回顾性分析经病理证实的26例脊索瘤患者的临床资料。肿瘤位于鞍区及鼻咽部者9例,位于中颅窝者5例,位于后颅窝者8例,混合型4例。手术入路:额颞入路-翼点开颅9例,鼻内镜下经蝶入路5例,颞下、颞枕及扩大中颅窝入路8例,4例未手术行放射治疗。结果:肿瘤全切8例,大部及次全切14例,围手术期未见死亡病例。25例患者获得随访;3例于术后1年内复发,其中2例死亡,余者颅神经损伤及肢体症状均有改善。结论:脊索瘤无典型临床表现,现多可通过术前影像学检查得以诊断,少数位于特殊位置者需与垂体瘤,颅咽管瘤,三叉神经鞘瘤等鉴别;肿瘤全切较为困难,根据需要选择相应的手术入路可明显提高手术效果;放疗可作为术后辅助治疗。 Objective: To investigate the CT and MRI manifestations and treatment of skull base chordoma. Methods: The clinical data of 26 patients with pathologically confirmed chordoma were retrospectively analyzed. Tumor located in the saddle area and nasopharynx in 9 cases, located in the cranial fossa in 5 cases, located in the posterior fossa in 8 cases, 4 cases of mixed type. Surgical approach: frontotemporal approach - wing point craniotomy in 9 cases, endoscopic transsphenoidal approach in 5 cases, infratemporal and temporal occipital and expansion of the middle cranial fossa approach in 8 cases, 4 cases of non-surgical radiotherapy. Results: Tumor resection in 8 cases, most of the subtotal resection in 14 cases, no perioperative deaths. Twenty-five patients were followed up. Three patients relapsed within one year after surgery. Two of them died and the other had cranial nerve injury and limb symptom improvement. Conclusion: There is no typical clinical manifestations of chordoma. Most of them can be diagnosed by preoperative imaging examination. A few patients in special position need to be distinguished from pituitary tumor, craniopharyngioma and trigeminal schwannoma. Tumor resection is difficult, according to Need to choose the appropriate surgical approach can significantly improve the surgical effect; radiotherapy can be used as adjuvant therapy.