Brain Stem Gliomas natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Natural History

Radiotherapy-induced neoplasms tend to be more aggressive in their natural history than their de novo counterparts.

Complications

Morbidity is due to the location of the space-occupying lesion and compression of surrounding structures; because these structures regulate basic body functions:

• Blood pressure
• Respiration
• Swallowing:
• Loss of motor and sensory functions

Sudden death can result from increased intracranial pressure and subsequent cerebral herniation. This may be a consequence either of edema induced by the tumor or of hemorrhage into the neoplasm.

Prognosis

The prognosis of brainstem gliomas depend on:

• The type of brain stem glioma
• Where the tumor is found in the brain and if it has spread within the brain stem
• Age of the child when diagnosed
  • Children younger than 3 years may have a more favorable prognosis
• Whether or not the child has a condition called neurofibromatosis type 1
  • Children with NF1 and brain stem gliomas may have a better prognosis than other patients who have intrinsic lesions
• Whether the tumor has been newly diagnosed or has recurred

As a general rule, dorsal exophytic tumors and cervicomedullary tumors tend to have a good prognosis with treatment, and diffuse type has the worst prognosis with treatment. The median survival for children with diffuse brainstem glioma is less than 1 year. In contrast, focal brainstem gliomas have a markedly improved prognosis, with 5-year overall survival exceeding 90%. Adults tend to have a better prognosis than childhood brainstem gliomas.

• Diffuse brainstem glioma
  • Terrible prognosis
  • 90-100% patients die within 2 years of diagnosis
• Focal (tectal glioma)
  • Excellent long term survival with CSF shunting (essentially benign lesions)
• Focal (other)
  • Good long-term prognosis with surgery
• (Dorsally) exophytic tumors
  • Good long-term prognosis with surgery