Zeiger M A, Pass H I, Doppman J D, Nieman L K, Chrousos G P, Cutler G B, Jensen R T, Norton J A
Surgical Metabolism Section, National Cancer Institute, National Institutes of Health, Bethesda, Md.
Surgery. 1992 Dec;112(6):994-1000; discussion 1000-1.
Non-small cell ectopic adrenocorticotropic hormone (ACTH) syndrome is a rare cause of hypercortisolism that may require surgery for either curative resection or palliative adrenalectomy.
We report our surgical experience with 41 patients with ectopic ACTH syndrome and no evidence of small cell lung cancer at initial evaluation.
All 41 patients had documented hypercortisolism secondary to ectopic production of ACTH. Based on imaging study results, we determined that 21 patients had localized/resectable disease; eight patients had metastatic disease, and 12 patients had occult disease at examination. Of the 21 patients with localized disease, 16 (76%) were cured of ectopic ACTH by surgery (15 bronchial carcinoid, one pheochromocytoma). Patients with bronchial carcinoid had the greatest probability for cure of ectopic ACTH syndrome, and patients with thoracic primary tumor were more likely to be cured than patients with abdominal primaries. Of the eight patients who had metastatic disease, none were cured of the disease; five patients underwent bilateral adrenalectomy, and three patients were given medical therapy. Only one patient was alive after 5 years. Of the 12 patients who had occult disease, four patients were eventually cured of the disease (three bronchial carcinoid, one thymic carcinoid); one patient died of disease (small cell lung cancer), and seven patients still have occult disease. Nine of 12 patients with occult disease underwent bilateral adrenalectomy for surgical management of hypercortisolism.
This study suggests that the most common primary focus of ectopic ACTH production is within the thorax with 25 of 34 (74%) identifiable tumors originating within either the thymus or bronchus. Adrenalectomy offers excellent palliation of hypercortisolism secondary to either occult or metastatic disease. Patients who initially have localized disease usually have bronchial carcinoids and have a high probability of cure with surgical resection (81%).
非小细胞异位促肾上腺皮质激素（ACTH）综合征是皮质醇增多症的一种罕见病因，可能需要手术进行根治性切除或姑息性肾上腺切除术。
我们报告了41例异位ACTH综合征患者的手术经验，这些患者在初始评估时无小细胞肺癌证据。
所有41例患者均有因异位分泌ACTH导致的皮质醇增多症记录。根据影像学检查结果，我们确定21例患者患有局限性/可切除疾病；8例患者有转移性疾病，12例患者在检查时有隐匿性疾病。在21例局限性疾病患者中，16例（76%）通过手术治愈了异位ACTH（15例支气管类癌，1例嗜铬细胞瘤）。支气管类癌患者治愈异位ACTH综合征的可能性最大，胸部原发性肿瘤患者比腹部原发性肿瘤患者更有可能治愈。在8例有转移性疾病的患者中，无一例治愈该疾病；5例患者接受了双侧肾上腺切除术，3例患者接受了药物治疗。5年后只有1例患者存活。在12例有隐匿性疾病的患者中，4例最终治愈了该疾病（3例支气管类癌，1例胸腺类癌）；1例患者死于该疾病（小细胞肺癌），7例患者仍有隐匿性疾病。12例隐匿性疾病患者中有9例接受了双侧肾上腺切除术以治疗皮质醇增多症。
本研究表明，异位ACTH产生的最常见原发部位在胸部，34例可识别肿瘤中有25例（74%）起源于胸腺或支气管。肾上腺切除术对隐匿性或转移性疾病继发的皮质醇增多症有很好的姑息作用。最初患有局限性疾病的患者通常患有支气管类癌，手术切除治愈的可能性很高（81%）。
