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男性化卵巢肿瘤:一例 Sertoli-Leydig 细胞瘤和 Brenner 瘤合并病例。

Virilising ovarian tumour: a case associating a Sertoli-Leydig cell tumour and a Brenner tumour.

机构信息

Service d'Endocrinologie et Maladies Métaboliques, Centre Hospitalier Universitaire Larrey, Toulouse, France.

出版信息

Gynecol Endocrinol. 2011 May;27(5):345-50. doi: 10.3109/09513590.2010.492883. Epub 2010 Jun 23.

Abstract

Ovarian Sertoli-Leydig cell tumours (SLCT), also termed arrhenoblastomas, are the most frequent virilising tumours in women of reproductive age. Very rare secretory Brenner tumours (BT) have been described, generally after the menopause. A 31-year-old woman sought medical advice for secondary amenorrhoea, progressive hirsutism and a 5-year history of virilisation syndrome with clitoromegaly. Testosterone was markedly high (285 ng/dl, N<85) with moderate elevation of delta 4-androstenedione (D4AD) (311 ng/dl, N <270), dehydroepiandrosterone sulfate (DHEAS) (366 μg/dl, N <340) and 17-hydroxyprogesterone (17OHP) (275 ng/dl). LH was 9 IU/l, FSH 4.3 IU/l, estradiol 60 pg/ml and progesterone 314 ng/100 ml. Cortisol was decreased (1.3 μg/dl) after the dexamethasone suppression test. Pelvic MRI showed a 5-cm right ovarian tumour with a 2.5 cm nodular component and cystic areas, and two nodules measuring 11 mm and 15 mm above the right and left ovaries. After right ovariectomy by laparoscopy, pathological examination concluded on a 3-cm SLCT and a 2-cm BT; the nodules above the ovaries were dysembryoplastic cysts. Postoperatively, testosterone level was normal after 24 h (26 ng/dl), estradiol and progesterone rapidly decreased, cyclic secretion then resumed and the patient menstruated at day 27. To our knowledge, this is the first report of an ovarian tumour associating a Sertoli-Leydig cell tumour and a Brenner tumour in a patient with virilisation syndrome which resolved after ovariectomy.

摘要

卵巢支持-间质细胞瘤(SLCT),也称为间质细胞瘤,是生育年龄妇女中最常见的男性化肿瘤。非常罕见的分泌性 Brenner 肿瘤(BT)已被描述,通常在绝经后。一名 31 岁女性因继发性闭经、进行性多毛症和 5 年的男性化综合征就诊,伴有阴蒂肥大。睾酮明显升高(285ng/dl,N<85),伴有 delta 4-雄烯二酮(D4AD)(311ng/dl,N<270)、脱氢表雄酮硫酸酯(DHEAS)(366μg/dl,N<340)和 17-羟孕酮(17OHP)(275ng/dl)中度升高。LH 为 9IU/l,FSH 为 4.3IU/l,雌二醇为 60pg/ml,孕酮为 314ng/100ml。地塞米松抑制试验后皮质醇降低(1.3μg/dl)。盆腔 MRI 显示右侧卵巢 5cm 肿瘤,有 2.5cm 结节性成分和囊性区域,以及右卵巢和左卵巢上方 11mm 和 15mm 的两个结节。腹腔镜右卵巢切除术术后,病理检查诊断为 3cm SLCT 和 2cm BT;卵巢上方的结节为发育不良性囊肿。术后 24 小时,睾酮水平正常(26ng/dl),雌二醇和孕酮迅速下降,随后恢复周期性分泌,患者在第 27 天月经来潮。据我们所知,这是首例报道卵巢肿瘤合并支持-间质细胞瘤和 Brenner 肿瘤的患者,男性化综合征在卵巢切除术后得到缓解。

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