Koshiyama Hiroyuki, Fujisawa Takeshi, Kuwamura Naomitsu, Nakamura Yoshio, Kanamori Hiroshi, Oida Emi, Hara Akira, Suzuki Takashi, Sasano Hironobu
Division of Diabetes & Endocrinology, Department of Medicine, Tazuke Kofukai Foundation Medical Research Institute Kitano Hospital, 2-4-20 Ohgi-machi, Kita-ku, Osaka 530-8480, Japan.
Endocrine. 2003 Aug;21(3):221-6. doi: 10.1385/ENDO:21:3:221.
The diagnosis of aldosterone-producing adenoma (APA) is challenging for endocrinologists, as APA does not always present with the typical constellation of clinical and laboratory features, such as hypertension, hypokalemia, suppressed plasma renin activity (PRA), and high plasma aldosterone concentration (PAC). Very recently, several studies have indicated that APA can be discovered even in normokalemic subjects with normal PRA more frequently than previously considered. Here we report a case of APA associated with chronic renal failure, which showed normokalemia and normal PRA. The patient was referred to our clinic for evaluation of an incidentally discovered adrenal mass with abnormally high PAC. After 6 yr, it was found that the right adrenal tumor showed a marked increase in size. Endocrinological examinations indicated normal PRA and markedly high PAC. Aldosterone showed a better response to the upright posture test than that to ACTH stimulation test. The diagnosis of APA was made based on the markedly high PAC to PRA ratio and the adrenocortical scintigraphy, which showed unequivocal uptake into the tumor. Right laparoscopic adrenalectomy was performed, revealing a right adrenocortical adenoma with massive hemorrhage. Histopathological examinations revealed the presence of two independent adrenocortical adenomas, one APA with predominant clear tumor cells and few c17 (17alpha-hydroxylase) immunoreactivity and the other, cortisol producing adenoma with compact cytoplasm and abundant C17 immunoreactivity. This case indicates a difficulty of diagnosis of "normoreninemic APA" with renal failure. This case is in line with the recent concept that APA is a continuous condition in which only a minority of patients have the classical clinical picture of primary aldosteronism such as hypokalemia. It is possible that normokalemic APA constitutes the most common presentation of the disease.
对于内分泌科医生而言,醛固酮瘤(APA)的诊断颇具挑战性,因为APA并不总是表现出典型的临床和实验室特征组合,如高血压、低钾血症、血浆肾素活性(PRA)受抑制以及血浆醛固酮浓度(PAC)升高。最近,多项研究表明,即使在PRA正常的血钾正常受试者中,也能比以往认为的更频繁地发现APA。在此,我们报告一例与慢性肾衰竭相关的APA病例,该病例表现为血钾正常和PRA正常。该患者因偶然发现肾上腺肿块且PAC异常升高而被转诊至我们的诊所。6年后,发现右侧肾上腺肿瘤大小显著增加。内分泌检查显示PRA正常但PAC明显升高。醛固酮对直立位试验的反应比对促肾上腺皮质激素(ACTH)刺激试验的反应更好。基于显著升高的PAC与PRA比值以及肾上腺皮质闪烁显像(显示肿瘤有明确摄取)做出了APA的诊断。实施了右侧腹腔镜肾上腺切除术,发现一个伴有大量出血的右侧肾上腺皮质腺瘤。组织病理学检查显示存在两个独立的肾上腺皮质腺瘤,一个是APA,主要为透明肿瘤细胞,17α羟化酶(c17)免疫反应性低,另一个是产生皮质醇的腺瘤,细胞质致密且C17免疫反应性丰富。该病例表明肾衰竭患者“正常肾素活性APA”诊断存在困难。此病例符合最近的概念,即APA是一种连续状态,只有少数患者具有原发性醛固酮增多症的典型临床表现,如低钾血症。血钾正常的APA有可能是该疾病最常见的表现形式。