A case of normoreninemic aldosterone-producing adenoma associated with chronic renal failure
A case of normoreninemic aldosterone-producing adenoma associated with chronic renal failure
复制标题
伴有慢性肾功能衰竭的去甲醛固酮腺瘤一例
作者：
H. Koshiyama;T. Fujisawa;N. Kuwamura;Yoshio Nakamura;H. Kanamori;E. Oida;A. Hara;Takashi Suzuki;H. Sasano
醛固酮腺瘤（阿帕）的诊断对于内分泌学家来说是具有挑战性的，因为阿帕并不总是表现出典型的临床和实验室特征，如高血压、低钾血症、血浆肾素活性抑制（PRA）和血浆醛固酮浓度高（PAC）。最近，一些研究表明，阿帕可以发现，甚至在正常血钾与正常PRA的受试者比以前认为的更频繁。在此我们报告一个阿帕合并慢性肾衰竭的病例，显示血钾正常且PRA正常。病人被转介到我们的诊所，以评估一个偶然发现的肾上腺肿块与异常高的PAC。6年后发现右肾上腺肿瘤明显增大。内分泌检查显示PRA正常，PAC明显升高。直立位试验对醛固酮的反应优于ACTH刺激试验。阿帕的诊断是基于PAC与PRA的比值明显升高和肾上腺皮质放射性造影，显示明确的肿瘤摄取。进行右侧腹腔镜肾上腺切除术，发现右侧肾上腺皮质腺瘤伴大出血。组织学检查发现存在两个独立的肾上腺皮质腺瘤，一个阿帕主要透明的肿瘤细胞和几个C17（17 α-羟化酶）免疫反应性和其他皮质醇产生腺瘤致密的细胞质和丰富的C17免疫反应性。此病例提示肾衰竭时“正常血肾原性淀粉样蛋白阿帕”的诊断困难。该病例符合最近的概念，即阿帕是一种持续性疾病，只有少数患者具有原发性醛固酮增多症的经典临床表现，如低钾血症。正常血钾型阿帕可能是该病最常见的表现。
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.