Presentation
Headache and seizures.
Patient Data
There is a 3.7 x 4.4 x 5 cm intra-axial mass lesion that appears to be within the 3rd ventricle, obstructing the Monro foramina bilaterally, causing bilateral obstructive hydrocephalus and marked trans ependymal edema.
Extension of the mass to the thalamus on both sides and left-sided globus pallidus is seen associated with marked vasogenic edematous changes.
After contrast njection, the mass shows irregular thick rim of enhancement with a large central necrotic changes.
Post op changes along with right frontal lobe asociated with an encephalomalacia tract is seen.
The pathological report was compatible with a diffuse midline glioma, H3 K27M-altered, and classified as WHO grade 4.
Case Discussion
MRI imaging findings are compatible with a diffuse midline glioma as described in the pathology report.
Diffuse midline glioma, H3 K27-altered is a specific entity that represents the majority of diffuse intrinsic pontine gliomas, although identical tumors are also found elsewhere in the midline (e.g. brainstem, spinal cord and thalamus) 1. They are aggressive tumors with a poor prognosis and are considered WHO grade 4 tumors regardless of histological features 1,2,3.