Ozbey N, Bozbora A, Kalayci G, Kapran Y, Yilmazbayhan D, Dizdaroglu F, Orhan Y, Sencer E, Molvalilar S
Department of Internal Medicine, Istanbul Faculty of Medicine, Turkey.
J Endocrinol Invest. 2000 Sep;23(8):536-41. doi: 10.1007/BF03343771.
Multiple peripheral pulmonary carcinoid tumors or their smaller counterparts (tumorlets of carcinoid type) are the most unusual form of carcinoids as a cause of ectopic corticotropin syndrome. Only three case reports were found in the literature. We describe a 35 year-old female patient with ectopic corticotropin secretion due to multiple peripheral pulmonary carcinoid tumors and tumorlets. A high-dose dexamethasone suppression test result led to the diagnosis of Cushing's disease in our case. But no tumor was identified on sella imaging and bilateral inferior petrosal sinus sampling was non-diagnostic. Computed tomography of the lungs revealed multiple acinar-nodular parenchymal infiltrations confined to the left lung. Corticotropin-dependent hypercortisolism persisted after bilateral adrenalectomy. A second operation was necessary to remove the hyperplastic adrenal remnants. Meanwhile, computed tomography findings of the thorax were unchanged. We decided to explore these nodules by open lung biopsy. During the procedure multiple nodules ranging 12 to 3 mm in diameter scattered throughout the left lung were observed and left pneumonectomy was performed. Histopathological diagnosis was multiple peripheral carcinoid tumors and tumorlets of carcinoid type showing positive immunostaining with corticotropin. This observation emphasizes a rare form of carcinoids as a cause of ectopic corticotropin secretion and its unusual response to high dose dexamethasone suppression test.
多发性外周肺类癌肿瘤或其较小的对应物（类癌型微瘤）是作为异位促肾上腺皮质激素综合征病因的最不常见的类癌形式。文献中仅发现3例病例报告。我们描述了一名35岁女性患者，因多发性外周肺类癌肿瘤和微瘤导致异位促肾上腺皮质激素分泌。在我们的病例中，高剂量地塞米松抑制试验结果导致了库欣病的诊断。但蝶鞍影像学检查未发现肿瘤，双侧岩下窦取样也未得出诊断结果。肺部计算机断层扫描显示多个腺泡结节状实质浸润局限于左肺。双侧肾上腺切除术后，促肾上腺皮质激素依赖性高皮质醇血症持续存在。需要进行第二次手术以切除增生的肾上腺残余组织。与此同时，胸部计算机断层扫描结果未改变。我们决定通过开胸肺活检来探查这些结节。在手术过程中，观察到多个直径为12至3毫米的结节散在分布于左肺，遂进行了左肺切除术。组织病理学诊断为多发性外周类癌肿瘤和类癌型微瘤，促肾上腺皮质激素免疫染色呈阳性。这一观察结果强调了类癌作为异位促肾上腺皮质激素分泌病因的一种罕见形式及其对高剂量地塞米松抑制试验的异常反应。
