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...

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Published in:Cancer genetics and cytogenetics 2009-01, Vol.188 (2), p.112-117
Main Authors: Zen, Paulo R.G, Capra, Marcelo E.Z, Silla, Lúcia M.R, Loss, Jiseh F, Fernandes, Mário S, Jacques, Sidia M.C, Paskulin, Giorgio A
Format: Article
Language:English
Subjects:
Brazil
Child, Preschool
Core Binding Factor Alpha 2 Subunit - genetics
Disease-Free Survival
ETS Translocation Variant 6 Protein
Female
Follow-Up Studies
Hematology, Oncology and Palliative Medicine
Humans
Immunophenotyping
Karyotyping
Male
Medical Education
Oncogene Proteins, Fusion - genetics
Precursor Cell Lymphoblastic Leukemia-Lymphoma - diagnosis
Precursor Cell Lymphoblastic Leukemia-Lymphoma - genetics
Prognosis
Prospective Studies
Proto-Oncogene Proteins c-ets - genetics
Remission Induction
Repressor Proteins - genetics
Survival Analysis
Time Factors
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Summary: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.
ISSN:0165-4608
1873-4456
DOI:10.1016/j.cancergencyto.2008.09.005