The clinical characteristics and treatment outcomeof 57 children and adolescents with primary central nervoussystem germ cell tumors

作     者：Xiao-Fei Sun Fei Zhang Zi-Jun Zhen Qun-Ying Yang Yun-Fei Xia Shao-Xiong Wu Jia Zhu Su-Ying Lu Juan Wang Fei-Fei Sun Rui-Qing Cai Yan Chen Peng-Fei Li 

作者机构：State Key Laboratory of Oncology in South China Collaborative Innovation Center of Cancer Medicine Department of Pediatric Oncology Sun Yat-sen University Cancer Center Department of Neurosurgery Sun Yat-sen University Cancer Center Department of Radiation Oncology Sun Yat-sen University Cancer Center 

出 版 物：《Chinese Journal of Cancer》 (Chinese Journal of Cancer)

年 卷 期：2014年第33卷第8期

页      面：395-401页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：中枢神经系统 手术治疗 生殖细胞 临床特点 青少年 原发性 儿童 综合治疗 

摘      要：Primary central nervous system germ cell tumors(CNS-GCTs) in children and adolescents have unique clinical features and methods of treatment compared with those in adults. There is little information about Chinese children and adolescents with CNS-GCTs. Therefore, in this study we retrospectively analyzed the clinical features and treatment outcome of Chinese children and adolescents with primary CNS-GCTs. Between January 2002 and December 2012, 57 untreated patients from a single institution were enrolled. They were diagnosed with CNS-GCTs after pathologic or clinical assessment. Of the 57 patients, 41 were males and 16 were females, with a median age of 12.8 years(range, 2.7 to 18.0 years) at diagnosis; 43(75.4%) had non-germinomatous germ cell tumors(NGGCTs) and 14(24.6%) had germinomas; 44(77.2%) had localized disease and 13(22.8%) had extensive lesions. Fifty-three patients completed the prescribed treatment, of which 18 underwent monotherapy of surgery, radiotherapy, or chemotherapy, and 35 underwent multimodality therapies that included radiotherapy combined with chemotherapy or surgery combined with chemotherapy and/or radiotherapy. PEB(cisplatin, etoposide, and bleomycin) protocol was the major chemotherapy regimen. The median follow-up time was 32.3 months(range, 1.2 to 139 months). Fourteen patients died of relapse or disease progression. The 3-year event-free survival(EFS) and overall survival rates for all patients were 72.2% and 73.8%, respectively. The 3-year EFS was 92.9% for germinomas and 64.8% for NGGCTs(P = 0.064). The 3-year EFS rates for patients with NGGCTs who underwent monotherapy and multimodality therapies were 50.6% and 73.5%, respectively(P = 0.042). Our results indicate that multimodality therapies including chemotherapy plus radiotherapy were better treatment option for children and adolescents with CNS-GCTs.