RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literature

Objectives Myeloid neoplasms occurring after cytotoxic therapy (MN-pCT), previously termed therapy-related myelodysplastic neoplasia (MDS) or therapy-related acute myelogenous leukemia (AML), pose significant treatment challenges due to high resistance, poor chemotherapy tolerance, and relapse.Metho...

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Main Authors: Miriam Sandnes, Øystein Sefland, Irini Ktoridou-Valen, Rakel Brendsdal Forthun, Hilde Kollsete Gjelberg, Lars Henrik Dahl Hamnvik, Håkon Reikvam, Tor Henrik Andersson Tvedt
Format: Article
Language:English
Published: Taylor & Francis Group 2025-12-01
Series:Hematology
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Online Access:https://www.tandfonline.com/doi/10.1080/16078454.2025.2483094
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author Miriam Sandnes
Øystein Sefland
Irini Ktoridou-Valen
Rakel Brendsdal Forthun
Hilde Kollsete Gjelberg
Lars Henrik Dahl Hamnvik
Håkon Reikvam
Tor Henrik Andersson Tvedt
author_facet Miriam Sandnes
Øystein Sefland
Irini Ktoridou-Valen
Rakel Brendsdal Forthun
Hilde Kollsete Gjelberg
Lars Henrik Dahl Hamnvik
Håkon Reikvam
Tor Henrik Andersson Tvedt
author_sort Miriam Sandnes
collection DOAJ
description Objectives Myeloid neoplasms occurring after cytotoxic therapy (MN-pCT), previously termed therapy-related myelodysplastic neoplasia (MDS) or therapy-related acute myelogenous leukemia (AML), pose significant treatment challenges due to high resistance, poor chemotherapy tolerance, and relapse.Methods We present a 73-year-old woman with therapy-related AML following treatment for myxofibrosarcoma, characterized by the rare RUNX1::MECOM fusion resulting from a t(3;21)(q26;q22) chromosomal translocation. We also review the current literature regarding cases of this rare translocation.Results The patient, previously treated with chemotherapy and radiotherapy for sarcoma, was diagnosed with pancytopenia and hypoplastic bone marrow with increased blasts. Cytogenetic analysis confirmed RUNX1::MECOM fusion. Furthermore, we discuss the molecular mechanisms underlying MECOM rearrangements, specifically the role of the EVI1 oncogene. AML associated with MECOM rearrangements is associated with poor prognosis and resistance to conventional therapies. While allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the only potential curative treatment, the high relapse rate limits its efficacy. Recent advancements in understanding the molecular drivers of MECOM-related AML suggest potential therapeutic strategies, including hypomethylating agents and novel combinations such as lenalidomide.Conclusion this case and literature review emphasizes the importance of long-term monitoring of cancer survivors treated with cytotoxic therapies, as well as awareness of rare translocations in MN-pCT.
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spelling doaj-art-c27cc24c48a14d8f80f40b72aa561b472025-08-20T02:16:44ZengTaylor & Francis GroupHematology1607-84542025-12-0130110.1080/16078454.2025.2483094RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literatureMiriam Sandnes0Øystein Sefland1Irini Ktoridou-Valen2Rakel Brendsdal Forthun3Hilde Kollsete Gjelberg4Lars Henrik Dahl Hamnvik5Håkon Reikvam6Tor Henrik Andersson Tvedt7Section for Hematology, Department of Medicine, Haukeland University Hospital, Bergen, NorwaySection for Hematology, Department of Medicine, Haukeland University Hospital, Bergen, NorwaySection for Hematology, Department of Medicine, Haukeland University Hospital, Bergen, NorwayDepartment for Medical Genetics and Molecular Medicine, Haukeland University Hospital, Bergen, NorwayDepartment of Pathology, Haukeland University Hospital, Bergen, NorwaySection for Hematology, Institute of Clinical Medicine, University of Oslo, Oslo, NorwaySection for Hematology, Department of Medicine, Haukeland University Hospital, Bergen, NorwaySection for Hematology, Institute of Clinical Medicine, University of Oslo, Oslo, NorwayObjectives Myeloid neoplasms occurring after cytotoxic therapy (MN-pCT), previously termed therapy-related myelodysplastic neoplasia (MDS) or therapy-related acute myelogenous leukemia (AML), pose significant treatment challenges due to high resistance, poor chemotherapy tolerance, and relapse.Methods We present a 73-year-old woman with therapy-related AML following treatment for myxofibrosarcoma, characterized by the rare RUNX1::MECOM fusion resulting from a t(3;21)(q26;q22) chromosomal translocation. We also review the current literature regarding cases of this rare translocation.Results The patient, previously treated with chemotherapy and radiotherapy for sarcoma, was diagnosed with pancytopenia and hypoplastic bone marrow with increased blasts. Cytogenetic analysis confirmed RUNX1::MECOM fusion. Furthermore, we discuss the molecular mechanisms underlying MECOM rearrangements, specifically the role of the EVI1 oncogene. AML associated with MECOM rearrangements is associated with poor prognosis and resistance to conventional therapies. While allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the only potential curative treatment, the high relapse rate limits its efficacy. Recent advancements in understanding the molecular drivers of MECOM-related AML suggest potential therapeutic strategies, including hypomethylating agents and novel combinations such as lenalidomide.Conclusion this case and literature review emphasizes the importance of long-term monitoring of cancer survivors treated with cytotoxic therapies, as well as awareness of rare translocations in MN-pCT.https://www.tandfonline.com/doi/10.1080/16078454.2025.2483094acute myeloid leukemiaRUNX1::MECOMmyeloid neoplasms post-cytotoxic therapyimmunphenotypetranslocationfusion transcript
spellingShingle Miriam Sandnes
Øystein Sefland
Irini Ktoridou-Valen
Rakel Brendsdal Forthun
Hilde Kollsete Gjelberg
Lars Henrik Dahl Hamnvik
Håkon Reikvam
Tor Henrik Andersson Tvedt
RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literature
Hematology
acute myeloid leukemia
RUNX1::MECOM
myeloid neoplasms post-cytotoxic therapy
immunphenotype
translocation
fusion transcript
title RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literature
title_full RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literature
title_fullStr RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literature
title_full_unstemmed RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literature
title_short RUNX1::MECOM rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment: a case presentation and review of the literature
title_sort runx1 mecom rearrangement in myeloid neoplasm post cytotoxic therapy following sarcoma treatment a case presentation and review of the literature
topic acute myeloid leukemia
RUNX1::MECOM
myeloid neoplasms post-cytotoxic therapy
immunphenotype
translocation
fusion transcript
url https://www.tandfonline.com/doi/10.1080/16078454.2025.2483094
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