138例儿童颅外恶性生殖细胞肿瘤临床研究

目的:研究儿童恶性生殖细胞瘤(malignant germ cell tumor,MGCT)的临床及预后特征。方法:回顾性分析1998年1月至2016年1月上海儿童医学中心收治的初发颅外MGCT患儿138例。对患儿的临床特点、疗效及预后做综合分析。结果:按病理分期,Ⅰ期患儿5年总生存期(overall survival,OS)为100. 00%,Ⅱ期患儿5年OS为94. 44%,Ⅲ期5年OS为96. 43%,Ⅳ期5年OS为88. 73%。多因素分析显示,病理分期对生存率的影响有统计学差异(P <0. 01),年龄、性别及部位对于生存率的影响无显著统计学差异。本研究中共有13例Ⅰ/Ⅱ期患儿接受手术后临床观察,有5例(38. 5%)在2年内出现疾病复发进展的情况并接受化疗,目前均达到临床缓解。结论:通过以手术联合含铂类药物化疗并根据临床危险度的不同分层治疗MGCT瘤患儿,可得到较好的临床疗效。

Clinical observation of 138 children with extracranial malignant germ cell tumors

Objective:To investigate the clinical and prognostic features of malignant germ cell tumor(MGCT).Methods:Between January 1998 and January 2016，138 patients with extracranial(all stages) MGCT were enrolled in Shanghai Children's Medical Center(SCMC).The clinical characteristics，therapeutic efficacy and prognosis were retrospectively analyzed.Results:The 5-year overall survival(OS) of pediatric patients was determined as 96.1%，in detail 100.00% at stage Ⅰ，94.43% at stage Ⅱ，96.44% at stage Ⅲ and 88.73% at stage Ⅳ.Multivariate analysis suggested that staging affected the survival significantly(P<0.01).13 cases through clinical observation on stage Ⅰ/Ⅱpediatric patients post surgery，5 cases(38.5%) were suffered from recurrence within 2 years and alleviated by chemotherapy treatment.Conclusion:The cisplatin-based combination chemotherapy can improve the outcome of childhood extracranial MGCT.