Developing and Distributing a Pediatric Spinal Cord High-grade Glioma Model
Spinal cord high-grade gliomas (HGG) of childhood are rare and clinically devastating. Recent genomic studies have demonstrated that the majority of pediatric spinal cord HGG exhibit the H3K27M mutation (1, 2) that is characteristic of and specific to other HGG of the midline of the childhood central nervous system such as diffuse intrinsic pontine glioma (DIPG) and thalamic glioma (3, 4). We recently established neurosphere cultures of a pediatric spinal cord HGG expressing the H3K27M mutation from tissue donated at the time of autopsy. Cultures were established from both the primary site in the cervical spinal cord as well as from tumor metastatic to the cerebrum and leptomeninges. To our knowledge, this is the first such culture of a pediatric spinal cord HGG.
In the present proposal, we seek support to fully characterize, expand and distribute the culture, and to develop the correlate orthotopic xenograft model.
Funded by the ALSF Cord Fund