OBJECTIVE When transsphenoidal surgery (TSS) doesn't cure Cushing's disease (CD), four treatments are available: drug treatment (DT); second TSS (2nd TSS); bilateral adrenalectomy (BA); pituitary radiotherapy (PR). DT is attractive but supposes long-term continuation, which we aimed to evaluate. DESIGN AND METHODS Retrospective study, in a center prioritizing 2nd TSS, of 36 patients, including 19 with TSS failure and 17 with recurrence, out of 119 patients with CD treated by a 1st TSS, average follow-up 6.1 years (IC95 5.27-6.91). Control was defined as normalisation of urinary free cortisol (UFC), final treatment (FT) as the treatment allowing control at last follow-up. We also analysed discontinuation rates of DT in published CD prospective clinical trials. RESULTS Control was achieved in 33/36 patients (92%). DT was initiated in 29/36 patients (81%), allowing at least one normal UFC in 23/29 patients (79%), but was discontinued before last follow-up in 18/29 patients (62%). DT was FT in 11/29 patients (38%), all treated with cortisol synthesis inhibitors. 2nd TSS was FT in 8/16 (50%); BA in 14/14 (100%); PR in in 0/5. In published trials discontinuation of DT was 11-51% at 1 year and 32-74% before 5 years. CONCLUSION DT allowed at least one normal UFC in 23/29 patients (79%) but obtained long-term control in only 11/29 (38%), as discontinuation rate was high, although similar to published data. Interestingly, a successful 2nd TSS was the cause for discontinuing efficient and well tolerated DT in 5 patients. Further studies will show whether different strategies with cortisol synthesis inhibitors may allow for a lower discontinuation rate in patients not candidates for a 2nd TSS, so that BA may be avoided in these patients.

中文翻译：

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垂体手术未治愈的库欣病应停止药物治疗。

目的 当经蝶手术 (TSS) 不能治愈库欣病 (CD) 时，有四种治疗方法：药物治疗 (DT)；第二个 TSS（第二个 TSS）；双侧肾上腺切除术（BA）；垂体放射治疗（PR）。DT 很有吸引力，但需要长期持续，我们的目的是评估这一点。设计和方法 回顾性研究，在优先考虑第二次 TSS 的中心，对 36 名患者进行了回顾性研究，其中 119 名 CD 患者接受了第一次 TSS 治疗，其中 19 名 TSS 失败，17 名复发，平均随访 6.1 年（IC95 5.27-6.91） ）。控制被定义为尿游离皮质醇（UFC）正常化，最终治疗（FT）为在最后随访时允许控制的治疗。我们还分析了已发表的 CD 前瞻性临床试验中 DT 的终止率。结果 33/36 名患者 (92%) 获得控制。29/36 名患者 (81%) 开始 DT，使 23/29 名患者 (79%) 至少有 1 次正常 UFC，但 18/29 名患者 (62%) 在最后一次随访前停止。11/29 名患者 (38%) 接受 DT 治疗，所有患者均接受皮质醇合成抑制剂治疗。第二个 TSS 是 8/16 的 FT（50%）；14/14 学士（100%）；PR 在 0/5 内。在已发表的试验中，1 年内停用 DT 的比例为 11-51%，5 年前停用 DT 的比例为 32-74%。结论 DT 允许 23/29 名患者 (79%) 至少出现一次正常 UFC，但只有 11/29 (38%) 患者获得长期控制，因为停药率很高，尽管与已发表的数据相似。有趣的是，第二次 TSS 的成功是 5 名患者停止有效且耐受性良好的 DT 的原因。进一步的研究将表明皮质醇合成抑制剂的不同策略是否可以降低不适合进行第二次 TSS 的患者的停药率，从而可以避免这些患者的 BA。