| Literature DB >> 29746225 |
Lindsey M Hoffman1, Sophie E M Veldhuijzen van Zanten1, Niclas Colditz1, Joshua Baugh1, Brooklyn Chaney1, Marion Hoffmann1, Adam Lane1, Christine Fuller1, Lili Miles1, Cynthia Hawkins1, Ute Bartels1, Eric Bouffet1, Stewart Goldman1, Sarah Leary1, Nicholas K Foreman1, Roger Packer1, Katherine E Warren1, Alberto Broniscer1, Mark W Kieran1, Jane Minturn1, Melanie Comito1, Emmett Broxson1, Chie-Schin Shih1, Soumen Khatua1, Murali Chintagumpala1, Anne Sophie Carret1, Nancy Yanez Escorza1, Timothy Hassall1, David S Ziegler1, Nicholas Gottardo1, Hetal Dholaria1, Renee Doughman1, Martin Benesch1, Rachid Drissi1, Javad Nazarian1, Nada Jabado1, Nathalie Boddaert1, Pascale Varlet1, Géraldine Giraud1, David Castel1, Stephanie Puget1, Chris Jones1, Esther Hulleman1, Piergiorgio Modena1, Marzia Giagnacovo1, Manila Antonelli1, Torsten Pietsch1, Gerrit H Gielen1, David T W Jones1, Dominik Sturm1, Stefan M Pfister1, Nicolas U Gerber1, Michael A Grotzer1, Elke Pfaff1, André O von Bueren1, Darren Hargrave1, Guirish A Solanki1, Filip Jadrijevic Cvrlje1, Gertjan J L Kaspers1, William P Vandertop1, Jacques Grill1, Simon Bailey1, Veronica Biassoni1, Maura Massimino1, Raphaël Calmon1, Esther Sanchez1, Brigitte Bison1, Monika Warmuth-Metz1, James Leach1, Blaise Jones1, Dannis G van Vuurden1, Christof M Kramm1, Maryam Fouladi1.
Abstract
Purpose Diffuse intrinsic pontine glioma (DIPG) is a brainstem malignancy with a median survival of < 1 year. The International and European Society for Pediatric Oncology DIPG Registries collaborated to compare clinical, radiologic, and histomolecular characteristics between short-term survivors (STSs) and long-term survivors (LTSs). Materials and Methods Data abstracted from registry databases included patients from North America, Australia, Germany, Austria, Switzerland, the Netherlands, Italy, France, the United Kingdom, and Croatia. Results Among 1,130 pediatric and young adults with radiographically confirmed DIPG, 122 (11%) were excluded. Of the 1,008 remaining patients, 101 (10%) were LTSs (survival ≥ 2 years). Median survival time was 11 months (interquartile range, 7.5 to 16 months), and 1-, 2-, 3-, 4-, and 5-year survival rates were 42.3% (95% CI, 38.1% to 44.1%), 9.6% (95% CI, 7.8% to 11.3%), 4.3% (95% CI, 3.2% to 5.8%), 3.2% (95% CI, 2.4% to 4.6%), and 2.2% (95% CI, 1.4% to 3.4%), respectively. LTSs, compared with STSs, more commonly presented at age < 3 or > 10 years (11% v 3% and 33% v 23%, respectively; P < .001) and with longer symptom duration ( P < .001). STSs, compared with LTSs, more commonly presented with cranial nerve palsy (83% v 73%, respectively; P = .008), ring enhancement (38% v 23%, respectively; P = .007), necrosis (42% v 26%, respectively; P = .009), and extrapontine extension (92% v 86%, respectively; P = .04). LTSs more commonly received systemic therapy at diagnosis (88% v 75% for STSs; P = .005). Biopsies and autopsies were performed in 299 patients (30%) and 77 patients (10%), respectively; 181 tumors (48%) were molecularly characterized. LTSs were more likely to harbor a HIST1H3B mutation (odds ratio, 1.28; 95% CI, 1.1 to 1.5; P = .002). Conclusion We report clinical, radiologic, and molecular factors that correlate with survival in children and young adults with DIPG, which are important for risk stratification in future clinical trials.Entities:
Mesh：
Year: 2018 PMID： 29746225 PMCID： PMC6075859 DOI： 10.1200/JCO.2017.75.9308
Source DB: PubMed Journal: J Clin Oncol ISSN： 0732-183X Impact factor: 44.544