Hernández Irma, Espinosa-de-los-Monteros Ana Laura, Mendoza Victoria, Cheng Sonia, Molina Mario, Sosa Ernesto, Mercado Moisés
Endocrinology Service, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mexico City, Mexico.
Arch Med Res. 2006 Nov;37(8):976-80. doi: 10.1016/j.arcmed.2006.05.015.
Differentiation between the two forms of ACTH-dependent Cushing's syndrome is a challenging task. Although the majority of these cases will be diagnosed as Cushing's disease secondary to an ACTH-secreting pituitary adenoma, 10-15% result from the ectopic ACTH secretion syndrome (EAS), which is usually due to neuroendocrine tumors. In the present study we report our experience with EAS in eight patients evaluated and treated during the past 10 years.
Our experience in the evaluation and management of EAS was retrospectively reviewed. The latter included a standard biochemical assessment (urinary free cortisol, low- and high-dose dexamethasone suppression tests), petrosal sinus sampling when indicated and imaging techniques such as pituitary MRI, total body CT and somatostatin receptor scintigraphy.
The ectopic nature of the ACTH hypersecretion was confirmed with inferior petrosal sinus samplings in all cases. CT scanning localized a putative tumor in 6/8 patients, whereas octreotide scintigraphy was positive in only five. In all cases, the source was traced to the lungs. However, upon performing thoracotomy, a documented ACTH-secreting bronchial carcinoid tumor was found in only four patients. Thus, 4/8 patients with EAS remained "occult." All of these patients underwent adrenalectomy for hypercortisolism control.
EAS is a rare cause of ACTH-dependent Cushing's syndrome. Truly "occult" tumors were frequent and these patients need to be under close surveillance for the detection of neuroendocrine tumors.
区分两种促肾上腺皮质激素（ACTH）依赖性库欣综合征是一项具有挑战性的任务。尽管这些病例中的大多数将被诊断为继发于分泌ACTH的垂体腺瘤的库欣病，但10% - 15%是由异位ACTH分泌综合征（EAS）引起的，这通常是由于神经内分泌肿瘤所致。在本研究中，我们报告了过去10年中对8例接受评估和治疗的EAS患者的经验。
我们对EAS评估和管理的经验进行了回顾性分析。后者包括标准的生化评估（尿游离皮质醇、低剂量和高剂量地塞米松抑制试验），必要时进行岩下窦采血以及垂体MRI、全身CT和生长抑素受体闪烁显像等成像技术。
所有病例经岩下窦采血均证实了ACTH分泌过多的异位性质。CT扫描在8例患者中的6例定位了疑似肿瘤，而奥曲肽闪烁显像仅在5例中呈阳性。所有病例中，肿瘤来源均追溯至肺部。然而，开胸手术后，仅在4例患者中发现了有记录的分泌ACTH的支气管类癌肿瘤。因此，8例EAS患者中有4例仍为“隐匿性”。所有这些患者均接受了肾上腺切除术以控制皮质醇增多症。
EAS是ACTH依赖性库欣综合征的罕见病因。真正的“隐匿性”肿瘤很常见，这些患者需要密切监测以检测神经内分泌肿瘤。
