PDF(Pubmed)
Abstract：
Ectopic ACTH-secreting pheochromocytoma is a very rare cause of Cushing´s syndrome, posing diagnostic and therapeutic challenges. We here report the case of a female patient with suspected severe Cushing´s syndrome associated with melanoderma, arterial hypertension resistant to triple therapy and unbalanced diabetes treated with insulin therapy. Biologically, urinary ethoxylated, 24-hour urinary free cortisol and ACTH were very high. Imaging showed a 3.5 cm left adrenal mass. The patient underwent left adrenalectomy after medical preparation, with good clinico-biological outcome. Anatomopathological examination confirmed the diagnosis of pheochromocytoma. This case study highlights the importance of measuring methoxylated derivatives in any patient with ACTH-dependent Cushing´s syndrome associated with an adrenal mass. The aim is to ensure early treatment and avoid life-threatening complications.
摘要：
异位分泌ACTH的嗜铬细胞瘤是库欣综合征的一个非常罕见的原因，构成诊断和治疗挑战。我们在这里报告一例女性患者，怀疑患有与黑斑病相关的严重库欣综合征，动脉高血压抵抗三联疗法和胰岛素治疗的不平衡糖尿病。生物学,尿乙氧基化，24小时尿游离皮质醇和ACTH非常高。影像学显示左侧肾上腺肿块3.5cm。患者在医疗准备后接受了左肾上腺切除术，具有良好的临床生物学结果。解剖病理学检查证实了嗜铬细胞瘤的诊断。此案例研究强调了在患有与肾上腺肿块相关的ACTH依赖性库欣综合征的任何患者中测量甲氧基化衍生物的重要性。目的是确保早期治疗并避免危及生命的并发症。
