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

作者：van Zanten Sophie E M Veldhuijzen^*; Baugh Joshua; Chaney Brooklyn; De Jongh Dennis; Aliaga Esther Sanchez; Barkhof Frederik; Noltes Johan; De Wolf Ruben; Van Dijk Jet; Cannarozzo Antonio; Damen Korbijn Carin M; Lieverst Jan A; Colditz Niclas; Hoffmann Marion; Warmuth Metz Monika; Bison Brigitte; Jones David T W; Sturm Dominik; Gielen Gerrit H; Jones Chris; Hulleman Esther; Calmon Raphael; Castel David; Varlet Pascale; Giraud Geraldine; Slavc Irene

来源：Journal of Neuro-Oncology, 2017, 132(2): 255-266.

DOI：10.1007/s11060-016-2363-y

• Diffuse intrinsic pontine glioma (DIPG)
• Collaboration
• International research-infrastructure
• SIOPE DIPG network
• SIOPE DIPG registry

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.

• 出版日期2017-4