Diffuse intrinsic pontine gliomas (DIPGs) are amongst the most challenging tumors to treat. Surgery is not an option, the effects of radiation therapy are temporary, and no chemotherapeutic agent has demonstrated significant efficacy. Numerous clinical trials of new agents and novel therapeutic approaches have been performed over the course of several decades in efforts to improve the outcome of children with DIPG, yet without success. The diagnosis of DIPG is based on radiographic findings in the setting of a typical clinical presentation, and tissue is not routinely obtained as the standard of care. The paradigm for treating children with these tumors has been based on that for supratentorial high-grade gliomas in adults as the biology of these lesions were presumed to be similar. However, recent pivotal studies demonstrate that DIPGs appear to be their own entity. Simply identifying this fact releases a number of constraints and opens opportunities for biologic investigation of these lesions, setting the stage to move forward in identifying DIPG-specific treatments. This review will summarize the current state of knowledge of DIPG, discuss obstacles to therapy, and summarize results of recent biologic studies.
Keywords: pons, glioma, brainstem, DIPG, diffuse, intrinsic, pediatric, pontine
Citation: Warren KE (2012) Diffuse intrinsic pontine glioma: poised for progress. Front. Oncol. 2:205. doi: 10.3389/fonc.2012.00205
Received: 05 October 2012; Accepted: 11 December 2012;
Published online: 28 December 2012.
Edited by:Charles G. Mullighan, St. Jude Children’s Research Hospital, USA
Reviewed by:Jacques Grill, Gustave Roussy Cancer Institute, France
Copyright: © 2012 Warren. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in other forums, provided the original authors and source are credited and subject to any copyright notices concerning any third-party graphics etc.
*Correspondence: Katherine E. Warren, Pediatric Neuro-Oncology Section, Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Building 10 CRC, Room 1-5750, Bethesda, MD 20892-1104, USA. e-mail: email@example.com