Rivera-Arkoncel Maria Luisa Cecilia, Pacquing-Songco Debby, Lantion-Ang Frances Lina
Section of Endocrinology, Diabetes and Metabolism, Department of Medicine, Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.
BMJ Case Rep. 2010 Dec 14;2010:bcr0720103139. doi: 10.1136/bcr.07.2010.3139.
Androgen secreting tumours are the least commonly encountered androgen excess disorders, having a prevalence of 0.2%. Androblastomas of the ovary comprise less than 0.5% of all ovarian tumours. Pure Leydig cell tumours are very rare and almost always show secretion of male sex hormones. A 41-year-old multipara Filipino woman presented with a 2-year history of amenorrhoea and virilisation characterised by hirsutism, androgenic alopecia, masculine habitus and clitoromegaly. Diagnostic evaluation showed markedly elevated serum testosterone and normal dehydroepiandrosterone sulfate. Normal ovaries were seen on initial transvaginal ultrasound. A low dose dexamethasone suppression test suggested an ovarian source. A left adrenal nodule was seen on CT scan. Doppler transvaginal ultrasound revealed a solid lobulated structure in the right ovary. The patient underwent surgery and histopathology showed a Leydig cell tumour, hilar type. Serum testosterone levels normalised 3 days after surgery. Specific clinical and biochemical investigation of androgen secreting neoplasms is very important for correct diagnosis of these rare tumours.
分泌雄激素的肿瘤是最不常见的雄激素过多症,患病率为0.2%。卵巢的成男性细胞瘤占所有卵巢肿瘤的比例不到0.5%。单纯的睾丸间质细胞瘤非常罕见,几乎总是表现出男性性激素分泌。一名41岁的菲律宾经产妇,有2年闭经和男性化病史,表现为多毛、雄激素性脱发、男性体型和阴蒂肥大。诊断评估显示血清睾酮显著升高,硫酸脱氢表雄酮正常。初次经阴道超声检查可见双侧卵巢正常。低剂量地塞米松抑制试验提示为卵巢来源。CT扫描发现左侧肾上腺有一个结节。经阴道多普勒超声显示右侧卵巢有一个实性分叶状结构。患者接受了手术,组织病理学显示为睾丸间质细胞瘤,门部型。术后3天血清睾酮水平恢复正常。对分泌雄激素的肿瘤进行特异性临床和生化检查对于正确诊断这些罕见肿瘤非常重要。