title: ETV6/RUNX1 fusion lacking prognostic effect in pediatric patients with acute lymphoblastic leukemia.
abstract: A Brazilian sample of 58 patients with acute lymphoblastic leukemia has been prospectively followed up with the objective of evaluating evolution of disease. Age ranged from 6 months to 16 years. Of the 58 patients, 11 were positive and 47 were negative for the ETV6/RUNX1 fusion (previously known as TEL/AML1). After a minimum follow-up period of 57 months, 2 of the ETV6/RUNX1(+) patients had died and 11 of the ETV6/RUNX1(-), for an overall survival of 77.6%. Among the 11 ETV6/RUNX1(+) patients (age range, 2 years to 13 years 7 months), all achieved a complete remission; the average overall survival was 64.2 months and the average event-free survival was 61.7 months. Among the 47 ETV6/RUNX1(-) patients, 4 did not have a complete remission; the average overall survival was of 60.8 months and the average event-free survival was 57.2 months. No significant difference was observed between overall survival and event-free survival, nor in any of the other data comparatively analyzed. The patients had a cure rate similar to that described in literature. In this small sample population, the presence of the ETV6/RUNX1 fusion did not identify statistical difference in prognosis.
authors.affiliation: Clinical Genetics and Post-Graduation Program in Pathology, Federal University of Health Sciences of Porto Alegre, Rua Sarmento Leite 245/403, Porto Alegre, 90050-170, RS, Brazil. paulozen@ufcspa.edu.br
authors.forename: Paulo R G
authors.initials: PR
authors.lastname: Zen
authors.forename: Marcelo E Z
authors.initials: ME
authors.lastname: Capra
authors.forename: Lúcia M R
authors.initials: LM
authors.lastname: Silla
authors.forename: Jiseh F
authors.initials: JF
authors.lastname: Loss
authors.forename: Mário S
authors.initials: MS
authors.lastname: Fernandes
authors.forename: Sidia M C
authors.initials: SM
authors.lastname: Jacques
authors.forename: Giorgio A
authors.initials: GA
authors.lastname: Paskulin
mesh_terms: D001938:Brazil; D002675:Child, Preschool; D050676:Core Binding Factor Alpha 2 Subunit / Q000235:genetics*; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000235:genetics*; D011379:Prognosis; D011446:Prospective Studies; D050783:Proto-Oncogene Proteins c-ets / Q000235:genetics*; D012074:Remission Induction; D012097:Repressor Proteins / Q000235:genetics*; D016019:Survival Analysis; D013997:Time Factors; D000097764:ETS Translocation Variant 6 Protein
chemical_list: D050676:Core Binding Factor Alpha 2 Subunit; D015514:Oncogene Proteins, Fusion; D050783:Proto-Oncogene Proteins c-ets; D012097:Repressor Proteins; D000097764:ETS Translocation Variant 6 Protein; C493728:RUNX1 protein, human