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Childhood Brain Stem Glioma Treatment (PDQ®)

Health Professional Version
Last Modified: 05/19/2014

Stage Information for Childhood Brain Stem Glioma

There is no generally applied staging system for childhood brain stem glioma.[1]

Brain stem gliomas are classified according to the following:

  • Location.
  • Radiographic appearance.
  • Histology (when obtained).

Brain stem gliomas may occur in the pons, midbrain, tectum, dorsum of the medulla at the cervicomedullary junction, or in multiple regions of the brain stem. The tumor may contiguously involve the cerebellar peduncles, cerebellum, the cervical spinal cord, and/or thalamus. The majority of childhood brain stem gliomas are diffuse astrocytomas that involve the pons (diffuse intrinsic pontine gliomas [DIPGs]), often with contiguous involvement of other brain stem sites.[2,3]

It is uncommon for these tumors to have spread outside the brain stem itself at the time of initial diagnosis. Spread of malignant brain stem tumors is usually contiguous, with metastasis via the subarachnoid space. Such dissemination may occur prior to local relapse but usually occurs simultaneously with or after local disease relapse.[4]

References
  1. Freeman CR, Farmer JP: Pediatric brain stem gliomas: a review. Int J Radiat Oncol Biol Phys 40 (2): 265-71, 1998.  [PUBMED Abstract]

  2. Laigle-Donadey F, Doz F, Delattre JY: Brainstem gliomas in children and adults. Curr Opin Oncol 20 (6): 662-7, 2008.  [PUBMED Abstract]

  3. Khatua S, Moore KR, Vats TS, et al.: Diffuse intrinsic pontine glioma-current status and future strategies. Childs Nerv Syst 27 (9): 1391-7, 2011.  [PUBMED Abstract]

  4. Sethi R, Allen J, Donahue B, et al.: Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma. J Neurooncol 102 (1): 121-7, 2011.  [PUBMED Abstract]