Diffuse intrinsic pontine gliomas: A systematic update on clinical trials and biology

Jansen, M.H.A.; van Vuurden, D.G.; Vandertop, W.P.; Kaspers, G.J.L.

doi:10.1016/j.ctrv.2011.06.007

« Previous Next »Cancer Treatment Reviews
Volume 38, Issue 1 , Pages 27-35, February 2012

Diffuse intrinsic pontine gliomas: A systematic update on clinical trials and biology

M.H.A. Jansen
- Department of Pediatrics, Division of Pediatric Oncology/Hematology, VU University Medical Center, Amsterdam, The Netherlands
- Neuro-Oncology Research Group, Cancer Center Amsterdam, VU University Medical Center, Amsterdam, The Netherlands
- Corresponding author. Address: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center, Room PK 4x027, De Boelelaan 1118, 1007MB Amsterdam, The Netherlands. Tel.: +31 20 4446201; fax: +31 20 4442422.
D.G. van Vuurden
- Department of Pediatrics, Division of Pediatric Oncology/Hematology, VU University Medical Center, Amsterdam, The Netherlands
- Neuro-Oncology Research Group, Cancer Center Amsterdam, VU University Medical Center, Amsterdam, The Netherlands
- Tel.: +31 20 44 42420x7212; fax: +31 20 44 42422.
W.P. Vandertop
- Neurosurgical Center Amsterdam, VU University Medical Center, Amsterdam, The Netherlands
- Neuro-Oncology Research Group, Cancer Center Amsterdam, VU University Medical Center, Amsterdam, The Netherlands
- Tel.: +31 20 44 43725; fax: +31 20 44 43784.
G.J.L. Kaspers
- Department of Pediatrics, Division of Pediatric Oncology/Hematology, VU University Medical Center, Amsterdam, The Netherlands
- Neuro-Oncology Research Group, Cancer Center Amsterdam, VU University Medical Center, Amsterdam, The Netherlands
- Tel.: +31 20 44 42420; fax: +31 20 44 42422.

Received 9 February 2011; received in revised form 17 May 2011; accepted 25 June 2011. published online 18 July 2011.

Abstract

Patients with diffuse intrinsic pontine gliomas (DIPG) have a poor prognosis. Although DIPG constitute only 10–15% of all pediatric brain tumors, they are the main cause of death in this group. Despite 26 clinical trials in newly diagnosed DIPG in the past 5years (including several targeted agents), there is no clear improvement in prognosis. However, knowledge on DIPG biology is increasing, mainly due to the (re)introduction of biopsies and autopsies, the possibility of gene expression profiling, and the development of in vivo models. Translation of this knowledge into clinical trials in combination with improved drug distribution methods may eventually lead to more effective treatment of this devastating disease.