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脊索瘤是不常见的发生在中线骨骼的低度恶性肿瘤,来源于颅底—脊髓轴的中线神经胚胎脊索残余.约占骨和软组织恶性肿瘤不到1%,原发骨肿瘤3%,在脊柱肿瘤中占第3位,在骶骨原发肿瘤中占40%,在颅内肿瘤占不到1%.脊索瘤原发于骶骨占50%,颅底斜坡及蝶骨至枕骨范围内占35%,腰椎及脊柱占15%.脊索瘤生长缓慢,其恶性表现为向相邻的骨及组织浸润生长,治疗后有较高的复发率,以颅内所占据的位置影响神经系统功能.脊索瘤全身转移从8%～43%.大多数转移病灶并无症状,只是尸检时才被发现.脊索瘤最常见发病年龄为50～70岁,颅底脊索瘤发病则以40～50岁居多.文献记录发病年龄15～82岁.脊索瘤患者主要症状是疼痛,肿块及神经功能障碍或缺损.颅底脊索瘤因其压迫和侵犯周围的重要神经结构,患者会有明显头痛、复视、鼻堵、颅神经缺损等症状.颅底脊索瘤虽缓慢进展,但最终可因肿瘤导致死亡.O’Conoell报告马萨诸塞总医院收治的62例颅底脊索瘤,均有颅底骨质破坏.腰、骶部脊索瘤主要症状是局部疼痛、局部触及明显较大的胀块,以及肿瘤压迫神经产生的相应症状.脊索瘤手术治疗应为首选治疗,手术根治切除或次全切
Chordoma is an uncommon low-grade malignancy that occurs in the midline of the skeleton. The spinal cord of the midline neurons originating from the skull base-spinal axis is less than 1% of the bone and soft tissue malignancies. The primary bone tumour is 3%. 3rd among spine tumors, accounting for 40% of primary tumors of the sacrum, and less than 1% of intracranial tumors. 50% of sacral bone originates from the sacrum, and 35% of the skull slope and sphenoid sacrum. %, lumbar spine and spine account for 15%. Chordoma growth is slow, the malignant manifestations of infiltration and growth to adjacent bone and tissue, a higher rate of recurrence after treatment, to occupy the position of the brain affect the nervous system function. Tumor metastasis from 8% to 43%. Most of the metastatic lesions are asymptomatic, but were found only at autopsy. The most common onset age of chordoma is 50 to 70 years, and the incidence of chordoma of the skull base is 40 to 50 years old. The literature records the age of onset at 15 to 82 years. The main symptoms of chordomas are pain, masses, and neurological dysfunction or defects. The skull base chordoma has significant headaches, diplopia, and nose due to its compression and invading the surrounding vital nerve structures. Blockage, cranial nerve defects and other symptoms. Although the skull base chordoma slowly progresses, but eventually Can cause death due to cancer. O’Conoell reported that 62 cases of skull base chordomas treated at the Massachusetts General Hospital all had skull base destruction. The main symptoms of lumbar and sacral chordomas were local pain and localized swelling. , And the corresponding symptoms of tumor compression nerve production. Chordoma surgical treatment should be the preferred treatment, surgical radical resection or subtotal cut