颅底脊索瘤临床诊疗进展

脊索瘤是一种罕见的来源于脊索胚胎残余组织的肿瘤，其中颅底脊索瘤约占1/3。颅底脊索瘤首选手术治疗，手术切除程度与预后高度相关。因为大部分肿瘤位于颅底腹侧面的硬膜外，适用于内镜经鼻入路切除，对脑组织的损伤很小。放疗是颅底脊索瘤重要的辅助治疗手段，术后残留肿瘤放疗可以明显延长患者无进展生存期。脊索瘤化疗仍处于临床试验阶段，传统化疗药物对于脊索瘤的疗效较差，有待发展新的靶向治疗或免疫治疗药物。随着综合治疗技术的不断进步，颅底脊索瘤的治疗效果将逐步提高。

Progress in clinical diagnosis and treatment of skull base chordoma

Chordoma is a rare tumor derived from the residual tissue of the notochord, and the skull base chordoma accounts for about one third. Surgical treatment is the first choice for skull base chordoma, and the degree of surgical resection is highly correlated with prognosis. Because most of the tumors are epidural and locate on the ventral side of the skull base, transnasal endoscopic approach is suitable for tumor resection with little damage to brain tissue. Radiotherapy is an important adjuvant treatment for skull base chordoma. Postoperative radiotherapy applied to the patients with residual tumor can significantly prolong their progression free survival. Chordoma chemotherapy is still in clinical trials. Due to poor efficacy of traditional chemotherapy drugs for chordoma, new targeted therapic or immunotherapeutic drugs are needed to be developed. With the continuous advancement of comprehensive treatment technology, the therapeutic effect of skull base chordoma will be gradually improved.