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

• Published in: Cancer research and treatment Vol. 53; no. 2; pp. 378 - 388
• Main Authors: Park, Meerim, Han, Jung Woo, Hahn, Seung Min, Lee, Jun Ah, Kim, Joo-Young, Shin, Sang Hoon, Kim, Dong-Seok, Yoon, Hong In, Hong, Kyung Taek, Choi, Jung Yoon, Kang, Hyoung Jin, Shin, Hee Young, Phi, Ji Hoon, Kim, Seung-Ki, Lee, Ji Won, Yoo, Keon Hee, Sung, Ki Woong, Koo, Hong Hoe, Lim, Do Hoon, Shin, Hyung Jin, Kim, Hyery, Koh, Kyung-Nam, Im, Ho Joon, Ahn, Seung Do, Ra, Young-Shin, Baek, Hee-Jo, Kook, Hoon, Jung, Tae-Young, Choi, Hyoung Soo, Kim, Chae-Yong, Park, Hyeon Jin, Lyu, Chuhl Joo
• Format: Journal Article
• Language: English
• Published: Korea (South) Korean Cancer Association 01.04.2021; 대한암학회
• Subjects: Central Nervous System Neoplasms - diagnosis; Central Nervous System Neoplasms - mortality; Central Nervous System Neoplasms - pathology; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Original; Progression-Free Survival; Rhabdoid Tumor - diagnosis; Rhabdoid Tumor - mortality; Rhabdoid Tumor - pathology; 의학일반; ATRT; Children; Radiotherapy; High-dose chemotherapy
• ISSN: 1598-2998; 2005-9256; 2005-9256
• DOI: 10.4143/crt.2020.756

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.