Prognostic factors in metastatic rhabdomyosarcomas: Results of a pooled analysis from United States and European cooperative groups
Prognostic factors in metastatic rhabdomyosarcomas: Results of a pooled analysis from United States and European cooperative groups
复制标题
DOI：
10.1200/jco.2007.14.7207
复制
发表时间：
2008-05-10
影响因子：
45.3
通讯作者：
Anderson, James R.
中科院分区：
文献类型：
--
作者：
Oberlin, Odile;Rey, Annie;Anderson, James R.
目的在一大群儿童转移性横纹肌肉瘤患者中确定与转移性横纹肌肉瘤预后相关的危险因素。年龄、组织学、原发灶部位(S)、转移灶个数等临床因素与无事件生存期(EFS)和总生存期(OS)相关。3年OS和EFS分别为34%(SE，1.7)和27%(SE，1.6)。单因素分析显示，年龄、肺泡组织学、原发肿瘤位于不利部位(定义为四肢和“其他”部位)、是否有3个或更多的转移灶、是否有骨或骨髓受累对3年EFS有显著的负面影响。经多因素分析，EFS与除组织学外的所有因素都有很强的相关性。年龄小于1岁或至少10岁的相对危险度为1.6，原发肿瘤不良部位的相对危险度为1.4，累及骨髓或骨髓的相对危险度为1.4，3个或以上转移部位的相对危险度为1.4。无上述四种不良因素的患者EFS为50%，有一、二、三或四种不良因素的患者EFS分别为42%、18%、12%和5%(P<；.0001)。结论该分析确定了转移性横纹肌肉瘤患者的亚群，其结果与当前的治疗方法不同，并提供了一种确定患者候选实验治疗方法的策略。
PurposeTo identify risk factors associated with outcome in children with metastatic rhabdomyosarcoma in a large cohort of patientsPatients and MethodsPooled data were obtained from 788 patients treated in nine studies performed by European and American cooperative groups. Clinical factors, including age, histology, site of primary, and site(s) and number of sites of metastatic disease, were correlated with event-free survival (EFS) and overall survival (OS).ResultsSeven hundred eighty-eight patients were eligible for analysis. The 3-year OS and EFS were 34% (SE, 1.7) and 27% (SE, 1.6), respectively. By univariate analysis, 3-year EFS was significantly and adversely influenced by age, alveolar histology, location of primary tumor in unfavorable site (defined as extremity and "other" sites), presence of three or more sites of metastatic disease, and the presence of bone or bone marrow involvement. By multivariate analysis, EFS was strongly correlated to all factors except histology. Relative risks were 1.6 for age younger than 1 year or at least 10 years, 1.4 for unfavorable site of primary tumor, 1.4 for bone or bone marrow involvement, 1.4 for three or more metastatic sites. EFS was 50% for patients without any of these four adverse factors and was respectively 42%, 18%, 12%, and 5% in patients with one, two, three, or four factors (P < .0001).ConclusionThis analysis identified subsets of patients with metastatic rhabdomyosaroma with different outcomes to current therapy and offers a strategy to define patient candidates for experimental approaches to treatment.