Espinosa-de-Los-Monteros Ana Laura, Ramírez-Rentería Claudia, Mercado Moisés
From the Endocrine Service and the Research Unit in Endocrine Diseases, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, IMSS, Mexico City, Mexico.
From the Endocrine Service and the Research Unit in Endocrine Diseases, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, IMSS, Mexico City, Mexico..
Endocr Pract. 2020 Dec;26(12):1435-1441. doi: 10.4158/EP-2020-0368.
Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a heterogeneous condition caused by neuroendocrine neoplasms (NENs) located in the lungs, thymus, or pancreas. Our purpose was to evaluate the long-term outcome of these patients.
Retrospective study at a referral center. The charts of 164 patients with Cushing syndrome, followed at our center from 1993 to 2019, were analyzed.
EAS was found in 16 patients (9.75%, 9 women, mean age 36.01 years) who had been followed for a median of 72 months. The source of EAS was a NEN in 10 patients (8 bronchial and 2 thymic carcinoid tumors) and a mixed corticomedullary tumor, consisting of a pheochromocytoma and an adrenocortical carcinoma in 1 patient. In 2 of the 6 patients initially considered to have occult EAS, the source of the ACTH excess became apparent after adrenalectomy, whereas in the remaining 4 (25%) patients, it has remained occult. Of the 11 patients in whom resection of the NEN was attempted, 10 patients achieved an early remission (91%), but 4 (25%) of these patients had a recurrence during follow-up (biochemically and clinically silent in 2 patients). Three patients died (18.75%): the young woman with the mixed corticomedullary tumor, a man with a thymic NEN that evolved into a neuroendocrine (NE) carcinoma after 11 years of follow-up, and a woman with a bronchial NEN.
The course of EAS varies according to tumor type and grade. Some patients have a protracted course, whereas others may evolve into neuroendocrine carcinomas.
ACTH = adrenocorticotropic hormone; CS = Cushing syndrome; CT = computed tomography; CV = coefficient of variation; EAS = ectopic ACTH syndrome; IQR = interquartile range; NEN = neuroendocrine neoplasm; SCCL = small cell carcinoma of the lung; TSS = transsphenoidal surgery; UFC = urinary free cortisol.
异位促肾上腺皮质激素（ACTH）综合征（EAS）是一种由位于肺、胸腺或胰腺的神经内分泌肿瘤（NENs）引起的异质性疾病。我们的目的是评估这些患者的长期预后。
在一家转诊中心进行回顾性研究。分析了1993年至2019年在我们中心随访的164例库欣综合征患者的病历。
16例患者（9.75%，9名女性，平均年龄36.01岁）被诊断为EAS，中位随访时间为72个月。EAS的来源在10例患者中为NEN（8例支气管和2例胸腺类癌肿瘤），1例患者为混合性皮质髓质肿瘤，由嗜铬细胞瘤和肾上腺皮质癌组成。在最初被认为患有隐匿性EAS的6例患者中，2例患者在肾上腺切除术后ACTH过量的来源变得明显，而其余4例（25%）患者的来源仍不明确。在尝试切除NEN的11例患者中，10例患者实现了早期缓解（91%），但其中4例（25%）患者在随访期间复发（2例患者生化和临床无症状）。3例患者死亡（18.75%）：患有混合性皮质髓质肿瘤的年轻女性、随访11年后胸腺NEN演变为神经内分泌（NE）癌的男性以及患有支气管NEN的女性。
EAS的病程因肿瘤类型和分级而异。一些患者病程迁延，而另一些患者可能演变为神经内分泌癌。
ACTH = 促肾上腺皮质激素；CS = 库欣综合征；CT = 计算机断层扫描；CV = 变异系数；EAS = 异位ACTH综合征；IQR = 四分位数间距；NEN = 神经内分泌肿瘤；SCCL = 肺小细胞癌；TSS = 经蝶窦手术；UFC = 尿游离皮质醇
