Claahsen-van der Grinten Hedi L, Otten Barto J, Sweep Fred C G J, Span Paul N, Ross H Alec, Meuleman Eric J H, Hermus Ad R M M
Radboud University Nijmegen Medical Centre, Department of Paediatric Endocrinology (833), PO Box 9101, 6500 HB Nijmegen, The Netherlands.
J Clin Endocrinol Metab. 2007 Sep;92(9):3674-80. doi: 10.1210/jc.2007-0337. Epub 2007 Jun 26.
In male patients with congenital adrenal hyperplasia (CAH), testicular adrenal rest tumors are frequently found that may interfere with gonadal function.
Our objective was to determine steroid-producing features of testicular adrenal rest tumors.
The study is descriptive and took place at a university medical center.
Eight adult CAH patients with bilateral testicular adrenal rest tumors were treated with testis-sparing surgery.
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.
Adrenal-specific steroids/enzymes were assessed.
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.
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.
在先天性肾上腺皮质增生症(CAH)男性患者中,常发现睾丸肾上腺残余瘤,其可能干扰性腺功能。
我们的目的是确定睾丸肾上腺残余瘤的类固醇生成特征。
本研究为描述性研究,在一所大学医学中心进行。
8例患有双侧睾丸肾上腺残余瘤的成年CAH患者接受了保留睾丸手术。
除1例患者外,所有患者在手术期间均进行精索静脉插管,并采集血样以测量肾上腺特异性类固醇21-脱氧皮质醇(21DF)、17-羟孕酮(17OHP)和雄烯二酮(A)。同时采集外周血测量相同参数。在肿瘤组织中测量肾上腺特异性酶CYP11B1和CYP11B2以及促肾上腺皮质激素(ACTH)和血管紧张素II(AII)受体的mRNA浓度。
评估肾上腺特异性类固醇/酶。
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±标准差)分别为37.8±56.3(21DF)、132.0±249(17OHP)和57.0±68.2(A)。在所有肿瘤中均检测到CYP11B1、CYP11B2以及ACTH和AII受体的mRNA,肿瘤中的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)浓度之间存在强相关性。
睾丸肾上腺残余瘤产生肾上腺特异性类固醇,表达肾上腺特异性酶以及ACTH和AII受体,证实其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残余瘤生长的另一个因素。