Oh Hyoung Chul, Koh Jung-Min, Kim Min Seon, Park Joong Yoel, Shong Young Kee, Lee Ki-Up, Kim Ghi Su, Hong Suck Joon, Koo Hyun Lyoung, Kim Won Bae
Division of Endocrinology and Metabolism, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Endocr J. 2003 Dec;50(6):739-44. doi: 10.1507/endocrj.50.739.
Ectopic ACTH syndrome is rarely caused by pheochromocytoma. We report a case of a 28-year-old woman with Cushing's syndrome due to ACTH-producing adrenal pheochromocytoma. She had delivered preterm baby at 32nd week of gestation with 'severe preeclampsia'. After delivery, persistent hypertension accompanied by severe headache led her to being misdiagnosed as Cushing's syndrome due to right adrenal adenoma (normal plasma ACTH level) and cerebral vasculitis of unknown etiology. She was referred to our hospital for surgical treatment. Repeated biochemical studies suggested coexistence of ectopic ACTH syndrome and pheochromocytoma. To reverse her clinical deterioration, right total and left subtotal adrenalectomy was performed with presumptive diagnosis of 1) right adrenal pheochromocytoma causing ectopic ACTH syndrome or 2) coexistence of ACTH-dependent Cushing's syndrome and right adrenal pheochromocytoma. Pathologic examination of right adrenal mass revealed pheochromocytoma which showed strong immunostaining for ACTH. Plasma ACTH and urinary cortisol excretion normalized after surgery, but she succumbed to multiple cerebral infarcts and disseminated intravascular coagulation. Pregnancy and inappropriately low plasma ACTH at initial evaluation might have hampered early diagnosis. To our knowledge, this is the first description of a case with ectopic ACTH syndrome due to pheochromocytoma associated with pregnancy.
异位促肾上腺皮质激素（ACTH）综合征很少由嗜铬细胞瘤引起。我们报告一例28岁患有库欣综合征的女性病例，病因是产生ACTH的肾上腺嗜铬细胞瘤。她在妊娠32周时因“重度子痫前期”早产。产后，持续高血压伴严重头痛导致她被误诊为因右肾上腺腺瘤（血浆ACTH水平正常）和病因不明的脑血管炎所致的库欣综合征。她被转诊至我院接受手术治疗。反复的生化检查提示异位ACTH综合征和嗜铬细胞瘤并存。为逆转其临床恶化情况，在初步诊断为1）导致异位ACTH综合征的右肾上腺嗜铬细胞瘤或2）ACTH依赖性库欣综合征与右肾上腺嗜铬细胞瘤并存的情况下，实施了右侧肾上腺全切术和左侧肾上腺次全切除术。右侧肾上腺肿块的病理检查显示为嗜铬细胞瘤，对ACTH呈强免疫染色。术后血浆ACTH和尿皮质醇排泄恢复正常，但她最终死于多发性脑梗死和弥散性血管内凝血。妊娠以及初始评估时血浆ACTH水平不适当降低可能阻碍了早期诊断。据我们所知，这是首例关于妊娠相关嗜铬细胞瘤导致异位ACTH综合征病例的描述。
