Background
Gliomas constitute a wide spectrum of neuroepithelial tumors arising
from glia or supporting cells (astrocytes, oligodendrocytes, and
ependymocytes) of the central nervous system (CNS)1,2.
Gliomas are responsible for nearly 24% of all primary brain and CNS
neoplasms and vastly differ in histology and behavior ranging from
benign ependymomal tumors to the most aggressive and lethal grade IV
glioblastoma multiforme3. Specifically, brainstem
gliomas are diverse groups of neoplasms that primarily affect children
and include low-grade focal brainstem gliomas, as well as high-grade
diffuse intrinsic pontine gliomas (DIPG)4,5. Although
80% of gliomas arise within the pons as DIPGs, the remaining low-grade
gliomas are located within the midbrain, dorsal medulla, or the
cervico-medullary junction. The aforementioned locations of these
neoplasms pose therapeutic challenges, and hence, may negatively affect
treatment outcomes.
Radiotherapy, chemotherapy, or combined treatment modalities are the
standard therapeutic options for gliomas6. Previous
studies have reported failure of chemotherapy in treating DIPGs due to
the lack of intra-tumor penetration7,8. Recently, the
unraveling of the genetic landscape of DIPGs together with
identification of the K27M mutation (mutation in both histones H3.1 and
H3.3) has improved the understanding of the pathogenesis of gliomas and
identification of novel targeted therapies9,10.
Despite a wealth of information on cancer staging, prediction of
survival and treatment strategies, little is known about the
determinants of cancer-specific survival in childhood brain stem glioma.
The Surveillance, Epidemiology, and End Results (SEER) database of
survival data from population-based cancer registries encompass
~28% of the American population11,12.
The aim of the current study was to characterize a comprehensive,
accurate and useful prognostic model using a population-based SEER
analysis to predict survival of pediatric cases of stem gliomas.