Risk factors and long-term outcome in pituitary-dependent Cushing's disease.
Risk factors and long-term outcome in pituitary-dependent Cushing's disease.
复制标题
DOI：
10.1210/jcem.81.7.8675592
复制
发表时间：
1996-07
期刊：
影响因子：
--
通讯作者：
N. Sonino;Maria Zielezny;G. A. Fava;Francesco Fallo;Marco Boscaro
中科院分区：
文献类型：
--
作者：
N. Sonino;Maria Zielezny;G. A. Fava;Francesco Fallo;Marco Boscaro
虽然经蝶窦垂体显微手术已成为治疗库兴氏病的首选方法，但其他手术，如双侧肾上腺切除和垂体放射治疗，目前仍在使用中。事实上，没有一种治疗方法被证明对这种情况完全令人满意。垂体手术或放疗后的治愈率和复发率以及双侧肾上腺切除术后Nelson综合征的发生率仍然是悬而未决的问题。在一家机构对162名垂体依赖型库欣病患者进行了研究，并在治疗后进行了至少2年的随访(中位数为7年)。根据治疗类型，患者被分成不同的亚组：经蝶垂体显微手术、双侧肾上腺切除术或垂体放射治疗。生存分析用于描述各亚组的治疗结果。此外，还评估了垂体部手术成功的预测因素。79例成功接受垂体手术的患者术后2年、5年、7年和10年的累积缓解率分别为93.7%、80.6%、78.5%和74.1%。在检查的8个危险因素中，以下因素具有统计学意义：年龄、临床严重程度、是否存在严重抑郁症、治疗前和治疗后的尿皮质醇水平以及治疗后的ACTH水平。103例患者中79例(76.7%)手术成功。手术失败与垂体腺瘤的缺失、临床严重程度和重度抑郁症的存在显著相关。在接受双侧肾上腺切除术的患者(n=63)中，估计2年后Nelson综合征的累积无症状率为87.1%，7年后为79.3%，10年后为71.2%。Nelson综合征的发生与术前尿皮质醇水平及既往有无垂体腺瘤密切相关。23例患者经垂体放疗治愈后，2年、5年、7年、10年的累积缓解率分别为100%、81.8%、71.6%、65.1%。以前的脑下垂体手术虽然没有成功，但似乎是复发的保护性因素。结果表明，通过脑下垂体手术或放射治疗治愈后复发是一个相当大的临床问题，而且会随着时间的推移而增加。我们的发现对患者的临床表现具有新的重要性，并指出了垂体手术或放疗后复发以及双侧肾上腺切除术后发展为纳尔逊综合征的高危亚群。
Although transphenoidal pituitary microsurgery has become the treatment of choice in Cushing's disease, other procedures, such as bilateral adrenalectomy and pituitary irradiation, are currently in use in its management. Indeed, no treatment has proven to be fully satisfactory for this condition. The rates of cure and recurrence after pituitary surgery or irradiation and the incidence of Nelson's syndrome after bilateral adrenalectomy are still open issues. A population of 162 patients with pituitary-dependent Cushing's disease was studied at 1 institution and had a follow-up of at least 2 yr after treatment (median, 7 yr). Patients were divided in subgroups according to the type of treatment: transsphenoidal pituitary microsurgery, bilateral adrenalectomy, or pituitary irradiation. Survival analysis was employed to characterize the outcome of treatment in each subgroup. Predictive factors for success of pituitary surgery were also evaluated. The estimated cumulative percentage of patients remaining in remission after successful pituitary surgery (n = 79) was 93.7% after 2 yr, 80.6% after 5 yr, 78.5% after 7 yr, and 74.1% after 10 yr. Of 8 risk factors examined, the following attained statistical significance: age, clinical severity, presence of major depression, pre- and posttreatment urinary cortisol levels, and posttreatment ACTH level. Pituitary surgery was successful in 79 of 103 patients (76.7%). Surgical failure was significantly associated with lack of pituitary adenoma and the clinical severity and presence of major depression. Of patients treated by bilateral adrenalectomy (n = 63), the estimated cumulative percentage remaining free of Nelson's syndrome was 87.1% after 2 yr, 79.3% after 7 yr, and 71.2% after 10 yr. The occurrence of Nelson's syndrome was significantly related to the pretreatment urinary cortisol level and the presence of pituitary adenoma at previous pituitary surgery. After cure by pituitary irradiation (n = 23), the estimated cumulative percentage of patients remaining in remission was 100% after 2 yr, 81.8% after 5 yr, 71.6% after 7 yr, and 65.1% after 10 yr. Previous pituitary surgery, although unsuccessful, appeared to be a protective factor for relapse. The results indicate that relapse after cure by either pituitary surgery or irradiation is a considerable clinical problem that increases over time. Our findings ascribe new importance to the clinical presentation of patients and indicate subgroups that are at high risk for relapse after pituitary surgery or irradiation and for developing Nelson's syndrome after bilateral adrenalectomy.