Characteristics of patients ≥10 years of age with diffuse intrinsic pontine glioma: a report from the International DIPG/DMG Registry

Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in th...

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Vydáno v:Neuro-oncology (Charlottesville, Va.) Ročník 24; číslo 1; s. 141
Hlavní autoři: Erker, Craig, Lane, Adam, Chaney, Brooklyn, Leary, Sarah, Minturn, Jane E, Bartels, Ute, Packer, Roger J, Dorris, Kathleen, Gottardo, Nicholas G, Warren, Katherine E, Broniscer, Alberto, Kieran, Mark W, Zhu, Xiaoting, White, Peter, Dexheimer, Phillip J, Black, Katie, Asher, Anthony, DeWire, Mariko, Hansford, Jordan R, Gururangan, Sridharan, Nazarian, Javad, Ziegler, David S, Sandler, Eric, Bartlett, Allison, Goldman, Stewart, Shih, Chie-Schin, Hassall, Tim, Dholaria, Hetal, Bandopadhayay, Pratiti, Samson, Yvan, Monje, Michelle, Fisher, Paul G, Dodgshun, Andrew, Parkin, Sarah, Chintagumpala, Murali, Tsui, Karen, Gass, David, Larouche, Valerie, Broxson, Emmett, Garcia Lombardi, Mercedes, Wang, Stacie Shiqi, Ma, Jie, Hawkins, Cynthia, Hamideh, Dima, Wagner, Lars, Koschmann, Carl, Fuller, Christine, Drissi, Rachid, Jones, Blaise V, Leach, James, Fouladi, Maryam
Médium: Journal Article
Jazyk:angličtina
Vydáno: England 05.01.2022
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ISSN:1523-5866, 1523-5866
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Abstract Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR). Patients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included. The primary outcome was overall survival (OS) categorized as long-term survivors (LTS) (≥24 months) or short-term survivors (STS) (<24 months). Among 1010 patients, 208 (21%) were ≥10 years of age at diagnosis; 152 were eligible with a median age of 12 years (range 10-26.8). Median OS was 13 (2-82) months. The 1-, 3-, and 5-year OS was 59.2%, 5.3%, and 3.3%, respectively. The 18/152 (11.8%) LTS were more likely to be older (P < .01) and present with longer symptom duration (P < .01). Biopsy and/or autopsy were performed in 50 (33%) patients; 77%, 61%, 33%, and 6% of patients tested had H3K27M (H3F3A or HIST1H3B), TP53, ATRX, and ACVR1 mutations/genome alterations, respectively. Two of 18 patients with IDH1 testing were IDH1-mutant and 1 was a LTS. The presence or absence of H3 alterations did not affect survival. Patients ≥10 years old with DIPG have a median survival of 13 months. LTS present with longer symptom duration and are likely to be older at presentation compared to STS. ATRX mutation rates were higher in this population than the general DIPG population.
AbstractList Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR).BACKGROUNDDiffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR).Patients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included. The primary outcome was overall survival (OS) categorized as long-term survivors (LTS) (≥24 months) or short-term survivors (STS) (<24 months).METHODSPatients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included. The primary outcome was overall survival (OS) categorized as long-term survivors (LTS) (≥24 months) or short-term survivors (STS) (<24 months).Among 1010 patients, 208 (21%) were ≥10 years of age at diagnosis; 152 were eligible with a median age of 12 years (range 10-26.8). Median OS was 13 (2-82) months. The 1-, 3-, and 5-year OS was 59.2%, 5.3%, and 3.3%, respectively. The 18/152 (11.8%) LTS were more likely to be older (P < .01) and present with longer symptom duration (P < .01). Biopsy and/or autopsy were performed in 50 (33%) patients; 77%, 61%, 33%, and 6% of patients tested had H3K27M (H3F3A or HIST1H3B), TP53, ATRX, and ACVR1 mutations/genome alterations, respectively. Two of 18 patients with IDH1 testing were IDH1-mutant and 1 was a LTS. The presence or absence of H3 alterations did not affect survival.RESULTSAmong 1010 patients, 208 (21%) were ≥10 years of age at diagnosis; 152 were eligible with a median age of 12 years (range 10-26.8). Median OS was 13 (2-82) months. The 1-, 3-, and 5-year OS was 59.2%, 5.3%, and 3.3%, respectively. The 18/152 (11.8%) LTS were more likely to be older (P < .01) and present with longer symptom duration (P < .01). Biopsy and/or autopsy were performed in 50 (33%) patients; 77%, 61%, 33%, and 6% of patients tested had H3K27M (H3F3A or HIST1H3B), TP53, ATRX, and ACVR1 mutations/genome alterations, respectively. Two of 18 patients with IDH1 testing were IDH1-mutant and 1 was a LTS. The presence or absence of H3 alterations did not affect survival.Patients ≥10 years old with DIPG have a median survival of 13 months. LTS present with longer symptom duration and are likely to be older at presentation compared to STS. ATRX mutation rates were higher in this population than the general DIPG population.CONCLUSIONPatients ≥10 years old with DIPG have a median survival of 13 months. LTS present with longer symptom duration and are likely to be older at presentation compared to STS. ATRX mutation rates were higher in this population than the general DIPG population.
Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR). Patients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included. The primary outcome was overall survival (OS) categorized as long-term survivors (LTS) (≥24 months) or short-term survivors (STS) (<24 months). Among 1010 patients, 208 (21%) were ≥10 years of age at diagnosis; 152 were eligible with a median age of 12 years (range 10-26.8). Median OS was 13 (2-82) months. The 1-, 3-, and 5-year OS was 59.2%, 5.3%, and 3.3%, respectively. The 18/152 (11.8%) LTS were more likely to be older (P < .01) and present with longer symptom duration (P < .01). Biopsy and/or autopsy were performed in 50 (33%) patients; 77%, 61%, 33%, and 6% of patients tested had H3K27M (H3F3A or HIST1H3B), TP53, ATRX, and ACVR1 mutations/genome alterations, respectively. Two of 18 patients with IDH1 testing were IDH1-mutant and 1 was a LTS. The presence or absence of H3 alterations did not affect survival. Patients ≥10 years old with DIPG have a median survival of 13 months. LTS present with longer symptom duration and are likely to be older at presentation compared to STS. ATRX mutation rates were higher in this population than the general DIPG population.
Author Chintagumpala, Murali
Samson, Yvan
Packer, Roger J
Shih, Chie-Schin
Black, Katie
Hansford, Jordan R
Dholaria, Hetal
Fouladi, Maryam
Dodgshun, Andrew
Zhu, Xiaoting
Gururangan, Sridharan
Wang, Stacie Shiqi
Bartels, Ute
Wagner, Lars
Dexheimer, Phillip J
DeWire, Mariko
Hawkins, Cynthia
Sandler, Eric
Garcia Lombardi, Mercedes
Minturn, Jane E
Hassall, Tim
Asher, Anthony
Broniscer, Alberto
Bandopadhayay, Pratiti
Fisher, Paul G
Monje, Michelle
Tsui, Karen
Kieran, Mark W
Broxson, Emmett
White, Peter
Dorris, Kathleen
Koschmann, Carl
Erker, Craig
Gottardo, Nicholas G
Parkin, Sarah
Hamideh, Dima
Lane, Adam
Nazarian, Javad
Drissi, Rachid
Leach, James
Bartlett, Allison
Larouche, Valerie
Chaney, Brooklyn
Ziegler, David S
Warren, Katherine E
Jones, Blaise V
Goldman, Stewart
Leary, Sarah
Gass, David
Fuller, Christine
Ma, Jie
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Keywords International DIPG Registry
DIPG
outcomes
10 years old
Language English
License The Author(s) 2021. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
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PublicationTitle Neuro-oncology (Charlottesville, Va.)
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Snippet Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this...
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SubjectTerms Adolescent
Adult
Astrocytoma
Brain Stem Neoplasms - genetics
Child
Diffuse Intrinsic Pontine Glioma
Glioma - genetics
Humans
Registries
Young Adult
Title Characteristics of patients ≥10 years of age with diffuse intrinsic pontine glioma: a report from the International DIPG/DMG Registry
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