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

In this clinical article, Dr. Roger Packer of the Children's National medical Center describes brainstem gliomas, which remain an extraordinarily difficult form of childhood cancer to treat. Approximately 80% of all brainstem gliomas arise in the pons, often with infiltration into contiguous brainstem and other central nervous system regions. Surgery is rarely needed to confirm the presence of a diffuse intrinsic brainstem glioma. Surgery may be of benefit in lower-grade, more exophytic lesions. Radiotherapy remains the only treatment that has demonstrated at least a transient benefit for patients with brainstem gliomas, but despite treatment with radiotherapy, over 90% of patients with diffuse intrinsic brainstem gliomas will die within 18 months of diagnosis. Chemotherapy, to date, has shown a limited role in the management of brainstem gliomas. Multiple studies are underway evaluating new biological agents or chemotherapeutic agents with radiosensitization properties during and after radiotherapy.

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.