颅底脊索瘤的分型、诊断与手术

Skull base chordoma

目的 探讨颅底脊索瘤的临床分型、诊断与手术。方法 1988年 9月至 2002年 12月收治 102例颅底脊索瘤病人,临床主要表现为颅神经功能损害、运动障碍、鼻咽部症状等。根据肿瘤起源和发展方向,分为鞍区型、颅中窝型、颅后窝型、鼻 (口 )咽型和混合型。99例病人 106次分别经枕下远外侧、乳突后、前方颅底、鼻蝶、额下、颞下、侧裂、口咽和联合入路手术。肿瘤未能全切除者术后辅以放疗或放射外科治疗。结果 99例手术者,肿瘤全切除 25例(25 3% ),次全或大部切除32例(32 3% ),部分切除 41例(41 4% ), 1例仅作活检。死亡 1例(1% )。2例发生脑脊液漏, 6例出现新的颅神经损害, 1例偏瘫。86例平均随访 5 6年, 21例恢复正常工作, 28例恢复部分工作, 17例生活自理, 9例不能自理, 11例死于肿瘤复发或其他原因。结论 根据临床表现和影像学资料,多数颅底脊索瘤术前可确诊,但有些病例需与侵袭性垂体腺瘤、鼻咽癌、软骨性肿瘤 (尤其软骨肉瘤 )鉴别。恰当的临床分型有助于手术入路的选择。治疗应首选手术,部分肿瘤可全切除,多数不能全切者术后辅以放疗,可达到缓解症状延长存活期的目的。

Objective To investigate the clinical classification, diagnosis and surgical treatment of skull base chordoma Methods One hundred and two patients with skull base chordoma were admitted from September 1988 to December 2002 The clinical manifestations included cranial nerve dysfunctions, motor disturbances and nasopharyngeal symptoms The chordoma could be divided into parasellar, middle fossa, posterior fossa, naso or oropharyngeal and mixed patterns based on the original site and growth direction of tumor Ninety nine patients underwent operations via suboccipital far lateral, retromastoid, anterior transbasal , nasosphenoidal, subfrontal, subtemporal, transsylvian fissure, oropharyngeal and combined approaches respectively Those patients whose tumor couldn't be removed totally recieved radiotherapy or radiosurgery after operation Results The tumor was totally removed in 25 cases (25 3%), subtotally in 32 (32 3%), partially in 41 (41 4%), and only biopsied in one After operation one patient died, two developed CSF rhinorrhea, six experienced cranial nerve dysfunction, and one occured hemiplegia Eighty six patients were followed up with average 5 6 years, 21 returned to normal works, 28 returned to part time works, 17 took care by oneself, 9 needed help, and 11 died from tumor recurrence or other causes Conclusions Most skull base chordomas can be diagnosed based on clinical and imaging features, although some should be differentiated from invasive pituitary adenoma, nasopharyngeal carcinoma and chondroid tumor Appropriate clinical classification may be useful in choice of surgical approach Surgical management should be the first choice for skull base chordoma Tumor can be totally removed in some patients Symptoms reduction and survival prolongation can be achived in those patients whose tumor couldn't be removed totally and radiotherapy was recieved