Gerhardt Louisa M S, Sabath Lisa, Müller Beat, Capraro Joel, Borm Katrin
Abteilung für Endokrinologie, Diabetologie und Metabolismus, Medizinische Universitätsklinik, Kantonsspital Aarau, Schweiz.
Dtsch Med Wochenschr. 2020 Jun;145(11):783-786. doi: 10.1055/a-1163-9873. Epub 2020 Jun 3.
A 58-year-old man presented with refractory hypokalemia and rapid weight gain. On examination, he had high blood pressure, central obesity and bilateral pitting edema.
Biochemical tests showed hypokalemic metabolic alkalosis due to ACTH-dependent hypercortisolism. CT of thorax and abdomen revealed a pulmonary and a right adrenal mass. Biopsy of the pulmonary mass led to the diagnosis of an ACTH-producing small cell lung cancer.
Under treatment with ketoconazole and chemotherapy a tumor response was reached and the hypercortisolism was controlled. Since the right adrenal mass remained stationary under chemotherapy, an incidental adrenal adenoma seemed the most likely diagnosis.
The diagnosis of paraneoplastic Cushing's syndrome can be challenging, since classical clinical features of hypercortisolism may still be absent, even if the underlying cancer is already advanced. Therefore high clinical suspicion is warranted, especially in patients presenting with new-onset refractory hypokalemia, metabolic alkalosis and arterial hypertension.
一名58岁男性出现难治性低钾血症和体重迅速增加。检查发现，他患有高血压、中心性肥胖和双侧凹陷性水肿。
生化检查显示，由于促肾上腺皮质激素（ACTH）依赖性皮质醇增多症导致低钾性代谢性碱中毒。胸部和腹部CT显示肺部有一个肿块以及右侧肾上腺有一个肿块。肺部肿块活检确诊为分泌ACTH的小细胞肺癌。
在酮康唑和化疗治疗下，肿瘤出现反应，皮质醇增多症得到控制。由于化疗期间右侧肾上腺肿块大小稳定，最可能的诊断似乎是偶发性肾上腺腺瘤。
副肿瘤性库欣综合征的诊断可能具有挑战性，因为即使潜在癌症已经进展，皮质醇增多症的典型临床特征仍可能不存在。因此，尤其是对于出现新发难治性低钾血症、代谢性碱中毒和动脉高血压的患者，高度的临床怀疑是必要的。
