Gliomatosis Cerebri SIOP Europe Task Force
In the last few years, some scientific publications have highlighted the fact that Gliomatosis cerebri (GC) shares some relevant pathways other invasive high-grade gliomas have. This has been reproduced in small case series of adult and pediatric GC. Additionally, the 2016 WHO classification has deleted GC as a distinct entity, rather being considered as a growth pattern identified rarely in higher grade glial tumors.
However, “GC phenotype” exists as a rare clinic and radiologic presentation and has an extremely unique infiltrative and aggressive biologic behavior. These cases harbor a very dismal prognosis and rapid progression with neurologic deterioration is the rule.
Therefore, it is not surprising the identification of genetic/epigenetic/metabolic alterations observed in other aggressive gliomas. However, in addition to these alterations, it is to be unveiled what other alterations characterize GC-like cases.
We strongly believe that the study of this phenotype may unveil novel mechanisms of tumor invasion and migration that may be relevant for this and other aggressive gliomas with similar poor prognosis.
Basic and Clinical Research Initiatives
The SIOPe HGG/DIPG created in Valencia in 2015 a dedicated task force for the study of GC led by Andres Morales La Madrid, MD (Barcelona, Spain). This group of physicians and researchers work collaboratively in basic and translational projects at this stage.
The first research project initiated by this group is the following: “Gliomatosis cerebri (GC) pre-clinical research project: Cell culture establishment/animal modeling, molecular characterization and migration studies”. The primary and secondary objectives of this project are the following:
Establish pediatric GC cell lines for pre-clinical projects including molecular profiling, drug testing, and animal modeling.
Explore migratory and invasion mechanisms by which GC cell infiltrate brain parenchyma.
We are in the process of putting together the largest pediatric GC case series from several SIOPe countries. All cases are being centrally reviewed (histology and radiology) and when tissue available, immunohistochemistry and molecular profiling are being performed. Our goal is to present some of these data in the upcoming second international meeting in Washington DC (June 2017) and publish
the paper before the end of 2017. The information obtained out of these cases will be paramount in characterizing better this condition and in identifying key players in tumor initiation and progression.
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