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大细胞/间变性髓母细胞瘤与预后不良相关——单机构回顾性分析

Large cell/anaplastic medulloblastoma is associated with poor prognosis-a retrospective analysis at a single institute.

作者信息

Huang Pin-I, Lin Shih-Chieh, Lee Yi-Yen, Ho Donald Ming-Tak, Guo Wan-Yuo, Chang Kai-Ping, Chang Feng-Chi, Liang Muh-Lii, Chen Hsin-Hung, Liu Yu-Ming, Yen Sang-Hue, Wong Tai-Tong, Chen Yi-Wei

机构信息

Division of Radiotherapy, Department of Oncology, Taipei Veterans General Hospital, 201, Sec. 2, Shih-Pai Road, Taipei, 11217, Taiwan.

Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan.

出版信息

Childs Nerv Syst. 2017 Aug;33(8):1285-1294. doi: 10.1007/s00381-017-3435-9. Epub 2017 May 9.

Abstract

PURPOSE

Medulloblastoma (MB) is the most commonly occurring malignant pediatric brain tumor worldwide. However, a recent study found that the treatment outcomes in those with high-risk disease receiving conventional treatment were suboptimal. This study aimed to assess outcomes and treatment strategies for specific histologic subtypes of pediatric MB.

METHODS

A total of 114 pediatric patients (age < 20 years) diagnosed with MB between March 1998 and August 2011 were retrospectively reviewed; 52 that were treated with surgery followed by adjuvant radiotherapy (RT) and chemotherapy (CHT) were included.

RESULTS

The 5-year overall survival (OS) and relapse-free survival (RFS) rates were 73 and 69%, respectively. Median time to relapse was 17 months with a median survival time of 6 months after relapse. Patients of average risk had a better 5-year OS rate compared with high-risk patients (p = 0.027). The 5-year RFS of high-risk patients was lower compared with average risk (p = 0.038). A greater proportion of patients with large cell/anaplastic (LC/A) MB had recurrence than classic MB with 5-year RFS rate of 34 and 76%, respectively (p = 0.001), and OS rate of 56 and 76%, respectively (p = 0.04).

CONCLUSION

High-risk group and histology of LC/A were the most significant factors associated with worse OS and RFS. Patients with LC/A-MB had higher relapse rates and worse survival than those with classic MB. LC/A-MB carries a high risk for recurrence and should be treated with the more aggressive strategies.

摘要

目的

髓母细胞瘤(MB)是全球最常见的儿童恶性脑肿瘤。然而,最近一项研究发现,接受传统治疗的高危疾病患者的治疗效果并不理想。本研究旨在评估儿童MB特定组织学亚型的治疗效果和治疗策略。

方法

回顾性分析了1998年3月至2011年8月期间诊断为MB的114例儿科患者(年龄<20岁);其中52例接受了手术,随后进行辅助放疗(RT)和化疗(CHT)。

结果

5年总生存率(OS)和无复发生存率(RFS)分别为73%和69%。复发的中位时间为17个月,复发后中位生存时间为6个月。中危患者的5年OS率高于高危患者(p = 0.027)。高危患者的5年RFS低于中危患者(p = 0.038)。大细胞/间变性(LC/A)MB患者的复发比例高于经典MB患者,5年RFS率分别为34%和76%(p = 0.001),OS率分别为56%和76%(p = 0.04)。

结论

高危组和LC/A组织学是与较差的OS和RFS相关的最显著因素。与经典MB患者相比,LC/A-MB患者的复发率更高,生存率更差。LC/A-MB具有较高的复发风险,应采用更积极的治疗策略。

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