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Atypical Teratoid/Rhabdoid Tumor in Taiwan: A Nationwide, Population-Based Study
| Content Provider | MDPI |
|---|---|
| Author | Liu, Yen-Lin Tsai, Min-Lan Chen, Chang-I Yar, Noi Tsai, Ching-Wen Lee, Hsin-Lun Kuo, Chia-Chun Ho, Wan-Ling Hsieh, Kevin Li-Chun Tseng, Sung-Hui Miser, James S. Chang, Chia-Yau Chang, Hsi Huang, Wen-Chang Wong, Tai-Tong Wu, Alexander T. H. Yen, Yu-Chun |
| Copyright Year | 2022 |
| Description | Background: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, highly aggressive embryonal brain tumor most commonly presenting in young children. Methods: We performed a nationwide, population-based study of AT/RT (ICD-O-3 code: 9508/3) in Taiwan using the Taiwan Cancer Registry Database and the National Death Certificate Database. Results: A total of 47 cases (male/female = 29:18; median age at diagnosis, 23.3 months (IQR: 12.5–87.9)) were diagnosed with AT/RT between 1999 and 2014. AT/RT had higher prevalence in males (61.70%), in children < 36 months (55.32%), and at infratentorial or spinal locations (46.81%). Survival analyses demonstrated that patients ≥ 3 years of age (n = 21 (45%)) had a 5y-OS of 41% (p < 0.0001), treatment with radiotherapy only (n = 5 (11%)) led to a 5y-OS of 60%, treatment with chemotherapy with or without radiotherapy (n = 27 (62%)) was associated with a 5y-OS of 45% (p < 0.0001), and patients with a supratentorial tumor (n = 11 (23%)) had a 5y-OS of 51.95%. Predictors of better survival on univariate Cox proportional hazard modeling and confirmed with multivariate analysis included older age (≥1 year), supratentorial sites, and the administration of radiotherapy, chemotherapy, or both. Gender had no effect on survival. Conclusion: Older age, supratentorial site, and treatment with radiotherapy, chemotherapy, or both significantly improves the survival of patients with AT/RT. |
| Starting Page | 668 |
| e-ISSN | 20726694 |
| DOI | 10.3390/cancers14030668 |
| Journal | Cancers |
| Issue Number | 3 |
| Volume Number | 14 |
| Language | English |
| Publisher | MDPI |
| Publisher Date | 2022-01-28 |
| Access Restriction | Open |
| Subject Keyword | Cancers Oncology Atypical Teratoid/rhabdoid Tumor Cns Tumors Pediatric Cancer Survival Outcome |
| Content Type | Text |
| Resource Type | Article |