Der-Shiun Wang1, Meng-Yao Lu2, Yung-Li Yang3, Dong-Tsamn Lin4, Kai-Hsin Lin5, Hsiu-Hao Chang6, Shiann-Tarng Jou7. 1. Department of Pediatrics, Tri-service General Hospital, National Defense Medical Center, Taipei, Taiwan; Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan; Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan. 2. Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Pediatrics, College of Medicine, National Taiwan University, Taipei, Taiwan. 3. Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Pediatrics, College of Medicine, National Taiwan University, Taipei, Taiwan; Department of Laboratory Medicine, National Taiwan University Hospital, Taipei, Taiwan. 4. Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Laboratory Medicine, National Taiwan University Hospital, Taipei, Taiwan. 5. Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan. 6. Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Pediatrics, College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address: changhh2001@ntu.edu.tw. 7. Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Pediatrics, College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address: stjou4@gmail.com.
Abstract
BACKGROUND/ PURPOSE: The Taiwan Pediatric Oncology Group (TPOG) initiated two consecutive protocols for treating pediatric patients with Langerhans cell histiocytosis (LCH) since 1994. However, the results have not been analyzed and reported. This study aimed to investigate the survival outcomes of childhood LCH at the National Taiwan University Hospital over the past 20 years. METHODS: Treatment of pediatric patients with LCH according to TPOG protocols at the National Taiwan University Hospital began in 1994. During 1994-2003, patients were treated using the TPOG LCH-94 protocol. After 2003, patients were treated using the TPOG LCH-2003 protocol. Clinical data of these patients were obtained retrospectively by reviewing electronic medical records. Patients were followed up until July 31, 2018. RESULTS: Fifty-three newly diagnosed pediatric patients with LCH were treated at National Taiwan University Hospital during 1994-2015. Twenty-nine (54.7%) were treated with the TPOG LCH-94 protocol, and 24 (45.3%) were treated with the TPOG LCH-2003 protocol. The 5-year event-free survival and overall survival rates were 96.2 ± 2.6% standard error (SE) and 98.1 ± 1.9% (SE), respectively. Overall survival and 5-year event-free survival between patients treated with the TPOG LCH-94 and TPOG LCH-2003 protocols showed no significant difference. Multisystem, liver, or spleen diseases were associated with significantly bad survival outcomes. Among at-risk-organ involvement in LCH, liver involvement was an independent factor for poor prognosis. CONCLUSION: Clinical outcomes of children with LCH in Taiwan was good. The results of this study may help in the better classification of risk grouping for protocol designs in the future.
BACKGROUND/ PURPOSE: The Taiwan Pediatric Oncology Group (TPOG) initiated two consecutive protocols for treating pediatric patients with Langerhans cell histiocytosis (LCH) since 1994. However, the results have not been analyzed and reported. This study aimed to investigate the survival outcomes of childhood LCH at the National Taiwan University Hospital over the past 20 years. METHODS: Treatment of pediatric patients with LCH according to TPOG protocols at the National Taiwan University Hospital began in 1994. During 1994-2003, patients were treated using the TPOG LCH-94 protocol. After 2003, patients were treated using the TPOG LCH-2003 protocol. Clinical data of these patients were obtained retrospectively by reviewing electronic medical records. Patients were followed up until July 31, 2018. RESULTS: Fifty-three newly diagnosed pediatric patients with LCH were treated at National Taiwan University Hospital during 1994-2015. Twenty-nine (54.7%) were treated with the TPOG LCH-94 protocol, and 24 (45.3%) were treated with the TPOG LCH-2003 protocol. The 5-year event-free survival and overall survival rates were 96.2 ± 2.6% standard error (SE) and 98.1 ± 1.9% (SE), respectively. Overall survival and 5-year event-free survival between patients treated with the TPOG LCH-94 and TPOG LCH-2003 protocols showed no significant difference. Multisystem, liver, or spleen diseases were associated with significantly bad survival outcomes. Among at-risk-organ involvement in LCH, liver involvement was an independent factor for poor prognosis. CONCLUSION: Clinical outcomes of children with LCH in Taiwan was good. The results of this study may help in the better classification of risk grouping for protocol designs in the future.