Rodrigues Pedro, Castedo José Luís, Damasceno Margarida, Carvalho Davide
Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar de São João EPE, Porto, Portugal.
Arq Bras Endocrinol Metabol. 2012 Oct;56(7):461-4. doi: 10.1590/s0004-27302012000700009.
Ectopic ACTH syndrome is a rare disease often associated with severe hypercortisolism. When feasible, optimal management is surgical excision of the tumor. A 33-year-old male patient was admitted to the hospital in 1993 with clinical manifestations suggestive of Cushing's syndrome. He presented high plasma ACTH and markedly elevated urinary free cortisol excretion that was not suppressed with high-dose dexamethasone administration. Pituitary MRI scan was normal. No central-to-peripheral ACTH gradient was present in bilateral inferior petrosal sinus sampling. Thoracic CT scan showed a 1.7 cm nodule at the left lung. Pulmonary fine needle cytology and immunocytochemical and ultrastructural studies, together with the presence of bone metastases, led to the diagnosis of an ACTH-producing neuroendocrine carcinoma. He was initially submitted to chemotherapy and has been on treatment with octreotide LAR since 1998, having shown a favorable clinical, biochemical and imaging response. We highlight the excellent long-term response to medical therapy with octreotide LAR, without tachyphylaxis, probably due to its antiproliferative effect.
异位促肾上腺皮质激素（ACTH）综合征是一种罕见疾病，常与严重的皮质醇增多症相关。在可行的情况下，最佳治疗方法是手术切除肿瘤。一名33岁男性患者于1993年入院，临床表现提示库欣综合征。他血浆ACTH水平高，尿游离皮质醇排泄显著升高，大剂量地塞米松给药后未被抑制。垂体磁共振成像（MRI）扫描正常。双侧岩下窦采样未显示中央至外周ACTH梯度。胸部CT扫描显示左肺有一个1.7厘米的结节。肺部细针细胞学检查、免疫细胞化学和超微结构研究，以及骨转移的存在，导致诊断为分泌ACTH的神经内分泌癌。他最初接受了化疗，自1998年以来一直使用长效奥曲肽治疗，临床、生化和影像学反应良好。我们强调了长效奥曲肽药物治疗的出色长期反应，无快速耐受现象，这可能归因于其抗增殖作用。
