title: Development of the SIOPE DIPG network, registry and imaging repository: a collaborative effort to optimize research into a rare and lethal disease.
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.
authors.affiliation: Department of Paediatrics, Division of Oncology/Haematology, VU University Medical Center, Amsterdam, The Netherlands. s.veldhuijzen@vumc.nl.
authors.forename: Sophie E M
authors.identifier: http://orcid.org/0000-0002-3888-0977
authors.initials: SEM
authors.lastname: Veldhuijzen van Zanten
authors.affiliation: Department of Pediatrics, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, USA.
authors.forename: Joshua
authors.initials: J
authors.lastname: Baugh
authors.affiliation: Department of Pediatrics, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, USA.
authors.forename: Brooklyn
authors.initials: B
authors.lastname: Chaney
authors.affiliation: 2TCI B.V., Breda, The Netherlands.
authors.forename: Dennis
authors.initials: D
authors.lastname: De Jongh
authors.affiliation: Department of Radiology and Nuclear Medicine, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Esther
authors.initials: E
authors.lastname: Sanchez Aliaga
authors.affiliation: Department of Radiology and Nuclear Medicine, VU University Medical Center, Amsterdam, The Netherlands.|Institutes of Neurology and Healthcare Engineering, UCL, London, UK.
authors.forename: Frederik
authors.initials: F
authors.lastname: Barkhof
authors.affiliation: KPMG, Amstelveen, The Netherlands.
authors.forename: Johan
authors.initials: J
authors.lastname: Noltes
authors.affiliation: KPMG, Amstelveen, The Netherlands.
authors.forename: Ruben
authors.initials: R
authors.lastname: De Wolf
authors.affiliation: Yellow Research B.V., Amsterdam, The Netherlands.
authors.forename: Jet
authors.initials: J
authors.lastname: Van Dijk
authors.affiliation: Trasferimento Tecnologico (TTO), Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Cannarozzo
authors.affiliation: Dutch Childhood Oncology Group (DCOG), The Hague, The Netherlands.
authors.forename: Carin M
authors.initials: CM
authors.lastname: Damen-Korbijn
authors.affiliation: Dutch Childhood Oncology Group (DCOG), The Hague, The Netherlands.
authors.forename: Jan A
authors.initials: JA
authors.lastname: Lieverst
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Child and Adolescent Health, University of Göttingen, Göttingen, Germany.
authors.forename: Niclas
authors.initials: N
authors.lastname: Colditz
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Child and Adolescent Health, University of Göttingen, Göttingen, Germany.
authors.forename: Marion
authors.initials: M
authors.lastname: Hoffmann
authors.affiliation: Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) Heidelberg, Heidelberg, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Warmuth-Metz
authors.affiliation: Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) Heidelberg, Heidelberg, Germany.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Bison
authors.affiliation: Institute of Neuropathology, University of Bonn Medical Center, Bonn, Germany.
authors.forename: David T W
authors.initials: DTW
authors.lastname: Jones
authors.affiliation: Institute of Neuropathology, University of Bonn Medical Center, Bonn, Germany.
authors.forename: Dominik
authors.initials: D
authors.lastname: Sturm
authors.affiliation: Institute of Neuropathology, University of Bonn Medical Center, Bonn, Germany.
authors.forename: Gerrit H
authors.initials: GH
authors.lastname: Gielen
authors.affiliation: Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, Sutton, UK.
authors.forename: Chris
authors.initials: C
authors.lastname: Jones
authors.affiliation: Neuro-oncology Research Group, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Esther
authors.initials: E
authors.lastname: Hulleman
authors.affiliation: Université Paris-Descartes, Hôpital Necker-Enfants malades, Paris, France.
authors.forename: Raphael
authors.initials: R
authors.lastname: Calmon
authors.affiliation: UMR 8203 CNRS, Gustave Roussy, Paris-Saclay University, Villejuif, France.
authors.forename: David
authors.initials: D
authors.lastname: Castel
authors.affiliation: Service de Neuropathologie, Hôpital Sainte-Anne, Paris, France.
authors.forename: Pascale
authors.initials: P
authors.lastname: Varlet
authors.affiliation: Institut Gustave Roussy, Villejuif, France.
authors.forename: Géraldine
authors.initials: G
authors.lastname: Giraud
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.
authors.forename: Irene
authors.initials: I
authors.lastname: Slavc
authors.affiliation: Immuno-oncology Centre Köln, Cologne, Germany.
