Elsheikh Arwa, Harbuz-Miller Inga, Vates Edward, Nead Michael, Shafiq Ismat
Endocrinology, Diabetes, and Metabolism, UCHealth Anschutz Outpatient Pavilion, Anschutz Medical Campus, Aurora, USA.
Endocrinology and Metabolism, University of Rochester School of Medicine and Dentistry, Rochester, USA.
Cureus. 2024 Nov 11;16(11):e73438. doi: 10.7759/cureus.73438. eCollection 2024 Nov.
Ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing is a rare syndrome. We present a case that illustrates the diagnostic and therapeutic challenges of ectopic Cushing. A 35-year-old woman presented to the outpatient clinic for evaluation of progressive weight gain, muscle weakness, easy bruising, uncontrolled hypertension, and hyperglycemia. Biochemical workup revealed elevated salivary cortisol and 24-hour urine cortisol; the baseline ACTH was elevated, consistent with ACTH-dependent hypercortisolemia. Imaging showed a pituitary microadenoma and a lung nodule. Inferior petrosal sinus sampling was suggestive of an ectopic source. Medical treatment was employed to manage acute hypercortisolemia with a resolution of symptoms. A biopsy of the lung nodule showed the neuroendocrine tumor. Surgical treatment with pulmonary wedge resection did not alleviate hypercortisolemia, leading to repeat surgery with radical lymph node dissection, which resulted in the resolution of hypercortisolemia. This case illustrates that radical lymph node dissection, along with tumor resection, has a high likelihood of cure.
异位促肾上腺皮质激素（ACTH）依赖性库欣综合征是一种罕见的综合征。我们报告一例病例，该病例说明了异位库欣综合征在诊断和治疗方面的挑战。一名35岁女性因进行性体重增加、肌肉无力、易瘀伤、难以控制的高血压和高血糖到门诊就诊。生化检查显示唾液皮质醇和24小时尿皮质醇升高；基础促肾上腺皮质激素升高，符合ACTH依赖性高皮质醇血症。影像学检查显示垂体微腺瘤和肺结节。岩下窦取样提示为异位来源。采用药物治疗来控制急性高皮质醇血症，症状得到缓解。肺结节活检显示为神经内分泌肿瘤。肺楔形切除术未能缓解高皮质醇血症，导致再次手术并进行根治性淋巴结清扫，从而使高皮质醇血症得到缓解。该病例表明，根治性淋巴结清扫联合肿瘤切除治愈的可能性很大。
