Testicular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue
Testicular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue
复制标题
DOI：
10.1210/jc.2007-0337
复制
发表时间：
2007-09-01
影响因子：
5.8
通讯作者：
Hermus, Ad R. M. M.
中科院分区：
文献类型：
--
作者：
Claahsen-van der Grinten, Hedi L.;Otten, Barto J.;Hermus, Ad R. M. M.
背景：在患有先天性肾上腺增生 (CAH) 的男性患者中，经常发现睾丸肾上腺剩余肿瘤可能会干扰性腺功能。目的：我们的目的是确定睾丸肾上腺剩余肿瘤产生类固醇的特征。设计和背景：该研究是描述性的，并在大学医疗中心进行。患者：8 名患有双侧睾丸肾上腺剩余肿瘤的成人 CAH 患者接受了睾丸保留手术治疗。一名患者在手术期间对精索静脉进行插管，并收集血样以测量肾上腺特异性类固醇 21-脱氧皮质醇 (21DF) 和 17-羟基孕酮 (17OHP) 和雄烯二酮 (A)。在同时采集的外周血中测量相同的参数。测量肿瘤组织中肾上腺特异性酶 CYP11B1 和 CYP11B2 以及 ACTH 和血管紧张素 II (AII) 受体的 mRNA 浓度。主要结果指标：评估肾上腺特异性类固醇/酶。结果：所有精索静脉样本中均可检测到 21DF、17OHP 和 A 水平。精索静脉与同时采集的外周血样本之间的比率（平均值+/-SD）为37.8+/-56.3（21DF）、132.0+/-249（17OHP）和57.0+/-68.2（A）。所有肿瘤中均检测到 CYP11B1、CYP11B2、ACTH 和 AII 受体 mRNA，肿瘤中 ACTH 受体 mRNA 与外周血中 21DF（r = 0.85；P = 0.015）、17OHP（r = 1；P = 0.01）和 A（r = 0.89；P = 0.007）浓度呈强相关。结论：睾丸肾上腺其余肿瘤产生肾上腺特异性类固醇并表达肾上腺特异性酶以及 ACTH 和 AII 受体，证实与肾上腺组织高度相似。由于肿瘤组织中存在 AII 受体，因此可以推测 AII 可能是导致睾丸肾上腺剩余肿瘤生长的另一个因素。
Context: In male patients with congenital adrenal hyperplasia (CAH), testicular adrenal rest tumors are frequently found that may interfere with gonadal function.Objective: Our objective was to determine steroid-producing features of testicular adrenal rest tumors.Design and Setting: The study is descriptive and took place at a university medical center.Patients: Eight adult CAH patients with bilateral testicular adrenal rest tumors were treated with testis-sparing surgery.Interventions: In all but one patient, spermatic veins were cannulated during surgery and blood samples collected to measure the adrenal-specific steroid 21-deoxycortisol (21DF) and 17-hydroxyprogesterone (17OHP) and androstenedione ( A). The same parameters were measured in simultaneously taken peripheral blood. mRNA concentrations of adrenal-specific enzymes CYP11B1 and CYP11B2 and ACTH and angiotensin II ( AII) receptors were measured in tumor tissue.Main Outcome Measures: Adrenal-specific steroids/ enzymes were assessed.Results: 21DF, 17OHP, and A levels were measurable in all spermatic vein samples. The ratio ( mean +/- SD) between spermatic vein and simultaneously taken peripheral blood samples was 37.8 +/- 56.3 ( 21DF), 132.0 +/- 249 ( 17OHP), and 57.0 +/- 68.2 ( A). CYP11B1, CYP11B2, and ACTH and AII receptor mRNAs were detected in all tumors with a strong correlation between ACTH receptor mRNA in tumors and 21DF (r = 0.85; P = 0.015), 17OHP ( r = 1; P = 0.01) and A ( r = 0.89; P = 0.007) concentrations in peripheral blood.Conclusion: Testicular adrenal rest tumors produce adrenal-specific steroids and express adrenal-specific enzymes and ACTH and AII receptors, confirming the strong resemblance with adrenal tissue. Because AII receptors are present in tumor tissue, it can be hypothesized that AII may be an additional factor responsible for testicular adrenal rest tumor growth.