Neuroendocrine neoplasms (NENs) causing ectopic Cushing's syndrome (ECS) are rare and challenging to treat. In this retrospective cohort study, we aimed to evaluate different approaches for bilateral adrenalectomy (BA) as a treatment option in ECS. Fifty-three patients with ECS caused by a NEN (35 females/18 men; mean ± SD age: 53 ± 15 years) were identified from medical records. Epidemiological and clinical parameters, survival, indications for surgery and timing, as well as duration of surgery, complications and surgical techniques, were collected and further analysed. The primary tumour location was thorax (n = 30), pancreas (n = 14) or unknown (n = 9). BA was performed in 37 patients. Median time from diagnosis of ECS to BA was 2 months (range 1–10 months). Thirty-two patients received different steroidogenesis inhibitors before BA to control hypercortisolaemia. ECS resolved completely after surgery in 33 patients and severe peri- or postoperative complications were detected in 12 patients. There were fewer severe complications in the endoscopic group compared to open surgery (p = .030). Posterior retroperitoneoscopic BA performed simultaneously by a two surgeon approach had the shortest operating time (p = .001). Despite the frequent use of adrenolytic treatment, BA was necessary in a majority of patients to gain control over ECS. Complication rate was high, probably as a result of the combination of metastatic disease and metabolic disorders caused by high cortisol levels. The two surgeon approach BA may be considered as the method of choice in ECS compared to other BA approaches as a result of fewer complications and a shorter operating time.

中文翻译：

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异位库欣综合征的肾上腺切除术：来自三级医疗中心的回顾性队列研究

引起异位库欣综合征 (ECS) 的神经内分泌肿瘤 (NEN) 罕见且难以治疗。在这项回顾性队列研究中，我们旨在评估双侧肾上腺切除术 (BA) 作为 ECS 治疗选择的不同方法。从医疗记录中确定了 53 名由 NEN 引起的 ECS 患者（35 名女性/18 名男性；平均 ± SD 年龄：53 ± 15 岁）。收集并进一步分析流行病学和临床参数、生存率、手术指征和时机，以及手术持续时间、并发症和手术技术。原发肿瘤位置为胸部（n  = 30）、胰腺（n  = 14）或未知（n = 9)。对 37 名患者进行了 BA。从诊断 ECS 到 BA 的中位时间为 2 个月（范围 1-10 个月）。32 名患者在 BA 前接受了不同的类固醇生成抑制剂以控制高皮质醇血症。33 名患者术后 ECS 完全消退，12 名患者出现严重的围手术期或术后并发症。与开放手术相比，内窥镜组的严重并发症更少（p  = .030）。两名外科医生同时进行的后腹腔镜 BA 手术时间最短（p = .001)。尽管经常使用肾上腺素溶解治疗，但大多数患者需要 BA 来控制 ECS。并发症发生率很高，可能是由于高皮质醇水平引起的转移性疾病和代谢紊乱的结合。与其他 BA 方法相比，两位外科医生方法 BA 可被视为 ECS 的首选方法，因为其并发症更少且手术时间更短。