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Overview

The brain stem consists of the midbrain, pons and medulla located deep in the posterior part of the brain. Tumors that arise along these structures are called brain stem gliomas. Most brain stem gliomas occur in the pons ("pontine gliomas"). The pontine tumors have a poorer prognosis than the less common midbrain and medullary gliomas.


Symptoms

Children with a brain stem glioma may experience the following symptoms. Sometimes, children with brain stem glioma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a brain stem glioma. If you are concerned about a symptom on this list, please talk with your child’s doctor.
  • Double vision or an inability to close the eyelids
  • Drooping of the face
  • Difficulty chewing and swallowing food
  • Weakness in the arms and legs, clumsiness or wobbliness, and difficulty walking
  • Difficulty talking
  • Headache
  • Vomiting


Diagnosis

Throughout the United States, brain magnetic resonance imaging (MRI), with and without gadolinium contrast, has become the "gold standard" for diagnosis of brain stem gliomas. Biopsy is almost never indicated for the diffusely infiltrative glioma involving the pons, unless the diagnosis of this tumor is in doubt. Biopsy may be indicated for brain stem tumors that are focal or atypical, especially when the tumor is progressive or when surgical excision may be possible.

Spread of either of these tumors (metastases) outside the brain stem to other sites in the brain or spine is unusual. Thus, staging tests to look for tumor spread, such as spine MRI or lumbar puncture (spinal tap), are not routinely performed at diagnosis.


Treatment

Since brain stem gliomas are relatively uncommon and require complex management, children with such tumors deserve evaluation in a comprehensive cancer center where the coordinated services of dedicated pediatric neurosurgeons, pediatric neurologists, pediatric oncologists, radiation oncologists, neuropathologists, and neuroradiologists are available. In particular, for diffusely infiltrative brain stem gliomas, because of their rarity and poor prognosis, children and their families should be encouraged to participate in clinical trials attempting to improve survival with innovative therapy.








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