title: Outcome and Prognostic Factors forETV6/RUNX1Positive Pediatric Acute Lymphoblastic Leukemia Treated at a Single Institution in Korea.
abstract: OBJECTIVE
  ETV6/RUNX1(+) acute lymphoblastic leukemia (ALL), which is the most common genetic subtype of pediatric ALL, has a favorable prognosis. In this study, we analyzed the outcome ofETV6/RUNX1(+) ALL patients treated at our institution with the aim of identifying significant prognostic variables.
  
  
  METHODS
  Sixty-three patients were diagnosed withETV6/RUNX1(+) ALL from 2005 to 2011. Prognostic variables studied included minimal residual disease (MRD) as detected byETV6/RUNX1(+) fusion, and the presence of additional cytogenetic abnormalities.
  
  
  RESULTS
  The 5-year event-free survival was 84.1±4.6%, with 10 patients relapsing at a median of 28.3 months from diagnosis for a 5-year cumulative incidence of relapse of 15.9±4.6%. Multivariate analysis revealed that the presence MRD, as detected by real-time quantitative-polymerase chain reaction or fluorescencein situhybridization forETV6/RUNX1fusion at end of remission induction, and the presence of additional structural abnormalities of 12p (translocations or inversions) negatively affected outcome. Despite treatment such as allogeneic hematopoietic cell transplantation, eight of the 10 relapsed patients died from disease progression for overall survival of 82.5±6.9%.
  
  
  CONCLUSIONS
  ETV6/RUNX1(+) ALL may be heterogeneous in terms of prognosis, and variables such as MRD at end ofremission induction or additional structural abnormalities of 12p could define a subset of patients who are likely to have poor outcome.
authors: Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Jae Wook
authors: JW
authors: Lee
authors: Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Seong-Koo
authors: SK
authors: Kim
authors: Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Pil-Sang
authors: PS
authors: Jang
authors: Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Nack-Gyun
authors: NG
authors: Chung
authors: Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Dae-Chul
authors: DC
authors: Jeong
authors: Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Myungshin
authors: M
authors: Kim
authors: Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Bin
authors: B
authors: Cho
authors: Division of Hematology and Oncology, Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors: Hack-Ki
authors: HK
authors: Kim
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D050676:Core Binding Factor Alpha 2 Subunit / Q000235:genetics*; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D007621:Karyotyping; D008297:Male; D018365:Neoplasm, Residual / Q000175:diagnosis; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000235:genetics* / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D060888:Real-Time Polymerase Chain Reaction; D012008:Recurrence; D056910:Republic of Korea; D016019:Survival Analysis; D014178:Translocation, Genetic; D016896:Treatment Outcome; D000097764:ETS Translocation Variant 6 Protein
chemical_list: D050676:Core Binding Factor Alpha 2 Subunit; D015514:Oncogene Proteins, Fusion; C102421:TEL-AML1 fusion protein; D000097764:ETS Translocation Variant 6 Protein
keywords: 12p abnormalities; Acute lymphoblastic leukemia; ETV6/RUNX1; Minimal residual disease