分泌雄激素卵巢肿瘤25例临床分析

Testosterone-producing ovarian tumors:a clinical study of 25 cases

目的分析和总结分泌雄激素卵巢肿瘤的临床特征、病理类型、诊断和治疗。方法回顾性分析25例男性化体征卵巢肿瘤的临床症状、手术前后血清激素水平、病理类型、治疗和预后。结果(1)分泌雄激素卵巢肿瘤发病中位数年龄为27岁,出现男性化100%(25/25),月经稀发40%(10/25)、闭经68%(17/25),平均病程20个月。(2)血清激素水平:手术前后血清总睾酮(T)为16.9±6.8vs.1.0±1.6nmol/L,术前92%(12/13)促肾上腺皮质激素(ACTH)在正常范围,50%(8/16)17-α羟孕酮(17-αOHP)升高,22%(2/9)皮质醇(F)升高,100%(12/12)24h尿游离皮质醇在正常范围。地塞米松抑制试验,T均无抑制(9/9),ACTH兴奋试验均为阴性(5/5)。(3)病理特征:60%(15/25)病理类型为卵巢支持-间质细胞肿瘤,32%(8/25)为脂质细胞肿瘤。Ia期患者23例,Ic期2例。肿瘤直径平均为(4.8±2.8)cm,均为单侧,以实性为主,少数为囊性。(4)治疗及预后:22例年轻患者行保留生育手术,3例无生育要求者行肿瘤细胞减灭术。术后肿瘤分化不良者行辅助化疗(方案PVB或PEB)。失访3例,22例患者预后较好,术后2～6个月患者多毛及声音变粗症状均有不同程度好转,5例已完成生育。结论分泌雄激素卵巢肿瘤好发于生育年龄,主要表现为去女性化和明显男性化,病程短,血清总T值显著升高。该类肿瘤一般较小,多为早期肿瘤,以手术治疗为主,总的预后较好。

Objective： To study the clinical characteristics of testosterone-producing ovarian tumors. Method： Twenty-five patients with testosterone-producing ovarian tumors diagnosed pathologically were treated in Peking Union Medical College Hospital from Jan 1986 to Oct 2006. Clinical characteristics of these 25 cases were analyzed retrospectively. Results： （1）The median age of patients was 27 years. There were 96% （24/25） of menstrual disturbance and 100%（25/25） of virilism. （2）Endocrinological tests： Total serum testosterone before and after operation was 16.9±6.8 vs 1.0±1.6 nmol/L （P=0.000）. Initial investigation showed normal level of ACTH in 92% （12/13）of the patients, increased level of 17-αOHP in 50%（8/16） and increased cortisol （F） in 22% （2/9）. All patients（12/12） have normal 24-hours urinary free cortisol level. Dexamethasone suppression test and ACTH stimulating test suggested non-adrenocorticotrophic hormone dependency of the hyper-androgenic state. （3） Pathological features： Of all tumors, 60 % （15/25） were Sertoli-Leydig cell tumors and 32% （8/25）were lipid cell tumors. All tumors were unilateral and most of them were solid. There were 23 cases of stage Ⅰa and 2 cases of stage Ⅰc. The median size of tumors was 4.8 cm in diameter. （4）Treatment and prognosis： Twenty-two younger patients had conservative surgery with reservation of fertility. Three patients without need for further fertility had cyto-reductive surgery. Patients with poor tumor differentiation accepted adjuvant chemotherapy. After removal of ovarian tumors, symptoms of virilism were reduced and five patients have already had healthy children. Conclusions： Testosterone-producing ovarian tumors are often seen in reproductive age. They produce defeminization followed by virilism. The level of total serum testosterone is increased markedly. Owing to their relatively small size, early detection of these tumors may be difficult. Removal of tumors or unilateral salpingo-oophorectomy is preferable for the preservation of fertility. The prognosis of these tumors is satisfactory after treatment.