Transsphenoidal microsurgery for Cushing's disease: Initial outcome and long-term results
Transsphenoidal microsurgery for Cushing's disease: Initial outcome and long-term results
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DOI：
10.1210/jc.2003-032180
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发表时间：
2004-12-01
影响因子：
5.8
通讯作者：
Wilson, CB
中科院分区：
文献类型：
--
作者：
Hammer, GD;Tyrrell, JB;Wilson, CB
未经治疗的库欣病和由此产生的糖皮质激素水平的慢性升高导致严重的代谢紊乱，包括糖尿病、肥胖症、高血压、肌肉萎缩和骨质疏松症。虽然经蝶窦切除术已成为治疗库欣病的标准方法，且初始成功率高，但与经蝶窦切除术治疗后复发或持续性库欣病患者相比，关于缓解期患者的长期发病率和死亡率的信息很少。因此，我们对289例库欣病患者进行了回顾性研究，这些患者在三级保健中心接受了由一名外科医生单独进行的经蝶窦ACTH分泌腺瘤显微手术（C。B。W.）。82%（n = 236）的患者实现了术后缓解，在I级（86%）和II级（83%）或0期（88%）、A期（94%）和B期（100%）肿瘤患者中观察到最佳初始缓解率。男性、肿瘤体积大、分期高预示初始预后差。对178例患者进行了长期随访，中位随访时间为11.1年（范围：0.6-24.1年）。150例初始缓解患者中有13例（9%）出现疾病复发，12例患者接受了额外治疗。在最后一次随访时，这些患者中只有2例有活动性疾病。然而，在随访超过6个月的28例初始持续性疾病患者中，10例患者在末次随访时仍有活动性疾病。尽管根据年龄和性别分布，与一般人群相比，初始缓解患者的总生存率与预期无显著差异，但与预期死亡率相比，初始持续性疾病患者的死亡率显著增加。因此，库欣病的成功治疗与正常的长期生存有关。这些结果表明，持续性库欣病患者需要早期积极干预，以防止这种过度死亡。
Untreated Cushing's disease and the resultant chronically elevated glucocorticoid levels lead to severe metabolic disturbances, including diabetes mellitus, obesity, hypertension, muscle wasting, and osteoporosis. Although transsphenoidal resection has become the standard of care for Cushing's disease with high initial success rates, little information is available on the long-term morbidity and mortality of patients in remission compared with patients with recurrent or persistent Cushing's disease after such treatment. We therefore conducted a retrospective study of 289 patients with Cushing's disease who underwent transsphenoidal microsurgery for an ACTH-secreting adenoma at a tertiary care center exclusively by one surgeon ( C. B. W.). Postoperative remission was achieved in 82% (n = 236) of patients, with best initial remission rates observed in patients with grade I (86%) and II (83%) or stage 0 (88%), A (94%), and B (100%) tumors. Male gender, larger tumor size, and higher stage predicted poorer initial outcome. Long-term follow-up was obtained on 178 patients, with a median follow-up time of 11.1 yr (range, 0.6-24.1 yr). Thirteen of 150 (9%) of patients in initial remission developed recurrent disease, and 12 patients underwent additional treatment. At last follow-up, only two of these patients had active disease. However, of the 28 patients with initial persistent disease who had follow-up greater than 6 months, 10 patients continued to have active disease at last follow-up. Although overall survival rates in patients with initial remission did not differ significantly from expected compared with the general population based on age and sex distribution, patients with initial persistent disease had a significant increase in mortality compared with the expected mortality. Thus, successful treatment of Cushing's disease is associated with normal long-term survival. These results suggest that patients with persistent Cushing's disease require early and aggressive intervention to attempt to prevent this excess mortality.