Santos Marcos A, Viégas Célia M P, Servidoni Renata A, Barros Mario H M, Pinel Maria Isabel, Araújo Carlos M M
Department of Radiation Oncology, Instituto Nacional de Câncer (INCA), Rio de Janeiro, Brazil.
Pediatr Blood Cancer. 2007 Apr;48(4):416-22. doi: 10.1002/pbc.21049.
To evaluate the importance of timing of radiation therapy in children with PNET/medulloblastoma, treated at the Brazilian National Cancer Institute (INCA).
The records of 101 children with confirmed diagnosis of medulloblastoma were retrospectively reviewed. Patients had a median follow-up of 48 months (0.5-241 months). The age varied from 0.8 to 17.5 years (median: 7.6 years) and 21.7% were 3 years old or younger.
According to the data collected from patients that received treatment for medulloblastoma from 1983 to 2001, the overall survival (OS) rate was 53% and the Disease Free Survival (DFS) rate was 40%. Multivariate analysis showed that under age 3 years, presence of neoplasic cells in the cerebrospinal fluid (CSF) at presentation or subtotal tumor resection resulted in a worse OS. The patients that received a biological effective dose (BED) greater than 44 Gy10 had better prognosis. Two-thirds of the patients had complete response after the initial treatment. Among them, 50% (34 patients) recurred, and of those 34 patients, 42% of them (14 patients) had recurrence in the posterior fossa.
Surgery with total resection of the tumor and absence of neoplasic cells in the CSF are effective predictors of better OS. Radiotherapy was more effective when a BED was greater than 44 Gy10.
评估在巴西国家癌症研究所(INCA)接受治疗的儿童原始神经外胚层肿瘤/髓母细胞瘤患者放疗时机的重要性。
对101例确诊为髓母细胞瘤的儿童记录进行回顾性分析。患者的中位随访时间为48个月(0.5 - 241个月)。年龄范围为0.8至17.5岁(中位年龄:7.6岁),21.7%的患者年龄为3岁或更小。
根据1983年至2001年接受髓母细胞瘤治疗患者收集的数据,总生存率(OS)为53%,无病生存率(DFS)为40%。多变量分析显示,3岁以下、就诊时脑脊液(CSF)中存在肿瘤细胞或肿瘤次全切除导致OS较差。接受生物等效剂量(BED)大于44 Gy10的患者预后较好。三分之二的患者在初始治疗后完全缓解。其中,50%(34例患者)复发,在这34例复发患者中,42%(14例患者)在后颅窝复发。
肿瘤全切除手术且脑脊液中无肿瘤细胞是OS较好的有效预测指标。当BED大于44 Gy10时,放疗更有效。