Phornphutkul C, Okubo T, Wu K, Harel Z, Tracy T F, Pinar H, Chen S, Gruppuso P A, Goodwin G
Department of Pediatrics, Brown University and Rhode Island Hospital, Providence, Rhode Island 02903, USA.
J Clin Endocrinol Metab. 2001 Feb;86(2):649-52. doi: 10.1210/jcem.86.2.7201.
A 7-yr-old girl presented with isosexual precocious puberty secondary to a feminizing adrenal adenoma. The adrenal tumor was found to express aromatase messenger ribonucleic acid. Enzyme kinetic studies revealed a high level of aromatase activity in the adrenal tumor, with a K(m) of 45 nmol/L and a maximum velocity of 25.6 pmol/mg.h. Aromatase activity was approximately 500-fold higher in the tumor than in adjacent normal adrenal tissue. Although histopathological examination of the tumor was most consistent with a benign adenoma, the aromatase transcripts present in the tumor corresponded to those previously associated with malignant as well as benign tumors. We consider the pattern of aromatase expression sufficient to warrant continued follow-up for tumor recurrence. Our case demonstrates that isosexual precocious puberty secondary to a feminizing adrenal tumor can be due to estrogen synthesis from the tumor itself rather than peripheral aromatization as had been previously theorized.
一名7岁女孩因女性化肾上腺腺瘤继发同性性早熟。发现肾上腺肿瘤表达芳香化酶信使核糖核酸。酶动力学研究显示肾上腺肿瘤中芳香化酶活性水平较高,米氏常数(K(m))为45 nmol/L,最大反应速度为25.6 pmol/mg·h。肿瘤中的芳香化酶活性比相邻正常肾上腺组织高约500倍。尽管肿瘤的组织病理学检查与良性腺瘤最为相符,但肿瘤中存在的芳香化酶转录本与先前与恶性及良性肿瘤相关的转录本相对应。我们认为芳香化酶的表达模式足以保证对肿瘤复发进行持续随访。我们的病例表明,女性化肾上腺肿瘤继发的同性性早熟可能是由于肿瘤自身合成雌激素,而非如先前理论所认为的外周芳香化作用。