Baugh Joshua, Bartels Ute, Leach James, Jones Blaise, Chaney Brooklyn, Warren Katherine E, Kirkendall Jenavieve, Doughman Renee, Hawkins Cynthia, Miles Lili, Fuller Christine, Hassall Tim, Bouffet Eric, Lane Adam, Hargrave Darren, Grill Jacques, Hoffman Lindsey M, Jones Chris, Towbin Alex, Savage Sharon A, Monje Michelle, Li Xiao-Nan, Ziegler David S, Veldhuijzen van Zanten Sophie, Kramm Christof M, van Vuurden Dannis G, Fouladi Maryam
Brain Tumor Center, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, USA.
Hospital for Sick Children, Toronto, ON, Canada.
J Neurooncol. 2017 Apr;132(2):323-331. doi: 10.1007/s11060-017-2372-5. Epub 2017 Jan 16.
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.
弥漫性脑桥内生型胶质瘤（DIPG）是一种罕见的、常致命的儿童脑肿瘤，仍然是一个重大的治疗挑战。2012年，由DIPG协作组织（29个私人基金会之间的慈善合作关系）资助的研究人员发起了国际DIPG注册库（IDIPGR），以增进对DIPG的了解。IDIPGR由全面的、去识别化但相互关联的临床、影像、组织病理学和基因组库组成，使用标准化的病例报告表进行统一的数据收集；系列影像和组织病理学分别由IDIPGR的神经放射学家和神经病理学家进行集中审查。经科学咨询委员会批准的研究，研究人员可以获取由IDIPGR协调的尸检获得的组织、基因组数据以及细胞培养物。从2012年4月到2016年12月，来自美国、加拿大、澳大利亚和新西兰的55个参与机构共招募了670例被诊断为DIPG的患者。放射学库包含来自448例患者的3558项研究。病理学库包含81例患者的组织，另有98个样本可供提交。来自7例尸检的新鲜DIPG组织已被送交研究人员以建立原代细胞培养物。生物信息学库包含66个肿瘤的二代测序数据。目前，来自世界各地的13位主要研究人员正在开展9个使用IDIPGR数据/组织的项目。IDIPGR是慈善机构和研究人员之间的一个成功联盟，为DIPG的跨学科和转化项目开发并维护了一个高度协作、以假设为驱动的研究基础设施，以改善患者的诊断、反应评估、治疗和预后。
