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Characteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry

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dc.contributor.author Bartlett, Allison L.
dc.contributor.author Lane, Adam
dc.contributor.author Chaney, Brooklyn
dc.contributor.author Yanez Escorza, Nancy
dc.contributor.author Black, Katie
dc.contributor.author Cochrane, Anne
dc.contributor.author Minturn, Jane
dc.contributor.author Bartels, Ute
dc.contributor.author Warren, Kathy
dc.contributor.author Hansford, Jordan
dc.contributor.author Ziegler, David
dc.contributor.author Diez, Blanca
dc.contributor.author Goldman, Stewart
dc.contributor.author Packer, Roger
dc.contributor.author Kieran, Mark
dc.contributor.author DeWire-Schottmiller, Mariko
dc.contributor.author Erker, Craig
dc.contributor.author Monje-Deisseroth, Michelle
dc.contributor.author Wagner, Lars
dc.contributor.author Koschmann, Carl
dc.date.accessioned 2023-01-10T10:55:21Z
dc.date.available 2023-01-10T10:55:21Z
dc.date.issued 2022-12-01
dc.identifier.citation Bartlett AL, Lane A, Chaney B, Escorza NY, Black K, Cochrane A, Minturn J, Bartels U, Warren K, Hansford J, Ziegler D, Diez B, Goldman S, Packer R, Kieran M, DeWire-Schottmiller M, Erker C, Monje-Deisseroth M, Wagner L, Koschmann C, Dorris K, Shih CS, Hassall T, Samson Y, Fisher P, Wang SS, Tsui K, Sevlever G, Zhu X, Dexheimer P, Asher A, Fuller C, Drissi R, Jones B, Leach J, Fouladi M. Characteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry. Neuro Oncol. 2022 Dec 1;24(12):2190-2199. doi: 10.1093/neuonc/noac123. es_ES
dc.identifier.uri https://doi.org/10.1093/neuonc/noac123
dc.identifier.uri https://repositorio.fleni.org.ar/xmlui/handle/123456789/767
dc.description.abstract Background: Children ≤36 months with diffuse intrinsic pontine glioma (DIPG) have increased long-term survival (LTS, overall survival (OS) ≥24 months). Understanding distinguishing characteristics in this population is critical to improving outcomes. Methods: Patients ≤36 months at diagnosis enrolled on the International DIPG Registry (IDIPGR) with central imaging confirmation were included. Presentation, clinical course, imaging, pathology and molecular findings were analyzed. Results: Among 1183 patients in IDIPGR, 40 were eligible (median age: 29 months). Median OS was 15 months. Twelve patients (30%) were LTS, 3 (7.5%) very long-term survivors ≥5 years. Among 8 untreated patients, median OS was 2 months. Patients enrolled in the registry but excluded from our study by central radiology review or tissue diagnosis had median OS of 7 months. All but 1 LTS received radiation. Among 32 treated patients, 1-, 2-, 3-, and 5-year OS rates were 68.8%, 31.2%, 15.6% and 12.5%, respectively. LTS had longer duration of presenting symptoms (P = .018). No imaging features were predictive of outcome. Tissue and genomic data were available in 18 (45%) and 10 patients, respectively. Among 9 with known H3K27M status, 6 had a mutation. Conclusions: Children ≤36 months demonstrated significantly more LTS, with an improved median OS of 15 months; 92% of LTS received radiation. Median OS in untreated children was 2 months, compared to 17 months for treated children. LTS had longer duration of symptoms. Excluded patients demonstrated a lower OS, contradicting the hypothesis that children ≤36 months with DIPG show improved outcomes due to misdiagnosis. es_ES
dc.language.iso eng es_ES
dc.publisher Oxford University Press es_ES
dc.rights info:eu-repo/semantics/openAccess
dc.rights.uri https://creativecommons.org/licenses/by/2.5/ar/
dc.subject Brain Neoplasms es_ES
dc.subject Neoplasias Encefálicas es_ES
dc.title Characteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry es_ES
dc.type info:eu-repo/semantics/article es_ES
dc.type info:eu-repo/semantics/publishedVersion
dc.description.fil Fil: Diez, Blanca. Fleni. Departamento de Neurología. Servicio de Neurooncología; Argentina.
dc.description.fil Fil: Bartlett, Allison L. Cincinnati Children's Hospital Medical Center; Estados Unidos.
dc.description.fil Fil: Lane, Adam. Cincinnati Children's Hospital Medical Center; Estados Unidos.
dc.description.fil Fil: Chaney, Brooklyn. Cincinnati Children's Hospital Medical Center; Estados Unidos.
dc.description.fil Fil: Yanez Escorza, Nancy. Cincinnati Children's Hospital Medical Center; Estados Unidos.
dc.description.fil Fil: Black, Katie. Cincinnati Children's Hospital Medical Center; Estados Unidos.
dc.description.fil Fil: Cochrane, Anne. Cincinnati Children's Hospital Medical Center; Estados Unidos. University of Cincinnati College of Medicine; Estados Unidos.
dc.description.fil Fil: Minturn, Jane. Children's Hospital of Philadelphia and Perelman School of Medicine; Estados Unidos.
dc.description.fil Fil: Bartels, Ute. University of Toronto and The Hospital for Sick Children; Canadá.
dc.description.fil Fil: Warren, Kathy. Dana Farber Cancer Institute/Boston Children's Hospital; Estados Unidos.
dc.description.fil Fil: Hansford, Jordan. University of Melbourne; Australia.
dc.description.fil Fil: Ziegler, David. Sydney's Children Hospital, Randwick; Australia. University of New South Wales; Australia.
dc.description.fil Fil: Goldman, Stewart. Ann & Robert H. Lurie Children's Hospital of Chicago; Estados Unidos.
dc.description.fil Fil: Packer, Roger. Children's National Hospital; Estados Unidos.
dc.description.fil Fil: Kieran, Mark. Dana-Farber/Boston Children's Cancer and Blood Disorders Center and Harvard Medical School; Estados Unidos.
dc.description.fil Fil: DeWire-Schottmiller, Mariko. Cincinnati Children's Hospital Medical Center; Estados Unidos.
dc.description.fil Fil: Erker, Craig. Dalhousie University and IWK Health Center; Canadá.
dc.description.fil Fil: Monje-Deisseroth, Michelle. Stanford University School of Medicine; Estados Unidos.
dc.description.fil Fil: Wagner, Lars. University of Kentucky; Estados Unidos.
dc.description.fil Fil: Koschmann, Carl. Mott Children's Hospital and University of Michigan School of Medicine; Estados Unidos.
dc.relation.ispartofVOLUME 24
dc.relation.ispartofNUMBER 12
dc.relation.ispartofPAGINATION 2190-2199.
dc.relation.ispartofCOUNTRY Inglaterra
dc.relation.ispartofCITY Oxford
dc.relation.ispartofTITLE Neuro-oncology
dc.relation.ispartofISSN 1523-5866
dc.type.snrd info:ar-repo/semantics/artículo es_ES


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