肠神经内分泌肿瘤引起的异位库欣综合征：一项系统综述。
完整摘要 · 中文译文
背景：异位库欣综合征是一种可能危及生命的疾病，最常由胸部或胰腺神经内分泌肿瘤引起。肠道神经内分泌肿瘤是一种极为罕见且特征不明确的病因，给诊断和治疗带来了重大挑战。我们旨在系统性地描述由肠道神经内分泌肿瘤引起的异位库欣综合征的临床表现、诊断路径、治疗策略和结局。 方法：对已发表的病例报告进行系统性综述，并补充一例未发表的典型病例。按照系统综述和荟萃分析优先报告条目（PRISMA）指南，检索了四个电子数据库，检索时间从建库至2026年2月。提取患者的个体数据，包括临床表现、肿瘤定位、治疗和结局，并进行描述性分析。 结果：共 identified 24例已发表病例。异位库欣综合征为87.5%患者的首发表现，且常呈现侵袭性临床表型（70.8%）。肿瘤定位往往具有挑战性。常规影像学检查在不到一半的病例中检测到原发病灶，而功能影像学检查，特别是镓-68生长抑素受体正电子发射断层扫描，显示出更高的检出率。87.5%的患者接受了手术切除，72.7%实现了生化缓解。然而，50%的患者需要针对高皮质醇血症的药物治疗，20.8%接受了双侧肾上腺切除术。复发率为14.3%。 结论： 肠道神经内分泌肿瘤是异位库欣综合征的一种罕见但在临床上具有相关性的病因。它们通常表现为严重的高皮质醇血症和肿瘤定位延迟。治疗既需要针对肿瘤的治疗，也需要快速的内分泌控制。尽管初始获得缓解，仍可能复发，这支持长期监测以及多模式诊断和治疗策略。
完整摘要 · 英文原文
BACKGROUND: Ectopic Cushing syndrome is a potentially life-threatening disorder most caused by thoracic or pancreatic neuroendocrine tumors. Intestinal neuroendocrine tumors are an exceptionally uncommon and poorly characterized source, creating important diagnostic and therapeutic challenges. We aimed to systematically characterize the clinical presentation, diagnostic pathways, treatment strategies, and outcomes of ectopic Cushing syndrome caused by intestinal neuroendocrine tumors. METHODS: Systematic review of published case reports, complemented by an illustrative unpublished case. Four electronic databases were searched from inception to February 2026 according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidance. Individual patient data on clinical presentation, tumor localization, treatment, and outcomes were extracted and analyzed descriptively. RESULTS: Twenty-four published cases were identified. Ectopic Cushing syndrome was the initial manifestation in 87.5% of patients and frequently presented with an aggressive clinical phenotype (70.8%). Tumor localization was often challenging. Conventional imaging detected the primary lesion in fewer than half of cases, whereas functional imaging, particularly gallium-68 somatostatin receptor positron emission tomography, showed higher detection rates. Surgical resection was performed in 87.5% of patients and biochemical remission was achieved in 72.7%. However, 50% required medical therapy for hypercortisolism and 20.8% underwent bilateral adrenalectomy. Recurrence occurred in 14.3%. CONCLUSIONS: Intestinal neuroendocrine tumors are a rare but clinically relevant cause of ectopic Cushing syndrome. They commonly present with severe hypercortisolism and delayed tumor localization. Management requires both tumor-directed treatment and rapid endocrine control. Recurrence may occur despite initial remission, supporting prolonged surveillance and a multimodal diagnostic and therapeutic approach.