Del Monte P, Bernasconi D, Bertolazzi L, Meozzi M, Badaracco B, Torre R, Marugo M
Department of Endocrinology, Ospedali Galliera, Genova, Italy.
Clin Endocrinol (Oxf). 1995 Mar;42(3):273-7. doi: 10.1111/j.1365-2265.1995.tb01875.x.
Recent studies suggest a possible connection between silent adrenal nodules and mild forms of 21-hydroxylase deficiency. It remains unclear whether the enzymatic deficiency is generalized or intrinsic to the adrenal mass. To help to clarify this, we have studied 17 alpha-OH-progesterone (17OH-P) response to ACTH stimulation in a group of patients with adrenal 'incidentaloma' in comparison with normal subjects. In patients who underwent surgical treatment, the test was repeated to evaluate possible modifications in 17OH-P behaviour after resection of the adrenal mass.
Fifteen subjects with incidentally discovered asymptomatic adrenal masses were studied. Basal hormone evaluations were normal, with normal cortisol suppression after low-dose dexamethasone. Iodocholesterol scanning, performed in 12 patients, showed normal bilateral adrenal uptake in 2 subjects and an increased uptake on the side of the lesion in 10 subjects. In every patient, ACTH stimulation was performed to evaluate the secretory response of cortisol, 17OH-P, progesterone and dehydroepiandrosterone sulphate. An identical test was performed in 10 control subjects with normal adrenal glands, matched for age and sex. In six patients with an adrenal lesion > 3.5 cm, the ACTH stimulation test was repeated one month after surgery.
The 17OH-P response to ACTH stimulation was significantly higher in subjects with adrenal 'incidentaloma' than in controls (P < 0.01). In particular, 10 subjects out of 15 (66%) evidenced a 17OH-P peak > 18 nmol/l at 60 minutes. No differences were seen in baseline 17OH-P or cortisol levels or in cortisol response to ACTH between the two groups. Dehydroepiandrosterone sulphate concentrations were significantly lower in patients with adrenal 'incidentaloma' than in normals. In six patients who had an increased 17OH-P response to ACTH on initial evaluation, the ACTH test was repeated one month after surgery. In five of these patients, 17OH-P response to ACTH was clearly reduced, suggesting that in these cases the enzymatic defect was restricted to the adenoma. In the other patient, however, stimulated 17OH-P levels remained unchanged. In this case, therefore, all of the adrenal tissue seems to be involved, suggesting a late-onset 21-hydroxylase deficiency. No significant modifications in cortisol response to ACTH were observed.
It seems therefore that in some cases of incidentaloma the steroidogenic enzymatic defect may be secondary to the adenomatous proliferation, while in others such defects may induce the development of silent adrenal nodules.
近期研究表明，无症状肾上腺结节与轻度21-羟化酶缺乏之间可能存在联系。目前尚不清楚酶缺乏是全身性的还是肾上腺肿块所特有的。为了阐明这一点，我们研究了一组肾上腺“偶发瘤”患者与正常受试者相比，促肾上腺皮质激素（ACTH）刺激后17α-羟孕酮（17OH-P）的反应。对于接受手术治疗的患者，术后重复该测试以评估切除肾上腺肿块后17OH-P行为的可能变化。
研究了15例偶然发现的无症状肾上腺肿块患者。基础激素评估正常，低剂量地塞米松后皮质醇抑制正常。12例患者进行了碘胆固醇扫描，其中2例双侧肾上腺摄取正常，10例病变侧摄取增加。对每位患者进行ACTH刺激试验，以评估皮质醇、17OH-P、孕酮和硫酸脱氢表雄酮的分泌反应。对10例年龄和性别匹配的肾上腺正常的对照受试者进行了相同的测试。对6例肾上腺病变>3.5 cm的患者，术后1个月重复ACTH刺激试验。
肾上腺“偶发瘤”患者对ACTH刺激的17OH-P反应显著高于对照组（P<0.01）。特别是，15例患者中有10例（66%）在60分钟时17OH-P峰值>18 nmol/L。两组之间的基线17OH-P或皮质醇水平以及皮质醇对ACTH的反应无差异。肾上腺“偶发瘤”患者的硫酸脱氢表雄酮浓度显著低于正常人。在初始评估时对ACTH的17OH-P反应增加的6例患者中，术后1个月重复ACTH试验。其中有5例患者对ACTH的17OH-P反应明显降低，表明在这些病例中酶缺陷仅限于腺瘤。然而，在另一例患者中，刺激后的17OH-P水平保持不变。因此，在这种情况下，似乎所有肾上腺组织均受累，提示为迟发性21-羟化酶缺乏。未观察到皮质醇对ACTH反应的显著变化。
因此，在某些偶发瘤病例中，类固醇生成酶缺陷可能继发于腺瘤性增殖，而在其他病例中，此类缺陷可能诱发无症状肾上腺结节的发生。
