14例婴幼儿阴道卵黄囊瘤的临床分析

Clinical analysis of 14 cases of vaginal yolk sac tumor in infants

目的探讨婴幼儿阴道卵黄囊瘤的临床特征、诊治及预后。方法回顾性分析2011年1月至2021年4月本院收治的14例婴幼儿阴道卵黄囊瘤患者的临床特征、彩超、磁共振成像(MRI)、血清甲胎蛋白(AFP)、病理结果及随访情况等资料。结果同期女性生殖道恶性肿瘤154例,阴道卵黄囊瘤占9.1%(14/154)。14例患儿平均年龄(12.3±3.4)个月,均出现阴道不规则流血,其中2例患儿阴道内肿物已自行脱落。血AFP为1024~7371 ng/ml,平均(4025.8±332.4)ng/ml,β-人绒毛膜促性腺激素(β-hCG)均正常。2例患儿取得标本经病理证实后直接化疗,化疗采用顺铂、依托泊苷、博来霉素(PEB)方案。其余12例均在麻醉下经小儿鼻窥镜或阴道镜辅助行肿物及部分阴道切除术,术后病理报告均提示卵黄囊瘤,给予PEB方案2例,卡铂、依托泊苷、博来霉素(JEB)方案10例。第3疗程开始时,14例患儿AFP均恢复至正常水平,并在第4个疗程结束后再次麻醉下用阴道镜探查,术中见原肿物生长处的阴道壁呈瘢痕样外观,质地稍硬,无明显肿物生长,余阴道壁无异常。2例因术前彩超提示局部阴道壁增厚,行局部多点活检术,术后病理仅提示纤维组织伴慢性炎症,未见肿瘤细胞,但仍给予了6个疗程的化疗。其余12例未行活检,共给予4个疗程化疗。14例患儿随访时间2~96个月,均存活,无复发。结论婴幼儿阴道卵黄囊瘤特征为不规律阴道流血伴AFP明显升高,主要治疗方法为肿物及部分阴道壁切除联合规范化疗。

Objective To analyze the clinical manifestations,diagnosis,treatment and prognosis of vaginal yolk sac tumor(YST)in infants.Methods The data of 14 infants with vaginal YSTs treated in our hospital from January 2011 to April 2021 were retrospectively analyzed,including clinical features,color Doppler ultrasound,magnetic resonance imaging(MRI),serum alpha-fetoprotein,pathological results and follow-up result.Results A total of 154 cases of malignant germ cell tumors(MGCTs)were hospitalized during the same time,vaginal YSTs accounted for 9.1%(14/154).The average age of the patients was(12.3±3.4)months.Irregular vaginal bleeding occurred in all 14 patients,and the vaginal tumor in 2 infants were discharged spontaneously.The AFP was 1024~7371 ng/ml,the average value was(4025.8±332.4)ng/ml,and theβ-h CG was normal.Two children were directly treated with chemotherapy after pathological confirmation.The chemotherapy regimen was cisplatin,etoposide,and bleomycin(PEB).The other 12 patients were all treated with vaginoscopy assisted tumor and partial vaginectomy under anaesthesia,postoperative pathological reports showed YST.Two cases were given PEB regimen,10 cases were given carboplatin,etoposide,bleomycin(JEB)regimen.The AFP of all the 14 patients regressed to normal levels at the beginning of the third cycle.After the fourth cycle,all patients underwent vaginoscopy-assisted vaginal exploration under anaesthesia.During the operation,it was found that the vaginal wall where the tumor grew had a scar-like appearance with a slightly hard texture,no obvious tumor growth,and the rest of the vaginal wall was normal.Two cases underwent local multi-point biopsy because of preoperative color Doppler ultrasound showed local vaginal wall thickening.The postoperative pathology only showed fibrous tissue with chronic inflammation,and no tumor cells were found,but they were still given a total of 6 courses of chemotherapy.The remaining 12 cases did not undergo biopsy and were given a total of 4 courses of treatment.All the patients were followed up for 2 to 96 months.There was no recurrence,and all the children survived.Conclusion Vaginal YST in infants is characterized by irregular vaginal bleeding with markedly elevated AFP.The main treatment method is resection of the tumor and partial vaginectomy combined with standard chemotherapy.