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Cancer Research and Treatment > Accepted Articles
doi: https://doi.org/10.4143/crt.2020.756    [Accepted]
Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System in Children Under the Age of 3 Years
Meerim Park1, Jung Woo Han2, Seung Min Hahn2, Jun Ah Lee1, Joo-Young Kim3, Sang-Hoon Shin4, Dong-Seok Kim5, Hong In Yoon6, Kyung Taek Hong7, Jung Yoon Choi7, Hyoung Jin Kang7, Hee Young Shin7, Ji Hoon Phi8, Seung-Ki Kim8, Ji Won Lee9, Keon Hee Yoo9, Ki Woong Sung9, Hong Hoe Koo9, Do Hoon Lim10, Hyung Jin Shin11, Hyery Kim12, Kyung Nam Koh12, Ho Joon Im12, Seung Do Ahn13, Young-Shin Ra14, Hee-Jo Baek15, Hoon Kook15, Tae-Young Jung16, Hyoung Soo Choi17, Chae-Yong Kim18, Hyeon Jin Park1, Chuhl Joo Lyu2
1Department of Pediatrics, Center for Pediatric Cancer, National Cancer Center, Goyang, Korea
2Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
3Department of Radiation Oncology, National Cancer Center, Goyang, Korea
4Neuro-Oncology Clinic, Center for Pediatric Cancer, National Cancer Center, Goyang, Korea
5Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea
6Department of Radiation Oncology, Yonsei University College of Medicine, Seoul, Korea
7Department of Pediatrics, Seoul National University Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
8Department of Neurosurgery, Seoul National University Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
9Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
10Department of Radiation Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
11Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
12Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
13Department of Radiation Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
14Department of Neurosurgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
15Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
16Department of Neurosurgery, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
17Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
18Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
Correspondence  Hyeon Jin Park ,Tel: 82-31-920-1654, Email: hjpark@ncc.re.kr
Chuhl Joo Lyu ,Tel: 82-2-2228-9149, Email: cj@yuhs.ac
Received: July 29, 2020;  Accepted: October 27, 2020.  Published online: October 28, 2020.
*Meerim Park and Jung Woo Han contributed equally to this work.
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 CNS 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 7 centers was performed between January 2005 and December 2016.
Results
Forty-three patients were enrolled. With a median follow-up of 90 months, 27 (64.3%) patients 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.
Key words: ATRT, Children under the age of 3 years, High dose chemotherapy, Radiotherapy
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