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Medulloblastoma: optimizing care with a multidisciplinary approach

Medulloblastoma is a malignant tumor of the cerebellum and the most frequent malignant brain tumor in children. The standard of care consists of maximal resection surgery, followed by craniospinal irradiation and chemotherapy. Such treatment allows long-term survival rates of nearly 70%; however, th...

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Autores principales: Thomas, Alice, Noël, Georges
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove Medical Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6498429/
https://www.ncbi.nlm.nih.gov/pubmed/31118657
http://dx.doi.org/10.2147/JMDH.S167808
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author Thomas, Alice
Noël, Georges
author_facet Thomas, Alice
Noël, Georges
author_sort Thomas, Alice
collection PubMed
description Medulloblastoma is a malignant tumor of the cerebellum and the most frequent malignant brain tumor in children. The standard of care consists of maximal resection surgery, followed by craniospinal irradiation and chemotherapy. Such treatment allows long-term survival rates of nearly 70%; however, there are wide disparities among patient outcomes, and in any case, major long-term morbidity is observed with conventional treatment. In the last two decades, the molecular understanding of medulloblastoma has improved drastically, allowing us to revolutionize our understanding of medulloblastoma pathophysiological mechanisms. These advances led to an international consensus in 2010 that defined four prognostic molecular subgroups named after their affected signaling pathways, that is, WNT, SHH, Group 3 and Group 4. The molecular understanding of medulloblastoma is starting to translate through to clinical settings due to the development of targeted therapies. Moreover, recent improvements in radiotherapy modalities and the reconsideration of craniospinal irradiation according to the molecular status hold promise for survival preservation and the reduction of radiation-induced morbidity. This review is an overview of the current knowledge of medulloblastoma through a molecular approach, and therapeutic prospects currently being developed in surgery, radiotherapy and targeted therapies to optimize the treatment of medulloblastoma with a multidisciplinary approach will also be discussed.
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spelling pubmed-64984292019-05-22 Medulloblastoma: optimizing care with a multidisciplinary approach Thomas, Alice Noël, Georges J Multidiscip Healthc Review Medulloblastoma is a malignant tumor of the cerebellum and the most frequent malignant brain tumor in children. The standard of care consists of maximal resection surgery, followed by craniospinal irradiation and chemotherapy. Such treatment allows long-term survival rates of nearly 70%; however, there are wide disparities among patient outcomes, and in any case, major long-term morbidity is observed with conventional treatment. In the last two decades, the molecular understanding of medulloblastoma has improved drastically, allowing us to revolutionize our understanding of medulloblastoma pathophysiological mechanisms. These advances led to an international consensus in 2010 that defined four prognostic molecular subgroups named after their affected signaling pathways, that is, WNT, SHH, Group 3 and Group 4. The molecular understanding of medulloblastoma is starting to translate through to clinical settings due to the development of targeted therapies. Moreover, recent improvements in radiotherapy modalities and the reconsideration of craniospinal irradiation according to the molecular status hold promise for survival preservation and the reduction of radiation-induced morbidity. This review is an overview of the current knowledge of medulloblastoma through a molecular approach, and therapeutic prospects currently being developed in surgery, radiotherapy and targeted therapies to optimize the treatment of medulloblastoma with a multidisciplinary approach will also be discussed. Dove Medical Press 2019-04-30 /pmc/articles/PMC6498429/ /pubmed/31118657 http://dx.doi.org/10.2147/JMDH.S167808 Text en © 2019 Thomas and Noël. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.
spellingShingle Review
Thomas, Alice
Noël, Georges
Medulloblastoma: optimizing care with a multidisciplinary approach
title Medulloblastoma: optimizing care with a multidisciplinary approach
title_full Medulloblastoma: optimizing care with a multidisciplinary approach
title_fullStr Medulloblastoma: optimizing care with a multidisciplinary approach
title_full_unstemmed Medulloblastoma: optimizing care with a multidisciplinary approach
title_short Medulloblastoma: optimizing care with a multidisciplinary approach
title_sort medulloblastoma: optimizing care with a multidisciplinary approach
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6498429/
https://www.ncbi.nlm.nih.gov/pubmed/31118657
http://dx.doi.org/10.2147/JMDH.S167808
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