title: The clinical characteristics and treatment outcome of 57 children and adolescents with primary central nervous system germ cell tumors.
abstract: 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.
authors.affiliation: State Key Laboratory of Oncology in South China; Collaborative Innovation Center of Cancer Medicine; Department of Pediatric Oncology, Sun Yat-sen University Cancer Center, Guangzhou, Guangdong 510060, P. R. China. sunxf@sysucc.org.cn.
authors.forename: Xiao-Fei
authors.initials: XF
authors.lastname: Sun
authors.forename: Fei
authors.initials: F
authors.lastname: Zhang
authors.forename: Zi-Jun
authors.initials: ZJ
authors.lastname: Zhen
authors.forename: Qun-Ying
authors.initials: QY
authors.lastname: Yang
authors.forename: Yun-Fei
authors.initials: YF
authors.lastname: Xia
authors.forename: Shao-Xiong
authors.initials: SX
authors.lastname: Wu
authors.forename: Jia
authors.initials: J
authors.lastname: Zhu
authors.forename: Su-Ying
authors.initials: SY
authors.lastname: Lu
authors.forename: Juan
authors.initials: J
authors.lastname: Wang
authors.forename: Fei-Fei
authors.initials: FF
authors.lastname: Sun
authors.forename: Rui-Qing
authors.initials: RQ
authors.lastname: Cai
authors.forename: Yan
authors.initials: Y
authors.lastname: Chen
authors.forename: Peng-Fei
authors.initials: PF
authors.lastname: Li
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001761:Bleomycin / Q000008:administration & dosage; D016543:Central Nervous System Neoplasms / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy / Q000706:statistics & numerical data; D018572:Disease-Free Survival; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local; D009373:Neoplasms, Germ Cell and Embryonal / Q000628:therapy*; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D001761:Bleomycin; D005047:Etoposide; D002945:Cisplatin