Favre J, Deruaz J P, Uske A, de Tribolet N
Neurosurgical Service, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
J Clin Neurosci. 1994 Jan;1(1):7-18. doi: 10.1016/0967-5868(94)90004-3.
Chordomas are uncommon tumours occuring from remnants of the notochord. They are mainly localized in the sacrococcygium, the spine and the skull base. We focused our study on skull base chordomas and reviewed 531 published cases and 6 cases diagnosed in our institution. The six cases diagnosed at our hospital are described with a special mention of a unique case presenting with meningitis. Clinical features, radiology, histopathology and survival with different treatments are reviewed. Skull base chordomas can occur at any age and are slightly more frequent in males. Patients complain most often of diplopia and headaches. Abducens nerve palsy is the commonest neurological finding. Radiological examination should include both CT-scan which shows extensive osteolysis, calcifications and contrast enhancement and MRI for topographical definition. Metastases rarely occur. Treatment remains controversial but combined surgery and radiotherapy is the best treatment with a 5 year survival rate of 75%.
脊索瘤是一种由脊索残余组织发生的罕见肿瘤。它们主要位于骶尾部、脊柱和颅底。我们的研究聚焦于颅底脊索瘤，并回顾了531例已发表病例以及我们机构诊断的6例病例。对我院诊断的6例病例进行了描述，并特别提及了1例表现为脑膜炎的独特病例。对临床特征、放射学、组织病理学以及不同治疗方法下的生存率进行了回顾。颅底脊索瘤可发生于任何年龄，男性略为多见。患者最常主诉复视和头痛。外展神经麻痹是最常见的神经学表现。放射学检查应包括CT扫描（显示广泛骨质溶解、钙化及强化）和MRI（用于明确解剖位置）。转移很少发生。治疗仍存在争议，但手术联合放疗是最佳治疗方法，5年生存率为75%。
