Endocrine Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Department of Clinical Pathology, Faculty of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.
J Med Case Rep. 2021 Oct 11;15(1):514. doi: 10.1186/s13256-021-03046-3.
Ectopic adrenocorticotropic hormone secretion syndrome occurs in 10% of all patients with adrenocorticotropic-hormone-dependent hypercortisolism. It is usually associated with overt malignancies or with occult and indolent tumors. This study aims to confirm the source of ectopic adrenocorticotropic hormone in four patients with ectopic Cushing's syndrome over time.
A 38-year-old Iranian man with Cushing's syndrome underwent bilateral adrenalectomy since the source of ectopic adrenocorticotropic hormone secretion was not localized and pituitary imaging was normal. A whole-body scan revealed a right-lung tumoral mass with mediastinal lymph node metastasis. The mass was assumed a lung carcinoid tumor with mediastinal adenopathy. Right-lung mid-zone lobectomy and mediastinal lymphadenectomy were done. In a 47-year-old Iranian man with Cushing's syndrome, whole-body computed tomography scan revealed a pulmonary nodule in the posterior segment of the left lower lobe of the lung. The third case was a 25-year-old Iranian man who presented with symptoms and signs of Cushing's syndrome. Pituitary magnetic resonance imaging revealed a microadenoma 5 × 9 mm. Whole-body scan showed abnormal focal somatostatin receptors analog avid lesion in the posterior aspect of inferior third of right lung, highly suggestive of ectopic adrenocorticotropic-hormone-producing tumor. The last case was a 43-year-old Iranian woman with Marfan syndrome with a history of mitral and aortic valve replacement and chronic dissection of the aorta, who presented with symptoms and signs of Cushing's syndrome. She underwent bilateral adrenalectomy 1 year later owing to failure to locate ectopic adrenocorticotropic hormone syndrome. Whole-body scan showed abnormally increased radiotracer uptake in the midline of the skull base and posterior aspect of the middle zone of left hemithorax and bed of left lobe of thyroid.
The clinical spectrum of ectopic adrenocorticotropic hormone secretion syndrome is wide, and distinguishing Cushing's disease from ectopic adrenocorticotropic hormone secretion syndrome is difficult. Initial failure to identify a tumor is common. Pulmonary carcinoid or occult source of ectopic adrenocorticotropic hormone secretion syndrome is usually the cause. In occult cases of ectopic adrenocorticotropic hormone in which the tumor cannot be localized, serial follow-up with serial computed tomography, magnetic resonance imaging, or scintigraphy is recommended for several years until the tumor can be localized and treated.
异位促肾上腺皮质激素分泌综合征发生在所有依赖促肾上腺皮质激素的皮质醇增多症患者的 10%。它通常与明显的恶性肿瘤或隐匿性和惰性肿瘤有关。本研究旨在随着时间的推移，确认四名异位库欣综合征患者异位促肾上腺皮质激素的来源。
一名 38 岁的伊朗男性患有库欣综合征，由于未能定位异位促肾上腺皮质激素分泌的来源，且垂体成像正常，他接受了双侧肾上腺切除术。全身扫描显示右侧肺部有一个肿瘤性肿块，伴有纵隔淋巴结转移。该肿块被认为是一种具有纵隔淋巴结转移的肺类癌肿瘤。进行了右肺中叶叶切除术和纵隔淋巴结切除术。一名 47 岁的伊朗男性患有库欣综合征，全身计算机断层扫描显示左肺下叶后段有一个肺结节。第三例是一名 25 岁的伊朗男性，出现库欣综合征的症状和体征。垂体磁共振成像显示 5×9mm 的微腺瘤。全身扫描显示在后下三分之一右肺的后部有异常的局部生长抑素受体类似物摄取病灶，高度提示异位促肾上腺皮质激素分泌肿瘤。最后一例是一名 43 岁的伊朗女性，患有马凡综合征，有二尖瓣和主动脉瓣置换术以及主动脉慢性夹层病史，出现库欣综合征的症状和体征。由于未能定位异位促肾上腺皮质激素综合征，她在 1 年后接受了双侧肾上腺切除术。全身扫描显示颅底中线和左半胸中部后区以及左叶甲状腺床的放射性示踪剂摄取异常增加。
异位促肾上腺皮质激素分泌综合征的临床谱很广，区分库欣病和异位促肾上腺皮质激素分泌综合征很困难。最初未能识别肿瘤很常见。肺类癌或隐匿性异位促肾上腺皮质激素分泌综合征的来源通常是病因。在不能定位异位促肾上腺皮质激素的隐匿性病例中，建议进行数年的连续随访，包括连续计算机断层扫描、磁共振成像或闪烁扫描，直到能够定位和治疗肿瘤。
