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Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System in Children under the Age of 3 Years

Authors
 Meerim Park  ;  Jung Woo Han  ;  Seung Min Hahn  ;  Jun Ah Lee  ;  Joo-Young Kim  ;  Sang Hoon Shin  ;  Dong-Seok Kim  ;  Hong In Yoon  ;  Kyung Taek Hong  ;  Jung Yoon Choi  ;  Hyoung Jin Kang  ;  Hee Young Shin  ;  Ji Hoon Phi  ;  Seung-Ki Kim  ;  Ji Won Lee  ;  Keon Hee Yoo  ;  Ki Woong Sung  ;  Hong Hoe Koo  ;  Do Hoon Lim  ;  Hyung Jin Shin  ;  Hyery Kim  ;  Kyung-Nam Koh  ;  Ho Joon Im  ;  Seung Do Ahn  ;  Young-Shin Ra  ;  Hee-Jo Baek  ;  Hoon Kook  ;  Tae-Young Jung  ;  Hyoung Soo Choi  ;  Chae-Yong Kim  ;  Hyeon Jin Park  ;  Chuhl Joo Lyu 
Citation
 CANCER RESEARCH AND TREATMENT, Vol.53(2) : 378-388, 2021-04 
Journal Title
 CANCER RESEARCH AND TREATMENT 
ISSN
 1598-2998 
Issue Date
2021-04
Keywords
ATRT ; Children ; High-dose chemotherapy ; Radiotherapy
Abstract
Purpose: Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. Materials and methods: A search of medical records from seven centers was performed between January 2005 and December 2016. Results: Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). Conclusion: Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.
Files in This Item:
T202101446.pdf Download
DOI
10.4143/crt.2020.756
Appears in Collections:
1. College of Medicine (의과대학) > Others (기타) > 1. Journal Papers
Yonsei Authors
Lyu, Chuhl Joo(유철주) ORCID logo https://orcid.org/0000-0001-7124-7818
Hahn, Seung Min(한승민) ORCID logo https://orcid.org/0000-0001-9832-6380
Han, Jung Woo(한정우) ORCID logo https://orcid.org/0000-0001-8936-1205
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/182853
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