authors.forename: Stefaan
authors.initials: S
authors.lastname: Van Gool
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospitals Leuven, Leuven, Belgium.|Department of Microbiology and Immunology, KU Leuven, Leuven, Belgium.
authors.forename: Sandra
authors.initials: S
authors.lastname: Jacobs
authors.affiliation: Department of Oncology and Hematology, Children's Hospital Zagreb, Zagreb, Croatia.
authors.forename: Filip
authors.initials: F
authors.lastname: Jadrijevic-Cvrlje
authors.affiliation: Department of Pediatric Hematology and Oncology, 2nd Faculty of Medicine, University Hospital Motol, Charles University, Prague, Czech Republic.
authors.forename: David
authors.initials: D
authors.lastname: Sumerauer
authors.affiliation: Pediatrics and Adolescent Medicine, University Hospital Rigshospitalet, Copenhagen, Denmark.
authors.forename: Karsten
authors.initials: K
authors.lastname: Nysom
authors.affiliation: Division of Hematology-Oncology and Stem Cell Transplantation, Children's Hospital, Helsinki University Hospital, Helsinki, Finland.
authors.forename: Virve
authors.initials: V
authors.lastname: Pentikainen
authors.affiliation: Helsinki University Hospital (HUH) and HUH Children and Adolescents, Helsinki, Finland.
authors.forename: Sanna-Maria
authors.initials: SM
authors.lastname: Kivivuori
authors.affiliation: Pediatric Oncology Unit, Oscar Lambret Comprehensive Cancer Center, Lille, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Leblond
authors.affiliation: Service de Pédiatrie Onco-Hématologie, CHRU Hautepierre Strasbourg, Strasbourg, France.
authors.forename: Natasha
authors.initials: N
authors.lastname: Entz-Werle
authors.affiliation: Pediatric Oncology and Hematology, Department of Pediatrics, University Hospital of Geneva, Genève, Switzerland.
authors.forename: Andre O
authors.initials: AO
authors.lastname: von Bueren
authors.affiliation: First Department of Pediatrics, National and Kapodistrian University of Athens, 'Aghia Sofia' Children's Hospital, Athens, Greece.
authors.forename: Antonis
authors.initials: A
authors.lastname: Kattamis
authors.affiliation: Paediatric Oncology Unit, Great Ormond Street Hospital (GOSH), London, UK.
authors.forename: Darren R
authors.initials: DR
authors.lastname: Hargrave
authors.affiliation: Second Department of Pediatrics, Semmelweis University, Budapest, Hungary.
authors.forename: Péter
authors.initials: P
authors.lastname: Hauser
authors.affiliation: Second Department of Pediatrics, Semmelweis University, Budapest, Hungary.
authors.forename: Miklos
authors.initials: M
authors.lastname: Garami
authors.affiliation: Pediatric Hematology-Oncology, The Children's Hospital, Reykjavik, Iceland.
authors.forename: Halldora K
authors.initials: HK
authors.lastname: Thorarinsdottir
authors.affiliation: Department of Paediatric Oncology, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland.
authors.forename: Jane
authors.initials: J
authors.lastname: Pears
authors.affiliation: Pediatric Radiotherapy Unit, Department of Diagnostic Radiology and Radiotherapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.affiliation: Department of Pediatric Oncology and Hematology, Hospital of Lithuanian University of Health Sciences Kaunas Clinic, Kaunas, Lithuania.
authors.forename: Giedre
authors.initials: G
authors.lastname: Rutkauskiene
authors.affiliation: Department of Radiation Oncology, University Medical Center Utrecht and Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands.
authors.forename: Geert O
authors.initials: GO
authors.lastname: Janssens
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, Haukeland University Hospital, Mons, Norway.
authors.forename: Ingrid K
authors.initials: IK
authors.lastname: Torsvik
authors.affiliation: Department of Oncology, The Children's Memorial Health Institute, Warsaw, Poland.
authors.forename: Marta
authors.initials: M
authors.lastname: Perek-Polnik
authors.affiliation: Pediatric Hemathology and Oncology Division, University Hospital S. João Alameda Hernani Monteiro, Porto, Portugal.
authors.forename: Maria J
authors.initials: MJ
authors.lastname: Gil-da-Costa
authors.affiliation: Department of Neuro-Oncology, Russian Scientific Center of Radiology, Moscow, Russia.
