【摘要】 背景青少年恶性实体肿瘤的临床特征及预后与儿童和成人不同。目的 总结北京地区多中心诊治的青少年恶性实体肿瘤的临床特征及预后。设计横断面调查。方法 回顾性分析2010年1月1日至2019年12月31日北京地区7家医院确诊的且当时年龄≥10岁的恶性实体肿瘤初发患儿的临床特征和预后。主要结局指标3年无事件生存率(EFS)和总生存率(OS)。结果 共411例恶性实体肿瘤患儿纳入分析，中位年龄150(120～216)月，男239例(58.2%),女172例。中枢神经系统肿瘤183例(44.5%),尤文肉瘤82例(20.0%),横纹肌肉瘤47例(11.4%),神经母细胞瘤33例(8.0%),非横纹肌肉瘤类软组织肉瘤29例(7.1%),颅外生殖细胞肿瘤18例(4.4%),其他肿瘤19例(4.6%)。411例恶性实体肿瘤患儿中，前3位户籍来自华北地区160例(38.9%),华东地区97例(23.6%),中南地区70例(17.0%)。治疗涉及1家、2家和≥3家医院患儿分别有129例(31.4%)、180例(43.8%)和102例(24.8%)。截至末次随访时间2020年6月30日，411例恶性实体肿瘤患儿中位随访时间26(1～120)个月，生存333例(81.0%),死亡78例，发生肿瘤复发/进展145例(35.3%)。3年总体EFS为60.3%,以中枢神经系统肿瘤最高(73.7%),横纹肌肉瘤最低(37.4%);3年总体OS为79.5%,以中枢神经系统肿瘤最高(87.9%)。78例死亡病例中位生存时间13(1～65)个月，其中中枢神经系统肿瘤22例(28.2%),横纹肌肉瘤18例(23.1%),尤文肉瘤16例(20.5%),神经母细胞瘤8例(10.3%),颅外恶性生殖细胞瘤3例(3.8%),非横纹肌肉瘤类软组织肉瘤5例(6.4%),其他肿瘤6例(7.7%)。结论 青少年恶性实体肿瘤以中枢神经系统肿瘤最多见，神经母细胞瘤、横纹肌肉瘤和颅外恶性生殖细胞瘤预后不佳。更多还原

【Abstract】 Background The clinical characteristics and prognosis of malignant solid tumors in adolescents are different from children and adults. Objective To summarize the clinical characteristics and prognosis of adolescent malignant solid tumors in multiple centers in Beijing. Design Cross-sectional survey. Methods This retrospective study analyzed the clinical characteristics and prognosis of adolescents over the age of 120 months with the initial diagnosis of malignant solid tumors in seven hospitals in Beijing from January 1, 2010 to December 31, 2019. Main outcome measures Three-year event free survival(EFS) and overall survival(OS). Results A total of 411 patients were enrolled in this study with a median age of 150 months(120-216 months). In total, 239 patients(58.2%) were males and 172(41.8%) were females. There were 183 patients(44.5%) with central nervous system tumors, 82(20%) with Ewing sarcoma, 47(11.4%) with rhabdomyosarcoma, 33(8%) with neuroblastoma, 29(7.1%) with non-rhabdomyosarcoma soft tissue sarcomas, 18(4.4%) with extracranial germ cell tumors and 19(4.6%) with other rare tumors. Among the 411 patients, the top three regions of registered residence were North China(160 cases, 38.9%), East China(97 cases, 23.6%), South Central China(70 cases, 17%). There were 129 patients(31.4%) treated at one hospital, 180 patients(43.8%) at two hospitals, and 102 patients(24.8%) at three or more hospitals. The median follow-up time of 411 patients was 26 months(1-120 months). At the end of follow-up, 333 patients(81.0%) survived, 78 patients(19%) died, and 145 patients(35.3%) had tumor relapse or progression. The 3-year EFS rate was 60.3%, and the 3-year OS rate was 79.5%. The median survival time of 78 cases of death was 13 months(1-65 months), including 22 patients(28.2%) with central nervous system tumors, 18(23.1%) with rhabdomyosarcoma, 16(20.5%) with Ewing sarcoma, 8(10.3%) with neuroblastoma, 3(3.8%) with extracranial malignant germ cell tumors, 5(6.4%) with non-rhabdomyomatous soft tissue sarcomas and 6(7.7%) with rare tumors. Conclusion Center nervous system tumors are the most common malignant solid tumors in adolescents. The prognosis of neuroblastoma, rhabdomyosarcoma and extracranial malignant germ cell tumor in adolescents is poor.更多还原