卵巢肿物合并第二性征发育异常患者的临床特点分析  

Clinical Characterization of Patients with Ovarian Mass Combined with Dysplasia of Secondary Sexual Characteristics

作　　者：刘倩[1] 周慧梅[1] 杨佳欣[1] 曹冬焱[1] 沈铿[1] 郎景和[1] LIU Qian;ZHOU Huimei;YANG Jiaxin;CAO Dongyan;SHEN Keng;LANG Jinghe(Department of Obstetrics and Gynecology,National Clinical Research Center for Obstetric and Gynecologic Diseases,PUMC Hospital,CAMS and PUMC,Beijing 100730,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院妇产科,国家妇产疾病临床研究中心,北京100730

出　　处：《中国医学科学院学报》2022年第1期17-23,共7页Acta Academiae Medicinae Sinicae

基　　金：中国医学科学院医学与健康科技创新工程项目(2017-I2M-1-002)。

摘　　要：目的探讨卵巢肿物合并第二性征发育异常的青春期前及青春期女性患者的临床特点。方法检索2012年1月至2019年11月北京协和医院收治的0~19岁卵巢肿物合并第二性征发育异常病例,回顾性分析其临床特点、手术方式、术后病理、治疗及预后,总结其诊疗思路。结果卵巢肿物合并第二性征发育异常患者共18例。7例(7/18,38.9%)青春期前出现第二性征发育,其中5例表现为性早熟(幼年型颗粒细胞瘤2例,性腺母细胞瘤、卵巢滤泡囊肿、46,XY单纯性腺发育不良合并无性细胞瘤各1例),2例出现男性化表现(类固醇细胞瘤、硬化性间质瘤各1例);11例(11/18,61.1%)青春期出现第二性征发育异常,其中8例月经初潮后出现男性化表现或月经异常(性索间质肿瘤7例,囊性颗粒细胞瘤1例),2例原发闭经(雄激素不敏感综合征合并睾丸支持细胞瘤1例,子宫内膜异位囊肿合并生殖道畸形1例),1例青春期无第二性征发育(46,XX性腺发育不全合并性腺浆液性囊腺瘤)。结论青春期前第二性征过早出现、青春期第二性征发育异常(发育不全或发育欠佳)和/或月经失调时应积极检测性激素水平,同时行影像学检查排除卵巢器质性病变,必要时行染色体核型分析。青春期前及青春期女性出现相关症状时卵巢肿物诊断首先警惕性索间质肿瘤,青春期原发闭经者需积极排除是否合并生殖道畸形,青春期原发闭经和/或无第二性征发育者需行染色体检查排除性腺发育异常。Objective To investigate the clinical characteristics of preadolescent and adolescent female patients with ovarian mass combined with dysplasia of secondary sexual characteristics.Methods This study retrospectively analyzed 18 cases of ovarian mass combined with dysplasia of secondary sexual characteristics aged 0-19 years admitted to Peking Union Medical College Hospital from January 2012 to November 2019.By analyzing the clinical manifestations,surgical methods,postoperative pathology,therapies and prognosis of the cases,we summarized the diagnosis and treatment ideas.Results Among the 18 cases,7(7/18,38.9%)developed secondary sex signs before puberty,including 5 cases showing precocity(including 2 cases of juvenile granulosa cell tumor,1 case of gonadoblastoma,1 case of ovarian follicular cyst,and 1 case of 46,XY simple gonadal dysplasia combined with dysgerminoma)and 2 cases presenting masculine manifestations(1 case of steroid cell tumor and 1 case of sclerosing stromal tumor).The rest 11(11/18,61.1%)cases showed abnormal development of secondary sexual characteristics during puberty,including 8 cases with masculine manifestations or abnormal menstruation after menarche(7 cases with sex cord stromal cell tumor and 1 case with cystic granulosa cell tumor),2 cases with primary amenorrhea(1 case with androgen insensitivity syndrome combined with testicular sertoli cell tumor and 1 case with endometriosis cyst combined with reproductive tract malformation),and 1 case diagnosed as 46,XX gonadal dysplasia with serous cystadenoma and no secondary sexual development during puberty.Conclusions Sex hormone levels should be actively tested in the case of prepubertal secondary sexual characteristics appearing early,pubertal secondary sexual characteristics being abnormal(underdevelopment),and/or menstrual abnormalities.Imaging examination should be performed to exclude ovarian organic lesions,and chromosome karyotype analysis should be performed if necessary.The diagnosis of ovarian mass in preadolescent and adolescent

关 键 词：卵巢肿物 生殖道畸形 性腺发育异常 第二性征 

分 类 号：R711[医药卫生—妇产科学]