【作者】 董峰歧； 许丽彦； 刘洋； 张晓强； 宋晓燕；

【Author】 Dong Feng-Qi Liu Yang Xu Li-Yan Zhang Xiao-Qiang Song Xiao-Yan Department of urinary surgery of Harbin Children’ s Hospital, Harbin, 150010

【机构】 哈尔滨市儿童医院泌尿外科；

【摘要】 目的:总结二例女婴阴道胚胎癌的诊断及治疗。方法:例1:女婴,8个月,因阴道外口葡萄状肿物半个月伴排尿困难,于2005年1月入院,行相关检查后,切除子宫及阴道,术后病理为阴道胚胎癌。例2:女婴,13个月,阴道不规则出血4个月。2006年3月入院后行阴道和子宫切除术,病理为阴道胚胎癌。二例均轻度贫血,无恶病质,术前AFP均高,术后三天AFP降到正常。术前术后给予化疗。术中快速冰冻切片均是恶性肿瘤。结果:二例均为一期,行根治术,切除子宫阴道,保留卵巢。化疗采用VCR、CTX、CDDP,例一已一年,例二已三月,目前均无瘤存活。结论:阴道胚胎癌是罕见高度恶性肿瘤。目前病因尚未明了,多数学者认为此病可能源于中肾管的中胚层组织,具有胚胎性和未成熟组织特性,又称胚胎性横纹肌肉瘤。病理主要是葡萄状肉瘤,常是多中心性肿瘤,为粉红色的结节,可充满阴道如葡萄状,有时可突出阴道外口,肿瘤组织疏松,呈黏液状。镜下见疏松组织中有散在的梭形细胞和星状细胞。间质为黏液样组织。有的核有异型性,可见横纹肌母细胞。临床表现早期即有阴道不规则出血,外生型可有排尿困难,早期多由淋巴转移,晚期由血行转移。治疗以手术为主,术中冰冻切片检查,如为恶性即行根治性切除,使患者避免二次手术的创伤和痛苦。本组二例均术中及术后病理确诊。根据美国IRS分期标准为一期。本病恶性程度较高,术后辅以化疗,对改善后有重要意义。现已证实VCR、CTX、CDDP联合用药有较好的抗横纹肌肉瘤作用,可提高无瘤存活率。本组2例术后采用此方案, 随访至今未见复发及转移。更多还原

【Abstract】 Objective: Summarize the diagnosis and treatment of 2 cases of children’ s vagina embryonal carcinoma. Methods: One girl, 8 months old, was in hospital because there was a botryoidalis tumor on the external aperture of the vagina. Uterus and vagina was cut in operation, and pathologic findings showed embryonal carcinoma after operation. The other, 13 months old, was suffered from irregular bleeding from the vagina for 4 months. Uterus and vagina was also cut in operation, and pathologic findings was the same to the one. They both were not suffered from cachexia, but anaemia lightly. The alpha fe-toprotein (AFP) was high before the operation and normal after the operation. Chemotherapy was used before and after the operation. Pathologic findings of frozen section during operation showed malignant Results: The 2 cases were belong to the stage one, uterus and vagina was cut in operation, ovaries remained. The 2 cases were given chemotherapy by PVB program VCR, CTX, and CDDP. Prognosis was ok. Conclusion: Vagina embryonal carcinoma is the rare malignant tumor, maybe comes from Meso-nephric mesoblastema, also named embryonal rhabdomyosarcoma. Pathologic findings is sarcoma botryoides. Clinical manifestation: irregular bleeding, dysuresia in exogenic tumor, lymphatic metastasis in early stage and hematogenous metastasis in advanced stage. Operation is the main method of the therapy. Frozen section during operation is necessary, if it were malignant, radical excision was performed. The 2 cases above is confirmed by pathologic findings during and after operation, they both belong to stage one by U.S. IRS. Chemotherapy after operation is important for prognosis. VCR, CTX and CDDP is effective to rhabdomyosarcoma.更多还原