4例婴儿阴道内胚窦瘤的综合治疗分析

A comprehensive treatment of infantile endodermal sinus tumor of vagina:An analysis of four infants

目的:介绍化疗结合手术切除的综合方案治疗婴儿阴道内胚窦瘤。方法:选取2019年11月至2022年10月于杭州市儿童医院、上海交通大学医学院附属新华医院收治的4例小于12个月婴儿阴道内胚窦瘤的临床治疗和随访并结合文献进行讨论。结果:4例经病理活检确诊为阴道内胚窦瘤后均先给予JEB/PEB(卡铂/顺铂、依托泊苷、博来霉素)方案化疗2~3个疗程,再次评估证实肿瘤显著缩小,但未完全消失,原发灶位于膨大扩张的阴道后壁,即给予原发灶手术切除,阴道修补。术后继续JEB/PEB方案化疗。完成预定治疗方案后4例分别随访12~36个月,均保持完全缓解状态。结论:鉴于婴儿阴道内胚窦瘤对化疗的敏感性,推荐采取化疗结合以保留器官功能为前提的局部阴道切除的综合性治疗为婴儿阴道内胚窦瘤的首选方案。

Objective:To introduce the comprehensive treatment of infantile endodermal sinus tumor of vagina assisted by surgical resection.Methods:To summarize the clinical data and follow-up treatment of 4 cases of endodermal sinus tumor of vagina in infants younger than 12 months from Hangzhou Children’s Hospital and Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from November 2019 to October 2022 and to discuss in combination with the literature.Results:All four patients confirmed by pathological biopsy were treated by chemotherapy with JEB/PEB(Carboplatin/Cisplatin,Etoposide,Bleomycin)for 2-3 courses.Re-evaluation showed that the tumor reduced significantly but not yet completely disappeared.As the primary lesion was located in the enlarged posterior wall of the vagina,the tumor was removed and the vaginal defect repaired.Four patients continued JEB/PEB chemotherapy after operation and were followed up for 12 months to 36 months respectively after completing the treatment plan,and all of them remained in complete remission status.Conclusion:As the vaginal endodermal sinus tumor in infant are sensitive to chemotherapy,it is suggested that multi-drug chemotherapy combined with local vaginal tumor resection should be the first choice for the treatment of infantile vaginal endodermal sinus tumor.