Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Endocr Pract. 2021 Sep;27(9):866-873. doi: 10.1016/j.eprac.2021.02.015. Epub 2021 Mar 9.
Ectopic adrenocorticotropic hormone syndrome (EAS) is a rare cause of Cushing's syndrome and diagnosis and management remain challenging. The aim of this study was to present the clinical spectrum of a group of EAS cases in a single center to explore better management strategies.
A retrospective study was conducted to identify 88 confirmed EAS cases at our hospital from 1984 to 2019. The clinical, biochemical, imaging, and pathological features were analyzed.
Of the 88 eligible patients with EAS, 38 (43.2%) cases of pulmonary neuroendocrine tumors (NETs) and a larger number of thymic/mediastinal NETs (29 cases, 33%) were identified. The clinical and biological features of EAS and Cushing's disease overlapped but were more severe in EAS. Inferior petrosal sinus sampling (97.4%) and computed tomography (85.4%) provided the highest positive diagnostic accuracy. Computed tomography is also a useful tool to identify tumors in chest cavity compared with nonchest lesions (91.2% vs 57.1%). Although a greater tumor size (4.54 cm vs 1.44 cm) and higher rate of insuppressible high-dose dexamethasone suppression test (83.3% vs 51.5%) were found in thymic/mediastinum NETs than in pulmonary NETs, the level of hormone production had no difference.
EAS had more common and severe clinical presentations than Cushing's disease, and multiple imaging approaches are required for reliable diagnosis. A higher proportion of thymic/mediastinal NETs was found in our study. For patients without a certain tumor source, long-term follow-up and further evaluations are needed.
异位促肾上腺皮质激素综合征（EAS）是库欣综合征的罕见病因，其诊断和治疗仍然具有挑战性。本研究旨在介绍单一中心一组 EAS 病例的临床特征，以探讨更好的治疗策略。
回顾性分析我院 1984 年至 2019 年间确诊的 88 例 EAS 患者的临床、生化、影像学和病理特征。
在 88 例符合条件的 EAS 患者中，有 38 例（43.2%）为肺部神经内分泌肿瘤（NETs），更多的是胸内/纵隔 NETs（29 例，33%）。EAS 的临床和生物学特征与库欣病重叠，但在 EAS 中更为严重。蝶鞍窦取样（97.4%）和计算机断层扫描（85.4%）提供了最高的阳性诊断准确率。与非胸部病变相比，计算机断层扫描也是识别胸部肿瘤的有用工具（91.2%比 57.1%）。尽管胸内/纵隔 NETs 的肿瘤直径较大（4.54cm 比 1.44cm），且不可抑制性大剂量地塞米松抑制试验阳性率较高（83.3%比 51.5%），但激素分泌水平并无差异。
EAS 的临床表现比库欣病更为常见和严重，需要多种影像学方法进行可靠诊断。本研究中发现更多的胸内/纵隔 NETs。对于没有明确肿瘤来源的患者，需要长期随访和进一步评估。
