Background/Objectives: Ectopic Cushing’s syndrome (ECS) is a rare, life-threatening condition caused by uncontrolled ACTH secretion from tumors, most commonly small-cell lung cancer (SCLC). ECS is traditionally reported in 1–6% of SCLC cases; however, recent data suggest it may be much higher. This study compares the clinical presentation of SCLC-related ECS (ECS-SCLC) with other ECS etiologies and analyzes the diagnosis, treatment, and outcomes of ECS-SCLC. Methods: We retrospectively analyzed the records of 39 ECS patients diagnosed between 2000 and 2024 at a tertiary endocrinology center. Seven cases (18%) were due to SCLC. Diagnosis was based on clinical signs, biochemical testing, imaging, and histopathology. Results: ECS-SCLC patients (five men, two women; median age 61), compared to other ECS etiologies, had a shorter time to diagnosis (median 1 vs. 2 months;p= 0.03), worse general condition (ECOG 4 vs. 3;p= 0.01), greater muscle weakness (Lovett scale median 2[IQR 1–2] vs. 2[IQR 2–3];p= 0.04), more severe hypokalemia (2.12 vs. 2.7 mmol/L;p= 0.03), and required higher potassium supplementation (200 vs. 120 mEq/day;p= 0.001). All ECS-SCLC patients experienced weight loss (median 5 kg). Cortisol-lowering therapy (metyrapone or osilodrostat) was initiated in six patients (mean initiation time 3.7 days), leading to clinical improvement. Oncological treatment (chemotherapy or radiotherapy) was administered in five patients after stabilization. The median follow-up time was 3 months. Conclusions: Early recognition of ECS-SCLC and a multidisciplinary approach are critical. Severe hypokalemia and muscle weakness should prompt timely evaluation for hypercortisolism. Cortisol-lowering therapy may improve clinical status and facilitate oncological treatment.
背景/目的：异位库欣综合征（ECS）是一种罕见且危及生命的疾病，由肿瘤（最常见于小细胞肺癌）不受控分泌促肾上腺皮质激素引起。传统报道显示，ECS在小细胞肺癌患者中的发生率为1-6%，但近期数据提示其实际发生率可能更高。本研究旨在比较小细胞肺癌相关ECS与其他病因所致ECS的临床表现，并分析小细胞肺癌相关ECS的诊断、治疗及预后。方法：我们回顾性分析了2000年至2024年间在某三级内分泌中心确诊的39例ECS患者的病历资料，其中7例（18%）由小细胞肺癌引起。诊断依据包括临床症状、生化检测、影像学及组织病理学检查。结果：与其他病因的ECS患者相比，小细胞肺癌相关ECS患者（男性5例，女性2例，中位年龄61岁）具有以下特征：诊断时间更短（中位1个月 vs 2个月，p=0.03）、全身状况更差（ECOG评分4分 vs 3分，p=0.01）、肌无力更显著（Lovett评分中位数2[四分位距1-2] vs 2[四分位距2-3]，p=0.04）、低钾血症更严重（2.12 vs 2.7 mmol/L，p=0.03）、需补充更高剂量的钾（200 vs 120 mEq/天，p=0.001）。所有小细胞肺癌相关ECS患者均出现体重减轻（中位减轻5公斤）。6例患者启动了皮质醇降低治疗（美替拉酮或osilodrostat，平均起始时间3.7天），治疗后临床症状得到改善。5例患者在病情稳定后接受了肿瘤治疗（化疗或放疗）。中位随访时间为3个月。结论：早期识别小细胞肺癌相关ECS并采取多学科协作诊疗至关重要。严重低钾血症与肌无力应及时提示高皮质醇血症的评估。皮质醇降低治疗可改善患者临床状况，并为后续肿瘤治疗创造条件。
肿瘤(癌症)患者之家