Department of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, 577 Matsushima, Kurashiki, 701-0192, Japan.
BMC Endocr Disord. 2021 Aug 12;21(1):163. doi: 10.1186/s12902-021-00818-2.
Various adrenal disorders including primary aldosteronism and Cushing's syndrome lead to the cause of hypertension. Although primary aldosteronism is sometimes complicated with preclinical Cushing's syndrome, concurrence of overt Cushing's syndrome and primary aldosteronism is very rare. In addition, it has been drawing attention recently that primary aldosteronism is brought about by the presence of aldosterone-producing cell cluster in adjacent adrenal cortex rather than the presence of aldosterone-producing adenoma.
A 67-year-old Japanese female was referred to our institution due to moon face and central obesity. Based on various clinical findings and data, we diagnosed this subject as overt Cushing's syndrome and primary aldosteronism. Furthermore, in immunostaining for cytochrome P450 (CYP) 11B1, a cortisol-producing enzyme, diffuse staining was observed in tumorous lesion. Also, in immunostaining for CYP11B2, an aldosterone-producing enzyme, CYP11B2 expression was not observed in tumorous lesion, but strong CYP11B2 expression was observed in adjacent adrenal cortex, indicating the presence of aldosterone-producing cell cluster.
We should bear in mind the possibility that concurrence of overt Cushing's syndrome and primary aldosteronism is accompanied by aldosterone-producing cell cluster in adjacent adrenal cortex.
各种肾上腺疾病，包括原发性醛固酮增多症和库欣综合征，都会导致高血压的发生。虽然原发性醛固酮增多症有时会并发临床前期库欣综合征，但显性库欣综合征和原发性醛固酮增多症同时存在非常罕见。此外，最近人们越来越关注到，醛固酮产生细胞簇存在于肾上腺皮质的相邻部位而不是醛固酮腺瘤的存在，导致了原发性醛固酮增多症。
一位 67 岁的日本女性因满月脸和中心性肥胖被转介至我院。根据各种临床发现和数据，我们诊断该患者患有显性库欣综合征和原发性醛固酮增多症。此外，在细胞色素 P450（CYP）11B1 的免疫染色中，一种产生皮质醇的酶，在肿瘤病变中观察到弥漫性染色。而且，在 CYP11B2 的免疫染色中，一种产生醛固酮的酶，在肿瘤病变中未观察到 CYP11B2 的表达，但在相邻肾上腺皮质中观察到强烈的 CYP11B2 表达，表明存在醛固酮产生细胞簇。
我们应该牢记显性库欣综合征和原发性醛固酮增多症同时存在的可能性，并且伴有肾上腺皮质的醛固酮产生细胞簇。
