Center for Neuroscience and Behavioral Medicine, Children's National Medical Center, 111 Michigan Ave., NW, 4th Floor, Suite 800, Washington, DC 20010, USA.
Neuro Oncol. 2013 Jan;15(1):97-103. doi: 10.1093/neuonc/nos267. Epub 2012 Oct 25.
The purpose of the trial was to determine the survival and incidence of secondary tumors in children with medulloblastoma receiving radiotherapy plus chemotherapy. Three hundred seventy-nine eligible patients with nondisseminated medulloblastoma between the ages of 3 and 21 years were treated with 2340 cGy of craniospinal and 5580 cGy of posterior fossa irradiation. Patients were randomized between postradiation cisplatin and vincristine plus either CCNU or cyclophosphamide. Survival, pattern of relapse, and occurrence of secondary tumors were assessed. Five- and 10-year event-free survivals were 81 ± 2% and 75.8 ± 2.3%; overall survivals were 87 ± 1.8% and 81.3 ± 2.1%. Event-free survival was not impacted by chemotherapeutic regimen, sex, race, age at diagnosis, or gender. Seven patients had disease relapse beyond 5 years after diagnosis; relapse was local in 4 patients, local plus supratentorial in 2, and supratentorial alone in 1. Fifteen patients experienced secondary tumors as a first event at a median time of 5.8 years after diagnosis (11 >5 y postdiagnosis). All non-CNS solid secondary tumors (4) occurred in regions that had received radiation. Of the 6 high-grade gliomas, 5 occurred >5 years postdiagnosis. The estimated cumulative 10-year incidence rate of secondary malignancies was 4.2% (1.9%-6.5%). Few patients with medulloblastoma will relapse ≥ 5 years postdiagnosis; relapse will occur predominantly at the primary tumor site. Patients are at risk for development of secondary tumors, many of which are malignant gliomas. This may become an increasing issue as more children survive.
本试验旨在观察接受放化疗的髓母细胞瘤患儿的生存和继发肿瘤的发生率。379 例 3 至 21 岁非播散性髓母细胞瘤患儿接受颅脊髓 2340cGy 和后颅窝 5580cGy 照射。患儿随机分为放疗后顺铂+长春新碱联合 CCNU 或环磷酰胺化疗组。评估患儿的生存情况、复发模式和继发肿瘤的发生情况。5 年和 10 年无事件生存率分别为 81±2%和 75.8±2.3%；总生存率分别为 87±1.8%和 81.3±2.1%。化疗方案、性别、种族、诊断时年龄和性别对无事件生存率无影响。7 例患儿在诊断后 5 年以上复发；4 例为局部复发，2 例为局部+幕上复发，1 例为幕上复发。15 例患儿在诊断后 5.8 年（11 例＞5 年）首次发生继发肿瘤。所有非 CNS 实体继发肿瘤（4 例）均发生在接受过放疗的部位。6 例高级别胶质瘤中，5 例发生于诊断后＞5 年。继发恶性肿瘤的估计 10 年累积发生率为 4.2%（1.9%-6.5%）。少数髓母细胞瘤患儿在诊断后≥5 年会复发，且复发多发生于原发肿瘤部位。患儿有发生继发肿瘤的风险，其中许多为恶性胶质瘤。随着更多患儿存活，这可能成为一个日益严重的问题。
