van Coevorden A, Laurent E, Rickaert F, van Reeth O, Van Cauter E, Mockel J
Department of Endocrinology, Free University of Brussels, Belgium.
J Endocrinol Invest. 1990 Apr;13(4):317-26. doi: 10.1007/BF03349570.
A case of ectopic ACTH syndrome with intermittent secretion in a 72-yr-old woman is described. Plasma and urinary cortisol levels were obtained at frequent intervals for a period of more than 10 months and varied erratically from the normal range to extremely high values. Nonsuppression by high doses of dexamethasone was documented during a period of hypersecretion. Normal circadian rhythmicity and normal responses to hypoglycemia were observed during an interval of dormance of the ectopic secretion. Hypokalemia did not develop. These findings, together with the occult nature of the primary tumor, resulted in unusual diagnostic difficulties. Liver masses were detected by echography and CT scan. Pathological examination of liver biopsies suggested a neuroendocrine tumor of foregut origin. While a multicentric primary apudoma secreting ACTH was a putative diagnosis, detailed and extensive microscopic post-mortem studies revealed a more likely primary tumor site in the pancreatic tail.
本文描述了一例72岁女性的异位促肾上腺皮质激素（ACTH）综合征，其具有间歇性分泌。在超过10个月的时间里，频繁测定血浆和尿皮质醇水平，结果在正常范围到极高值之间不规则波动。在高分泌期，记录到高剂量地塞米松不能抑制皮质醇分泌。在异位分泌静止期，观察到正常的昼夜节律和对低血糖的正常反应。未出现低钾血症。这些发现，加上原发肿瘤隐匿的性质，导致了不同寻常的诊断困难。通过超声检查和CT扫描检测到肝脏肿块。肝脏活检的病理检查提示为起源于前肠的神经内分泌肿瘤。虽然推测诊断为多中心原发性促肾上腺皮质激素分泌性腺瘤，但详细而广泛的尸检显微镜检查显示，更可能的原发肿瘤部位是胰尾。
