| Title: |
Characteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry |
| Authors: |
Bartlett, Allison L; Lane, Adam; Chaney, Brooklyn; Escorza, Nancy Yanez; Black, Katie; Cochrane, Anne; Minturn, Jane; Bartels, Ute; Warren, Kathy; Hansford, Jordan; Ziegler, David; Diez, Blanca; Goldman, Stewart; Packer, Roger; Kieran, Mark; DeWire-Schottmiller, Mariko; Erker, Craig; Monje-Deisseroth, Michelle; Wagner, Lars; Koschmann, Carl; Dorris, Kathleen; Shih, Chie-Schin; Hassall, Tim; Samson, Yvan; Fisher, Paul; Wang, Stacie S; Tsui, Karen; Sevlever, Gustavo; Zhu, Xiaoting; Dexheimer, Phillip; Asher, Anthony; Fuller, Christine; Drissi, Rachid; Jones, Blaise; Leach, James; Fouladi, Maryam |
| Contributors: |
Cure Starts Now Foundation; The Cure Starts Now Australia; Brooke Healey Foundation; Wayland Villars Foundation; Aidan’s Avengers; Aubreigh’s Army; Austin Strong; Cure Brain Cancer; Jeffrey Thomas Hayden Foundation; Laurie’s Love Foundation; Love Chloe Foundation; Musella Foundation; Pray Hope Believe; Reflections Of Grace; Storm the Heavens Fund; Whitley’s Wishes; Gabriella’s Smile Foundation; The Gold Hope Project; The Isabella and Marcus Foundation; Lauren’s Fight for Cure; Robert Connor Dawes Foundation; Ryan’s Hope; Benny’s World; Lily Larue Foundation; Marlee’s Mission; RUN DIPG; American Childhood Cancer Organization; The DIPG Collaborative; Snapgrant.com; Kyler Strong Foundation; Keris Kares |
| Source: |
Neuro-Oncology ; volume 24, issue 12, page 2190-2199 ; ISSN 1522-8517 1523-5866 |
| Publisher Information: |
Oxford University Press (OUP) |
| Publication Year: |
2022 |
| Description: |
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. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1093/neuonc/noac123 |
| DOI: |
10.1093/neuonc/noac123/45418291/noac123.pdf |
| Availability: |
https://doi.org/10.1093/neuonc/noac123; https://academic.oup.com/neuro-oncology/advance-article-pdf/doi/10.1093/neuonc/noac123/45418291/noac123.pdf; https://academic.oup.com/neuro-oncology/article-pdf/24/12/2190/47369168/noac123.pdf |
| Rights: |
https://academic.oup.com/pages/standard-publication-reuse-rights |
| Accession Number: |
edsbas.3D2B9671 |
| Database: |
BASE |