Brain stem gliomas

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Brain stem gliomas
DIPG.jpg
T1 and FLAIR axial images of diffuse pontine glioma
Tumor Class Astrocytoma
WHO Grade II to IV
Survival 9-18 Months
Treatment Radiation
Histologic Features
Micro highly cellular tumor
Radiographic Features
MRI enlargement of the pons without enhancement


Contents

Definition

Brainstem Glioma is a general term for tumors that arise in the brainstem. These are subcategorized as Diffuse, Focal, Dorsal Exophytic, and Cervicomedullary.

Epidemiology

Brainstem gliomas as a whole account for 10-20% of pediatric brain tumors. There is an incidence of 2 per 1 million children in the USA.

Categories of Brainstem Glioma

Diffuse

These tumors account for 80% of brainstem gliomas.

  1. symptoms are subtle but progressive and include ataxia and cranial nerve dysfunction (usually bilateral) plus cerebellar and pyramidal tract findings
  2. Diffuse enlargement of the pons is noted on MRI with very little enhancement.

Focal

  1. history of symptoms spans months to years along with work up for multiple medical problems
  2. tectal tumors present with hydrocephalus while tegmental tumors may present with occulomotor paresis


Exophytic

  1. tumors grow from subependymal glial tissue out into the fourth ventricle
  2. typically present with hydrocephalus symptoms

Cervicomedullary tumors

  1. typically arise from lower medulla or upper cervical area and cause nausea, vomiting, dysphagia, aspiration; if arising from cervical cord then torticollis, pain, motor deficits

Treatment

The treatment of diffuse pontine gliomas is essentially limited to focal radiation with treatment of hydrocephalus if also present. The prognosis is poor with a median survival of 9-12 months. Most children experience improvement of their symptoms after radiation.

Focal, exophytic and cervicomedullary tumors may be amenable to surgery. Dorsal exophytic are generally the most surgically accessible tumors.

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