Brain stem gliomas are classified according to their location, radiographic appearance, and 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, fibrillary astrocytomas that involve the pons (diffuse intrinsic pontine gliomas [DIPG]), often with contiguous involvement of other brain stem sites.[1,2] The prognosis is extremely poor for these tumors. The biology of diffuse pontine glioma is limited by the lack of pathological tissue available for study, but this is slowly being remedied by occasional upfront biopsies or analysis of immediate postmortem autopsy specimens. Overexpression of epidermal growth factor receptor, platelet-derived growth factor receptor alpha, and poly (ADP-ribose) polymerase (PARP-1) have been identified.[3,4] Focal pilocytic astrocytomas have a more favorable prognosis. These most frequently arise in the tectum of the midbrain, focally, within the pons, or at the cervicomedullary junction where they are often exophytic, and have a far better prognosis than diffuse intrinsic tumors.[5,6,7]
Primary tumors of the brain stem are most often diagnosed based on clinical findings and on neuroimaging studies using magnetic resonance imaging.[8,9] Histologic confirmation of presumed DIPG is usually unnecessary. Biopsy or resection may be indicated for brain stem tumors that are not diffuse and intrinsic or when there is diagnostic uncertainty based on imaging findings. New approaches with stereotactic needle biopsy may make biopsy safer.
Central nervous system atypical teratoid/rhabdoid tumor is a disease in which malignant (cancer) cells form in the tissues of the brain.
Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT) is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children younger than three years of age, although it can occur in older children and adults.
About half of these tumors form in the cerebellum or brain stem. The cerebellum is the part of the brain that...
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Liu AK, Brandon J, Foreman NK, et al.: Conventional MRI at presentation does not predict clinical response to radiation therapy in children with diffuse pontine glioma. Pediatr Radiol 39 (12): 1317-20, 2009.
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