DIPG/DIPT Discussion

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Tuesday, July 13, 2010

French Present DIPG Biopsy Data at ISPNO

Back in the summer of 2005 the Institut Gustave Roussy started a study with upfront stereotactic biopsy in children with diffuse intrinsic pontine gliomas.


In the July 2007 edition of The Journal of Neurosurgery, the results neurosurgical results of 24 children who underwent biopsy were published. In this there was no mortality and transient morbidity in two patients.

At the 2010 International Society of Pediatric Neuro-Oncology in Vienna, the French group presented pathologic data regarding the upfront biopsy results with pediatric DIPG. There were 60 children who underwent biopsy and which there were 35 frozen sample in which they could undertake array comparative genomic hybridization or gene expression profiles. The median age of the children was 7.3 years and the overall survival was 10.5 months. Histopathologically, there were 10 grade II, 14 grade III, 10 grade IV and one that could not be graded.

Neither the MRI characteristics or the tumor grade were correlated with survival.

Two patterns were found by genetic analysis. One pattern showed enrichment for neural patterning genes (particularly regarding PDGFRa). The other had angiogenic and adhesion genes. The patterns did not relate to tumor grade. The first group had a worse prognosis with a majority of children in this group having overall survival less than the median survival.

The authors concluded that they have found two DIPG molecular subgroups ‘characterized by chromosomal regions and potentially therapeutic targets’.
These results seem to dovetail well with the previously reported post-mortem study in March in JCO from Sick Kids in Toronto.

Whole-genome profiling of pediatric diffuse intrinsic pontine gliomas highlights platelet-derived growth factor receptor alpha and poly (ADP-ribose) polymerase as potential therapeutic targets
J Clin Oncol. 2010 Mar 10;28(8):1337-44. Epub 2010 Feb 8.