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J Clin Oncol. 2018 Jul 1;36(19):1963-1972. doi: 10.1200/JCO.2017.75.9308. Epub 2018 May 10.

Clinical, Radiologic, Pathologic, and Molecular Characteristics of Long-Term Survivors of Diffuse Intrinsic Pontine Glioma (DIPG): A Collaborative Report From the International and European Society for Pediatric Oncology DIPG Registries.

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1
Lindsey M. Hoffman and Nicholas K. Foreman, University of Colorado Denver; Lindsey M. Hoffman and Nicholas K. Foreman, Children's Hospital Colorado, Aurora, CO; Sophie E.M. Veldhuijzen van Zanten, Esther Hulleman, Gertjan J.L. Kaspers, Esther Sanchez, and Dannis G. van Vuurden, Vrije Universiteit University Medical Center, Amsterdam; William P. Vandertop, Academy of Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands; Niclas Colditz, Marion Hoffmann, Brigitte Bison, Monika Warmuth-Metz, and Christof M. Kramm, University Medical Center Goettingen, Goettingen; Torsten Pietsch and Gerrit H. Gielen, University of Bonn Medical Center, Bonn; David T.W. Jones, Dominik Sturm, Stefan M. Pfister, and Elke Pfaff, German Cancer Research Center, Hopp-Children's Cancer Center at the Nationale Centrum für Tumorerkrankungen Heidelberg, and German Consortium for Translational Cancer Research; Dominik Sturm, Stefan M. Pfister, and Elke Pfaff, Heidelberg University Hospital, Heidelberg, Germany; Joshua Baugh, Brooklyn Chaney, Adam Lane, Christine Fuller, Nancy Yanez Escorza, Renee Doughman, Rachid Drissi, James Leach, Blaise Jones, and Maryam Fouladi, Cincinnati Children's Hospital Medical Center, Cincinnati; Emmett Broxson, Wright State University and The Children's Medical Center, Dayton, OH; Lili Miles, Nemours Children's Hospital, Orlando, FL; Cynthia Hawkins, Ute Bartels, and Eric Bouffet, The Hospital for Sick Children, Toronto, Ontario; Anne Sophie Carret, Centre Hospitalier Universitaire Sainte-Justine; Nada Jabado, McGill University, Montreal, Quebec, Canada; Stewart Goldman, Ann and Robert H Lurie Children's Hospital of Chicago, Chicago, IL; Sarah Leary, Seattle Children's Hospital, University of Washington and Fred Hutchinson Cancer Research Center, Seattle, WA; Roger Packer, Children's National Health System; Javad Nazarian, Children's National Medical Center, Washington, DC; Katherine E. Warren, National Cancer Institute, Bethesda, MD; Alberto Broniscer, St Jude Children's Research Hospital, Memphis, TN; Mark W. Kieran, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA; Jane Minturn, Children's Hospital of Philadelphia and The Perelman School of Medicine at the University of Pennsylvania, Philadelphia; Melanie Comito, The Pennsylvania State University, Hershey, PA; Chie-Schin Shih, Indiana University, Indianapolis, IN; Soumen Khatua, The University of Texas MD Anderson Cancer Center; Murali Chintagumpala, Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, TX; Timothy Hassall, Lady Cilento Children's Hospital, Brisbane, Queensland; David S. Ziegler, Kids Cancer Centre, Sydney Children's Hospital, Randwick; David S. Ziegler, University of New South Wales, Sydney, New South Wales; Nicholas Gottardo and Hetal Dholaria, Princess Margaret Hospital for Children, Perth, Western Australia, Australia; Martin Benesch, Medical University of Graz, Graz, Austria; Nathalie Boddaert. Stephanie Puget, and Raphaël Calmon, Hôpital Necker Enfants Malades; Pascale Varlet, Hôpital Sainte-Anne, Université Paris V Descartes, Sorbonne Paris Cité, Paris; Géraldine Giraud, David Castel, and Jacques Grill, Gustave Roussy, Université Paris-Sud, Université Paris-Saclay, Villejuif, France; Géraldine Giraud, Uppsala University, Uppsala, Sweden; Chris Jones, The Institute of Cancer Research, Sutton; Darren Hargrave, Great Ormond Street Hospital, London; Guirish A. Solanki, Birmingham Women's and Children's Hospital, Birmingham; Simon Bailey, Great North Children's Hospital, Victoria Wing, Royal Victoria Infirmary, Newcastle upon Tyne, United Kingdom; Piergiorgio Modena and Marzia Giagnacovo, Sant' Anna Como General Hospital, Como; Manila Antonelli, Sapienza University of Rome, Rome; Veronica Biassoni and Maura Massimino, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico, Istituto Nazionale dei Tumori, Milan, Italy; Nicolas U. Gerber and Michael A. Grotzer, University Children's Hospital of Zurich, Zurich; André O. von Bueren, University Hospital of Geneva and University of Geneva, Geneva, Switzerland; and Filip Jadrijevic Cvrlje and Gertjan J.L. Kaspers, Children's Hospital Zagreb, Zagreb, Croatia.

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.

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