Hypercortisolism Due to Paraganglioma Secreting Adrenocorticotropin and Catecholamines.

JCEM case reports Pub Date : 2025-04-10 eCollection Date: 2025-05-01 DOI:10.1210/jcemcr/luaf052
Drew W Cox, Aleona C Zuzek, Matthew Genco, Tammy Holm, Shailendra B Patel
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Abstract

A paraganglioma is a neuroendocrine tumor classically associated with catecholamine production. We describe a 71-year-old woman with an incidentally identified para-aortic mass who later developed hyperglycemia, hypertension, hypokalemia, and leukocytosis. Work-up ultimately revealed significantly elevated adrenocorticotropin (ACTH), cortisol, and metanephrines, and biopsy of the mass suggested paraganglioma cosecretion of both ACTH and catecholamines. Using osilodrostat to decrease her excess cortisol production, she underwent successful surgical paraganglioma resection. Pathology of the mass demonstrated a paraganglioma with ACTH-producing cells, confirming the diagnosis of ectopic Cushing syndrome (CS). Following resection, the patient had resolution of hypertension and hyperglycemia and normalization of the hypothalamic-pituitary-adrenal axis. We describe the work-up and important perioperative and long-term management considerations for patients with hypercortisolism from ectopic CS and catecholamine excess.

副神经节瘤分泌促肾上腺皮质激素和儿茶酚胺所致的高皮质醇症。
副神经节瘤是一种典型的与儿茶酚胺产生有关的神经内分泌肿瘤。我们描述了一位71岁的女性,偶然发现了主动脉旁肿块,后来发展为高血糖、高血压、低钾血症和白细胞增多症。最终检查显示促肾上腺皮质激素(ACTH)、皮质醇和肾上腺素显著升高,肿块活检提示副神经节瘤促肾上腺皮质激素和儿茶酚胺共同分泌。使用奥西洛司他来减少她过量的皮质醇分泌,她成功地接受了手术切除副神经节瘤。肿块病理显示副神经节瘤伴acth产生细胞,确认异位库欣综合征(CS)的诊断。切除后,患者高血压和高血糖得到缓解,下丘脑-垂体-肾上腺轴恢复正常。我们描述了对异位CS和儿茶酚胺过量引起的高皮质醇患者的检查和重要的围手术期和长期管理考虑。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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