Close examination of steroidogenesis disorders in a DOC- and progesterone-producing adrenocortical carcinoma
Close examination of steroidogenesis disorders in a DOC- and progesterone-producing adrenocortical carcinoma
复制标题
仔细检查产生 DOC 和孕激素的肾上腺皮质癌中的类固醇生成障碍
DOI：
10.1007/s12020-008-9123-5
复制
发表时间：
2009
期刊：
影响因子：
3.7
通讯作者：
K. Nakao
中科院分区：
文献类型：
--
作者：
Masakatsu Sone;H. Shibata;K. Homma;N. Tamura;J. Akahira;S. Hamada;M. Yahata;N. Fukui;H. Itoh;H. Sasano;K. Nakao
我们报告一个肾上腺皮质癌合并高血压、低血钾及闭经的病例。一位27岁的女性因为左侧肾上腺意外瘤而住进我院。她表现为高血压、低钾血症和闭经;她的血浆肾素活性低，但血浆醛固酮浓度正常，皮质醇和雄激素也正常。相比之下，她的血清脱氧皮质酮（DOC）、18-羟基脱氧皮质酮和孕酮浓度较高，她的尿类固醇谱显示17-脱氧类固醇和11-脱氧类固醇（孕酮、DOC、11-脱氢皮质酮和11-脱氧皮质醇）和3 β-羟基5-烯类固醇（双烯醇酮、17-羟基双烯醇酮和DHEA）分泌升高。17-OH烯醇酮与17-OH烯醇酮、17-OH孕酮与孕酮、皮质酮与DOC、皮质醇与11-脱氧皮质醇、孕酮与17-OH烯醇酮、雄烯二酮与DHEA的代谢物比值降低，分别表明17 α-羟化酶、11 β-羟化酶和3 β-HSD活性受损。肿瘤切除后，所有肾上腺类固醇的水平均恢复正常。根据韦斯标准，该肿瘤被诊断为肾上腺皮质癌，类固醇生成酶的免疫组化分析显示肿瘤组织中类固醇生成紊乱。对于肾上腺皮质癌，肿瘤细胞内单个类固醇产生酶的异质性可导致各种类固醇中间体的分泌过多，即使类固醇终产物在正常范围内。
We report a case of hypertension, hypokalemia, and amenorrhea accompanying an adrenocortical carcinoma. A 27-year-old woman was admitted to our hospital because of a left adrenal incidentaloma. She presented with hypertension, hypokalemia, and amenorrhea; her plasma renin activity was low, but her plasma aldosterone concentration was normal, as were cortisol and androgens. By contrast, her serum concentrations of deoxycorticosterone (DOC), 18-hydroxydeoxycorticosterone, and progesterone were high, and her urinary steroid profile showed elevated secretion of 17-deoxysteroids and 11-deoxysteroids (progesterone, DOC, 11-dehydrocorticosterone, and 11-deoxycortisol), and 3β-hydroxy 5-en steroids (pregnenolone, 17-hydroxypregnenolone, and DHEA). Decreased ratios of metabolites of (1) 17-OHpregnenolone to pregnenolone and 17-OHprogesterone to progesterone, (2) corticosterone to DOC and cortisol to 11-deoxycortisol, and (3) progesterone to pregnenolone, 17-OHprogesterone to 17-OHpregnenolone and androstenedione to DHEA suggested the impairment of 17α-hydroxylase, 11β-hydroxylase, and 3β-HSD activities, respectively. After the tumor was removed, levels of all adrenal steroids were normalized. Based on the Weiss criteria, the tumor was diagnosed as an adrenocortical carcinoma, and immunohistochemical analysis of steroidogenic enzymes revealed disorganized steroidogenesis in the tumor tissue. With adrenocortical carcinomas, heterogeneity of individual steroid producing enzymes within tumor cells can lead to hypersecretion of various steroid intermediates, even when steroid end products are within the normal range.
影响因子：
2
作者：
Homma, K;Hasegawa, T;Matsuo, N
通讯作者：
Matsuo, N