经蝶窦显微外科手术治疗541例库欣病

目的 探讨经蝶窦入路显微外科手术治疗库欣病的疗效。方法 总结北京协和医院神经外科1981年至2007年收治的541例库欣病的临床资料。结果CT和（或）MRI显示微腺瘤、大腺瘤、巨大腺瘤和未见异常的比例分别为66.2％( 358/541)、14.0％( 76/541)、0.6％( 3/541)和19.2％(104/541)。病理为垂体ACTH腺瘤、增生和既未见肿瘤又未见增生的比例分别为75.4％(408/541)、8.3％ (45/541)和16.3％ (88/541)。病理明确腺瘤、增生以及病理阴性者术后近期（半年内）治愈率分别是85.0％( 347/408)、24.4％ (11/45)和45.5％(40/88)。随访0.5 -25.0年（平均3.1年），复发率13.3％(53/398)，复发时间0.5 -16.5年（平均3.4年）。结论 经蝶窦选择性垂体腺瘤切除加瘤周垂体组织大部分切除是治疗库欣病安全有效的首选方法。对首次手术无效或复发性库欣病，可以再次经蝶窦手术或垂体放疗。

Transsphenoidal microsurgery in 541 patients with Cushing's disease

Objective To analyze the transsphenoidal microsurgical effect of Cushing's disease. Method The clinical data of 541 Cushing's disease cases were analyzed retrospectively in this study from 1981 to 2007. Results CT/MRI revealed microadenoma in 358 cases (66.2％ ), macroadenoma in 76 ( 14.0％ ) ,huge adenoma in 3 ( 0.6％ ) and negative results in 104 ( 19. 2％ ). Pathological examination confirmed pituitary ACTH adenoma in 408 ( 75.4％ ), pituitary ACTH cell hyperplasia in 45 ( 8. 3％ ), no evidence of adenoma or hyperplasia in 88 ( 16. 3％ ). In our group, the cure rate were 85.0％ ( 347/408 ),24. 4％ (11/45) and 45.5％ (40/88) respectively in Cushing's disease patients with pituitary ACTH adenoma, pituitary ACTH cell hyperplasia and negative pathological results. The follow up period ranged from half year to 25 years( average 3.1 years). Recurrence rate was 13. 3％. Average recurrent time was 3.4 years (0.5 - 16.5 years ). Conclusions Transsphenoidal selective adenomectomy plus resection of parsadenomatous pituitary tissue is the first choice in the treatment of Cushing's disease. Repeated transsphenoidal pituitary surgery or pituitary radiotherapy could be underwent for patients after failed surgery of recurrenct cases.