authors.forename: Olga
authors.initials: O
authors.lastname: Zheludkova
authors.affiliation: Department of Children Oncology and Haematology, City Clinical Hospital #31 of Saint-Petersburg, St.-Petersburg, Russian Federation.
authors.forename: Liudmila
authors.initials: L
authors.lastname: Shats
authors.affiliation: Department of Pediatric Oncology/ Haematology, Children University Hospital, Kosice, Slovakia.
authors.forename: Ladislav
authors.initials: L
authors.lastname: Deak
authors.affiliation: Department of Pediatrics, Division of Haematooncology, University Medical Center Ljubljana, Ljubljana, Slovenia.
authors.forename: Lidija
authors.initials: L
authors.lastname: Kitanovski
authors.affiliation: Department of Pediatric Hematology and Oncology, Hospital Sant Joan de Déu, Barcelona, Spain.
authors.forename: Ofelia
authors.initials: O
authors.lastname: Cruz
authors.affiliation: Department of Pediatric Hematology and Oncology, Hospital Sant Joan de Déu, Barcelona, Spain.
authors.forename: Andres
authors.initials: A
authors.lastname: Morales La Madrid
authors.affiliation: Department of Pediatric Hematology and Oncology, Department of Woman and Child Health, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Stefan
authors.initials: S
authors.lastname: Holm
authors.affiliation: Department of Oncology, University Children's Hospital of Zurich, Zurich, Switzerland.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Gerber
authors.affiliation: Cerrahpasa Medical Faculty & Oncology Institute, Pediatric Hematology-Oncology, Istanbul University, Istanbul, Turkey.
authors.forename: Rejin
authors.initials: R
authors.lastname: Kebudi
authors.affiliation: Children's Brain Tumour Research Centre, The Medical School, University of Nottingham, Nottingham, UK.
authors.forename: Richard
authors.initials: R
authors.lastname: Grundy
authors.affiliation: Jefatura de Servicio de Oncología, Hospital de Pediatría, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Distrito Federal, Mexico.
authors.forename: Enrique
authors.initials: E
authors.lastname: Lopez-Aguilar
authors.affiliation: Division of Pediatric Oncology, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico.
authors.forename: Marta
authors.initials: M
authors.lastname: Zapata-Tarres
authors.affiliation: Stichting Semmy, Weesp, The Netherlands.
authors.forename: John
authors.initials: J
authors.lastname: Emmerik
authors.affiliation: The DIPG Collaborative, Cincinnati, USA.
authors.forename: Tim
authors.initials: T
authors.lastname: Hayden
authors.affiliation: Great North Childrens Hospital, Victoria Wing, Royal Victoria Infirmary, Newcastle upon Tyne, UK.
authors.forename: Simon
authors.initials: S
authors.lastname: Bailey
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
authors.forename: Veronica
authors.initials: V
authors.lastname: Biassoni
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
authors.affiliation: Institut Gustave Roussy, Villejuif, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Grill
authors.affiliation: Neurosurgical Center Amsterdam, Academic Medical Center and VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: William P
authors.initials: WP
authors.lastname: Vandertop
authors.affiliation: Department of Paediatrics, Division of Oncology/Haematology, VU University Medical Center, Amsterdam, The Netherlands.|Academy of Princess Máxima Center for Pediatric Oncology, Lundlaan 6, 3584 EA, Utrecht, The Netherlands.
authors.forename: Gertjan J L
authors.initials: GJL
authors.lastname: Kaspers
authors.affiliation: Department of Pediatrics, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, USA.
authors.forename: Maryam
authors.initials: M
authors.lastname: Fouladi
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Child and Adolescent Health, University of Göttingen, Göttingen, Germany.
authors.forename: Christof M
authors.initials: CM
authors.lastname: Kramm
authors.affiliation: Department of Paediatrics, Division of Oncology/Haematology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Dannis G
authors.initials: DG
authors.lastname: van Vuurden
mesh_terms: D020295:Brain Stem Neoplasms / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D005060:Europe; D005260:Female; D005910:Glioma / Q000000981:diagnostic imaging*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007255:Information Services; D007391:International Cooperation; D008279:Magnetic Resonance Imaging; D008297:Male; D011149:Pons / Q000000981:diagnostic imaging; D012042:Registries; D055815:Young Adult
keywords: Collaboration; Diffuse intrinsic pontine glioma (DIPG); International research-infrastructure; SIOPE DIPG network; SIOPE DIPG registry