Ewing Sarcoma

Ewing sarcomas are characterized by oncogenic ews/ets translocations. However, these oncofusions do not determine the outcome. Patient fate is determined by metastasis, and the complex spreading process is far from being completely understood. Ewing sarcomas are childhood cancers with a low mutation...

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Langue:anglais
Publié: MDPI - Multidisciplinary Digital Publishing Institute 2025
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Accès en ligne:ONIX_20250812T110751_9783725834471_19
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collection Directory of Open Access Books
description Ewing sarcomas are characterized by oncogenic ews/ets translocations. However, these oncofusions do not determine the outcome. Patient fate is determined by metastasis, and the complex spreading process is far from being completely understood. Ewing sarcomas are childhood cancers with a low mutational rate, making biomarkers elusive. Mutations increase with relapse and mutagenic therapy exposure. A silent tumor genome limits targeted therapy approaches. Nevertheless, precision oncology approaches aim to enhance the therapeutic index of conventional chemotherapy with novel small molecules targeting epigenetics, metabolism, and stress responses. Although their genome is generally silent, Ewing sarcomas reactivate endogenous retro-elements. Their activation is linked to an inflammatory response and a prometastatic modulation of the microenvironment. Moreover, in most cases, these tumors are characterized by a scarcity of T cell infiltrates and a predominance of immunosuppressive myeloid cells, leading to inflammation and immunosuppression. These suppressive myeloid cells shield the tumor against adaptive immune attack, hampering the efficacy of chimeric antigen receptor or T cell receptor transgenic T cells. Overcoming these immunosuppressive mechanisms may enhance immunotherapeutic efficacy. One approach is the utilization of oncolytic viruses, which are genetically engineered to depend on their lytic cycle from metastatic drivers. They can induce immunogenic cell death and, particularly when in combination with cell cycle inhibitors, also have the potential to overcome barriers to immunotherapy.
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spelling doab-20.500.12854ir-1652632025-08-12T09:15:17Z Ewing Sarcoma Burdach, Stefan Dirksen, Uta Sorensen, Poul H. Ewing sarcoma translational research epigenetics targeted therapies T cells immunotherapy late effects and prevention thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general thema EDItEUR::P Mathematics and Science::PS Biology, life sciences Ewing sarcomas are characterized by oncogenic ews/ets translocations. However, these oncofusions do not determine the outcome. Patient fate is determined by metastasis, and the complex spreading process is far from being completely understood. Ewing sarcomas are childhood cancers with a low mutational rate, making biomarkers elusive. Mutations increase with relapse and mutagenic therapy exposure. A silent tumor genome limits targeted therapy approaches. Nevertheless, precision oncology approaches aim to enhance the therapeutic index of conventional chemotherapy with novel small molecules targeting epigenetics, metabolism, and stress responses. Although their genome is generally silent, Ewing sarcomas reactivate endogenous retro-elements. Their activation is linked to an inflammatory response and a prometastatic modulation of the microenvironment. Moreover, in most cases, these tumors are characterized by a scarcity of T cell infiltrates and a predominance of immunosuppressive myeloid cells, leading to inflammation and immunosuppression. These suppressive myeloid cells shield the tumor against adaptive immune attack, hampering the efficacy of chimeric antigen receptor or T cell receptor transgenic T cells. Overcoming these immunosuppressive mechanisms may enhance immunotherapeutic efficacy. One approach is the utilization of oncolytic viruses, which are genetically engineered to depend on their lytic cycle from metastatic drivers. They can induce immunogenic cell death and, particularly when in combination with cell cycle inhibitors, also have the potential to overcome barriers to immunotherapy. 2025-08-12T09:15:15Z 2025-08-12T09:15:15Z 2025 book ONIX_20250812T110751_9783725834471_19 9783725834471 9783725834488 https://directory.doabooks.org/handle/20.500.12854/165263 eng image/jpeg Attribution 4.0 International https://mdpi.com/books https://mdpi.com/books/pdfview/book/10936 MDPI - Multidisciplinary Digital Publishing Institute 10.3390/books978-3-7258-3448-8 10.3390/books978-3-7258-3448-8 46cabcaa-dd94-4bfe-87b4-55023c1b36d0 9783725834471 9783725834488 204 open access
spellingShingle Ewing sarcoma
translational research
epigenetics
targeted therapies
T cells
immunotherapy
late effects and prevention
thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general
thema EDItEUR::P Mathematics and Science::PS Biology, life sciences
Ewing Sarcoma
title Ewing Sarcoma
title_full Ewing Sarcoma
title_fullStr Ewing Sarcoma
title_full_unstemmed Ewing Sarcoma
title_short Ewing Sarcoma
title_sort ewing sarcoma
topic Ewing sarcoma
translational research
epigenetics
targeted therapies
T cells
immunotherapy
late effects and prevention
thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general
thema EDItEUR::P Mathematics and Science::PS Biology, life sciences
topic_facet Ewing sarcoma
translational research
epigenetics
targeted therapies
T cells
immunotherapy
late effects and prevention
thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general
thema EDItEUR::P Mathematics and Science::PS Biology, life sciences
url ONIX_20250812T110751_9783725834471_19