The international diffuse intrinsic pontine glioma registry: an infrastructure to accelerate collaborative research for an orphan disease

  • Joshua Baugh
  • Ute Bartels
  • James Leach
  • Blaise Jones
  • Brooklyn Chaney
  • Katherine E. Warren
  • Jenavieve Kirkendall
  • Renee Doughman
  • Cynthia Hawkins
  • Lili Miles
  • Christine Fuller
  • Tim Hassall
  • Eric Bouffet
  • Adam Lane
  • Darren Hargrave
  • Jacques Grill
  • Lindsey M. Hoffman
  • Chris Jones
  • Alex Towbin
  • Sharon A. Savage
  • Michelle Monje
  • Xiao-Nan Li
  • David S. Ziegler
  • Sophie Veldhuijzen van Zanten
  • Christof M. Kramm
  • Dannis G. van Vuurden
  • Maryam Fouladi
Clinical Study

DOI: 10.1007/s11060-017-2372-5

Cite this article as:
Baugh, J., Bartels, U., Leach, J. et al. J Neurooncol (2017). doi:10.1007/s11060-017-2372-5

Abstract

Diffuse intrinsic pontine glioma (DIPG), a rare, often fatal childhood brain tumor, remains a major therapeutic challenge. In 2012, investigators, funded by the DIPG Collaborative (a philanthropic partnership among 29 private foundations), launched the International DIPG Registry (IDIPGR) to advance understanding of DIPG. Comprised of comprehensive deidentified but linked clinical, imaging, histopathological, and genomic repositories, the IDIPGR uses standardized case report forms for uniform data collection; serial imaging and histopathology are centrally reviewed by IDIPGR neuro-radiologists and neuro-pathologists, respectively. Tissue and genomic data, and cell cultures derived from autopsies coordinated by the IDIPGR are available to investigators for studies approved by the Scientific Advisory Committee. From April 2012 to December 2016, 670 patients diagnosed with DIPG have been enrolled from 55 participating institutions in the US, Canada, Australia and New Zealand. The radiology repository contains 3558 studies from 448 patients. The pathology repository contains tissue on 81 patients with another 98 samples available for submission. Fresh DIPG tissue from seven autopsies has been sent to investigators to develop primary cell cultures. The bioinformatics repository contains next-generation sequencing data on 66 tumors. Nine projects using data/tissue from the IDIPGR by 13 principle investigators from around the world are now underway. The IDIPGR, a successful alliance among philanthropic agencies and investigators, has developed and maintained a highly collaborative, hypothesis-driven research infrastructure for interdisciplinary and translational projects in DIPG to improve diagnosis, response assessment, treatment and outcome for patients.

Keywords

Brain tumor Diffuse intrinsic pontine glioma Registry 

Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Joshua Baugh
    • 1
  • Ute Bartels
    • 2
  • James Leach
    • 1
  • Blaise Jones
    • 1
  • Brooklyn Chaney
    • 1
  • Katherine E. Warren
    • 3
  • Jenavieve Kirkendall
    • 1
  • Renee Doughman
    • 1
  • Cynthia Hawkins
    • 2
  • Lili Miles
    • 1
  • Christine Fuller
    • 1
  • Tim Hassall
    • 4
  • Eric Bouffet
    • 2
  • Adam Lane
    • 1
  • Darren Hargrave
    • 5
  • Jacques Grill
    • 6
  • Lindsey M. Hoffman
    • 1
  • Chris Jones
    • 7
  • Alex Towbin
    • 1
  • Sharon A. Savage
    • 3
  • Michelle Monje
    • 8
  • Xiao-Nan Li
    • 9
  • David S. Ziegler
    • 10
    • 11
  • Sophie Veldhuijzen van Zanten
    • 12
  • Christof M. Kramm
    • 13
  • Dannis G. van Vuurden
    • 12
  • Maryam Fouladi
    • 1
  1. 1.Brain Tumor Center, Cancer and Blood Diseases InstituteCincinnati Children’s Hospital Medical CenterCincinnatiUSA
  2. 2.Hospital for Sick ChildrenTorontoCanada
  3. 3.National Cancer Institute, National Institutes of HealthBethesdaUSA
  4. 4.Lady Cilento Children’s HospitalBrisbaneAustralia
  5. 5.Great Ormond Street Hospital for Children NHS TrustLondonUK
  6. 6.Gustave Roussy InstituteVillejuifFrance
  7. 7.Institute of Cancer ResearchSuttonUK
  8. 8.Stanford University and Lucile Packard Children’s HospitalPalo AltoUSA
  9. 9.Texas Children’s HospitalHoustonUSA
  10. 10.Sydney Children’s HospitalRandwickAustralia
  11. 11.Children’s Cancer InstituteUniversity of New South WalesKensingtonAustralia
  12. 12.VU University Medical CenterAmsterdamThe Netherlands
  13. 13.University of GöttingenGöttingenGermany