Acute lymphoblastic leukemia (ALL) is the number one cancer in children worldwide. Survival with ALL in children has shown a steady improvement over time with contemporary chemotherapy. This study aimed to determine the incidence rates and lifetime health impacts of pediatric ALL in Taiwan. A total of 3,854 patients with the diagnosis of ALL (ICD-9-CM code: 2040) were collected from the Registry for Catastrophic Illness (RCI) between 1997 and 2015 to assure the diagnostic accuracy. We included 2,044 cases for the final analysis. All patients were followed-up until the end of 2017 by linkage with the National Mortality Registry of Taiwan. A survival extrapolation method was applied and validated to estimate the lifetime survival function for life expectancy (LE) and loss-of-life expectancy (loss-of-life LE). The cohort included 1,222 males and 822 females. The average incidence rates (IRs) of pediatric ALL from 1997 to 2015 for age strata of <1, 1-4, 5-9, 10-14, 15-18 were 1.24, 3.39, 2.21, 1.56, and 0.97 per 100,000 person-year, respectively. The cumulative incidence rate up to age 18 (CIR0-18) of pediatric ALL increased after 2001, and was more pronounced in males than females. Most patients received treatments based on the protocol of Taiwan pediatric oncology group (TPOG)-ALL-97 (23.0%) or TPOG-ALL-2002 (62.7%). After extrapolation of survival to age 80, we found LE and loss-of-LE of pediatric ALL were 54.5 and 15.2 years, respectively. Future studies should explore long-term survival for different groups at risk of pediatric ALL and impacts of ALL on the society.

BACKGROUND: Diagnostic mIBG (meta-iodobenzylguanidine) scans are an integral component of response assessment in children with high-risk neuroblastoma. The role of end of induction (EOI) Curie Scores (CS) was previously described in patients undergoing a single autologous hematopoietic cell transplant (AHCT) as consolidation therapy. OBJECTIVE: We now examine the prognostic significance of CS in patients randomized to tandem AHCT on the Children’s Oncology Group (COG) trial ANBL0532. STUDY DESIGN: A retrospective analysis of mIBG scans obtained from patients enrolled in COG ANBL0532 was performed. Evaluable patients had mIBG-avid, International Neuroblastoma Staging System (INSS) stage 4 disease, did not progress during induction therapy, consented to consolidation randomization, and received a tandem AHCT (n=80). Optimal CS cut points maximized the outcome difference (≤ vs >CS cut-off) according to the Youden index. RESULTS: For recipients of tandem AHCT, the optimal cut point at diagnosis was CS=12, with superior EFS from study enrollment for patients with CS<12 (3-year EFS 74.2±7.9%) vs CS>12 (59.2±7.1%) (p=0.002). At EOI, the optimal cut point was CS=0, with superior end-induction EFS for patients with CS=0 (72.9±6.4%) vs CS>0 (46.5±9.1%) (p=0.002). CONCLUSION: In the setting of tandem transplantation for children with high-risk neuroblastoma, Curie scores at diagnosis and end-induction may identify a more favorable patient group. Patients treated with tandem AHCT who exhibited a CS<12 at diagnosis or CS=0 at EOI had superior EFS compared to those with CS above these cut points.