摘要： 目的·分析接受放射治疗（放疗）的Ⅲ~Ⅳ期中高危及极高危神经母细胞瘤（neuroblastoma，NB）患儿生存及预后的影响因素。方法·纳入2008—2018年在上海交通大学医学院附属上海儿童医学中心血液肿瘤科、附属仁济医院放射诊疗科，以及附属新华医院放射诊疗科接受放疗的NB患儿共132例，治疗的方案包括化学治疗、手术、自体骨髓干细胞移植（autologous bone marrow transplantation，ABMT）、放疗，以及后期13-顺式-视黄酸维持治疗。采用SPSS 19.0软件进行无事件生存（event-free survival，EFS）率和总体生存（overall survival，OS）率的计算，以及单因素和多因素生存分析。结果·至随访结束，整组患儿1年、2年、3年、5年EFS率分别为87.41%、55.25%、45.02%、38.67%，OS率分别为96.02%、83.54%、72.15%、57.79%。中位随访时间为40.3个月。单因素生存分析提示患儿疾病的分期、危险度的分组及有无脑转移、血液乳酸脱氢酶及铁蛋白水平均与EFS和OS有关（均
P<0.05）；有无骨髓浸润为EFS的相关因素（
P=0.007），但与OS无关；患儿性别、是否接受ABMT治疗、
MYCN原癌基因扩增情况、有无肝脏转移等因素与EFS和OS均无明显相关性。多因素分析发现有无脑转移为EFS以及OS的独立预后因素。结论·
MYCN基因的扩增情况、是否接受ABMT均可能对Ⅲ~Ⅳ期NB患儿放疗后疗效及总体生存情况无明显影响，但有无脑转移是与放疗后生存及疾病进展相关的独立预后因素。
关键词: 神经母细胞瘤, 放射治疗, 预后因素, 脑转移 Abstract: Objective·To analyze the factors affecting the survival and prognosis of the children with medium-, high-, and very high-risk neuroblastoma (NB) in stage Ⅲ?Ⅳ after radiation therapy.
Methods·A total of 132 cases of neuroblastoma patients from 2008 to 2018 who received local radiotherapy in the Department of Hematology and Oncology, Shanghai International Children′s Medical Center, Shanghai Jiao Tong University School of Medicine, and the Department of Radiation Oncology, Renji Hospital and Xinhua Hospital were included. Treatment options included chemotherapy, surgery, autologous bone marrow transplantation (ABMT), radiotherapy, and later 13-cis-RA maintenance therapy. SPSS 19.0 software was used for the calculation of event-free survival (EFS) and overall survival (OS) rates, as well as univariate and multivariate survival analysis.
Results·By the end of the follow-up, the EFS rates for the all patients were 87.41% in 1 year, 55.25% in 2 years, 45.02% in 3 years, and 38.67% in 5 years, and the OS rates were 96.02% in 1 year, 83.54% in 2 years, 72.15% in 3 years, and 57.79% in 5 years, respectively. The medium follow-up time was 40.3 months. Univariate survival analysis suggested that the staging, risk grouping, brain metastasis or not, blood lactic acid dehydrogenase and serum ferritin levels were associated with EFS and OS (P<0.05). The presence or absence of bone marrow infiltration was a related factor of EFS (P=0.007), but not OS-related. Factors such as the child′s gender, whether the child was treated with ABMT, the amplification of MYCN proto-oncogene, and the presence of liver metastasis had no significant correlation with EFS or OS. Multivariate survival concluded that brain metastasis was an independent prognosis factor for EFS and OS.
Conclusion·The amplification of MYCN gene and ABMT treatment may not significantly affect the radiotherapy efficacy and overall survival of stage Ⅲ?Ⅳ NB children. However, the presence of brain metastasis is an independent prognostic factor related to survival and disease progression after radiotherapy.
Key words: neuroblastoma (NB), radiotherapy, prognostic factor, brain metastasis PDF全文下载地址:
点我下载PDF 