Leinonen P, Ranta T, Siegberg R, Pelkonen R, Heikkilä P, Kahri A
Third Department of Medicine, University of Helsinki, Finland.
Clin Endocrinol (Oxf). 1991 Jan;34(1):31-5. doi: 10.1111/j.1365-2265.1991.tb01732.x.
A 60-year-old woman was evaluated for persistently elevated serum testosterone concentrations after bilateral ovariectomy. Her serum cortisol, androstenedione, dehydroepiandrosterone sulphate and 17-hydroxyprogesterone levels were normal, and decreased after dexamethasone administration. Those of testosterone (17.8-18.4 nmol/l) were remarkably high (normal range 0.7-2.8 nmol/l), were not suppressed by dexamethasone, but clearly increased after hCG administration (up to 128 nmol/l). Computed tomography revealed an adenoma in the right adrenal gland and adrenal scintigraphy under dexamethasone suppression visualized this adenoma. A right adrenalectomy was performed. (1) The tumour was histologically and ultrastructurally adrenocortical adenoma of zona reticularis cell type. (2) The adenoma tissue contained hCG receptors (198 fmol/g). (3) During tissue culture both ACTH and hCG were capable of maintaining its testosterone production, which was attenuated with time without stimulation. (4) The adenoma tissue did not elaborate 21-hydroxylated steroids in contrast to normal adrenal tissue. Thus the aberrant endocrine behaviour of this gonadotrophin-responsive testosterone-secreting adenoma of adrenal zona reticularis cell origin can be explained by ectopic functional hCG receptors and the lack of 21-hydroxylase activity.
一名60岁女性在双侧卵巢切除术后因血清睾酮浓度持续升高而接受评估。她的血清皮质醇、雄烯二酮、硫酸脱氢表雄酮和17-羟孕酮水平正常，地塞米松给药后降低。睾酮水平（17.8 - 18.4 nmol/l）显著升高（正常范围0.7 - 2.8 nmol/l），不受地塞米松抑制，但在人绒毛膜促性腺激素（hCG）给药后明显升高（高达128 nmol/l）。计算机断层扫描显示右肾上腺有一个腺瘤，地塞米松抑制下的肾上腺闪烁显像显示了这个腺瘤。进行了右肾上腺切除术。（1）肿瘤在组织学和超微结构上为网状带细胞型肾上腺皮质腺瘤。（2）腺瘤组织含有hCG受体（198 fmol/g）。（3）在组织培养过程中，促肾上腺皮质激素（ACTH）和hCG都能够维持其睾酮分泌，在无刺激的情况下，睾酮分泌随时间减弱。（4）与正常肾上腺组织不同，腺瘤组织不产生21-羟化类固醇。因此，这种起源于肾上腺网状带细胞的促性腺激素反应性睾酮分泌腺瘤的异常内分泌行为可以通过异位功能性hCG受体和缺乏21-羟化酶活性来解释。
