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Published in: Child's Nervous System 8/2017

01-08-2017 | Original Paper

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

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

Published in: Child's Nervous System | Issue 8/2017

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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.
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Metadata
Title
Large cell/anaplastic medulloblastoma is associated with poor prognosis—a retrospective analysis at a single institute
Authors
Pin-I Huang
Shih-Chieh Lin
Yi-Yen Lee
Donald Ming-Tak Ho
Wan-Yuo Guo
Kai-Ping Chang
Feng-Chi Chang
Muh-Lii Liang
Hsin-Hung Chen
Yu-Ming Liu
Sang-Hue Yen
Tai-Tong Wong
Yi-Wei Chen
Publication date
01-08-2017
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 8/2017
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-017-3435-9

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