黑斑息肉综合征合并多发妇科肿瘤1例及文献复习  

Peutz-Jeghers syndrome with multiple gynecological tumors:a case report with a review of literatures

作　　者：杨心运 朱琳玲[1] 王文文[1] 梁云[1] 谢臻蔚[1] 金帆[1] YANG Xin-yun;ZHU Lin-ling;WANG Wen-wen;LIANG Yun;XIE Zhen-wei;JIN Fan(Women＇s Hospital,School of Medicine,Zhejiang University,Hangzhou 310006,China)

机构地区：[1]浙江大学医学院附属妇产科医院,浙江杭州310006

出　　处：《中国实用妇科与产科杂志》2018年第8期931-937,共7页Chinese Journal of Practical Gynecology and Obstetrics

基　　金：浙江省医药卫生科技计划项目(2014KYA247)

摘　　要：黑斑息肉综合征(Peutz-Jeghers syndrome,PJS)女性患者常伴发多种妇科肿瘤,且肿瘤类型较为罕见,常在临床中出现误诊漏诊。文章报道1例PJS患者以腹胀及白带增多为临床表现,查体及辅助检查提示盆腹腔巨大包块、宫颈膨大坚硬,最终确诊同时合并宫颈微偏腺癌、卵巢环小管性索间质细胞瘤和黏液性囊腺癌。另外,文章对PJS患者合并妇科肿瘤的文献进行回顾性总结,对PJS女性患者合并多发妇科肿瘤的病例临床特征及治疗随访进行了分析,为PJS相关的妇科肿瘤诊断治疗提供思路。Peutz-Jeghers syndrome（PJS）is often found complicated by multiple gynecological tumors in female patients.The pathological types of these tumors were always rare so missed diagnosis and misdiagnosis were common.This article focused on one case of a female patient with PJS complicated multiple genital tract neoplasms.The patient was presented with huge pelvic mass and watery vaginal discharge.The final diagnosis of the patient was ovarian sex cord tumor with annular tubules,ovarian mucinous cystadenocarcinoma and cervical minimal deviation adenocarcinoma.In addition,the article reviewed the literature of PJS patients with multiple genital tract tumors, and reviewed the clinical features and follow-ups of treatment among the female patients,which helped provide ideas for the diagnosis and treatment of PJS-related multiple genital tract tumors.

关 键 词：PEUTZ-JEGHERS综合征 微偏腺癌 宫颈 环状小管性索间质瘤 卵巢 

分 类 号：R737.31[医药卫生—肿瘤]