Background: Diffuse intrinsic pontine gliomas represent a unique subtype of
primary brain tumors occuring in a specific location and age. Their growth demonstrates
early invasion and, following diagnosis, rapid growth not responsive to common
therapies. Until recently, the genetic and cellular basis of these tumors was unknown.
Genetic evidence implicates mutations in the histone genes in the origin of these tumors.
Methods: Surgical biopsies performed on selected patients have resulted in the
establishment of anatomically accurate mouse models that have been used to
examine patterns of growth and response to new therapeutic agents.
Results: Human derived pontine glioma models recapitulate the invasive patterns of
growth. The grade of the original tumor affects the latency of tumor growth after
Conclusion: The use of human-derived xenograft models allows for improved pre-clinical testing of new
therapeutic targets in a tumor- and organ-specific manner.