摘要
目的探讨幼女原发性阴道内胚窦瘤的临床特点、治疗与结局。方法回顾性分析1997年1月至2017年12月间21例经病理证实的原发性阴道内胚窦瘤,患儿4个月~4岁,中位年龄11个月,<1岁者14例,所有患儿借助宫腔镜下阴道手术,明确病理诊断。给予PEB(顺铂、依托泊苷、博莱霉素)方案为主的化疗,监测患儿血清甲胎蛋白(AFP)水平。结果 21例患儿均表现为阴道出血或血性分泌物,部分出现阴道脱落肿物。接受化疗3~13个疗程,中位5个疗程,20例患儿达到完全缓解(95.2%)。中位随访时间44个月(4个月~20年),19例存活,生存率90.5%;1例肿瘤进展死亡,2例复发,但再次化疗后达到完全缓解。1例患儿在初始治疗期间死于感染和心脏衰竭。结论幼女阴道内胚窦瘤非常罕见,对化疗十分敏感,治疗以保守手术和经典的PEB方案化疗为主,联合血清标志物AFP和阴道检查,评估疗效。
Objective To analyze the clinical characteristics,treatment,and outcomes of children with primary vaginal endodermal sinus tumor. Methods A retrospective analysis was performed on clinical and pathological data of 21 children with pathologically confirmed primary vaginal endodermal sinus tumor between January 1997 and December 2017.The patients ranged from 4 months to 4 years old,with median age being 11 months old. There were 14 cases younger than one year old. All patients were treated with chemotherapy,mainly PEB（cisplatin,etoposide,bleomycin） after an IV port implanted into subclavian vein. Detection of serum α-fetoprotein（AFP） level,examination under anesthesia and biopsy by hysteroscope were performed before and after treatment to evaluate the tumor status. Results Bleeding or blood-tinged discharge was the most common clinical presentation. The patients received chemotherapy for a median of5 courses（ranged from 3 to 13 courses）. Twenty patients obtained complete remission（95.2%）. The patients were followed up for a median of 44 months（ranged from 4 months to 20 years）. Nineteen patients（90.5%） remained alive.One child died of disease progression. One child received 4 courses of PEB chemotherapy and died of infection and heart failure. Two patients were recurrent but obtained complete remission after chemotherapy again. Conclusion Vaginal endodermal sinus tumors are rare in children and extremely sensitive to chemotherapy. To allowpreservation of sexual and reproductive function,PEB chemotherapy with conservative surgery should be considered for children with vaginal endodermal sinus tumor. Combination of serum AFP with vaginal examination are reliable evaluating methods.
作者
王涛
曹冬焱
俞梅
张颖
刘巍
宋红梅
沈铿
郭丽娜
杨佳欣
WANG Tao1 ,CAO Dongyan1 ,YU Mei1, ZHANG Ying1 ,LIU Wei2 , SONG Hongmei3, SHEN Keng1 , GUO Lina4, YANG Jiaxin4(1. Department of Obstetrics and Gynecology; 2. Department of Interventional Radiology; 3. Department of Pediatrics; 4. Department of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Science ＆ Peking Union Medical College, Beijing 100730, Chin)
出处
《山东大学学报（医学版）》
CAS
北大核心
2018年第5期41-45,共5页
Journal of Shandong University:Health Sciences
基金
国家科技部十一五国家科技支撑计划(2008BAI57B02)
关键词
阴道内胚窦瘤
幼女
化疗
Vaginal endoderlnal sinus tumor
Children
Chemotherapy
