Barani Claire, Goyers Jean François, Hoton Delphine, Maiter Dominique, Furnica Raluca Maria
Department of Endocrinology, Cliniques Universitaires Saint-Luc UCL, Bruxelles, Belgium.
Department of Radiology, Clinique CHC MontLégia, Liége, Belgium.
JCEM Case Rep. 2025 Jul 10;3(8):luaf143. doi: 10.1210/jcemcr/luaf143. eCollection 2025 Aug.
Ectopic ACTH secretion is a rare, severe condition covering a wide spectrum of tumors of different location and aggressiveness. The usual poor prognosis of patients with ectopic ACTH secretion requires prompt management. We report a case of a patient with high surgical risk and intolerance to cortisol-lowering drug, in which hypercortisolism was successfully treated with radiofrequency ablation of a lung carcinoid tumor. A 78-year-old woman was referred for a rapidly progressive ACTH-dependent Cushing syndrome (CS). Biological tests suggested the presence of CS due to ectopic ACTH production. Imaging studies detected an 11-mm left upper pulmonary lobe nodule. A computed tomography-guided biopsy of the lesion confirmed a well-differentiated ACTH-positive carcinoid tumor with a Ki67 index <10%. The patient started therapy with ketoconazole but developed severe gastrointestinal side effects. Because of a very high surgical risk, lung resection was prohibited. Radiofrequency ablation of the lung carcinoid tumor was performed without major complication. The procedure allowed a progressive control of hypercortisolism with rapid improvement of CS that persists after 1 year. We show that radiofrequency ablation may be an efficient alternative treatment for the management of high surgical risk patients with ectopic ACTH syndrome secondary to a well-differentiated lung carcinoid tumor.
异位促肾上腺皮质激素(ACTH)分泌是一种罕见的严重病症,涵盖了多种不同位置和侵袭性的肿瘤。异位ACTH分泌患者通常预后较差,需要及时治疗。我们报告了一例手术风险高且对降低皮质醇药物不耐受的患者,其通过对肺类癌肿瘤进行射频消融成功治疗了皮质醇增多症。一名78岁女性因快速进展的促肾上腺皮质激素依赖性库欣综合征(CS)前来就诊。生物学检查提示存在因异位ACTH产生导致的CS。影像学研究发现左肺上叶有一个11毫米的结节。对该病变进行计算机断层扫描引导下活检,证实为分化良好的ACTH阳性类癌肿瘤,Ki67指数<10%。患者开始使用酮康唑治疗,但出现了严重的胃肠道副作用。由于手术风险极高,禁止进行肺切除术。对肺类癌肿瘤进行射频消融,未出现重大并发症。该手术使皮质醇增多症得到逐步控制,CS迅速改善,且1年后仍持续改善。我们表明,射频消融可能是治疗因分化良好的肺类癌肿瘤导致的异位ACTH综合征且手术风险高的患者的一种有效替代治疗方法。