Risk factors and long-term outcome in pituitary-dependent Cushing's disease
Risk factors and long-term outcome in pituitary-dependent Cushing's disease
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DOI：
10.1210/jc.81.7.2647
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发表时间：
1996-07-01
影响因子：
5.8
通讯作者：
Boscaro, M
中科院分区：
文献类型：
--
作者：
Sonino, N;Zielezny, M;Boscaro, M
尽管经蝶垂体显微手术已成为库欣病的首选治疗方法，但其他手术，例如双侧肾上腺切除术和垂体照射，目前也在其治疗中使用。事实上，没有一种治疗方法已被证明能够完全令人满意地治疗这种情况。垂体手术或放射治疗后的治愈率和复发率以及双侧肾上腺切除术后尼尔森综合征的发生率仍然是悬而未决的问题。在 1 个机构对 162 名垂体依赖性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（中位时间为 7 年）。根据治疗类型将患者分为亚组：经蝶垂体显微手术、双侧肾上腺切除术或垂体照射。采用生存分析来描述每个亚组的治疗结果。还评估了垂体手术成功的预测因素。成功垂体手术后仍处于缓解状态的患者的估计累积百分比（n = 79）为 2 年后 93.7%、5 年后 80.6%、7 年后 78.5% 和 10 年后 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、重性抑郁的存在、治疗前后尿皮质醇水平以及治疗后 ACTH 水平。 103 名患者中有 79 名 (76.7%) 的垂体手术取得了成功。手术失败与垂体腺瘤的缺乏以及严重抑郁症的临床严重程度和存在显着相关。在接受双侧肾上腺切除术治疗的患者 (n = 63) 中，估计 2 年后未出现尼尔森综合征的累计百分比为 87.1%，7 年后为 79.3%，10 年后为 71.2%。尼尔森综合征的发生与治疗前尿皮质醇水平以及既往垂体手术时是否存在垂体腺瘤显着相关。通过垂体照射治愈后（n = 23），估计2年后仍处于缓解状态的患者累积百分比为100％，5年后为81.8％，7年后为71.6％，10年后为65.1％。以前的垂体手术虽然不成功，但似乎是复发的保护因素。结果表明，通过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，并且随着时间的推移而增加。我们的研究结果对患者的临床表现赋予了新的重要性，并指出了垂体手术或放疗后复发以及双侧肾上腺切除后发生尼尔森综合征的高风险亚组。
Although transsphenoidal 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.