Long-term follow-up of the multicenter, multidisciplinary treatment study HIT-LGG-1996 for low-grade glioma in children and adolescents of the German Speaking Society of Pediatric Oncology and Hematology
Long-term follow-up of the multicenter, multidisciplinary treatment study HIT-LGG-1996 for low-grade glioma in children and adolescents of the German Speaking Society of Pediatric Oncology and Hematology
复制标题
DOI：
10.1093/neuonc/nos202
复制
发表时间：
2012-10-01
期刊：
影响因子：
15.9
通讯作者：
Faldum, Andreas
中科院分区：
文献类型：
--
作者：
Gnekow, Astrid K.;Falkenstein, Fabian;Faldum, Andreas
Hirntumorstudien（HIT）-LGG-1996方案为患有低级别胶质瘤（LGG）的儿科患者提供了一种综合治疗策略，即观察、手术、辅助放疗和化疗，以推迟幼儿的放疗开始。在目前的研究中，我们试图确定进展和生存的临床因素。在1996年10月1日至2004年3月31日期间，前瞻性招募了1031例患者，分为观察组（n 668）和非手术组，以年龄依赖性方式分层12个月的长春新碱-卡铂化疗（n 216）和常规放疗/近距离放射治疗（n 147）。患者的中位年龄为6.9岁; 28例患者患有间脑综合征，44例患者患有播散，108例患者患有1型神经纤维瘤病（NF-1）。肿瘤主要位于幕上中线（40.4），主要组织学类型为毛细胞型星形细胞瘤（67.9）。中位观察时间为9.3年，10年总生存期（OS）为0.94，10年无事件生存期（EFS）为0.47。放疗后10年无进展生存率为0.62，化疗后为0.44。216例化疗患者中有61例在初诊后0.38 ~ 7a接受了放疗。多因素分析发现间脑综合征和不完全切除是OS和EFS的不利因素，年龄> 11岁是OS的不利因素，幕上中线位置是EFS的不利因素。播散、年龄<1岁和非毛细胞组织学是放疗后进展的不利因素（138例患者）;间脑综合征、播散和年龄<11岁是化疗后的不利因素（210例患者）。NF-1患者和男孩经历了长期的肿瘤稳定与化疗。一个全国性的多模式治疗策略对儿童LGG是可行的。延长随访的结果与治疗组的单机构系列相当。四分之三的幸存化疗患者尚未接受放射治疗。患有或不患有间脑综合征和播散的婴儿在诊断或治疗后死亡和进展的风险最高。
The Hirntumorstudien (HIT)-LGG-1996 protocol offered a comprehensive treatment strategy for pediatric patients with low-grade glioma (LGG), ie, observation, surgery, adjuvant radiotherapy, and chemotherapy to defer the start of irradiation in young children. In this current study, we sought to determine clinical factors for progression and survival. Between October 1, 1996 and March 31, 2004, 1031 patients were prospectively recruited into an observation arm (n 668) and a nonsurgical arm stratifying 12 months of vincristine-carboplatin chemotherapy (n 216) and conventional radiotherapy/brachytherapy (n 147) in an age-dependent manner. Median patient age was 6.9 years; 28 patients had diencephalic syndrome, 44 had dissemination, and 108 had neurofibromatosis type 1(NF-1). Main tumor location was the supratentorial midline (40.4), and the main histology was pilocytic astrocytoma (67.9). Following a median observation of 9.3 years, 10-year overall survival (OS) was 0.94 and 10-year event-free survival (EFS) was 0.47. Ten-year progression-free survival was 0.62 following radiotherapy and 0.44 following chemotherapy. Sixty-one of 216 chemotherapy patients received radiotherapy 0.38.7 years after initial diagnosis. By multivariate analysis, diencephalic syndrome and incomplete resection were found to be unfavorable factors for OS and EFS, age epsilon 11 years for OS, and supratentorial midline location for EFS. Dissemination, age 1 year, and nonpilocytic histology were unfavorable factors for progression following radiotherapy (138 patients); and diencephalic syndrome, dissemination, and age epsilon 11 years were unfavorable factors following chemotherapy (210 patients). NF-1 patients and boys experienced prolonged tumor stabilization with chemotherapy. A nationwide multimodal treatment strategy is feasible for pediatric LGG. Extended follow-up yielded results comparable to single-institution series for the treatment groups. Three-quarters of surviving chemotherapy patients have not yet received radiation therapy. Infants with or without diencephalic syndrome and dissemination bear the highest risk for death and progression following diagnosis or treatment.