Genetic and histopathological spectrum of paediatric diffuse intrinsic pontine gliomas

Sick Kids instituted an brainstem glioma project back in 2003 in hopes of trying to understand diffuse intrinsic pontine glioma.   To do this the researchers reached out to families with an autopsy-based protocol asking for consideration of post-mortem tissue donation.    This decade commitment and collaborative effort against DIPG has lead to another DIPG abstract for the upcoming SNO/CBTF supported 2013 Pediatric Neuro-Oncology Basic and Translational Research Conference.  This one comes from Sick Kids in conjunction with Oren Becher from Duke.

The researchers examined 71diffuse intrinsic pontine tumors:

• 65 were high grade gliomas, 8 were low grade and 3 were PNETs.
• survival was not related to grade.
• leptomeningeal spread was present in one third of cases.
• 68% had K27M-H3.
• these tumors were histologically heterogenous, however increased homogeneity  was found of the histone mutation.
• the histone mutations were also present in low grade tumors that had worse outcomes.

The authors conclude with a call for "incorporation of histological and molecular data"- specifically stating  "histone mutational status at biopsy"- in designing new therapies for DIPG.

There has been a rash of publication regarding K27M mutations recently.   Several of the other abstracts for this meeting feature this histone mutation in DIPG.   I am sure more will be coming in the future.

Reference:
Genetic and histopathological spectrum of paediatric diffuse intrinsic pontine gliomas
https://soc-neuro-onc.conference-services.net/reports/template/onetextabstract.xml?xsl=template/onetextabstract.xsl&conferenceID=3467&abstractID=73797

Inhibition of PRC2 Activity by a Gain-of-Function H3 Mutation Found in Pediatric Glioblastoma

http://www.sciencemag.org/content/early/2013/03/27/science.1232245