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

Lookup NU author(s): Professor Simon BaileyORCiD

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This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).

Abstract

© 2017 The Author(s) 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.

Publication metadata

Author(s): van Zanten SEMV, Baugh J, Chaney B, de Jongh D, Aliaga ES, Barkhof F, Noltes J, de Wolf R, van Dijk J, Cannarozzo A, Damen-Korbijn CM, Lieverst JA, Colditz N, Hoffmann M, Warmuth-Metz M, Bison B, Jones DTW, Sturm D, Gielen GH, Jones C, Hulleman E, Calmon R, Castel D, Varlet P, Giraud G, Slavc I, van Gool S, Jacobs S, Jadrijevic-Cvrlje F, Sumerauer D, Nysom K, Pentikainen V, Kivivuori S-M, Leblond P, Entz-Werle N, von Bueren AO, Kattamis A, Hargrave DR, Hauser P, Garami M, Thorarinsdottir HK, Pears J, Gandola L, Rutkauskiene G, Janssens GO, Torsvik IK, Perek-Polnik M, Gil-da-Costa MJ, Zheludkova O, Shats L, Deak L, Kitanovski L, Cruz O, la Madrid AM, Holm S, Gerber N, Kebudi R, Grundy R, Lopez-Aguilar E, Zapata-Tarres M, Emmerik J, Hayden T, Bailey S, Biassoni V, Massimino M, Grill J, Vandertop WP, Kaspers GJL, Fouladi M, Kramm CM, van Vuurden DG, on behalf of the members of the SIOPE DIPG Network

Publication type: Article

Publication status: Published

Journal: Journal of Neuro-Oncology

Year: 2017

Volume: 132

Issue: 2

Pages: 255-266

Print publication date: 01/04/2017

Online publication date: 21/07/2017

Acceptance date: 25/12/2016

Date deposited: 02/06/2017

ISSN (print): 0167-594X

ISSN (electronic): 1573-7373

Publisher: Springer New York LLC

URL: https://doi.org/10.1007/s11060-016-2363-y

DOI: 10.1007/s11060-016-2363-y

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