Diffuse leptomeningeal glioneuronal tumor

Case contributed by Yaïr Glick


Known diffuse leptomeningeal glioneural tumor. Follow-up MR.

Patient Data

Age: child
Gender: Male

Left frontal ventriculoperitoneal shunt. Frontal and parietal burr holes on both sides - attempts at shunt insertion and for the evacuation of an extra-axial hematoma.
Extensive meningeal enhancement, including nodular foci. The enhancement does not extend to the brainstem or the spinal cord.
Pronounced communicating hydrocephalus (the Sylvian aqueduct is patent and shows a flow void on T2WI).
Numerous small white matter high-signal foci on FLAIR, none of which now enhance, showing a reduction in both size and number compared to a study done 2 months prior (not shown). Similar foci in the cerebellum.

Case Discussion

The patient had initially presented with a longstanding headache of several months' duration, plus unsteadiness and vomiting for the past few days. Papilledema was noted on physical examination.

The MR study shown here is one of many performed for the follow-up of diffuse leptomeningeal glioneuronal tumor, diagnosed by immunostaining of biopsy specimens from the dura and brain. After the patient started receiving cobimetinib (MEK inhibitor) treatment, he improved slowly but remarkably. This was mirrored in later follow-up MR studies (not shown), where the meningeal enhancement had all but vanished.

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