Abstract: 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 of 5 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. 山 东 大 学 学 报 (医 学 版)56卷5期 -王涛,等.幼女原发性阴道内胚窦瘤21例临床分析 \=-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 allow preservation 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.

Key words: Vaginal endodermal sinus tumor, Children, Chemotherapy