Diffuse intrinsic pontine gliomas (DIPG) comprise 70-80% of all brainstem gliomas in children. Approximately 300 children develop brainstem gliomas per year. Prognosis for patients with DIPGs remains dismal with a median survival of less than 1 year and no improvement in survival has been realized in more than three decades. No effective chemotherapeutic regimens are currently available.Little is known about the biology of these lesions because they are typically not biopsied and surgical resection is impossible given their location.We are establishing an International Diffuse Intrinsic Pontine Glioma Registry to provide a comprehensive database of clinical, radiologic and pathologic data linked to a bioinformatics repository of molecular data of patients. This is a new project, currently under development. Two initial studies are planned.
|Baugh, Joshua; Bartels, Ute; Leach, James et al. (2017) The international diffuse intrinsic pontine glioma registry: an infrastructure to accelerate collaborative research for an orphan disease. J Neurooncol 132:323-331|