biostudies-literatureBartlett ALThe Gold Hope ProjectCure Brain CancerReflections Of GraceRyan’s HopeLaurie’s Love FoundationWhitley’s WishesKeris KaresLove Chloe FoundationStorm the Heavens FundThe Cure Starts Now AustraliaLily Larue FoundationCure Starts Now FoundationThe Isabella and Marcus FoundationSnapgrant.comThe DIPG CollaborativePray Hope BelieveAidan’s AvengersWayland Villars FoundationNINDS NIH HHSMarlee’s MissionRUN DIPGAmerican Childhood Cancer OrganizationRobert Connor Dawes FoundationBenny’s WorldKyler Strong FoundationAubreigh’s ArmyMusella FoundationAustin StrongBrooke Healey FoundationLauren’s Fight for CureJeffrey Thomas Hayden FoundationGabriella’s Smile Foundation2190-2199https://www.ebi.ac.uk/biostudies/studies/S-EPMC9713498biostudies-literatureUnknown24(12)<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusions</h4>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.Neuro-oncologyCharacteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry.PMC9713498R01 NS124607Packer RFuller CZiegler DDexheimer PJones BLeach JBartels UMonje-Deisseroth MLane AGoldman SHansford JDorris KBlack KErker CShih CSWagner LSamson YCochrane ADrissi RSevlever GBartlett ALMinturn JFouladi MEscorza NYKieran MChaney BFisher PKoschmann CHassall TZhu XDiez BDeWire-Schottmiller MWang SSTsui KWarren KAsher AfalseCharacteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry.<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusions</h4>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.2022-01-01T00:00:00Z2022 Dec2025-04-20T00:15:23.442Z2025-04-20T00:15:23.442ZS-EPMC97134983555245210.1093/neuonc/noac123