Swyer综合征合并一侧卵巢无性细胞瘤和性腺母细胞瘤一例诊治失误分析  

Swyer syndrome with ovary gonadoblastoma and disgerminoma:report of a case and review of the literature

作　　者：奚文裕[1] 李金萍[1,2] 李爱斌[3] 

机构地区：[1]武汉大学第一临床学院,2009级硕士430060 [2]天津市第一中心医院 [3]武汉大学人民医院生殖中心

出　　处：《中华临床医师杂志（电子版）》2011年第13期3786-3789,共4页Chinese Journal of Clinicians(Electronic Edition)

摘　　要：目的通过对1例Swyer综合征合并卵巢无性细胞瘤和性腺母细胞瘤误诊误治的回顾性分析,总结经验教训以提高临床上对该病的认识和诊治水平。方法对1例17岁女性因腹部包块就诊的病历的诊治经过进行回顾性分析,讨论诊治失误及其主要原因,并对Swyer综合征的发病机理及其研究进展、临床特点和如何提高诊治水平进行阐述。结果患者为女性表型,第二性征发育欠佳,术后病检示卵巢无性细胞瘤和性腺母细胞瘤,染色体核型为46XY。结论 Swyer综合征患者因其内外生殖器均为女性,故早期就诊较少。其条索状性腺恶变率高,临床上一经诊断,需尽早切除发育不良的性腺。Objective This report was to review a case of Swyer syndrome and discuss its differential diagnosis,treatment and prognosis.Methods A case of a 17 year old woman with abdominal mass who had been given a exploratory laparotomy was reported and discussed.The pathogenesis,dinical features,diagnosis and treatment of Swyer syndrome were analysed.Results As the patient's karyotype was 46,XY,with her secondary sexual characters merely developed and the pathological section,the diagnosis was made.Conclusions Patients with Swyer syndrome have a high risk of neoplastic transformation.When the diagnosis has been made,the dysplastic gonad should be removed as early as possible.

关 键 词：性腺发育不全 46 XY 性腺母细胞瘤 无性细胞瘤 诊断 治疗 

分 类 号：R737.31[医药卫生—肿瘤]