Abstract
Diffuse intrinsic pontine glioma (DIPG) is a lethal pediatric brain tumor. While there are a number of in vivo rodent models for evaluating tumor biology and response to therapy, these models require significant time and resources. Here, we established the chick-embryo chorioallantoic (CAM) assay as an affordable and time efficient xenograft model for testing a variety of treatment approaches for DIPG. We found that patient-derived DIPG tumors develop in the CAM and maintain the same genetic and epigenetic characteristics of native DIPG tumors. We monitored tumor response to pharmaco- and radiation therapy by 3-D ultrasound volumetric and vasculature analysis. In this study, we established and validated the CAM model as a potential intermediate xenograft model for DIPG and its use for testing novel treatment approaches that include pharmacotherapy or radiation.
Highlights
Diffuse midline gliomas harboring the H3K27M mutation, historically referred to as diffuse intrinsic pontine gliomas (DIPG) are rare and aggressive brain tumors predominately found in children [1, 2]
While countless clinical trials have failed to show any therapeutic benefit, recent advances in DIPG biology have shown that a majority of DIPG tumors have a mutation in a histone H3 gene resulting in a methionine (M) in place of lysine (K) at position 27 (H3K27M) [4,5,6]
While recent breakthroughs in the molecular understanding of DIPG tumors have led to potential novel therapeutic targets, in vivo testing requires significant resources and time
Summary
Diffuse midline gliomas harboring the H3K27M mutation, historically referred to as diffuse intrinsic pontine gliomas (DIPG) are rare and aggressive brain tumors predominately found in children [1, 2]. The most prominent epigenetic change in H3K27M tumors is the global reduction of H3K27 trimethylation and is thought to be one of the main drivers of tumorigenesis [7, 10,11,12]. With this knowledge, the World Health Organization (WHO) reclassified DIPGs to include H3K27M mutation status as well as the signature loss of trimethylation
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