Otsuka Fumio, Miyoshi Tomoko, Murakami Kazutoshi, Inagaki Kenichi, Takeda Masaya, Ujike Kazuhiro, Ogura Toshio, Omori Masako, Doihara Hiroyoshi, Tanaka Yasushi, Hashimoto Kozo, Makino Hirofumi
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan.
Am J Hypertens. 2005 Oct;18(10):1364-8. doi: 10.1016/j.amjhyper.2005.01.019.
We report a 55-year-old woman with ectopic adrenocorticotropin (ACTH) secretion caused by extra-adrenal pheochromocytoma. The patient presented with a 6-month history of hypertension and diabetes mellitus. Her serum and urinary cortisol levels were extremely high and dexamethasone failed to suppress the cortisol secretion. Her plasma ACTH levels were also elevated (>300 pg/mL) and irresponsive to corticotropin-releasing hormone (CRH) or metyrapone administration. Gel filtration analysis of the patient's plasma detected the existence of large molecular weight ACTH being eluted with a major peak of authentic 1-39 ACTH. Abdominal computed tomographic scan and magnetic resonance imaging revealed a 5-cm paraganglioma located underneath the left kidney, in which (123)I-MIBG tracer specifically accumulated. Bilateral adrenal glands were diffusely enlarged. After surgical removal of the paraganglioma, the patient's clinical symptoms improved and biochemistry normalized including plasma ACTH, urinary free cortisol, and urinary catecholamines. Subsequent histologic evaluation of the transected paranglioma tissue revealed ACTH, synaptin, and chromogranin-A histologically immunostaining. Culture of primary cells collected from the resected paraganglioma demonstrated of in vitro production of ACTH, noradrenaline, and adrenaline. This is the first report of ectopic ACTH syndrome induced by an extra-adrenal abdominal paraganglioma.
我们报告了一名55岁女性，其患有由肾上腺外嗜铬细胞瘤引起的异位促肾上腺皮质激素（ACTH）分泌。该患者有6个月的高血压和糖尿病病史。她的血清和尿皮质醇水平极高，地塞米松未能抑制皮质醇分泌。她的血浆ACTH水平也升高（>300 pg/mL），对促肾上腺皮质激素释放激素（CRH）或甲吡酮给药无反应。对患者血浆进行凝胶过滤分析检测到存在与真实的1-39 ACTH主峰一起洗脱的大分子量ACTH。腹部计算机断层扫描和磁共振成像显示左肾下方有一个5厘米的副神经节瘤，其中（123）I-MIBG示踪剂特异性聚集。双侧肾上腺弥漫性增大。手术切除副神经节瘤后，患者的临床症状改善，生化指标恢复正常，包括血浆ACTH、尿游离皮质醇和尿儿茶酚胺。随后对切除的副神经节瘤组织进行组织学评估，显示ACTH、突触素和嗜铬粒蛋白A组织学免疫染色。对从切除的副神经节瘤收集的原代细胞进行培养，证明其在体外可产生ACTH、去甲肾上腺素和肾上腺素。这是关于肾上腺外腹部副神经节瘤诱发异位ACTH综合征的首例报告。
