Diffuse midline glioma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Acute onset of cranial nerve six palsy. Associated with nausea and vomiting.

Patient Data

Age: 6 years
Gender: Female
ct

Diffuse low-density throughout the pons extending into the cerebellar vermis and midbrain more on the left than the right.

mri

There is an expansile infiltrative mass centered within the pons which extends to surround the basilar artery within the prepontine cistern. Infiltrative T2 prolongation extends cephalad into both cerebral peduncles (left greater than right), as well as the left middle cerebellar peduncle.
Furthermore, there is an enhancing centrally necrotic component within the dorsal aspect of the brainstem that protrudes into the fourth ventricle.

There is bilateral cerebellar tonsillar ectopia resulting in crowding of the foreman magnum.

Case Discussion

This is a case of a diffuse midline glioma (formerly known as diffuse intrinsic pontine glioma).

The patient underwent a suboccipital craniectomy with resection of intrinsic brainstem tumor using neuronavigation, intraoperative ultrasound and microscope for Fluorescein fluorescence. Histologically, there were rare malignant glial cells admixed with necrosis. The presence of hyperchromasia, moderate cellularity, an infiltrative growth pattern, and elevated Ki-67 were suggestive of a largely low-grade glioma with potential for progression. The Ki-67 index was regionally up to 5-6% of tumor nuclei. IDH-1 was negative.

Co-author:
Travis Bevington, MD

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