Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System in Children under the Age of 3 Years
- Author:
Meerim PARK
1
;
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
Author Information
- Publication Type:Original Article
- From:Cancer Research and Treatment 2021;53(2):378-388
- CountryRepublic of Korea
- Language:English
-
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.
