Department of Cardio-Thoracic Surgery, Shenzhen University General Hospital, Xueyuan Avenue 1098, Nanshan District, 518055, Shenzhen, China.
Department of Thoracic Surgery, University Medical Center Mainz, Johannes Gutenberg University Mainz, Mainz, Germany.
BMC Surg. 2022 Jun 11;22(1):226. doi: 10.1186/s12893-022-01674-0.
Ectopic Cushing syndrome (ECS) is a sporadic condition. Even uncommon is an ECS that derives from a carcinoid tumor of the thymus. These tumors may pose several diagnostic and therapeutic conundrums. This report discusses the differential diagnosis, clinicopathological findings, and effective treatment of a rare case of ECS using a minimally invasive approach.
A 29-year-old woman with Cushing syndrome presented with facial flushing. Physical examination revealed hypertension (blood pressure: 141/100 mmHg). A mediastinal tumor was discovered to be the cause of the patient's chronic hypokalemia and hypercortisolemia. Cortisol levels increased in the morning, reaching 47.7 ug/dL. The levels of the hormones ACTH, aldosterone, and renin were determined to be 281 pg/mL, 3.0 ng/dL, and 2.1 pg/mL, respectively. The presence of hypertension, hypokalemia, and alkalinity suggested Cushing's syndrome, which was proven to be ACTH-dependent ECS by a dexamethasone suppression test. A chest CT scan revealed inflammation in the posterior basal region of the right lower lobe. The superior anterior mediastinum was characterized by round-shaped isodensity lesions with distinct borders. She underwent thoracoscopic anterior mediastinal tumor excision via the subxiphoid technique (R0 resection); following surgery, her blood pressure returned to normal, and the hypernatremia/hypopotassemia resolved. The tumor was determined to be a thymic carcinoid. Most notably, cortisol levels fell to half of their presurgical levels after one hour of surgery, and other abnormalities corrected substantially postoperatively.
Thoracoscopic excision of thymic tumors by subxiphoid incision may be a useful treatment option for ECS caused by neuroendocrine tumors of the thymus.
异位库欣综合征(ECS)是一种散发性疾病。即使是源自胸腺类癌的 ECS 也很少见。这些肿瘤可能会带来一些诊断和治疗上的难题。本报告讨论了一例罕见的 ECS 病例,采用微创方法,通过对其进行鉴别诊断、临床病理表现和有效治疗。
一位 29 岁的女性因库欣综合征出现面部潮红。体格检查发现血压升高(血压:141/100mmHg)。纵隔肿瘤被发现是导致患者慢性低钾血症和高皮质醇血症的原因。皮质醇水平在早晨升高,达到 47.7ug/dL。激素 ACTH、醛固酮和肾素的水平分别为 281pg/mL、3.0ng/dL 和 2.1pg/mL。高血压、低钾血症和碱中毒提示库欣综合征,通过地塞米松抑制试验证实为 ACTH 依赖性 ECS。胸部 CT 扫描显示右下肺后基底区炎症。上前纵隔呈圆形等密度病变,边界清楚。她接受了经剑突下入路的胸腔镜前纵隔肿瘤切除术(R0 切除);手术后,她的血压恢复正常,高钠血症/低钾血症得到缓解。肿瘤被确定为胸腺类癌。值得注意的是,手术一小时后皮质醇水平下降到术前水平的一半,其他异常也在术后得到了显著纠正。
经剑突下入路胸腔镜切除胸腺肿瘤可能是治疗由胸腺神经内分泌肿瘤引起的 ECS 的一种有用方法。