Development of the SIOPE DIPG network, registry and imaging repository: a collaborative effort to optimize research into a rare and lethal disease

Sophie E.M. Veldhuijzen van Zanten*, Joshua Baugh, Brooklyn Chaney, Dennis De Jongh, Esther Sanchez Aliaga, Frederik Barkhof, Johan Noltes, Ruben De Wolf, Jet Van Dijk, Antonio Cannarozzo, Carin M. Damen-Korbijn, Jan A. Lieverst, Niclas Colditz, Marion Hoffmann, Monika Warmuth-Metz, Brigitte Bison, David T.W. Jones, Dominik Sturm, Gerrit H. Gielen, Chris JonesEsther Hulleman, Raphael Calmon, David Castel, Pascale Varlet, Géraldine Giraud, Irene Slavc, Stefaan Van Gool, Sandra Jacobs, Filip Jadrijevic-Cvrlje, David Sumerauer, Karsten Nysom, Virve Pentikainen, Sanna Maria Kivivuori, Pierre Leblond, Natasha Entz-Werle, Andre O. von Bueren, Antonis Kattamis, Darren R. Hargrave, Péter Hauser, Miklos Garami, Halldora K. Thorarinsdottir, Jane Pears, Lorenza Gandola, Giedre Rutkauskiene, Geert O. Janssens, Ingrid K. Torsvik, Marta Perek-Polnik, William P. Vandertop, Gertjan J.L. Kaspers, Dannis G. van Vuurden, on behalf of the members of the SIOPE DIPG Network

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Diffuse intrinsic pontine glioma (DIPG) is a rare and deadly childhood malignancy. After 40 years of mostly single-center, often non-randomized trials with variable patient inclusions, there has been no improvement in survival. It is therefore time for international collaboration in DIPG research, to provide new hope for children, parents and medical professionals fighting DIPG. In a first step towards collaboration, in 2011, a network of biologists and clinicians working in the field of DIPG was established within the European Society for Paediatric Oncology (SIOPE) Brain Tumour Group: the SIOPE DIPG Network. By bringing together biomedical professionals and parents as patient representatives, several collaborative DIPG-related projects have been realized. With help from experts in the fields of information technology, and legal advisors, an international, web-based comprehensive database was developed, The SIOPE DIPG Registry and Imaging Repository, to centrally collect data of DIPG patients. As for April 2016, clinical data as well as MR-scans of 694 patients have been entered into the SIOPE DIPG Registry/Imaging Repository. The median progression free survival is 6.0 months (95% Confidence Interval (CI) 5.6–6.4 months) and the median overall survival is 11.0 months (95% CI 10.5–11.5 months). At two and five years post-diagnosis, 10 and 2% of patients are alive, respectively. The establishment of the SIOPE DIPG Network and SIOPE DIPG Registry means a paradigm shift towards collaborative research into DIPG. This is seen as an essential first step towards understanding the disease, improving care and (ultimately) cure for children with DIPG.

Original languageEnglish
Pages (from-to)255-266
Number of pages12
JournalJournal of Neuro-Oncology
Volume132
Issue number2
DOIs
Publication statusPublished - 1 Apr 2017

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