Shosuke Sunami1, Masahiro Sekimizu2, Tetsuya Takimoto3, Tetsuya Mori4, Tetsuo Mitsui5, Reiji Fukano6, Akiko Moriya Saito7, Tomoyuki Watanabe8, Koichi Ohshima9, Junichiro Fujimoto3, Atsuko Nakazawa10, Ryoji Kobayashi11, Keizo Horibe12, Masahito Tsurusawa13. 1. Department of Pediatrics, Japanese Red Cross Narita Hospital, Narita, Japan. 2. Department of Pediatrics, National Hospital Organization, Nagoya Medical Center, Aichi, Japan. 3. Clinical Research Center, National Center for Child Health and Development, Tokyo, Japan. 4. Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan. 5. Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan. 6. Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan. 7. Laboratory of Clinical, Epidemiological and Health Services Research, Clinical Research Center, National Hospital Organization, Nagoya Medical Center, Aichi, Japan. 8. Department of Nutritional Science, Faculty of Psychological and Physical Science, Aichi Gakuin University, Aichi, Japan. 9. Department of Pathology, School of Medicine, Kurume University, Kurume, Japan. 10. Department of Pathology, National Center for Child Health and Development, Tokyo, Japan. 11. Department of Pediatrics, Sapporo Hokuyu Hospital, Sapporo, Japan. 12. Clinical Research Center, National Hospital Organization, Nagoya Medical Center, Aichi, Japan. 13. Advanced Medical Research Center, Aichi Medical University, Aichi, Japan.
Abstract
BACKGROUND: Childhood advanced lymphoblastic lymphoma (LBL) has a favorable outcome with an event-free survival (EFS) rate of over 80% in response to treatment strategies for acute lymphoblastic leukemia (ALL). However, no progress has been made in this outcome over the past 10 years. PROCEDURE: We conducted the first nationwide prospective study of childhood advanced LBL to assess the efficacy and safety of ALL-directed therapy with an intensified maintenance phase. We omitted local radiotherapy including prophylactic cranial radiotherapy except for patients with initial central nervous system disease. The total duration of the treatment was 24 months. RESULTS: For the 136 patients analyzed in this study, 5-year overall survival (OS) was 82.9% and 5-year EFS was 77.9%. Thirty events were observed and 14 occurred before the initiation of intensified maintenance phase. Of 14 events, nine were observed as mediastinal enlargement. There was no significant difference in outcome when stratified according to gender or by immunophenotype. The 5-year EFS according to clinical stage in patients with T-cell LBL (T-LBL) was 70.6% for stage III and 88.9% for stage IV (P = 0.037). CONCLUSIONS: Our first nationwide study provided about 80% cure rate with only one case of toxic death in childhood advanced LBL. However, our intensified maintenance therapy could not improve the survival outcome. There was a trend of better EFS in Japanese patients with T-LBL stage IV than T-LBL stage III.
BACKGROUND: Childhood advanced lymphoblastic lymphoma (LBL) has a favorable outcome with an event-free survival (EFS) rate of over 80% in response to treatment strategies for acute lymphoblastic leukemia (ALL). However, no progress has been made in this outcome over the past 10 years. PROCEDURE: We conducted the first nationwide prospective study of childhood advanced LBL to assess the efficacy and safety of ALL-directed therapy with an intensified maintenance phase. We omitted local radiotherapy including prophylactic cranial radiotherapy except for patients with initial central nervous system disease. The total duration of the treatment was 24 months. RESULTS: For the 136 patients analyzed in this study, 5-year overall survival (OS) was 82.9% and 5-year EFS was 77.9%. Thirty events were observed and 14 occurred before the initiation of intensified maintenance phase. Of 14 events, nine were observed as mediastinal enlargement. There was no significant difference in outcome when stratified according to gender or by immunophenotype. The 5-year EFS according to clinical stage in patients with T-cell LBL (T-LBL) was 70.6% for stage III and 88.9% for stage IV (P = 0.037). CONCLUSIONS: Our first nationwide study provided about 80% cure rate with only one case of toxic death in childhood advanced LBL. However, our intensified maintenance therapy could not improve the survival outcome. There was a trend of better EFS in Japanese patients with T-LBL stage IV than T-LBL stage III.
Authors: Robert J Hayashi; Stuart S Winter; Kimberly P Dunsmore; Meenakshi Devidas; Zhiguo Chen; Brent L Wood; Michelle L Hermiston; David T Teachey; Sherrie L Perkins; Rodney R Miles; Elizabeth A Raetz; Mignon L Loh; Naomi J Winick; William L Carroll; Stephen P Hunger; Megan S Lim; Thomas G Gross; Catherine M Bollard Journal: J Clin Oncol Date: 2020-06-17 Impact factor: 44.544