Diffuse hemispheric glioma H3 G34 mutant

Case contributed by Alexandre Niro
Diagnosis almost certain

Presentation

Diplopia, progressive left-sided weakness, and headaches. No past history.

Patient Data

Age: 10 years
Gender: Male

On FLAIR sequence, ill-defined abnormality located in the right frontal and parietal deep white matter.

Extension through the splenium of the corpus callosum to the contralateral hemisphere. The cortex seems spared.

No significative mass effect: considering the volume of this abnormality, we would expect a more important mass effect.

No abnormality on susceptibility-weighted imaging: no calcification or flow void.

Hyperintensity on DWI, with facilitated diffusion on ADC (therefore DWI signal is due to T2 shinethrough).

No enhancement in the lesion or elsewhere in the brain.

Absence of hypersignal on pc-ASL (consistent with the DSC perfusion not shown here)

Of note: presence of cystic cavities (on the superior subcortical part and nearby the corpus callosum). On sagittal and coronal, they seem to have a linear and radial distribution following the perivascular spaces, and were considered as dilatations.

Case Discussion

This is the case of a young boy with progressive motor impairment on the left side of his body, headaches, and diplopia. He had no past medical history and was not on any medication.

Imaging of his brain (followed by spinal imaging, which was normal and is not shown) was performed urgently. It revealed an infiltrative mass located in the deep white matter of his right frontal and parietal lobes, without any mass effect but with the involvement of the corpus callosum.

The lesion’s atypical pattern of radial multifocal cystic cavities led the radiologist to consider pseudo-tumoral perivascular space dilatations as a differential diagnosis.

A biopsy was performed a few days later.

The pathological diagnosis was diffuse hemispheric glioma, H3 G34 mutant.

The patient subsequently underwent chemotherapy and radiotherapy.

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