Diffuse brain stem gliomas are the leading cause of death in children with CNS neoplasms and survival statistics have remained static over the past 20 years.
15-20% of all pediatric brain tumors are brain stem gliomas.
Far more common in children than in adults (75% occur < age 20).
Peak incidence for all brain stem gliomas is 6-7 years.
Possible slight male predominance.
Etiology
There is no known etiology for high grade diffuse brain stem gliomas.
There is an increased incidence of low grade brain stem gliomas in patients with neurofibromatosis (NF) type 1.
In association with NF-1:
- Tumors are typically low grade and indolent
- Generally have an excellent prognosis, often with little or no intervention.
There are no molecular or genetic markers consistently linked to the development of brain stem gliomas.
Summary of Brain stem Glioma Features:
Proportion of all pediatric brain tumors |
10-15% |
Patients diagnosed under age 20 |
75% |
Median age |
4-6 years |
| Proportion of diffusely infiltrating tumors | 70-80% |
| Most common site of origin | Pons |
| Racial Bias | None |
