Murakami Y, Sasaki M, Sasano H, Suzuki T, Kitamura N, Tomoi M, Yorifuji H, Koshimura K, Kato Y
Department of Medicine, Shimane Medical University, Izumo, Japan.
Endocr J. 1995 Apr;42(2):283-8. doi: 10.1507/endocrj.42.283.
A 28-year old female patient with virilization due to left adrenocortical adenoma was studied. The patient had clinical features of hyperandrogenism such as hirsutism and a low pitched voice, but not of hypercorticoidism. Plasma testosterone and dehydroepiandrosterone-sulfate (DHEA-S) were high. Although the basal plasma cortisol concentration and urinary excretion of 17-hydroxycorticosteroids (17-OHCS) were within the normal range, the absence of diurnal variation in plasma cortisol and loss of suppressibility by dexamethasone suggested constitutive secretion of cortisol by the tumor. Inappropriate cortisol secretion was also supported by blunted ACTH response to provocative stimuli. After successful removal of the left adrenal tumor, such endocrinological abnormalities were all normalized. Immunohistochemical analysis revealed that tumor cells were positively stained for C21 hydroxylase cytochrome P-450 (P-450C21) and P-450(11) beta which convert 17-hydroxy (OH) progesterone to cortisol as well as P-450SCC, 3 beta-hydroxysteroid dehydrogenase and P-450(17) alpha which are involved in testosterone biosynthesis. These findings suggest that adrenocortical adenoma secretes predominantly testosterone and constitutively cortisol in a young woman patient with virilization.
对一名因左肾上腺皮质腺瘤导致男性化的28岁女性患者进行了研究。该患者具有多毛症和嗓音低沉等高雄激素血症的临床特征，但无皮质醇增多症的表现。血浆睾酮和硫酸脱氢表雄酮（DHEA-S）水平升高。虽然基础血浆皮质醇浓度和17-羟皮质类固醇（17-OHCS）的尿排泄量在正常范围内，但血浆皮质醇缺乏昼夜变化以及地塞米松抑制试验结果阴性提示肿瘤持续分泌皮质醇。促肾上腺皮质激素（ACTH）对刺激的反应减弱也支持了皮质醇分泌异常。成功切除左肾上腺肿瘤后，这些内分泌异常均恢复正常。免疫组织化学分析显示，肿瘤细胞对将17-羟孕酮转化为皮质醇的细胞色素P-450 21羟化酶（P-450C21）和P-450（11）β呈阳性染色，同时对参与睾酮生物合成的细胞色素P-450侧链裂解酶（P-450SCC）、3β-羟类固醇脱氢酶和P-450（17）α也呈阳性染色。这些发现表明，在一名患有男性化的年轻女性患者中，肾上腺皮质腺瘤主要分泌睾酮并持续分泌皮质醇。
