报告颅内脊索瘤47例,特点为中年发病,是慢性进行性多发颅神经损害,颅骨X线片示鞍部及斜坡广泛性骨质破坏,1/3以上病例有肿瘤钙化斑。按肿瘤所在部位可分三型:鞍部型、中颅凹型及斜坡-后颅凹型,每型都有其神经系统临床特征及特有的造影表现,这种分型对手术入路的选择有一定帮助。本病预后差,术后2年内死亡者占56.7％,5年生存率为13.3％。 Report of 47 cases of intracranial chordoma, characterized by middle-aged onset, is a chronic progressive multiple cranial nerve damage, skull X-ray showed extensive saddle and slope destruction, more than 1/3 cases of tumor calcified plaque. According to the site of the tumor can be divided into three types: saddle type, in the cranial concave and slope - posterior fossa concave type, each type has its clinical features and unique neurological imaging, this classification of the surgical approach to the choice of some help . The poor prognosis of the disease, died within 2 years after surgery accounted for 56.7%, 5-year survival rate was 13.3%.