The purpose of the registry is to support research related to DIPG. Data and samples from the registry are available both to researchers affiliated with the registry and to external researchers. Research proposals from internal and external investigators will be evaluated by an advisory committee for scientific merit and appropriate use of resources before approval.
To request access to registry data or samples for a research project, please contact:
Current Research Projects
Clinical, radiographic, pathological, and biologic characteristics of long-term survivors of DIPG
Principal investigators: Darren Hargrave and Jacques Grill
Purpose: To describe the clinical, radiographic, pathological and biologic characteristics of long-term survivors with DIPG and correlate key variables with outcome.
Background: Although patients with DIPG have a dismal prognosis, long-term survivors have been reported in the literature, including in the context of clinical trials with robust inclusion/exclusion criteria. There have been very few reports specifically analysing long-term survivors due to the rarity of such patients. Apart from very young age, it is unclear whether these patients differ from other DIPG patients in terms of baseline characteristics, clinical presentation, imaging, tumour biology and treatment regimens. The establishment of an International DIPG registry (bringing together comprehensive data from multiple clinical trials and national registries) would allow the study of long-term DIPG survivors.
Methods: All patients registered in the International DIPG registry will be eligible for study. In order to be eligible for this study cases must fulfill pre-defined DIPG diagnostic criteria based on clinical and radiological data (i.e. minimum MRI images will be required) plus, if available, pathology data. Cases who have an overall survival of greater than 2 years will be studied. Data collection will include: demographics (age, gender, ethnicity, relevant past medical history & family history), symptoms & signs at diagnosis and their duration, MRI features at diagnosis and during disease (including if available advanced imaging techniques), treatment regimen and (clinical and radiological) response to treatment. Radiology will be reviewed by a panel of independent neuroradiologists to confirm the diagnosis of DIPG and documented response. Biology: If a biopsy has been performed, the material will be collected and reviewed by a panel of independent neuropathologists for histological diagnosis. In cases of long-term survivors who ultimately have succumbed to their illness, any autopsy samples will also be reviewed by the panel. If tumour tissue has already had molecular analysis or there is available tissue there will be further analysis of the underlying molecular pathology of the long term survival cases.
The data from long-term survivors will be compared to matched historical cohorts. Multivariate regression analyses will be performed to compare the outcome variables.
An epidemiological study to determine incidence patterns of DIPG in North America 2000-2010
Principal investigators: Ute Bartels and Eric Bouffet
Background: Currently, there are no epidemiologic data on the incidence patterns and trends of DIPG. Most information on the incidence comes from limited institutional series. The Canadian Pediatric Brain Tumor Consortium has representation from all the Canadian university-affiliated teaching hospitals where close to 100% of the children with brain tumors are being treated (the exception are some teenagers referred to adult services), and therefore, a true cross-Canada epidemiologic survey is feasible. In the US, we will approach all the Pediatric Brain Tumor Consortium institutions as well as other large volume pediatric neuro-oncology programs (e.g. Boston, Seattle, Atlanta, Children’s Hospital of Los Angeles) to garner support for enrollment of patients on the registry. This approach should provide us access to the vast majority of patients with DIPG in the US. We propose to study the epidemiology of DIPG across Canada and the US between 2000 and 2010. We will study the annual incidence (variation, trend), the seasonal variations in incidence, clusters (by collecting postal codes/ZIP codes), and geographical distribution. A better understanding of the incidence of these tumors is necessary in planning future strategies and protocol development. Furthermore, identifying clinical features that may be associated with prognosis would be important in future clinical trials.
Method: All patients diagnosed with DIPGs between the ages of 0-21 who are in the registry will be included. Proven pathologic diagnosis is not required and we do not expect pathology to be available in the majority of patients. Clinical, radiologic data on patients with DIPG will be sent to the registry. The registry personnel will enroll the patients and enter all data into the registry. The data required for this study will be extracted from the registry data base by the registry personnel. The central neuroradiology panel will review at least two MRI sequences at diagnosis including 1 Axial T2 or FLAIR picture of the tumor in the area of largest dimension and 1Sagittal T2 or Flair picture of the tumour in the area of largest dimension. All imaging studies will be reviewed centrally by the neuroradiology panel to further classify as focal or diffuse pontine glioma. Descriptive statistics and incidence trends will be calculated.