Presentation
Headache, difficulty in walking, altered mental status.
Patient Data
A large mass is seen centered on the pons extending to the midbrain and medulla oblongata as well; returning a high signal on the T2WI and FLAIR images and, a low signal on T1WI weighted images with patchy post-contrast enhancement. No blooming artifact is noted in the lesion to suggest hemorrhage/calcification. No diffusion restriction is seen in this lesion.
The mass is compressing the fourth ventricular and resultant proximal triventricular acute hydrocephalus (evident by trans-ependymal CSF leakage).
Turtuisity of the optic nerves is noted.
The features are suggestive of pontine large glioma as described.
Case Discussion
The features are highly suggestive of brainstem glioma. Unfortunately, the patient was lost of follow-up and no histology result is available.
The term Diffuse brainstem gliomas was used to describe infiltrating astrocytomas arising in the brainstem, usually in children. It is now removed from the 2016 update to the WHO classification of CNS tumors and replaced by a variety of entities defined based on molecular characteristics. The majority of these tumors would now be classified as diffuse midline glioma, H3 K27M–mutant.
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