幼少女原发性阴道恶性肿瘤25例临床特点

  目的  探讨幼少女原发性阴道恶性肿瘤的临床特点、治疗和结局。  方法  回顾性分析北京协和医院1980年1月至2015年3月收治的25例经病理确诊的幼少女原发性阴道恶性肿瘤患者的临床病理资料, 总结其临床特点、治疗方法、疗效及结局。  结果  患儿年龄为8个月~12岁, 中位年龄11个月。25例患儿中阴道内胚窦瘤16例(64%)、胚胎型横纹肌肉瘤7例(28%)、透明细胞癌2例(8%)。最常见的临床表现为阴道出血或血性分泌物(76%), 其次为阴道口肿物(24%)。所有患儿均行保守手术, 仅1例患儿术后行放射治疗。小儿阴道恶性肿瘤对化疗多敏感, 术后根据组织类型及高危因素, 分别给予PEB(顺铂、依托泊苷、博莱霉素)、PVB(顺铂、长春新碱、博莱霉素)、IVA(异环磷酰胺、长春新碱、更生霉素)或VAC(长春新碱、更生霉素、环磷酰胺)等方案化疗。中位随访时间44个月(6个月~18年), 22例存活, 生存率88%;3例患儿(1例胚胎型横纹肌肉瘤, 2例透明细胞癌)死于疾病进展, 病死率为12%, 复发和死亡大多发生在治疗后2年内。  结论  幼少女原发性阴道恶性肿瘤极为罕见, 内胚窦瘤是阴道最常见的恶性肿瘤, 其次为胚胎型横纹肌肉瘤和透明细胞癌。阴道内胚窦瘤和胚胎型横纹肌肉瘤多对化疗敏感, 治疗以联合化疗为主, 预后较好。阴道透明细胞癌对化疗不敏感, 治疗以手术切除和放疗为主, 预后较差。早期发现、早期治疗是提高生存率的关键。

Clinical Characteristics of 25 Cases with Primary Vaginal Malignancies in Young Girls

  Objective  To analyze the clinical characteristics, treatment, and outcomes of children with primary vaginal malignancies.  Methods  We conducted a retrospective analysis on clinical and pathological data of all children with pathologically confirmed primary vaginal malignancies treated at Peking Union Medical College Hospital between January 1980 and March 2015. The clinical characteristics, therapeutic methods, treatment effect, and outcomes of the children were summarized.  Results  Twenty-five patients (median age, 11 months; range, 8 months to 12 years) were identified, including 16 (64%) endodermal sinus tumor (EST), 7 (28%) embryonal rhabdomyosarcoma (ERMS), and 2 (8%) clear-cell adenocarcinoma (CCA). Bleeding or blood-tinged discharge was the most common clinical presentation (76%), followed by a protruding mass (24%). All patients were performed conservative surgery. Only one patient received radiotherapy. Childhood genital malignancies were generally sensitive to chemotherapy.According to the histological type and high risk factors, different chemotherapy regimens were administrated after surgery, such as PEB (cisplatin, etoposide, bleomycin), PVB (cisplatin, vincristine, bleomycin), IVA (ifosfamide, vincristine, actinomycin), or VAC (vincristine, actinomycin, cyclophosphamide). The patients were followed up for a median of 44 months (range, 6 months to 18 years). Twenty-two patients (88%) remained alive. Three patients (12%) died of disease progression (1 ERMS, and 2 CCA). Recurrence and death occurred mostly in the first two years after treatment.  Conclusions  Vaginal primary malignancies are extremely rare in children, among which EST is the most common type, followed by ERMS and CCA. EST and ERMS at the vagina are sensitive to chemotherapy, therefore the main therapeutic method is combined chemotherapy, and the prognosis is good. CCA is not sensitive to chemotherapy, thus the main therapeutic methods are surgery and radiotherapy, and the prognosis is poor. Early recognition of symptoms and early treatment is the key to improve survival.