Klotz R K, Müller-Holzner E, Fessler S, Reimer D U, Zervomanolakis I, Seeber B, Mattle V, Wildt L
Department of Obstetrics and Gynecology, Innsbruck Medical University, Innsbruck, Austria.
Exp Clin Endocrinol Diabetes. 2010 May;118(5):291-7. doi: 10.1055/s-0029-1225351. Epub 2010 Mar 2.
Progressive hirsutism can be a symptom of an androgen-producing tumor, especially in postmenopausal women. We report a case of a 58-year-old woman who complained of progressive hirsutism, nervousness, irritability, anxiousness and an increased libido. Examination showed an unusual redness of her head, décolleté, palms and soles of her feet. Basal laboratory tests revealed a profound elevation of testosterone levels (7.5 microg/l) and normal levels of androstendione, dehydroepiandrosterone-sulfate, 17alpha-hydroxy-progesterone and thyroid-stimulating hormone. Also remarkable was that her red blood count, hemoglobin and hematocrit values were elevated while erythropoietin was within normal limits. Functional laboratory tests ruled out heterozygous C21-hydroxylase deficiency and showed a moderate insulin resistance on the oral glucose tolerance test. Transvaginal ultrasound revealed a slightly hyperdensic area of 6 mm in the left ovary. Magnetic resonance imaging showed a contrast medium-accumulating area of 2 cm in the left ovary. Since the patient was initially reluctant to undergo surgery, a GnRH-analogue (triptoreline) was administered VIA intramuscular injection once per month for two months and testosterone levels were lowered to less than one third of the initial level (2 microg/l). Surgery was eventually performed with laparoscopic bilateral salpingoophorectomy, hysteroscopy and uterine curettage. The histologic examination revealed a Leydig cell tumor in the hilus and stroma of the left ovary. Postoperatively testosterone levels dropped dramatically and instantly into the normal range. Within months, the red blood count and hematocrit levels were within normal limits. The patient's face became more feminine, the redness of her face and hirsutism regressed. Her anxiousness and nervosity resolved and the insulin sensitivity improved. In this paper, polyglobulia, the metabolic and psychological changes due to hyperandrogenism are discussed, as well as the phenomenon that the tumor responded to a GnRH-analogue. Such a response implies that the tumor is either under gonadotropin control or that GnRH analogues have direct effects via receptors on tumorous Leydig cells.
进行性多毛症可能是雄激素分泌性肿瘤的症状，尤其在绝经后女性中。我们报告一例58岁女性，她主诉进行性多毛症、紧张、易怒、焦虑和性欲增强。检查发现她的头部、颈部、手掌和脚底有异常发红。基础实验室检查显示睾酮水平显著升高（7.5微克/升），而雄烯二酮、硫酸脱氢表雄酮、17α-羟孕酮和促甲状腺激素水平正常。同样值得注意的是，她的红细胞计数、血红蛋白和血细胞比容值升高，而促红细胞生成素在正常范围内。功能性实验室检查排除了杂合子C21-羟化酶缺乏症，口服葡萄糖耐量试验显示中度胰岛素抵抗。经阴道超声显示左卵巢有一个6毫米的稍高密度区。磁共振成像显示左卵巢有一个2厘米的造影剂聚集区。由于患者最初不愿接受手术，每月通过肌肉注射给予一次促性腺激素释放激素类似物（曲普瑞林），共两个月，睾酮水平降至初始水平的三分之一以下（2微克/升）。最终进行了腹腔镜双侧输卵管卵巢切除术、宫腔镜检查和刮宫术。组织学检查显示左卵巢门部和间质有一个Leydig细胞瘤。术后睾酮水平立即大幅下降至正常范围。数月内，红细胞计数和血细胞比容水平恢复正常。患者的面部变得更具女性化，面部发红和多毛症消退。她的焦虑和紧张情绪缓解，胰岛素敏感性提高。本文讨论了红细胞增多症、高雄激素血症引起的代谢和心理变化，以及肿瘤对促性腺激素释放激素类似物的反应现象。这种反应意味着肿瘤要么受促性腺激素控制，要么促性腺激素释放激素类似物通过肿瘤Leydig细胞上的受体产生直接作用。
