Department of Endocrinology and Metabolism, Medizinische Klinik-Innenstadt, University Hospital Munich, D-80336 Munich, Germany.
J Clin Endocrinol Metab. 2010 May;95(5):2065-72. doi: 10.1210/jc.2009-1929. Epub 2010 Feb 26.
Patients with 21-hydroxylase deficiency (21-OHD) have been shown to develop adrenal adenomas and, in males, testicular adrenal rest tumors (TARTs) at a high percentage.
The aim of this study was to evaluate the interrelation of adrenal masses and TARTs as well as factors stimulating tumor growth of orthotopic and ectopic adrenal tissue in 21-OHD.
In a cross-sectional study, 26 adult male patients with classic 21-OHD (15 salt wasting, 11 simple virilizing; age range, 18-48 yr) were clinically assessed according to their hormonal control. Magnetic resonance imaging of the adrenals (26 of 26) and of the testes (18 of 26) was performed. Adrenal size and morphology was compared to 26 age-matched controls.
Combined adrenal volume of 21-OHD patients was significantly higher (median, 9.3 ml; range, 3.2-124.5 ml) in comparison to controls (median, 7.4 ml; range, 5.5-10.8 ml; P = 0.005). Morphologically, adrenals were classified as normal without nodules in 27% of 21-OHD patients compared to 69% of controls. None of the controls, but 42% of 21-OHD patients had an overall adrenal volume higher than 11 ml. Ten of 18 patients had TARTs with a median volume of 3.3 ml (range, 0.4-21.6 ml). Total adrenal volume and tumor size but not TART volume correlated positively with current parameters of hormonal control (androstenedione, morning 17-OHP in serum, pregnanetriol in 24-h urine; P < 0.001 for each). Baseline ACTH was independent of adrenal and TART volume. There was no correlation of total adrenal or adrenal tumor size with TART volume.
These data provide indirect evidence that different factors regulate the growth of orthotopic adrenal tissue and ectopic adrenal remnants in TARTs.
已有研究表明，21-羟化酶缺乏症（21-OHD）患者会发生肾上腺腺瘤，且男性患者会发生睾丸肾上腺残迹瘤（TART）。
本研究旨在评估 21-OHD 患者中，肾上腺肿块和 TART 之间的关系，以及刺激原位和异位肾上腺组织肿瘤生长的因素。
在一项横断面研究中，根据激素控制情况，对 26 例成年男性经典 21-OHD 患者（15 例盐耗竭，11 例单纯性男性化；年龄范围 18-48 岁）进行临床评估。对 26 例患者的肾上腺（26/26）和睾丸（26/26）进行磁共振成像检查。将肾上腺大小和形态与 26 名年龄匹配的对照组进行比较。
21-OHD 患者的联合肾上腺体积明显高于对照组（中位数 9.3 ml；范围 3.2-124.5 ml 比中位数 7.4 ml；范围 5.5-10.8 ml；P = 0.005）。形态学上，27%的 21-OHD 患者肾上腺无结节，而对照组为 69%。对照组中无一例患者的总体肾上腺体积大于 11 ml，而 21-OHD 患者中有 42%的患者存在这种情况。18 例患者中有 10 例存在 TART，其体积中位数为 3.3 ml（范围 0.4-21.6 ml）。总的肾上腺体积和肿瘤大小与当前的激素控制参数呈正相关（血清中雄烯二酮、清晨 17-羟孕酮、24 小时尿中孕三醇；每项 P < 0.001）。基础 ACTH 与肾上腺和 TART 体积无关。总的肾上腺或肾上腺肿瘤大小与 TART 体积之间无相关性。
这些数据提供了间接证据，表明不同的因素调节原位肾上腺组织和 TART 中异位肾上腺残迹的生长。
