Brainstem gliomas in childhood

Introduction
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By Roger J Packer MD

Brainstem gliomas in childhood are also known as or subsume Brainstem astrocytomas in childhood, Brainstem tumors in childhood, and Pontine gliomas in childhood. -ed

The management of brainstem gliomas remains problematic. Approximately 80% of children have diffuse, intrinsic pontine tumors, which carry a dismal prognosis. Children with cervicomedullary and tectal tumors have markedly better outcomes. Dr. Roger Packer of Children’s National Medical Center reviews outcomes after recent clinical trials and new biologic insights that open exciting innovative avenues of molecularly-targeted treatment.

Key Points

  • Brainstem gliomas occur most frequently in children, with the majority being diffuse pontine gliomas.
  • Approximately 20% of childhood brainstem gliomas are not diffuse pontine tumors; these non-diffuse pontine lesions, such as tectal gliomas and exophytic cervicomedullary tumors, are often low-grade gliomas and have a relatively good prognosis.
  • Over 90% of children with diffuse pontine gliomas will die of disease within 18 months of diagnosis.
  • Radiotherapy remains the only effective, albeit transient, therapy for diffuse pontine gliomas.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Anesthesia
References cited
Contributors