Overview
Brain Tumor: Brain Stem Glioma
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.Characteristics
- Named for its location at the base of the brain
- Can range from low grade to high grade
- Occurs most often in children between three and ten years of age, but can occur in adults
Incidence
Brain stem tumors account for 10 percent of pediatric brain tumors. The peak incidence is between ages 5 and 10.
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.
Neurosurgery : - Surgery is usually not possible or advisable for diffusely infiltrative brain stem gliomas. By their very nature, these tumors invade diffusely throughout the brain stem, growing between normal nerve cells. Aggressive surgery would cause severe damage to neural structures vital for arm and leg movement, eye movement, swallowing, breathing, and even consciousness.
Surgery with less than total removal can be performed for many focal brain stem gliomas. Such surgery often results in quality long-term survival, without administering chemotherapy or radiotherapy immediately after surgery, even when a child has residual tumor. Surgery is particularly useful for tumors that grow out (exophytic) from the brain stem.
Focal brain stem tumors that arise at the back of the midbrain (tectal gliomas) should be managed conservatively, without surgical removal. Nevertheless, shunt placement or ventriculostomy for hydrocephalus (see below) is frequently necessary. These tumors have been described to be stable for many years without any intervention other than shunting.
Radiotherapy : - Radiotherapy limited to the involved area of tumor is the mainstay of treatment for diffusely infiltrative brain stem gliomas. A total radiation dosage ranging from 5400 to 6000cGy, administered in daily fractions of 150 to 200 cGy over 6 weeks, is standard. Hyperfractionated (twice-daily) radiotherapy was used previously to deliver higher irradiation dosages, but such did not lead to improved survival. Hyperfractionated radiotherapy has been associated with a greater dependency on steroids (see below).
Chemotherapy and other drug therapies : - The role of chemotherapy in diffusely infiltrative brain stem gliomas remains unclear. Studies to date with chemotherapy have shown little improvement in survival, although efforts through the Children's Oncology Group (COG) and Pediatric Brain Tumor Consortium (PBTC) are underway to explore further the use of chemotherapy and other drugs. Drugs utilized to increase the effect of radiotherapy (radiosensitizers) have thus far shown no added benefit, but promising new agents are under investigation.
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