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典型类癌肿瘤引发异位 ACTH 综合征病例:手术的显著疗效。

Typical Carcinoid Tumor Cases Causing Ectopic ACTH Syndrome: Dramatic Response to Surgery.

机构信息

Department of Thoracic Surgery, Gazi University, Ankara, Turkey.

出版信息

Port J Card Thorac Vasc Surg. 2023 Apr 4;30(1):53-56. doi: 10.48729/pjctvs.258.

Abstract

Although Cushing's Syndrome (CS) is usually caused by pituitary/adrenal adenomas, in the remaining few cases, Adrenocorticotrophic hormone (ACTH) is secreted by extrapituitary neuroendocrine tumors (NET). In typical pulmonary / bronchial carcinoid tumors leading to ectopic ACTH syndrome (EAS). The main principle of treatment is the localization of the ACTH-secreting tumor and its surgical removal. In this case report, we aimed to present two typical carcinoid cases, whose ACTH and cortisol levels returned to normal after lung surgery for ectopic ACTH. One of the cases, a 32-year-old female patient with CS symptoms and signs, was referred to our department after detecting a lesion in the left lower lobe in the thorax CT, which did not show an obvious focus on cranial MRI. The other patient, a 36-year-old male, had previously undergone adrenalectomy for Cushing's syndrome and was admitted to the emergency department due to adrenal insufficiency. The lesion seen in the right lower lobe on thorax CT was evaluated as an ectopic ACTH focus. After the tumors of the patients were resected according to surgical principles, CS clinic regressed, and ACTH and cortisol values returned to normal. Pathological examinations of the surgically resected tumors were reported as typical carcinoid. With surgically removed typical carcinoids tumors, excellent longterm survival results can be achieved and hypercortisolism can be controlled.

摘要

虽然库欣综合征(CS)通常由垂体/肾上腺腺瘤引起,但在剩下的少数情况下,促肾上腺皮质激素(ACTH)由垂体外神经内分泌肿瘤(NET)分泌。在典型的肺/支气管类癌肿瘤导致异位 ACTH 综合征(EAS)中。治疗的主要原则是定位分泌 ACTH 的肿瘤并进行手术切除。在本病例报告中,我们旨在介绍两例典型类癌病例,它们的 ACTH 和皮质醇水平在肺外分泌 ACTH 的手术后恢复正常。其中一例为 32 岁女性 CS 症状和体征患者,在胸部 CT 检测到左下叶病变后转至我科,头部 MRI 未见明显焦点。另一位患者为 36 岁男性,因库欣综合征曾行肾上腺切除术,因肾上腺功能不全入住急诊部。胸部 CT 上看到的右下叶病变被评估为异位 ACTH 焦点。根据手术原则切除患者的肿瘤后,CS 临床症状消退,ACTH 和皮质醇值恢复正常。手术切除的肿瘤的病理检查报告为典型类癌。通过手术切除典型类癌肿瘤,可以实现极好的长期生存结果并控制皮质醇过多症。

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