女性生殖道横纹肌肉瘤13例分析

Analysis of 13 cases of rhabdomyosarcoma in the female genital tract

目的探讨女性生殖道横纹肌肉瘤（RMS）患者的临床和病理特点、治疗方法及预后。方法收集1973年1月至2014年12月中国医学科学院肿瘤医院收治的13例生殖道RMS患者的临床病理资料，并对所有患者进行随访，随访截止日期为2015年4月1日，中位随访时间为25．0个月（1～228个月）。回顾性分析女性生殖道RMS患者的临床和病理特点、治疗方式、复发情况、复发后治疗情况以及预后。结果13例生殖道RMS患者的年龄为6～54岁，中位年龄21．0岁；原发灶部位：阴道6例，子宫颈7例；病理类型：胚胎型RMS（ERMS）11例，腺泡型RMS（ARMS）2例。按照国际妇产科联盟（FIGO）分期标准，I期6例、Ⅱ期3例、Ⅲ期1例、Ⅳ期3例，其中位生存时间分别为112．5、153．0、9．0、3．5个月；按照横纹肌肉瘤国际协作组（IRSG）分期标准，I期10例、Ⅳ期3例，其中位生存时间分别为112．5、3．5个月。女性生殖道RMS患者治疗方式主要为手术联合化疗，13例生殖道RMS患者中，9例在初始治疗时接受了手术治疗，中位生存时间为108．0个月（9～228个月），其中6例术后接受了辅助化疗，中位生存时间为152．0个月（9～228个月），3例术后未接受辅助治疗，中位生存时间25．0个月（9—108个月）；另外4例患者在初始治疗时未接受手术治疗，中位生存时间613个月（1～117个月）。13例生殖道RMS患者中有2例在初始治疗时接受了联合放疗，生存时间分别为4、198个月。随访期内，13例生殖道RMS患者中，8例无瘤生存，5例死亡；中位生存时间为25．0个月（1—228个月），5年累积生存率为58．6％。结论女性生殖道RMS早期患者的预后较好，晚期患者预后较差，其标准治疗模式为手术联合化疗，是否需要辅助放疗尚有待进一步研究。

Objective To investigate the clinical features, treatments and prognostic factors of rhabdomyosarcoma （RMS） in the female genital tract. Methods A retrospective analysis was performed on 13 cases of RMS in the female genital tract. Clinical characteristics, treatments and prognosis were compared and analyzed statistically. Results The median age was 21.0 years （range, 6 to 54 years）. There were 6 cases vaginal RMS and 7 cases cervical RMS, included 11 cases of embryonal RMS （ERMS） and 2 cases of alveolar RMS （ARMS）. According to the Federation International of Gynecology and Obstetrics （FIG{）） staging system, there were 6 cases of stage I, 3 cases of stage ]], 1 case of stage m and 3 cases of stage IV, the median survival time were respectively 112.5, 153.0, 9.0 and 3.5 months. According to the Intergroup Rhabdomyosarcom Study Group （IRSG） staging system, there were 10 cases of stage I and 3 cases of the stage IV＇, and their median survival time were respectively 112.5 and 3.5 months. Nine patients received surgery and the median survival time was 108.0 months （range, 9 to 228 months）, 6 of them received chemotherapy after surgery and the median survival time was 152.0 months （range, 9 to 228 months）, the other 3 cases did not receive any therapy after surgery and the median survival time was 25.0 months （range, 9 to 108 months）. Four patients did not receive surgery and the median survival time was 6.3 months （range, 1 to 117 months）. There were 2 cases received combined treatment included radiotherapy and the survival time were respectively 4 and 198 months. There were 8 cases who was survival without disease and 5 cases died of cancer. The median survival time in 13 patients was 25.0 months （range, 1 to 228 months） and the 5-year overall survival rate was 58.6%. Conclusions The prognosis of early stage of RMS in the female genital tract is good. While, the prognosis of advanced stage is poor. The standard treatment strategy is combination of surgery and chemotherapy, whether radiotherapy could improve the prognosis still need further study.