PMID- 31882550 OWN - NLM STAT- MEDLINE DCOM- 20200602 LR - 20200602 IS - 1790-6245 (Electronic) IS - 1109-6535 (Print) IS - 1109-6535 (Linking) VI - 17 IP - 1 DP - 2020 Jan-Feb TI - Chromosome Translocation t(14;21)(q11;q22) Activates Both OLIG1 and OLIG2 in Pediatric T-cell Lymphoblastic Malignancies and May Signify Adverse Prognosis. PG - 41-48 LID - 10.21873/cgp.20166 [doi] AB - BACKGROUND/AIM: The chromosome translocation t(14;21)(q11;q22) was reported in four pediatric T-cell lymphoblastic leukemias and was shown to activate the OLIG2 gene. MATERIALS AND METHODS: A pediatric T-cell lymphoblastic lymphoma was investigated using G-banding chromosome analysis, fluorescence in situ hybridization (FISH), and immunocytochemistry. RESULTS: The malignant cells carried a t(14;21)(q11;q22) aberration. The translocation moves the enhancer elements of TRA/TRD from band 14q11 to 21q22, a few thousands kbp downstream of OLIG1 and OLIG2, resulting in the production of both OLIG1 and OLIG2 proteins. CONCLUSION: The translocation t(14;21)(q11;q22) occurs in some pediatric T-cell lymphoblastic malignancies. Activation of both OLIG1 and OLIG2 by t(14;21)(q11;q22) in T-lymphoblasts and the ensuing deregulation of thousands of genes could explain the highly malignant disease and resistance to treatment that has characterized this small group of patients. CI - Copyright(c) 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved. FAU - Panagopoulos, Ioannis AU - Panagopoulos I AD - Section for Cancer Cytogenetics, Institute for Cancer Genetics and Informatics, The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway ioannis.panagopoulos@rr-research.no. FAU - Gorunova, Ludmila AU - Gorunova L AD - Section for Cancer Cytogenetics, Institute for Cancer Genetics and Informatics, The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway. FAU - Johannsdottir, Inga Maria Rinvoll AU - Johannsdottir IMR AD - Department of Pediatric Cancer and Blood Disorders, Oslo University Hospital, Oslo, Norway. AD - National Advisory Unit on Late Effects after Cancer Treatment, Oslo University Hospital, Oslo, Norway. FAU - Andersen, Kristin AU - Andersen K AD - Section for Cancer Cytogenetics, Institute for Cancer Genetics and Informatics, The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway. FAU - Holth, Arild AU - Holth A AD - Department of Pathology, The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway. FAU - Beiske, Klaus AU - Beiske K AD - Department of Pathology, The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway. AD - Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway. FAU - Heim, Sverre AU - Heim S AD - Section for Cancer Cytogenetics, Institute for Cancer Genetics and Informatics, The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway. AD - Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway. LA - eng PT - Case Reports PT - Journal Article PL - Greece TA - Cancer Genomics Proteomics JT - Cancer genomics & proteomics JID - 101188791 RN - 0 (Basic Helix-Loop-Helix Transcription Factors) RN - 0 (Nerve Tissue Proteins) RN - 0 (OLIG1 protein, human) RN - 0 (OLIG2 protein, human) RN - 0 (Oligodendrocyte Transcription Factor 2) SB - IM MH - Adolescent MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Basic Helix-Loop-Helix Transcription Factors/*genetics MH - Chromosome Banding MH - Chromosomes, Human, Pair 14/*genetics MH - Chromosomes, Human, Pair 21/*genetics MH - Fatal Outcome MH - Humans MH - In Situ Hybridization, Fluorescence MH - Male MH - Nerve Tissue Proteins/*genetics MH - Oligodendrocyte Transcription Factor 2/*genetics MH - Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy/genetics/*pathology MH - Prognosis MH - *Translocation, Genetic PMC - PMC6937122 OTO - NOTNLM OT - OLIG1 expression OT - OLIG2 expression OT - Pediatric T-cell lymphoblastic lymphoma OT - TRA/TRD enhancer OT - chromosome translocation t(14;21)(q11;q22) OT - unfavorable prognosis COIS- The Authors declare that they have no potential conflicts of interest. EDAT- 2019/12/29 06:00 MHDA- 2020/06/03 06:00 PMCR- 2020/01/03 CRDT- 2019/12/29 06:00 PHST- 2019/10/27 00:00 [received] PHST- 2019/11/11 00:00 [revised] PHST- 2019/11/15 00:00 [accepted] PHST- 2019/12/29 06:00 [entrez] PHST- 2019/12/29 06:00 [pubmed] PHST- 2020/06/03 06:00 [medline] PHST- 2020/01/03 00:00 [pmc-release] AID - 17/1/41 [pii] AID - 10.21873/cgp.20166 [doi] PST - ppublish SO - Cancer Genomics Proteomics. 2020 Jan-Feb;17(1):41-48. doi: 10.21873/cgp.20166.