Second Department of Internal Medicine, Hamamatsu University School of Medicine, Shizuoka 431-3192, Japan.
International Center, Hamamatsu University School of Medicine, Shizuoka 431-3192, Japan.
Endocr J. 2024 Oct 1;71(10):995-1002. doi: 10.1507/endocrj.EJ24-0128. Epub 2024 Jun 25.
Cushing's syndrome, a clinical condition characterized by hypercortisolemia, exhibits distinct clinical signs and is associated with cyclic cortisol secretion in some patients. The clinical presentation of cyclic Cushing's syndrome can be ambiguous and its diagnosis is often challenging. We experienced a 72-year-old woman with cyclic ACTH-dependent Cushing's syndrome caused by a pulmonary carcinoid tumor. Diagnosis was challenging because of the extended trough periods, and the responsible lesion was initially unidentified. A subsequent follow-up computed tomography revealed a pulmonary lesion, and ectopic ACTH secretion from this lesion was confirmed by pulmonary artery sampling. Despite the short peak secretion period of ACTH (approximately one week), immunostaining of the surgically removed tumor confirmed ACTH positivity. Interestingly, stored plasma chromogranin A levels were elevated during both peak and trough periods. The experience in evaluating this patient prompted us to investigate the potential use of plasma chromogranin A as a diagnostic marker of ACTH-dependent Cushing's syndrome. A retrospective study was conducted to determine the efficacy of plasma chromogranin A in three patients with ectopic ACTH syndrome (EAS), including the present case, and six patients with Cushing's disease (CD) who visited our hospital between 2018 and 2021. Notably, plasma chromogranin A levels were higher in patients with EAS than in those with CD. Additionally, a chromogranin A level in the present case during the trough phase was lower than that in the peak phase, and was similar to those in CD patients. The measurement of plasma chromogranin A levels could aid in differentiating EAS from CD.
库欣综合征是一种以皮质醇增多为特征的临床病症，表现出独特的临床特征，且部分患者的皮质醇呈周期性分泌。周期性库欣综合征的临床表现可能不典型，其诊断往往具有挑战性。我们曾遇到一例 72 岁女性，因肺类癌肿瘤引起的 ACTH 依赖性周期性库欣综合征。由于低谷期时间延长，且病变最初无法识别，导致诊断具有挑战性。随后的 CT 随访显示肺部有一个病灶，通过肺动脉取样证实该病灶存在异位 ACTH 分泌。尽管 ACTH 的分泌高峰持续时间较短（约一周），但手术切除的肿瘤免疫染色证实了 ACTH 阳性。有趣的是，在高峰和低谷期间，储存的血浆嗜铬粒蛋白 A 水平均升高。在评估该患者的过程中，我们探讨了使用血浆嗜铬粒蛋白 A 作为 ACTH 依赖性库欣综合征诊断标志物的可能性。我们进行了一项回顾性研究，以确定血浆嗜铬粒蛋白 A 在 3 例异位 ACTH 综合征（EAS）患者（包括本病例）和 2018 年至 2021 年期间来我院就诊的 6 例库欣病（CD）患者中的应用效果。值得注意的是，EAS 患者的血浆嗜铬粒蛋白 A 水平高于 CD 患者。此外，本病例低谷期的嗜铬粒蛋白 A 水平低于高峰期，与 CD 患者相似。检测血浆嗜铬粒蛋白 A 水平有助于区分 EAS 和 CD。
