Onoda Naoyoshi, Ishikawa Tetsuro, Nishio Kohei, Tahara Hideki, Inaba Masaaki, Wakasa Kenichi, Sumi Tomohiko, Yamazaki Takanori, Shigematsu Kazuto, Hirakawa Kosei
Department of Surgical Oncology, Osaka City University Graduate School of Medicine, Osaka, Japan.
Endocr J. 2009;56(3):495-502. doi: 10.1507/endocrj.k08e-268. Epub 2009 Mar 7.
Synchronous associations of Cushing's syndrome (CS) and primary aldosteronism (PA) with multiple adrenocortical adenomas secreting each hormone independently have rarely been reported. Herein, we describe a unique case of PA associated with CS with detailed clinical and pathological investigations. Bilateral adrenal masses with clinical symptoms of CS and PA were found in a 43-year-old woman. Venous sampling demonstrated excess secretion of cortisol, and aldosterone from right, and left tumor, respectively. A bilateral laparoscopic partial adrenalectomy was undergone. The right adrenal tumor (3 cm) was yellow in color with abundant lipofuscin granules, and was composed of both eosinophilic compact cells and clear cells. In situ hybridization showed that both mRNAs for HSD3B2 and CYP17A1 were strongly expressed in the tumor, suggesting cortisol synthesis. Left adrenal tumor (2.4 cm) was golden-yellow in color, and composed of clear cells only. Expression of HSD3B2 and CYP11B mRNAs were observed in the tumor compatible with the aldosterone synthesis. Furthermore, minute nodules were found at the surface of normal-appearing cortex on both sides of the adrenal glands, and the expression of HSD3B2 and CYP11B mRNAs was clearly demonstrated within the nodules, indicating aldosterone synthesis. We diagnosed that the present case had 1) cortisol-producing right adrenocortical adenoma, 2) aldosterone producing left adrenocortical adenoma, and 3) cortical minute nodules with aldosterone production in both adrenal glands compatible with idiopathic adrenal hyperplasia. We reviewed the cases reported, and discussed the significance of the minute nodules in the adrenal cortex, often found in association with the adrenocortical adenoma.
库欣综合征(CS)与原发性醛固酮增多症(PA)伴多个独立分泌每种激素的肾上腺皮质腺瘤的同步关联鲜有报道。在此,我们描述了一例PA合并CS的独特病例,并进行了详细的临床和病理研究。一名43岁女性被发现双侧肾上腺肿块伴有CS和PA的临床症状。静脉采血显示右侧和左侧肿瘤分别过量分泌皮质醇和醛固酮。患者接受了双侧腹腔镜肾上腺部分切除术。右侧肾上腺肿瘤(3厘米)呈黄色,含有丰富的脂褐素颗粒,由嗜酸性致密细胞和透明细胞组成。原位杂交显示肿瘤中HSD3B2和CYP17A1的mRNA均强烈表达,提示皮质醇合成。左侧肾上腺肿瘤(2.4厘米)呈金黄色,仅由透明细胞组成。肿瘤中观察到与醛固酮合成相符的HSD3B2和CYP11B mRNA表达。此外,在双侧肾上腺外观正常的皮质表面发现微小结节,结节内HSD3B2和CYP11B mRNA表达明显,提示醛固酮合成。我们诊断该病例有1)分泌皮质醇的右侧肾上腺皮质腺瘤,2)分泌醛固酮的左侧肾上腺皮质腺瘤,3)双侧肾上腺产生醛固酮的皮质微小结节,符合特发性肾上腺增生。我们回顾了已报道的病例,并讨论了肾上腺皮质微小结节(常与肾上腺皮质腺瘤相关)的意义。