Diffuse midline glioma

Case contributed by Hoe Han Guan
Diagnosis probable

Presentation

Right sided upper limb and lower limb weakness, associated with imbalance and unsteady gait.

Patient Data

Age: 5 years
Gender: Male
ct

Left sided brainstem lesion with centered at the pons. This lesion has higher peripheral attenuation (which can be due to high cellularity tumor) with central hypodensity which can represent necrotic or cystic component. Superiorly, it extends into the midbrain, left cerebral peduncle and left thalamus. Anteriorly, it encases the basilar artery. Posteriorly, the forth ventricle is partially effaced.
Basal cisterns are partially effaced.

mri

Left sided brainstem lesion with epicentered at pons. Superior extension into the midbrain, left cerebral peduncle and left thalamus. It has hyperintensity on T2W, hypointensity on T1WI with minimal contrast enhancement post contrast. Center of the lesion is cystic in nature which can represent necrotic component. On DWI/ADC, restricted diffusion at the periphery of the lesion. No susceptibility/blooming artefacts on hemo sequence to suggest calcification or blood products.
Extra-axial extension of the lesion into the prepontine cistern and left cerebellopontine angle. Anteriorly, the mass encases the basilar artery and left posterior cerebral artery, but these vessels preserved their flow void on MRI. Small hyperintensity within the proximal basilar artery can represent small thrombosis.
Posteriorly, partial effacement of forth ventricle without obstructive hydrocephalus or periventricular hyperintensity. Left middle cerebellar peduncle is involved.

Case Discussion

The information on the histopathology or molecular characteristics of this tumor is not available as biopsy is not performed, it is likely that the brainstem lesion represents a diffuse midline glioma H3 K27M–mutant

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