Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapy
Diffuse intrinsic pontine glioma (DIPG) is a lethal brainstem tumor that most commonly occurs in young children between the ages of five and ten. DIPG is the leading cause of brain tumor-related death in childhood, with most patients succumbing to the disease within two years of diagnosis. Attempts...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2023-06-01
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| Series: | Pediatric Hematology Oncology Journal |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2468124523000104 |
| _version_ | 1797790035639009280 |
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| author | Andrew Groves Pratiti Bandopadhayay Tabitha M. Cooney |
| author_facet | Andrew Groves Pratiti Bandopadhayay Tabitha M. Cooney |
| author_sort | Andrew Groves |
| collection | DOAJ |
| description | Diffuse intrinsic pontine glioma (DIPG) is a lethal brainstem tumor that most commonly occurs in young children between the ages of five and ten. DIPG is the leading cause of brain tumor-related death in childhood, with most patients succumbing to the disease within two years of diagnosis. Attempts to improve survival through clinical trials have been futile thus far, with standard of care remaining focal radiation which provides only modest benefit. While tumor tissue is not considered necessary for diagnosis and management unless there are atypical features, the development of stereotactic biopsy through neurosurgical technical advancements and the brave donations of patients and their families has allowed for a vast increase in our understanding of DIPG tumorigenesis. This practice also allowed for the development of in-vitro and in-vivo models which have proven instrumental for testing pre-clinical hypotheses and designing the next generation of biologically-informed clinical trials. In this review, we will detail the epigenetic and molecular drivers of DIPG, as well as the current outlook for targeted therapies. |
| first_indexed | 2024-03-13T01:59:02Z |
| format | Article |
| id | doaj.art-1fb92aeb403d4033a03e822146660add |
| institution | Directory Open Access Journal |
| issn | 2468-1245 |
| language | English |
| last_indexed | 2024-03-13T01:59:02Z |
| publishDate | 2023-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatric Hematology Oncology Journal |
| spelling | doaj.art-1fb92aeb403d4033a03e822146660add2023-07-02T04:17:02ZengElsevierPediatric Hematology Oncology Journal2468-12452023-06-01827379Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapyAndrew Groves0Pratiti Bandopadhayay1Tabitha M. Cooney2Division of Hematology/Oncology, University of Iowa Stead Family Children's Hospital, Iowa City, IA, USA; Corresponding author. Division of Pediatric Hematology/Oncology, University of Iowa Hospitals and Clinics 1327 BT, 200 Hawkins Dr, Iowa City, IA, 52242, USA.Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA, USA; Department of Pediatrics, Harvard Medical School, USA; Broad Institute of MIT and Harvard, USADana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA, USA; Department of Pediatrics, Harvard Medical School, USADiffuse intrinsic pontine glioma (DIPG) is a lethal brainstem tumor that most commonly occurs in young children between the ages of five and ten. DIPG is the leading cause of brain tumor-related death in childhood, with most patients succumbing to the disease within two years of diagnosis. Attempts to improve survival through clinical trials have been futile thus far, with standard of care remaining focal radiation which provides only modest benefit. While tumor tissue is not considered necessary for diagnosis and management unless there are atypical features, the development of stereotactic biopsy through neurosurgical technical advancements and the brave donations of patients and their families has allowed for a vast increase in our understanding of DIPG tumorigenesis. This practice also allowed for the development of in-vitro and in-vivo models which have proven instrumental for testing pre-clinical hypotheses and designing the next generation of biologically-informed clinical trials. In this review, we will detail the epigenetic and molecular drivers of DIPG, as well as the current outlook for targeted therapies.http://www.sciencedirect.com/science/article/pii/S2468124523000104DIPGDiffuse intrinsic pontine gliomaEpigeneticsTargeted therapy |
| spellingShingle | Andrew Groves Pratiti Bandopadhayay Tabitha M. Cooney Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapy Pediatric Hematology Oncology Journal DIPG Diffuse intrinsic pontine glioma Epigenetics Targeted therapy |
| title | Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapy |
| title_full | Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapy |
| title_fullStr | Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapy |
| title_full_unstemmed | Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapy |
| title_short | Diffuse intrinsic pontine glioma: Insights into oncogenesis and opportunities for targeted therapy |
| title_sort | diffuse intrinsic pontine glioma insights into oncogenesis and opportunities for targeted therapy |
| topic | DIPG Diffuse intrinsic pontine glioma Epigenetics Targeted therapy |
| url | http://www.sciencedirect.com/science/article/pii/S2468124523000104 |
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