Diffuse brainstem gliomas are the leading cause of death in children with CNS neoplasms and survival statisitics have remained static over the past 20 years.
15-20% of all pediatric brain tumors are brainstem gliomas.
Far more common in children than in adults (75% occur < age 20).
Peak incidence for all brainstem gliomas is 6-7 years.
Possible slight male predominance.
Etiology
- Increased incidence of brainstem gliomas in patients with neurofibromatosis (NF) type 1.
- Up to 14% of children with NF-1 develop these tumors. They are typically low grade and indolent - regardless of diffuse or focal presentation. In this setting they generaly have an excellent prognosis, often with little or no intervention.
- There are no molecular or genetic markers consistently linked to the development of brainstem gliomas.