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Published in: Journal of Neuro-Oncology 3/2020

01-05-2020 | Radiotherapy | Clinical Study

Effect of early radiotherapy initiation and high-dose chemotherapy on the prognosis of pediatric atypical teratoid rhabdoid tumors in different age groups

Authors: Wan-Chin Yang, Hsiu-Ju Yen, Muh-Lii Liang, Hsin-Hung Chen, Yi-Yen Lee, Feng-Chi Chang, Shih-Chieh Lin, Tai-Tong Wong, Yu-Wen Hu, Yi-Wei Chen

Published in: Journal of Neuro-Oncology | Issue 3/2020

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Abstract

Purpose

The optimal treatment strategy for pediatric atypical teratoid rhabdoid tumor (ATRT) is inconclusive. This study evaluated the prognostic value of early radiotherapy (RT) and high-dose chemotherapy with autologous stem cell rescue (HDC/ASCR) in pediatric ATRT.

Methods

This pooled analysis included ATRT patients treated at our institution and from other studies who were identified by a search of the PubMed electronic database. The effect of patient demographics and treatment profiles on progression-free survival (PFS) and overall survival (OS) were analyzed using Cox regression.

Results

Overall, 34 patients from our institution and 436 patients from 35 published studies were included. In multivariable analysis, patients with gross total resection (GTR), early RT (time to RT interval < 2 months), and HDC/ASCR had both better PFS [hazard ratio (HR) 0.46, p\(<\) 0.001; HR 0.64, p = 0.011; and HR 0.51, p = 0.005, respectively] and OS (HR 0.55, p = 0.002; HR 0.48, p = 0.004; and HR 0.42, p < 0.001, respectively). For patients aged < 3 years, both RT and HDC/ASCR were significant favorable factors for PFS (HR 0.32 and 0.46, respectively) and OS (HR 0.40 and 0.36, respectively), while early RT was not prognostic. For patients aged ≥ 3 years, early RT was significantly associated with better PFS (HR 0.51) and HDC/ASCR did not affect PFS, and neither was related to OS.

Conclusion

Both early RT initiation and HDC/ASCR were important components in the treatment of pediatric ATRT. However, the optimal treatment strategies might differ by age.
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Metadata
Title
Effect of early radiotherapy initiation and high-dose chemotherapy on the prognosis of pediatric atypical teratoid rhabdoid tumors in different age groups
Authors
Wan-Chin Yang
Hsiu-Ju Yen
Muh-Lii Liang
Hsin-Hung Chen
Yi-Yen Lee
Feng-Chi Chang
Shih-Chieh Lin
Tai-Tong Wong
Yu-Wen Hu
Yi-Wei Chen
Publication date
01-05-2020
Publisher
Springer US
Published in
Journal of Neuro-Oncology / Issue 3/2020
Print ISSN: 0167-594X
Electronic ISSN: 1573-7373
DOI
https://doi.org/10.1007/s11060-020-03456-1

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